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Sample records for extrahepatic biliary atresia

  1. Extrahepatic biliary atresia in a border collie.

    Science.gov (United States)

    Schulze, C; Rothuizen, J; van Sluijs, F J; Hazewinkel, H A; van den Ingh, T S

    2000-01-01

    Progressive lameness and leg pain were the predominant clinical signs in a 17-week-old male border collie presented for examination. On clinical investigation, extrahepatic cholestasis in association with rickets due to inadequate vitamin D resorption was diagnosed. The dog was treated parenterally with vitamin D and a cholecystoduodenostomy was performed. At 25 days postsurgery the lameness had resolved and bone structure was radiographically normal. However, at six weeks postsurgery, the dog's condition deteriorated rapidly and euthanasia was finally performed at eight weeks postsurgery. At postmortem examination, Toxocara canis nematodes were found to have invaded the biliary system via the anastomosis between the gallbladder and duodenum, causing biliary and hepatic toxocariasis. The cause of the primary extrahepatic cholestasis was atresia of the common bile duct at the hepatic end. The liver tissue showed microscopic lesions of chronic extrahepatic cholestasis as well as acute inflammation associated with the nematode invasion. There was no postmortem evidence of bone lesions. Extrahepatic biliary atresia is extremely rare in animals and has not been described before in dogs. In contrast, it represents the most common cause of congenital cholestasis in children, occurring in approximately one per 10,000 to 15,000 live births.

  2. Post-operative abdominal CT scanning in extrahepatic biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Day, D L; Mulcahy, P F; Letourneau, J G; Dehner, L P

    1989-07-01

    A retrospective review of the abdominal CT scans of 26 children with extrahepatic biliary atresia was performed, and the results were correlated with available surgical and pathologic data. Associated congenital anomalies or acquired abnormalities were identified in these patients. Congenital anomalies included polysplenia, venous anomalies and bowel stenosis. Acquired abnormalities developed secondary to cirrhosis, portal hypertension, intrahepatic biliary duct dilatation, and hepatic ischemia. Despite frequent episodes of ascending cholangitis in these children, no hepatic abscesses were identified by CT or by pathologic examination. In conclusion, abdominal CT scanning of children with extrahepatic biliary atresia can define congenital and acquired abnormalities and provide important anatomic data for the surgeons before liver transplantation. (orig.).

  3. [Extrahepatic biliary atresia: diagnostic methods].

    Science.gov (United States)

    Cauduro, Sydney M

    2003-01-01

    To emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct relationship with the surgical re-establishment of the biliary flow before the second month of life. To discuss several complementary methods with the aim of selecting the ones that present better evidence, and avoiding delays in diagnosis and worse prognostic. Bibliographical researching regarding the period of 1985-2001, in Medline and MdConsult, using the following key words: neo-natal cholestasis; extrahepatic biliary atresia; neo-natal hepatitis. National and foreign articles were also elected based on the bibliography of consulted publications, and when necessary, for better understanding of the theme, opinions emitted in theses and textbooks were referred. The revision of the consulted bibliography led to the assumption that early diagnosis of EHBA and surgical treatment to reestablish the biliary flow up to 60 days of life are fundamental in order to achieve good results. Among several complementary methods of diagnosis, cholangiography by MR, US and the hepatic biopsy are the ones that provide the largest success indexes. The referring of patients bearers of EHBA to centers of references in Brazil, is still made tardily, probably due to lack of enlightenment of the doctors of primary attention, allied to bureaucratic and technological difficulties. The experience in England in relation to the "Yellow Alert" program, allowed that the number of children referred to surgical treatment before the 60 days of life increased significantly. Among the complementary methods, the MR cholangiography, ultrasonography and hepatic biopsy should be used, depending on the technological resources of the diagnosis units.

  4. Abnormalities of intrahepatic bile ducts in extrahepatic biliary atresia.

    Science.gov (United States)

    Raweily, E A; Gibson, A A; Burt, A D

    1990-12-01

    The infantile cholangiopathies are a group of conditions associated with neonatal jaundice, which include extrahepatic biliary atresia, paucity of intra-hepatic bile ducts and disorders associated with persistence of fetal biliary structures, the so-called ductal plate malformations. Although previously regarded as distinct entities, it has recently been suggested that they may represent parts of a disease spectrum in which the principal process is one of bile duct destruction, the morphological manifestations in individual cases being influenced by the stage of intra-uterine development at which such injury occurs and by the site within the biliary system at which there is maximum damage. To further examine this concept, we have studied liver biopsy specimens from 37 neonates with extrahepatic biliary atresia, with particular reference to abnormalities of the intrahepatic bile ducts. Paucity of intrahepatic ducts, defined as a bile duct: portal tract ratio of less than 0.9, was identified in six cases (16.2%). In eight cases (21.6%) we found concentric tubular ductal structures similar to those observed in ductal plate malformations. In one case, both abnormalities could be demonstrated. Our findings support the concept that there is overlap between the various types of infantile cholangiopathy.

  5. Emergencies in neonatal management: jaundice and biliary atresia

    OpenAIRE

    Clemente, Maria Grazia; Dessanti, Antonio

    2016-01-01

    Biliary atresia is a severe and progressive inflammatory process of unknown cause, which initially involves the extrahepatic bile ducts but which quickly proceeds towards the intrahepatic bile tree leading rapidly to biliary cirrhosis. Biliary atresia is the major reason for liver transplantation during childhood. The extrahepatic bile ducts in biliary atresia become connective fibrotic cords which is irreversibly damaged.

  6. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras Extrahepatic biliary atresia: current concepts and future directions

    Directory of Open Access Journals (Sweden)

    Elisa de Carvalho

    2007-04-01

    Full Text Available OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal indicação de transplante hepático na faixa etária pediátrica. Quanto à etiologia, o citomegalovírus, o reovírus e o rotavírus têm sido os agentes mais estudados como possíveis desencadeadores da obstrução imunomediada da árvore biliar. A resposta imune, especialmente o predomínio da resposta TH1 e do interferon gama, a susceptibilidade genética e as alterações do desenvolvimento embrionário da árvore biliar são aspectos que podem participar na etiopatogênese da obliteração das vias biliares extra-hepáticas. Ainda hoje, o único tratamento disponível é a portoenterostomia, cujos resultados são melhores quando realizada nos primeiros 2 meses de vida. Em relação ao prognóstico, as crianças não tratadas vão a óbito na totalidade, por complicações relacionadas à hipertensão portal e à cirrose hepática, e mesmo os casos tratados necessitam, em sua maioria, do transplante hepático. CONCLUSÃO: A atresia das vias biliares extra-hepáticas continua sendo a principal indicação de transplante hepático em crianças, e a mudança deste panorama depende de um melhor entendimento da etiopatogenia da obstrução biliar nos diferentes fenótipos da doença. Investigações futuras a respeito do papel do interferon gama e de outras citocinas são necessárias para avaliar se esses aspectos seriam potenciais alvos de intervenção terapêutica.OBJECTIVE: To provide an updated review on extrahepatic biliary atresia, focusing

  7. microRNA-222 modulates liver fibrosis in a murine model of biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Shen, Wen-jun; Dong, Rui; Chen, Gong, E-mail: chengongzlp@hotmail.com; Zheng, Shan

    2014-03-28

    Highlights: • The RRV infected group showed cholestasis, retardation and extrahepatic biliary atresia. • miR-222 was highly expressed, and PPP2R2A was inhibited in the murine biliary atresia model. • miR-222 profoundly modulated the process of fibrosis in the murine biliary atresia model. • miR-222 might represent a potential target for improving biliary atresia prognosis. - Abstract: microRNA-222 (miR-222) has been shown to initiate the activation of hepatic stellate cells, which plays an important role in the pathogenesis of liver fibrosis. The aim of our study was to evaluate the role of miR-22 in a mouse model of biliary atresia (BA) induced by Rhesus Rotavirus (RRV) infection. New-born Balb/c mice were randomized into control and RRV infected groups. The extrahepatic bile ducts were evaluated. The experimental group was divided into BA group and negative group based on histology. The expression of miR-222, protein phosphatase 2 regulatory subunit B alpha (PPP2R2A), proliferating cell nuclear antigen (PCNA) and phospho-Akt were detected. We found that the experimental group showed signs of cholestasis, retardation and extrahepatic biliary atresia. No abnormalities were found in the control group. In the BA group, miR-222, PCNA and Akt were highly expressed, and PPP2R2A expression was significantly inhibited. Our findings suggest that miR-222 profoundly modulated the process of fibrosis in the murine BA model, which might represent a potential target for improving BA prognosis.

  8. microRNA-222 modulates liver fibrosis in a murine model of biliary atresia

    International Nuclear Information System (INIS)

    Shen, Wen-jun; Dong, Rui; Chen, Gong; Zheng, Shan

    2014-01-01

    Highlights: • The RRV infected group showed cholestasis, retardation and extrahepatic biliary atresia. • miR-222 was highly expressed, and PPP2R2A was inhibited in the murine biliary atresia model. • miR-222 profoundly modulated the process of fibrosis in the murine biliary atresia model. • miR-222 might represent a potential target for improving biliary atresia prognosis. - Abstract: microRNA-222 (miR-222) has been shown to initiate the activation of hepatic stellate cells, which plays an important role in the pathogenesis of liver fibrosis. The aim of our study was to evaluate the role of miR-22 in a mouse model of biliary atresia (BA) induced by Rhesus Rotavirus (RRV) infection. New-born Balb/c mice were randomized into control and RRV infected groups. The extrahepatic bile ducts were evaluated. The experimental group was divided into BA group and negative group based on histology. The expression of miR-222, protein phosphatase 2 regulatory subunit B alpha (PPP2R2A), proliferating cell nuclear antigen (PCNA) and phospho-Akt were detected. We found that the experimental group showed signs of cholestasis, retardation and extrahepatic biliary atresia. No abnormalities were found in the control group. In the BA group, miR-222, PCNA and Akt were highly expressed, and PPP2R2A expression was significantly inhibited. Our findings suggest that miR-222 profoundly modulated the process of fibrosis in the murine BA model, which might represent a potential target for improving BA prognosis

  9. Biliary atresia and cerebellar hypoplasia in polysplenia syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Vanderdood, Kurt; Op de Beeck, Bart; Desprechins, Brigitte; Osteaux, Michel [Department of Radiology, Free University Brussels, AZ-VUB, Laarbeeklaan 101, 1090 Brussels (Belgium)

    2003-09-01

    We report a 3.5-month-old boy with polysplenia syndrome who demonstrated hemiazygos continuation of the inferior vena cava, extrahepatic biliary atresia, multiple splenunculi, bowel malrotation, and the rare finding of brainstem and cerebellar hypoplasia. A possible pathogenesis for cerebellar hypoplasia in this syndrome is suggested after review of the literature. The importance of seeking associated anomalies in biliary atresia, which may be possible indicators of polysplenia syndrome, is stressed since these patients need appropriate management when surgery is considered. (orig.)

  10. Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report

    Directory of Open Access Journals (Sweden)

    Madhavi Nori

    2013-01-01

    Full Text Available Extrahepatic biliary atresia (EHBA is an uncommon cause of neonatal jaundice. Antenatal Magnetic Resonance Imaging (MRI diagnosis of EHBA has not been published to the best of our knowledge till date. EHBA with cystic component is likely to be mistaken for choledochal cyst. A case that was antenatally predicted and postnatally confirmed by surgery and histopathology is being reported. All imaging signs are analyzed herewith. Imaging helps in the prediction of EHBA and also helps in early postnatal surgical referral which in turn improves the results of Kasai′s portoenterostomy.

  11. Pregnancy Complicated by Portal Hypertension Secondary to Biliary Atresia

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    O. E. O'Sullivan

    2013-01-01

    Full Text Available Biliary atresia is a rare idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extrahepatic biliary ducts. As the disease is progressive all cases will develop portal fibrosis, cirrhosis, and portal hypertension with the sequelae of varices, jaundice, and eventually liver failure requiring a transplant. Survival rates have improved considerably with many females living well in to be childbearing age. Due to the complexity of the disease these pregnancies are considered, high risk. We report the antenatal, intrapartum, and postpartum managements of a pregnancy complicated by biliary atresia. Furthermore, we highlight the importance of a multidisciplinary team approach in optimizing obstetric care for this high risk group.

  12. Atresia biliar extra-hepática: métodos diagnósticos Extrahepatic biliary atresia: diagnostic methods

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    Sydney M. Cauduro

    2003-04-01

    Full Text Available OBJETIVO: enfatizar a importância do diagnóstico precoce da atresia biliar extra-hepática e sua relação direta com o restabelecimento cirúrgico do fluxo biliar antes do segundo mês de vida, discutindo os diversos métodos complementares utilizados, objetivando selecionar os de maior evidência, evitando retardo diagnóstico e, conseqüentemente, piora do prognóstico. MÉTODO: pesquisa bibliográfica referente ao período de 1985 a 2001, no Medline e MdConsult, através das palavras-chaves: colestase neonatal, atresia biliar extra-hepática e hepatite neonatal. Também foram selecionados artigos nacionais e estrangeiros a partir da bibliografia das publicações consultadas e, quando necessário, para melhor entendimento do tema, opiniões emitidas em teses e livros-textos foram referidas. RESULTADOS: a revisão da bibliografia consultada permite afirmar que o diagnóstico precoce das AVBEH e tratamento cirúrgico para restabelecer o fluxo biliar até 60 dias de vida são fundamentais na obtenção de bons resultados. Entre os diversos métodos complementares de diagnóstico, a colangiografia por RM, US e a biópsia hepática são os que proporcionam os maiores índices de acerto. CONCLUSÕES: o encaminhamento de pacientes portadores de AVBEH para centros de referências no Brasil ainda é efetuado tardiamente, provavelmente pela falta de esclarecimento dos médicos de atenção primária, aliada às dificuldades burocráticas e tecnológicas. A experiência da Inglaterra em relação ao programa "Alerta Amarelo", permitiu que o número de crianças encaminhadas para tratamento cirúrgico antes dos 60 dias de vida aumentasse significativamente. Entre os métodos complementares, a colangiografia por RM, a US e a biópsia hepática deverão ser utilizados, dependendo dos recursos tecnológicos das unidades de diagnóstico.OBJECTIVE: to emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct

  13. Immunological gap in the infectious animal model for biliary atresia.

    Science.gov (United States)

    Czech-Schmidt, G; Verhagen, W; Szavay, P; Leonhardt, J; Petersen, C

    2001-11-01

    Extrahepatic biliary atresia (EHBA), the etiology of which still remains unclear, occurs exclusively in newborns and has recently been simulated in an animal model. It is possible to trigger an EHBA corresponding to the human disease by means of intraperitoneal infection of newborn Balb/c mice with rhesus rotavirus (RRV). The aim of the present study was to determine the conditions and circumstances for inducing biliary atresia in this model focusing on first-line immunological aspects. Newborn as well as pregnant Balb/c mice were intraperitoneally infected with RRV. The highest incidence of cholestasis (86%) was achieved by infection with 10(6) PFU/ml RRV within the first 12 h postpartum, resulting in EHBA with a lethality of 100%. However, the later the newborn mouse is infected, the less likelihood there is that EHBA is triggered. Additionally, the incidence of biliary atresia in this model depends on the quantity of the virus that is given intraperitoneally. However, the development of biliary atresia is not correlated to the virus in the liver. The antepartum infection of pregnant mice does not induce EHBA in the offspring. Female mice that are immunized against RRV protect their newborns from developing RRV-induced cholestasis and EHBA. This protection is transmitted transplacentally and not by breast milk. It is obvious that a temporary immunological gap is essential for virally induced EHBA. Further studies should focus on specific parameters of the immune system of newborn mice in this biliary atresia model. Copyright 2001 Academic Press.

  14. Ultrasound-guided percutaneous cholecysto-cholangiography for the exclusion of biliary atresia in infants

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Kyung Min; Ryeom, Hun Kyu; Choe, Byung Ho; Kim, Kap Cheol; Kim, Jong Yeol; Lee, Jong Min; Kim, Hye Jeong; Lee, Hee Jung [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    2006-08-15

    The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder.

  15. Ultrasound-guided percutaneous cholecysto-cholangiography for the exclusion of biliary atresia in infants

    International Nuclear Information System (INIS)

    Shin, Kyung Min; Ryeom, Hun Kyu; Choe, Byung Ho; Kim, Kap Cheol; Kim, Jong Yeol; Lee, Jong Min; Kim, Hye Jeong; Lee, Hee Jung

    2006-01-01

    The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder

  16. Biliary atresia: evaluation on two distinct periods at a reference pediatric service.

    Science.gov (United States)

    Queiroz, Thais Costa Nascentes; Ferreira, Alexandre Rodrigues; Fagundes, Eleonora Druve Tavares; Roquete, Mariza Leitão Valadares; Penna, Francisco José

    2014-01-01

    Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

  17. BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

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    Thais Costa Nascentes QUEIROZ

    2014-03-01

    Full Text Available Context Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that pre­sents with biliary obstruction exclusively in the neonatal period. Objectives To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. Methods Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. Results Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001. On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15, but difference was found when comparing the age at surgery (P = 0.002. Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. Conclusions Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

  18. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras Extrahepatic biliary atresia: current concepts and future directions

    OpenAIRE

    Elisa de Carvalho; Cláudia Alexandra Pontes Ivantes; Jorge A. Bezerra

    2007-01-01

    OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed) pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal in...

  19. Detection of Epstein Barr Virus by Chromogenic In Situ Hybridization in cases of extra-hepatic biliary atresia

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    Farahmand Fatemeh

    2008-04-01

    Full Text Available Abstract Introduction Extra-hepatic biliary atresia (EHBA is an important cause of neonatal cholestasis. Several infectious agents have been proposed as etiologic factors such as Rotavirus and Reovirus. There is limited data on the role of Epstein Barr virus (EBV infection in EHBA, so we decided to study the presence of EBV virus in a series of 16 proven EHBA cases by Chromogenic in situ hybridization (CISH technique. Methods In the current study a total of 16 liver wedge biopsies of proven cases of EHBA were selected in a period of 4 years. CISH staining for EBV-encoded RNA (EBER transcript was performed. Results The review of H&E-stained slides of liver biopsies revealed fibrosis and marked ductular proliferation. In CISH-stained slides, EBV trace was observed in hepatocytes in two cases and in biliary epithelium in one case of EHBA. Discussion Considering the association of hepatitis with the Epstein-Barr virus in later life, it is likely that EBV hepatitis and its complications occur in the neonatal/perinatal period. Since EHBA is a relatively rare disease, a similar study on wedge biopsies of this number of proven cases of EHBA has not been performed to date. Current observation proposes the need for a study of larger series and employing other methods for confirming the etiologic role of EBV in EHBA cases.

  20. congenital Biliary atresia

    African Journals Online (AJOL)

    embryonic form), comprising 10-35% of cases2. The pathology of the extrahepatic biliary system widely varies in these ... hepatic duct, with cystic structures found in the porta hepatis. .... Nelson Textbook of. Paediatrics 15th edition : Chapter 302.

  1. ATRESIA BILIER

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    Julinar Julinar

    2009-09-01

    Full Text Available AbstrakAtresia bilier merupakan penyakit yang jarang terjadi dan penyababnya belum diketahui secara pasti. Karakteristik dari penyakit ini adalah terjadinya inflamasi progresif pada duktus bilier sehingga terjadi obstruksi ekstrahepatal yang akhirnya dapat menyebabkan fibrosis dan sirosis hepar. Atresia bilier ada 2 tipe yaitu: 1. Syndromic atau fetal, disertai beberapa kelainan kongenital (10-20%. 2. non syndromic, tanpa disertai kelainan kongenital yang lain (80-90%. Atresia bilier akan berakibat fatal tanpa penanganan yang cepat. Kelainan ini dapat ditangani dengan metode operasi Kasai prosedure yang dapat mengalirkan kembali aliran empedu hampir 80% jika dilakukan secepatnya, gold periode >60 hari. Diagnosis dini sangat penting untuk keberhasilan operasi Kasai. Pada penulisan ini akan dilaporkan sebuah kasus atresia bilier tipe fetal, seorang anak laki-laki berusia 58 hari, dengan keluhan tampak kuning sejak usia 3 minggu disertai dengan buang air besar berwarna pucat, buang air kecil berwarna seperti teh pekat. Diagnosis ditegakkan berdasarkan gejala klinis, laboratorium, USG dan biopsi hepar yang sangat mendukung diagnosis atresia bilier. Operasi Kasai tidak efektif karena disertai dengan komplikasi kholangitis yang akhirnya menyebabkan sirosis hepatis pada 5 bulan kehidupan.Kata kunci : Atresia bilier, Kasai procedure, kholangitis, sirosis hapatisAbstractBiliary atresia is a disease of unknown etiology, characterized by progressive fibro inflammatory of the bile duct and liver that result obstruction of extrahepatic bile duct, leading to the fIbrosis and liver cirrhosis. It has two form of biliary atresia : 1. syndromic of fetal biliary atresia (10-20% with various congenital anomalies, 2. non syndromic biliary atresia (80-90% with isolated anomaly. In this case we report on an infant with the second form of biliary atresia, with diagnosis and operation was not based on liver biopsy, but on clinical features, laboratorium finding and USG

  2. Risk factors for extrahepatic biliary tract carcinoma in men

    DEFF Research Database (Denmark)

    Ahrens, Wolfgang; Timmer, Antje; Vyberg, Mogens

    2007-01-01

    OBJECTIVES: To identify risk factors of carcinoma of the extrahepatic biliary tract in men. METHODS: Newly diagnosed and histologically confirmed patients, 35-70 years old, were interviewed between 1995 and 1997 in Denmark, Sweden, France, Germany and Italy. Population controls were frequency...... for extrahepatic biliary tract carcinoma in men (odds ratio 2.49; 95% confidence interval 1.32-4.70), particularly for gall bladder tumors (odds ratio 4.68; 95% confidence interval 1.85-11.84). For a body mass index [height (m) divided by squared weight (kg)] >30 at age 35 years, an excess risk was observed (odds...... as a strong risk factor for extrahepatic biliary tract carcinoma, whereas we did not find any strong lifestyle-associated risk factors. Inconsistent results across studies concerning the association of extrahepatic biliary tract carcinoma with overweight and obesity may be explained by the different...

  3. The Effects of Travel Burden on Outcomes After Resection of Extrahepatic Biliary Malignancies: Results from the US Extrahepatic Biliary Consortium.

    Science.gov (United States)

    O'Connor, Sean C; Mogal, Harveshp; Russell, Gregory; Ethun, Cecilia; Fields, Ryan C; Jin, Linda; Hatzaras, Ioannis; Vitiello, Gerardo; Idrees, Kamran; Isom, Chelsea A; Martin, Robert; Scoggins, Charles; Pawlik, Timothy M; Schmidt, Carl; Poultsides, George; Tran, Thuy B; Weber, Sharon; Salem, Ahmed; Maithel, Shishir; Shen, Perry

    2017-12-01

    Surgical resection of extrahepatic biliary malignancies has been increasingly centralized at high-volume tertiary care centers. While this has improved outcomes overall, increased travel burden has been associated with worse survival for many other malignancies. We hypothesized that longer travel distances are associated with worse outcomes for these patients as well. Data was analyzed from the US Extrahepatic Biliary Consortium database, which retrospectively reviewed patients who received resection of extrahepatic biliary malignancies at 10 high-volume centers. Driving distance to the patient's treatment center was measured for 1025 patients. These were divided into four quartiles for analysis: travel distances were associated with decreased overall survival, especially in the 3rd quartile of our study. Patients traveling longer distances also had a lower household income, suggesting that these patients have significant barriers to care.

  4. Fatores relacionados ao prognóstico da atresia biliar pós-portoenterostomia Factors related to the post-portoenterostomy prognosis of biliary atresia

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    Jorge Luiz dos Santos

    2002-01-01

    ós-portoenterostomia.Objective: this study considered the presence of congenital anomalies, ductal plate malformation, area of fibrosis and, mainly, the patient's age in cases of biliary atresia submitted to surgery. The present study verified the influence of these factors on the follow-up of a biliary atresia sample. Methods: a sample of 47 patients with biliary atresia was evaluated in a cross-sectional study. Their histologic specimens were stained for antibody anticytokeratin 19 and CAM 5.2 through immunohistochemistry in order to study biliary structures, and for picrosirius red to evaluate the area of fibrosis. The study of biliary structures was performed by two pathologists and the first author of the study. They were "blind" with regard to the clinical follow-up. The area of fibrosis was quantitatively evaluated. Data on the patients with regard to age, death and occurrence of liver transplantation were searched on the patients records. Results: age at portoenterostomy varied between 24 and 251 days of life (90.4 ±44.8 days and follow-up was available in 32 cases (72%. The nine cases (19% with extrahepatic congenital anomalies associated to biliary atresia did not present different prognosis from the remaining patients. Age at portoenterostomy influenced the prognosis (p=0.016. The area of fibrosis was different on patients aged less than 60 days and those aged more than 90 days at portoenterostomy (p=0.023, but did not influence the prognosis. The presence of ductal plate malformation, as well, did not influence the follow-up. Conclusions: age at portoenterostomy was the only factor that influenced prognosis on this sample of biliary atresia. It is necessary to increase the biliary atresia sample to check the influence of congenital extrahepatic anomalies on the follow-up post-portoenterostomy.

  5. Postoperative follow-up studies in biliary atresia using radioisotope

    Energy Technology Data Exchange (ETDEWEB)

    Kanto, Kei; Ishida, Haruo; Hayashi, Akira; Kamagata, Shoichiro; Sanbonmatsu, Toru; Matsufuji, Hiroshi; Ishii, Katsumi

    1988-09-01

    With increasing numbers of long survival patients in biliary atresia, associated diseases such as liver cirrhosis and portal hypertension seem to be more important in their course. We use liver scintigraphy, hepatobiliary scintigraphy and transrectal portal scintigraphy as the follow-up study. Three studies generally correlate the present state of the patients, but there seems to be dissociation in the group of cirrhosis without icterus which are encountered most often in biliary atresia. That can be seen in hepatobiliary scintigraphy especially. So we emphasis that to choose several isotope studies are essential in determination of the postoperative state in biliary atresia.

  6. Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia

    DEFF Research Database (Denmark)

    Madsen, Stine Skipper; Kvist, Nina; Thorup, Jørgen

    2015-01-01

    INTRODUCTION: Biliary atresia is the leading cause of liver transplantation in children. It affects 1:15,000 in Denmark. With a national birth rate of 60,000, four children are born every year with biliary atresia. Early correction of biliary obstruction is essential to prevent fatal biliary...... cirrhosis. The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of children with elevated level of serum bilirubin after two weeks of age. Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 μmol/l, and/or more than 20% of total bilirubin...

  7. Diagnostic Accuracy 99mTc-DISIDA Scintigraphy in Biliary Atresia

    International Nuclear Information System (INIS)

    Hyun, In Young; Lee, Dong Soo; Lee, Kyung Han; Kim, Jong Ho; Chung, June Key; Suh, Jung Key; Lee, Myung Chul; Koh, Chang Soon

    1994-01-01

    We evaluated the diagnostic accuracy of 99m Tc-DISIDA scintigraphy as a mean of differentiating biliary atresia from neonatal hepatitis. 99m Tc-DISIDA scintigraphy was visually interpreted by assessing the presence or absence of radioactivity in the intestine or gall bladder. In patients without intestinal radioactivity, we measured the hepatic retention index and the hepatic uptake index. The hepatic retention index was expressed as the amount of change of liver activity from 5 minutes to 30 minutes postinjection. The hepatic uptake index was graded visually with 5 minute images using the following scoring scheme :grade 0 (normal hepatic uptake), grade 1 (decreased hepatic up take), grade 2 (hepatic uptake equal to cardiac uptake), and grade 3 (hepatic uptake less than cardiac uptake). Age, total bilirubin, and hepatic uptake index were compared between the biliary atresia and the neonatal hepatitis group, between neonatal hepatitis patients with and without intestinal radioactivity, and between the biliary atresia and neonatal hepatitis patients with absent intestinal radioactivity. The results were as follows : l) None of the 30 biliary atresia patients showed intestinal radioactivity, while 31/40 neonatal hepatitis patients showed intestinal radioactivity. The sensitivity, specificity, and accuracy of the presence of intestinal radioactivity or the diagnosis of biliary atresia was 100%, 78%, and 87%, respectively. 2) In patients with absent intestinal radioactivity the mean hepatic retention index was 1.5 ± 0.6 in the 16 biliary atresia patient,s, and 1.1 ± 0.2 in the 7 neonatal hepatitis patients(p 99m Tc-DISIDA scintigraphy is accurate in the differential diagnosis of biliary atresia and neonatal hepatitis. In patients without intestinal radioactivity, the hepatic retention index and hepatic uptake index, along with the patients age and total bilirubin level may supplement diagnosis and improve diagnostic accuracy.

  8. Biliary atresia: the Canadian experience.

    Science.gov (United States)

    Schreiber, Richard A; Barker, Collin C; Roberts, Eve A; Martin, Steven R; Alvarez, Fernando; Smith, Lesley; Butzner, J Decker; Wrobel, Iwona; Mack, David; Moroz, Stanley; Rashid, Mohsin; Persad, Rabin; Levesque, Dominique; Brill, Herbert; Bruce, Garth; Critch, Jeff

    2007-12-01

    To determine the outcomes of Canadian children with biliary atresia. Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed. 349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS). This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.

  9. Ultrasonographic findings of type IIIa biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seung Seob; Kim, Myung Joon; Lee, Mi Jung; Yoon, Choon Sik; Han, Seok Joo; Koh, Hong [Dept. of Radiology, Research Institute of Radiological Science, Severance Hospital, Yensei University College of Medicine, Seoul (Korea, Republic of)

    2014-12-15

    To describe the ultrasonographic (US) findings of type IIIa biliary atresia. We retrospectively reviewed a medical database of patients pathologically confirmed to have biliary atresia, Kasai type IIIa, between January 2002 and May 2013 (n=18). We evaluated US findings including the visible common bile duct (CBD), triangular cord thickness, gallbladder size and shape, and subcapsular flow on color Doppler US; laboratory data; and pathological hepatic fibrosis grades. We divided them into two groups-those with visible (group A) and invisible (group B) CBD on US-and compared all parameters between the two groups. CBD was visible on US in five cases (27.8%; group A) and invisible in 13 cases (72.2%; group B). US was performed at an earlier age in group A than in group B (median, 27 days vs. 60 days; P=0.027) with the maximal age of 51 days. A comparison of the US findings revealed that the triangular cord thickness was smaller (4.1 mm vs. 4.9 mm; P=0.004) and the gallbladder length was larger (20.0 mm vs. 11.7 mm; P=0.021) in group A. The gallbladder shape did not differ between the two groups, and the subcapsular flow was positive in all cases of both groups. There was no significant difference in the laboratory data between the two groups. Upon pathological analysis, group A showed low-grade and group B showed low- to high-grade hepatic fibrosis. When CBD is visible on US in patients diagnosed with type IIIa biliary atresia, other US features could have a false negative status. A subcapsular flow on the color Doppler US would be noted in the type IIIa biliary atresia patients.

  10. Successful Outcome and Biliary Drainage in an Infant with Concurrent Alpha-1-Antitrypsin Deficiency and Biliary Atresia

    Directory of Open Access Journals (Sweden)

    Andrew W. Wang

    2017-01-01

    Full Text Available We describe the rare instance of concomitant biliary atresia and alpha-1-antitrypsin deficiency and the first documented successful portoenterostomy in this scenario. The potential for dual pathology must be recognized and underscores that prompt diagnosis of biliary atresia, despite concomitant alpha-1-antitrypsin deficiency, is essential to afford potential longstanding native liver function.

  11. Reconstruction of the mouse extrahepatic biliary tree using primary human extrahepatic cholangiocyte organoids

    DEFF Research Database (Denmark)

    Sampaziotis, Fotios; Justin, Alexander W; Tysoe, Olivia C

    2017-01-01

    The treatment of common bile duct (CBD) disorders, such as biliary atresia or ischemic strictures, is restricted by the lack of biliary tissue from healthy donors suitable for surgical reconstruction. Here we report a new method for the isolation and propagation of human cholangiocytes from....... The resulting bioengineered tissue can reconstruct the gallbladder wall and repair the biliary epithelium following transplantation into a mouse model of injury. Furthermore, bioengineered artificial ducts can replace the native CBD, with no evidence of cholestasis or occlusion of the lumen. In conclusion, ECOs...

  12. Acurácia diagnóstica do espessamento ecogênico periportal à ultra-sonografia e da histopatologia no diagnóstico diferencial da atresia biliar Accuracy of echogenic periportal enlargement image in ultrasonographic exams and histopathology in differential diagnosis of biliary atresia

    Directory of Open Access Journals (Sweden)

    Mariza L. V. Roquete

    2008-08-01

    histopathology, in isolation or in combination, for diagnostic differentiation between biliary atresia and intrahepatic cholestasis. METHODS: This was a retrospective study carried out between January 1990 and December 2004. Fifty-one cases of biliary atresia and 45 of intrahepatic cholestasis were analyzed. Histopathology was performed blind by a pathologist. The triangular cord sign was identified in ultrasound reports as the only diagnostic sign of biliary atresia. Sensitivity, specificity and accuracy were calculated for the triangular cord sign and histology both in isolation and in combination. The gold standard for diagnosis of biliary atresia was the appearance of the extrahepatic biliary tree via laparotomy. RESULTS: The triangular cord sign alone had sensitivity of 49%, specificity of 100% and accuracy of 72.5%. Histopathology compatible with extrahepatic biliary obstruction alone had 90.2% sensitivity, 84.6% specificity and 87.8% accuracy. The triangular cord sign and histopathology in isolation or combination resulted in sensitivity of 93.2%, specificity of 85.7% and accuracy of 90.3%. CONCLUSIONS: Finding the triangular cord sign on ultrasound is an indication for laparotomy. If the triangular cord sign is negative, liver biopsy is indicated; if histopathology reveals signs of biliary atresia, explorative laparotomy is indicated. In cases where the triangular cord sign is absent and histopathology indicates neonatal hepatitis or other intrahepatic cholestasis, clinical treatment or observation are recommended in accordance with the diagnosis.

  13. The Role of ARF6 in Biliary Atresia.

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    Mylarappa Ningappa

    Full Text Available Altered extrahepatic bile ducts, gut, and cardiovascular anomalies constitute the variable phenotype of biliary atresia (BA.To identify potential susceptibility loci, Caucasian children, normal (controls and with BA (cases at two US centers were compared at >550000 SNP loci. Systems biology analysis was carried out on the data. In order to validate a key gene identified in the analysis, biliary morphogenesis was evaluated in 2-5-day post-fertilization zebrafish embryos after morpholino-antisense oligonucleotide knockdown of the candidate gene ADP ribosylation factor-6 (ARF6, Mo-arf6.Among 39 and 24 cases at centers 1 and 2, respectively, and 1907 controls, which clustered together on principal component analysis, the SNPs rs3126184 and rs10140366 in a 3' flanking enhancer region for ARF6 demonstrated higher minor allele frequencies (MAF in each cohort, and 63 combined cases, compared with controls (0.286 vs. 0.131, P = 5.94x10-7, OR 2.66; 0.286 vs. 0.13, P = 5.57x10-7, OR 2.66. Significance was enhanced in 77 total cases, which included 14 additional BA genotyped at rs3126184 only (p = 1.58x10-2, OR = 2.66. Pathway analysis of the 1000 top-ranked SNPs in CHP cases revealed enrichment of genes for EGF regulators (p<1 x10-7, ERK/MAPK and CREB canonical pathways (p<1 x10-34, and functional networks for cellular development and proliferation (p<1 x10-45, further supporting the role of EGFR-ARF6 signaling in BA. In zebrafish embryos, Mo-arf6 injection resulted in a sparse intrahepatic biliary network, several biliary epithelial cell defects, and poor bile excretion to the gall bladder compared with uninjected embryos. Biliary defects were reproduced with the EGFR-blocker AG1478 alone or with Mo-arf6 at lower doses of each agent and rescued with arf6 mRNA.The BA-associated SNPs identify a chromosome 14q21.3 susceptibility locus encompassing the ARF6 gene. arf6 knockdown in zebrafish implicates early biliary dysgenesis as a basis for BA, and also

  14. Hepatic ADC value correlates with cirrhotic severity of patients with biliary atresia

    International Nuclear Information System (INIS)

    Mo, Yuan Heng; Jaw, Fu Shan; Ho, Ming Chih; Wang, Yung Cheng; Peng, Steven Shinn Forng

    2011-01-01

    Introduction: At least 40% of survivors of biliary atresia have progressive cirrhosis even after undergoing Kasai operation. The values of hepatic apparent diffusion coefficient and apparent-diffusion-coefficient-related indices were applied to biliary atresia patients and correlated with cirrhotic severity scores of model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte, and Child-Pugh systems. Materials and methods: Thirty-three biliary atresia patents (mean = 1140, 61–4314 days of age) received magnetic resonance image examinations due to complications of biliary atresia from April 2008 to August 2009. Two non-breath-hold diffusion weighted imaging sequences were performed with motion-probing gradients in three directions with two b values: 0/100 and 0/500 s/mm 2 ; 1000 ms/61.1 ms, time to repeat/time to echo; number of excitation, 1.0; 8 mm section thickness; 40 cm × 40 cm field of view; 128 × 256 matrix in all biliary atresia patients and 18 control subjects. We used the Spearman rank correlation test to analyze the relationship among the scores of model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte and Child-Pugh scores and right hepatic apparent diffusion coefficients, apparent diffusion coefficient using b factor of 500-albumin product and alanine transaminase/apparent diffusion coefficient with b factor of 500 ratio. Results: The right hepatic apparent diffusion coefficient using b factor of 100, apparent diffusion coefficient with b factor of 500 and product of apparent diffusion coefficient with b factor of 500-albumin level were significantly negatively correlated (p ≤ 0.0125) with model for end-stage liver disease or pediatric end-stage liver disease model, Child-Turcotte, and Child-Pugh scores of biliary atresia patients. The ratio of alanine transaminase level/right hepatic apparent diffusion coefficient with b factor of 500 was also significantly (p ≤ 0

  15. Silencing of the rotavirus NSP4 protein decreases the incidence of biliary atresia in murine model.

    Directory of Open Access Journals (Sweden)

    Jiexiong Feng

    Full Text Available Biliary atresia is a common disease in neonates which causes obstructive jaundice and progressive hepatic fibrosis. Our previous studies indicate that rotavirus infection is an initiator in the pathogenesis of experimental biliary atresia (BA through the induction of increased nuclear factor-kappaB and abnormal activation of the osteopontin inflammation pathway. In the setting of rotavirus infection, rotavirus nonstructural protein 4 (NSP4 serves as an important immunogen, viral protein 7 (VP7 is necessary in rotavirus maturity and viral protein 4 (VP4 is a virulence determiner. The purpose of the current study is to clarify the roles of NSP4, VP7 and VP4 in the pathogenesis of experimental BA. Primary cultured extrahepatic biliary epithelia were infected with Rotavirus (mmu18006. Small interfering RNA targeting NSP4, VP7 or VP4 was transfected before rotavirus infection both in vitro and in vivo. We analyzed the incidence of BA, morphological change, morphogenesis of viral particles and viral mRNA and protein expression. The in vitro experiments showed NSP4 silencing decreased the levels of VP7 and VP4, reduced viral particles and decreased cytopathic effect. NSP4-positive cells had strongly positive expression of integrin subunit α2. Silencing of VP7 or VP4 partially decreased epithelial injury. Animal experiments indicated after NSP4 silencing, mouse pups had lower incidence of BA than after VP7 or VP4 silencing. However, 33.3% of VP4-silenced pups (N = 6 suffered BA and 50% of pups (N = 6 suffered biliary injury after VP7 silencing. Hepatic injury was decreased after NSP4 or VP4 silencing. Neither VP4 nor VP7 were detected in the biliary ducts after NSP4. All together, NSP4 silencing down-regulates VP7 and VP4, resulting in decreased incidence of BA.

  16. Branchial cleft anomaly, congenital heart disease, and biliary atresia: Goldenhar complex or Lambert syndrome?

    Science.gov (United States)

    Cohen, J; Schanen, N C

    2000-01-01

    The features of Goldenhar complex have been well-described and classically include branchial arch abnormalities, epibulbar dermoid and vertebral abnormalities. We have identified an infant with these features in association with complex congenital heart disease and intrahepatic biliary atresia. Although Lambert described an autosomal recessive disorder with an association of biliary atresia and branchial arch abnormalities, none of those cases had epibulbar dermoid. Diagnostic considerations in this case include inclusion of biliary atresia as a new feature in the expanding spectrum of the Goldenhar complex, versus Lambert syndrome with epibulbar dermoid.

  17. Successful surgical management of an extrahepatic biliary cystadenocarcinoma

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    Márcio Lobo Guimarães

    2011-10-01

    Full Text Available Extrahepatic bile duct cancer is an uncommon disease, and few cases are curable by surgery. We report a case of extrahepatic biliary cystadenocarcinoma (BCAC associated with atrophy of the left hepatic lobe. A 54-year old male was admitted with painless obstructive jaundice and a hepatic palpable mass noticed one month before presentation. Liver functions tests were consistent with cholestatic damage and serum carbohydrate antigen 19.9 (CA 19-9 was increased before treatment. Magnetic resonance imaging (MRI disclosed dilatation of the left hepatic bile duct with irregular wall thickening close to the hepatic confluence, and atrophy of left hepatic lobe. The patient was submitted to en bloc extended left hepatectomy with resection of caudate lobe, hilar lymphadenectomy, and suprapancreatic biliary tree resection. All surgical margins were grossly negative, and postoperative course was uneventful, except for a minor bile leak. The patient was discharged on the 15th postoperative day; he is alive without tumor recurrence one year after primary therapy. Although technically challenging, extended en bloc resection is feasible in adults with extrahepatic BCAC and can improve survival with acceptable and manageable morbidity.

  18. Association between biliary complications and technique of hilar division (extrahepatic vs. intrahepatic in major liver resections

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    Gamaletsos Evangelos

    2006-08-01

    Full Text Available Abstract Background Division of major vascular and biliary structures during major hepatectomies can be carried out either extrahepatically at the porta hepatic or intrahepatically during the parenchymal transection. In this retrospective study we test the hypothesis that the intrahepatic technique is associated with less early biliary complications. Methods 150 patients who underwent major hepatectomies were retrospectively allocated into an intrahepatic group (n = 100 and an extrahepatic group (n = 50 based on the technique of hilar division. The two groups were operated by two different surgical teams, each one favoring one of the two approaches for hilar dissection. Operative data (warm ischemic time, operative time, blood loss, biliary complications, morbidity and mortality rates were analyzed. Results In extrahepatic patients, operative time was longer (245 ± 50 vs 214 ± 38 min, p Conclusion Intrahepatic hilar division is as safe as extrahepatic hilar division in terms of intraoperative blood requirements, morbidity and mortality. The extrahepatic technique is associated with more severe bile leaks and biliary injuries.

  19. Extrahepatic biliary obstruction; postoperative morbidity and mortality

    International Nuclear Information System (INIS)

    Hussain, Z.; Khan, K.I.; Vaseem, M.; Rana, S.H.

    2010-01-01

    The objectives of this study are to evaluate the surgical management, both definitive and palliative, in selected patients with biliary obstruction and to find out the postoperative morbidity and mortality in these patients. Duration of the study is two years conducted from June 2002 to May 2004. The study was carried out at. the surgical. unit 4 of the Combined Military Hospital and surgical department of the Military Hospital. Thirty eight cases of biliary obstruction were included. A convenient sampling technique was followed. Data analyzed by using SPSS version 10.0 for windows on computer. Descriptive statistics like frequency, percentage, average etc were computed for data presentation. Any inferential test-was not found to be applicable for this descriptive type case series. We selected 38 patients with features of extrahepatic biliary obstruction. Out of these (n 38) 15 patients (39.5%) suffered from benign diseases while those having malignant diseases were 23 (60.5%). 19 (50%) patients died within two years of follow up while 19 (50%) were the survivors. Mortality was maximum for the malignant cases. In benign cases only one patient died. Maximum deaths 6 (31.6%) occurred in the period of up to one month of operation. 20 patients had one or another complication of operation and hence the morbidity came out to be 52%. According to our results the mortality and morbidity related to extrahepatic biliary obstruction in our patients was higher compared to other studies which can only be reduced by early detection and treatment. (author)

  20. Extrahepatic biliary obstrution secondary to neuroendocrine tumor of the common hepatic duct

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    Faraz A. Khan

    2017-01-01

    Conclusion: NET of the extrahepatic biliary tree are a rare entity. Typical presentation is with painless jaundice and other symptoms related to obstruction of the biliary tree and the diagnosis is usually made post-operatively.

  1. A Point Mutation in the Rhesus Rotavirus VP4 Protein Generated through a Rotavirus Reverse Genetics System Attenuates Biliary Atresia in the Murine Model.

    Science.gov (United States)

    Mohanty, Sujit K; Donnelly, Bryan; Dupree, Phylicia; Lobeck, Inna; Mowery, Sarah; Meller, Jaroslaw; McNeal, Monica; Tiao, Greg

    2017-08-01

    Rotavirus infection is one of the most common causes of diarrheal illness in humans. In neonatal mice, rhesus rotavirus (RRV) can induce biliary atresia (BA), a disease resulting in inflammatory obstruction of the extrahepatic biliary tract and intrahepatic bile ducts. We previously showed that the amino acid arginine (R) within the sequence SRL (amino acids 445 to 447) in the RRV VP4 protein is required for viral binding and entry into biliary epithelial cells. To determine if this single amino acid (R) influences the pathogenicity of the virus, we generated a recombinant virus with a single amino acid mutation at this site through a reverse genetics system. We demonstrated that the RRV mutant (RRV VP4-R446G ) produced less symptomatology and replicated to lower titers both in vivo and in vitro than those seen with wild-type RRV, with reduced binding in cholangiocytes. Our results demonstrate that a single amino acid change in the RRV VP4 gene influences cholangiocyte tropism and reduces pathogenicity in mice. IMPORTANCE Rotavirus is the leading cause of diarrhea in humans. Rhesus rotavirus (RRV) can also lead to biliary atresia (a neonatal human disease) in mice. We developed a reverse genetics system to create a mutant of RRV (RRV VP4-R446G ) with a single amino acid change in the VP4 protein compared to that of wild-type RRV. In vitro , the mutant virus had reduced binding and infectivity in cholangiocytes. In vivo , it produced fewer symptoms and lower mortality in neonatal mice, resulting in an attenuated form of biliary atresia. Copyright © 2017 American Society for Microbiology.

  2. A Technique to Define Extrahepatic Biliary Anatomy Using Robotic Near-Infrared Fluorescent Cholangiography.

    Science.gov (United States)

    Maker, Ajay V; Kunda, Nicholas

    2017-11-01

    Bile duct injury is a rare but serious complication of minimally invasive cholecystectomy. Traditionally, intraoperative cholangiogram has been used in difficult cases to help delineate anatomical structures, however, new imaging modalities are currently available to aid in the identification of extrahepatic biliary anatomy, including near-infrared fluorescent cholangiography (NIFC) using indocyanine green (ICG).1-5 The objective of the study was to evaluate if this technique may aid in safe dissection to obtain the critical view. Thirty-five consecutive multiport robotic cholecystectomies using NIFC with ICG were performed using the da Vinci Firefly Fluorescence Imaging System. All patients received 2.5 mg ICG intravenously at the time of intubation, followed by patient positioning, draping, and establishment of pneumoperitoneum. No structures were divided until the critical view of safety was achieved. Real-time toggling between NIFC and bright-light illumination was utilized throughout the case to define the extrahepatic biliary anatomy. ICG was successfully administered to all patients without complication, and in all cases the extrahepatic biliary anatomy was able to be identified in real-time 3D. All procedures were completed without biliary injury, conversion to an open procedure, or need for traditional cholangiography to obtain the critical view. Specific examples of cases where x-ray cholangiography or conversion to open was avoided and NIFC aided in safe dissection leading to the critical view are demonstrated, including (1) evaluation for aberrant biliary anatomy, (2) confirmation of non-biliary structures, and (3) use in cases where the infundibulum is fused to the common bile duct. NIFC using ICG is demonstrated as a useful technique to rapidly identify and aid in the visualization of extrahepatic biliary anatomy. Techniques that selectively utilize this technology specifically in difficult cases where the anatomy is unclear are demonstrated in order

  3. Biliary Atresia – An Easily Missed Cause of Jaundice amongst ...

    African Journals Online (AJOL)

    Back ground: Biliary atresia is characterized by biliary obstruction, it has an incidence of 1:15000 and presents with jaundice, acholic stools / dark urine and hepatomegaly. This disease rapidly leads to liver cirrhosis and liver failure if untreated surgically. The main objective was to establish the epidemiology of patients ...

  4. Subdural hemorrhage: A unique case involving secondary vitamin K deficiency bleeding due to biliary atresia.

    Science.gov (United States)

    Miyao, Masashi; Abiru, Hitoshi; Ozeki, Munetaka; Kotani, Hirokazu; Tsuruyama, Tatsuaki; Kobayashi, Naho; Omae, Tadaki; Osamura, Toshio; Tamaki, Keiji

    2012-09-10

    Extrahepatic biliary atresia (EHBA) is a rare disease characterized by progressive and obliterative cholangiopathy in infants and is one of the major causes of secondary vitamin K deficiency bleeding (VKDB) due to cholestasis-induced fat malabsorption. Breast feeding increases the tendency of bleeding in EHBA patients because breast milk contains low amounts of vitamin K. A 2-month-old female infant unexpectedly died, with symptoms of vomiting and jaundice prior to death. She had been born by uncomplicated vaginal delivery and exhibited normal growth and development with breastfeeding. There was no history of trauma. She received vitamin K prophylaxis orally. In an emergency hospital, a CT scan showed a right intracranial hematoma and mass effect with midline shift to the left. In the postmortem examination, severe atresia was observed in the whole extrahepatic bile duct. Histologically, cholestasis, periductal fibrosis, and distorted bile ductules were noted. The gallbladder was not identified. A subdural hematoma and cerebellar tonsillar herniation were found; however, no traumatic injury in any part of the body was observed. Together, these findings suggest that the subdural hemorrhage was caused by secondary vitamin K deficiency resulting from a combination of cholestasis-induced fat malabsorption and breastfeeding. Subdural hemorrhage by secondary VKDB sometimes occurs even when vitamin K prophylaxis is continued. This case demonstrated that intrinsic factors, such as secondary VKDB (e.g., EHBA, neonatal hepatitis, chronic diarrhea), should also be considered in infant autopsy cases presenting with subdural hemorrhage. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  5. Ultrasonographic differentiation of biliary atresia and neonatal hepatitis: Reestablishment of size criteria of the gallbladder

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Woo Sun; Cheon, Jung Eun; Koh, Young Hwan; Kim, In One; Yeon, Kyung Mo [Seoul National University College of Medicine and Institude of Radiation Medicion, Seoul (Korea, Republic of)

    2001-12-15

    To reestablish the size criterion of the gallbladder on ultrasonography (US) for the differentiation diagnosis of biliary atresia from neonatal hepatitis. Abdominal US ws performed in 201 patients with jaundice and 40 patients without evidence of jaundice or hepatobiliary illness (all with the age less than 4 months). US was performed in fasting (fasting for at least 4 hours) to measure the length of the gallbladder and calculated the area of the gallbladder lumen. The morphology of the gallbladder was classified into three types: normal, elongated and atretic. To evaluate the contractibility of the gallbladder, the length of the gallbladder and area of the gallbladder lumen was again measured 1 hour after feeding. The final diagnosis included biliary atresia in 79 patients and neonatal hepatitis in 83 patients. Differences in the length, area, and morphology of the gallbladder were statistically significant among three groups, the normal group, neonatal hepatitis group and biliary atresia group (length and area of gallbladder; normal group>neonatal hepatitis>biliary atresia). The differences in the length and area of gallbladder between pre- and postmeal state were statistically significant in the normal and neonatal hepatitis groups whereas those of biliary atresia were not significant (p=0.85). When the empirical size criterion of the gallbladder (<15 mm in length) was applied, the sensitivity, specificity and diagnostic accuracy for the differential diagnosis of biliary atresia from hepatitis were 52%, 82%, and 67%, respectively. Meanwhile, if the area criterion(<30 mm{sup 2} in area) was applied, the sensitivity, the specificity and diagnostic accuracy were 67%, 85%, and 75%, respectively. Ultrasonographic evaluation of the morphology as well as size of the gallbladder are helpful in the differential diagnosis of biliary atresia from neonatal hepatitis. Therefore, since the measurement of the area of gallbladder lumen on US reflect both size and morphology of

  6. Hepatocellular carcinoma in the native liver of a 38-year-old female patient with biliary atresia

    Directory of Open Access Journals (Sweden)

    Yutaka Kanamori

    2015-11-01

    Full Text Available We report a rare case of hepatocellular carcinoma in native liver in a case of biliary atresia. The patient was a 38-year-old female with three children who had an aggressive tumor, resulting in her subsequent death. We also review 14 reports, published previously in the English language medical literature, concerning hepatocellular carcinoma originating from native liver in biliary atresia cases and discuss the possible etiology, and propose more careful follow up for the patients with biliary atresia who suffer from repetitive cholangitis and/or experience the child delivery.

  7. Expression of Bcl-2 and Bax in extrahepatic biliary tract carcinoma and dysplasia

    Science.gov (United States)

    Li, Sheng-Mian; Yao, Shu-Kun; Yamamura, Nobuyoshi; Nakamura, Toshitsugu

    2003-01-01

    AIM: To compare the difference of expression of Bcl-2 and Bax in extrahepatic biliary tract carcinoma and dysplasia, and to analyze the role of Bcl-2 and Bax proteins in the progression from dysplasia to carcinoma and to evaluate the correlation of Bcl-2/Bax protein expression with the biological behaviors. METHODS: Expressions of Bcl-2 and Bax were examined immunohistochemically in 27 cases of extrahepatic biliary tract carcinomas (bile duct carcinoma: n = 21, carcinoma of ampulla of Vater: n = 6), and 10 cases of atypical dysplasia. Five cases of normal biliary epithelial tissues were used as controls. A semiquantitative scoring system was used to assess the Bcl-2 and Bax reactivity. RESULTS: The expression of Bcl-2 was observed in 10 out of 27 (37.0%) invasive carcinomas, 1 out of 10 dysplasias, none out of 5 normal epithelial tissues. Bax expression rate was 74.1% (20/27) in invasive carcinoma, 30% (3/10) in dysplasia, and 40% (2/5) in normal biliary epithelium. Bcl-2 and Bax activities were more intense in carcinoma than in dysplasia, with no significant difference in Bcl-2 expression (P = 0.110), and significant difference in Bax expression (P = 0.038). Level of Bax expression was higher in invasive carcinoma than in dysplasia and normal tissue (P = 0.012). Bcl-2 expression was correlated to Bax expression (P = 0.0059). However, Bcl-2/Bax expression had no correlation with histological subtype, grade of differentiation, or level of invasion. CONCLUSION: Increased Bcl-2/Bax expression from dysplasia to invasive tumors supports the view that this is the usual route for the development of extrahepatic biliary tract carcinoma. Bcl-2/Bax may be involved, at least in part, in the apoptotic activity in extrahepatic biliary carcinoma. PMID:14606101

  8. Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia

    DEFF Research Database (Denmark)

    Madsen, Stine Skipper; Kvist, Nina; Thorup, Jørgen

    2015-01-01

    . This percentage value has caused diagnostic trouble over the years. The objective of the present study was to investigate the possibility of changing the recommendations. METHODS: This was a retrospective analysis of the medical records of children operated for biliary atresia in the 1993-2012 period. RESULTS......: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion. FUNDING: none. TRIAL REGISTRATION...

  9. European biliary atresia registries: summary of a symposium

    DEFF Research Database (Denmark)

    Petersen, C.; Harder, D.; Abola, Z.

    2008-01-01

    , centralization of surgery). In conclusion, EBAR represents the first attempt at an overall evaluation of the outcome of BA from a pan-European perspective. The natural history and outcome of biliary atresia is of considerable relevance to a European population. It is essential that there is further support...

  10. A prospective pilot study: Can the biliary tree be visualized in children younger than 3 months on Magnetic Resonance Cholangiopancreatography?

    Energy Technology Data Exchange (ETDEWEB)

    Siles, Pascale [La Timone Children' s Hospital, Department of Radiology, Marseille (France); Aschero, Audrey; Gorincour, Guillaume; Bourliere-Najean, Brigitte; Petit, Philippe [La Timone Children' s Hospital, Department of Pediatric Radiology, Marseille (France); Roquelaure, Bertrand [La Timone Children' s Hospital, Department of Pediatrics, Marseille (France); Delarue, Arnauld [La Timone Children' s Hospital, Department of Pediatric Surgery, Marseille (France)

    2014-09-15

    Magnetic resonance cholangiopancreatography (MRCP) could aid in the diagnosis of biliary atresia, a hepatic pathology with thin, irregular or interrupted biliary ducts. There is little published evidence of MRCP appearances in normal neonates and young infants. To assess the use of MR cholangiopancreatography in visualizing the biliary tree in neonates and infants younger than 3 months with no hepatobiliary disorder, and to assess this visibility in relationship to the child's age, weight, and sedation and fasting states. Between December 2008 and October 2010 our department performed MRI of the brain, orbits and face on 16 full-term neonates and infants. Each child was younger than 3 months (90 days) and without any hepatobiliary disorders. The children were scanned with a respiratory-gated 0.54 x 0.51 x 0.4-mm{sup 3} 3-D MRCP sequence. We used a reading grid to assess subjectively the visibility of the extrahepatic bile ducts along with extrahepatic bile duct confluence. The visibility of the extrahepatic bile duct confluence was assessed against age, weight, and sedation and fasting states. The extrahepatic bile duct confluence was seen in 10 children out of 16 (62.5%). In the neonate sub-group (corrected age younger than 30 days), the MRCP was technically workable and the extrahepatic bile duct confluence was seen in four cases out of eight (50%). This visualization was up to 75% in the subgroup older than 30 days. However, statistically there was no significant difference in visibility of the extrahepatic bile duct confluence in relationship to age, weight or MRCP performance conditions (feeding, fasting or sedation). The complete normal biliary system (extrahepatic bile duct confluence included) is not consistently visualized in infants younger than 3 months old on non-enhanced MRCP. Thus the use of MRCP to exclude a diagnosis of biliary atresia is compromised at optimal time of surgery. (orig.)

  11. A prospective pilot study: Can the biliary tree be visualized in children younger than 3 months on Magnetic Resonance Cholangiopancreatography?

    International Nuclear Information System (INIS)

    Siles, Pascale; Aschero, Audrey; Gorincour, Guillaume; Bourliere-Najean, Brigitte; Petit, Philippe; Roquelaure, Bertrand; Delarue, Arnauld

    2014-01-01

    Magnetic resonance cholangiopancreatography (MRCP) could aid in the diagnosis of biliary atresia, a hepatic pathology with thin, irregular or interrupted biliary ducts. There is little published evidence of MRCP appearances in normal neonates and young infants. To assess the use of MR cholangiopancreatography in visualizing the biliary tree in neonates and infants younger than 3 months with no hepatobiliary disorder, and to assess this visibility in relationship to the child's age, weight, and sedation and fasting states. Between December 2008 and October 2010 our department performed MRI of the brain, orbits and face on 16 full-term neonates and infants. Each child was younger than 3 months (90 days) and without any hepatobiliary disorders. The children were scanned with a respiratory-gated 0.54 x 0.51 x 0.4-mm 3 3-D MRCP sequence. We used a reading grid to assess subjectively the visibility of the extrahepatic bile ducts along with extrahepatic bile duct confluence. The visibility of the extrahepatic bile duct confluence was assessed against age, weight, and sedation and fasting states. The extrahepatic bile duct confluence was seen in 10 children out of 16 (62.5%). In the neonate sub-group (corrected age younger than 30 days), the MRCP was technically workable and the extrahepatic bile duct confluence was seen in four cases out of eight (50%). This visualization was up to 75% in the subgroup older than 30 days. However, statistically there was no significant difference in visibility of the extrahepatic bile duct confluence in relationship to age, weight or MRCP performance conditions (feeding, fasting or sedation). The complete normal biliary system (extrahepatic bile duct confluence included) is not consistently visualized in infants younger than 3 months old on non-enhanced MRCP. Thus the use of MRCP to exclude a diagnosis of biliary atresia is compromised at optimal time of surgery. (orig.)

  12. Extrahepatic biliary cystadenoma with mesenchymal stroma: a true biliary cystadenoma? A case report.

    LENUS (Irish Health Repository)

    Hennessey, Derek B

    2012-02-01

    Biliary cystadenomas are benign but potentially malignant cystic neoplasms, which classically contain mesenchymal stroma similar to ovarian tissue. We report a case of an extra-hepatic biliary cystadenoma with mesenchymal stroma along with a discussion of current pathological opinion. CASE PRESENTATION: A 54-year-old female presented with abdominal pain, abnormal liver function tests and a mass on ultrasound. Computerized Tomography identified a complex multi-locular cyst in the common hepatic duct. Radical excision of the lesion and a Roux-en-Y loop bilio-enteric anastomosis was performed. Histology confirmed the presence of a benign biliary cystadenoma with ovarian type stroma. CONCLUSION: Biliary cystadenomas classically contain mesenchymal stroma similar to ovarian tissue. It now appears that cystadenomas without mesenchymal stroma appear to be more akin to similar cystic lesions of the pancreas, and may represent a dissimilar neoplasm. Therefore, malignant transformation can occur, so complete excision is recommended.

  13. Physical development and cognitive performance in a monozygotic twins for biliary atresia: Report of a case and literature reviewing

    Directory of Open Access Journals (Sweden)

    Yang Chen

    2016-08-01

    Full Text Available A case of monozygotic twins, one of whom suffered from biliary atresia and the other one was apparently healthy, is described herein. Children with biliary atresia appear to be vulnerable to developmental lags in the physical and cognitive performance, due to malnutrition and deteriorated liver function. In our case where the child with biliary atresia, who underwent a successful kasai operation, has achieved comparable growth development and cognitive performance in comparision with her sister, which suggest that the early diagnosis and timly kasai operation could allow children with biliary artesia to grow well in the short-term following time.

  14. Sonic hedgehog (SHH) and glioblastoma-2 (Gli-2) expressions are associated with poor jaundice-free survival in biliary atresia.

    Science.gov (United States)

    Jung, Hae Yoen; Jing, Jin; Lee, Kyoung Bun; Jang, Ja-June

    2015-03-01

    Biliary atresia (BA) causes biliary obstruction in neonates. Although the Kasai operation can successfully treat certain BA cases, many patients exhibit recurrent jaundice and secondary biliary cirrhosis requiring liver transplantation. Consequently, studies of the prognostic factors of the Kasai operation are needed. Accordingly, sonic hedgehog (SHH) pathway expression at the extrahepatic bile duct (EHBD), an important bile duct repair mechanism, will be investigated via immunohistochemistry in patients with BA to examine the association with post-Kasai operation prognosis. Fifty-seven EHBD specimens were obtained during Kasai operations from 1992 to 2009. The SHH, patched (PTCH), and glioblastoma-2 (Gli-2) immunohistochemical staining results were analyzed quantitatively. Overall, 57.9% of patients had bile flow normalization after the Kasai operation; 43.1% did not. High preoperative serum total bilirubin, direct bilirubin, and aspartate aminotransferase levels were associated with sustained jaundice post-Kasai operation, as was an age ≥65days at the time of surgery (all pjaundice relapse. Strong Gli-2 and SHH expression in the EHBD might be a poor prognostic factor in Kasai operation-treated patients with BA. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Role of biliary tract cytology in the evaluation of extrahepatic cholestatic jaundice

    Science.gov (United States)

    Gupta, Mamta; Pai, Radha R.; Dileep, Devi; Gopal, Sandeep; Shenoy, Suresh

    2013-01-01

    Background: Endoscopic evaluation is critical in assessing the cause of obstructive jaundice. Cytological techniques including bile aspiration and biliary brushings have become the initial diagnostic modality. Aim: The aim of this study is to evaluate the role of endoscopic biliary tract cytology as a diagnostic tool in the evaluation of extrahepatic cholestatic jaundice. Materials and Methods: A total of 56 biliary tract specimens including 34 bile aspirations and 22 biliary brushings from 41 consecutive patients who had presented with obstructive jaundice and underwent endoscopic retrograde cholangiopancreatography (ERCP) were assessed by cytological examination. The smears prepared were analyzed for standard cytological features. Results: Cytologic diagnosis was adenocarcinoma in 13 (31.7%) cases, atypical in 2 (4.9%), reactive in 3 (7.3%) and benign changes in 19 (46.3%) cases. 4 (9.8%) cases were non-diagnostic. Serum bilirubin was significantly elevated in the malignant group. Biliary stricture was the most common finding on ERCP (68.3%). On cytological examination, presence of solitary, intact atypical cells, enlarged nuclei, irregular nuclear membrane, coarse chromatin and nucleoli were important cytologic criteria for differentiating malignant from benign biliary specimens. Conclusions: Regular use of bile cytology and brushings during ERCP evaluation of extrahepatic cholestatic jaundice is invaluable in obtaining a morphologic diagnosis. A systematic approach, use of strict cytomorphologic criteria and inclusion of significant atypia as malignant diagnosis may improve the sensitivity. PMID:24130407

  16. MRI-based decision tree model for diagnosis of biliary atresia.

    Science.gov (United States)

    Kim, Yong Hee; Kim, Myung-Joon; Shin, Hyun Joo; Yoon, Haesung; Han, Seok Joo; Koh, Hong; Roh, Yun Ho; Lee, Mi-Jung

    2018-02-23

    To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice. We retrospectively reviewed features of MRI and ultrasonography (US) performed in infants with jaundice between January 2009 and June 2016 under approval of the institutional review board, including the maximum diameter of periportal signal change on MRI (MR triangular cord thickness, MR-TCT) or US (US-TCT), visibility of common bile duct (CBD) and abnormality of gallbladder (GB). Hepatic subcapsular flow was reviewed on Doppler US. We performed conditional inference tree analysis using MRI findings to generate a decision tree model. A total of 208 infants were included, 112 in the BA group and 96 in the non-BA group. Mean age at the time of MRI was 58.7 ± 36.6 days. Visibility of CBD, abnormality of GB and MR-TCT were good discriminators for the diagnosis of BA and the MRI-based decision tree using these findings with MR-TCT cut-off 5.1 mm showed 97.3 % sensitivity, 94.8 % specificity and 96.2 % accuracy. MRI-based decision tree model reliably differentiates BA in infants with jaundice. MRI can be an objective imaging modality for the diagnosis of BA. • MRI-based decision tree model reliably differentiates biliary atresia in neonatal cholestasis. • Common bile duct, gallbladder and periportal signal changes are the discriminators. • MRI has comparable performance to ultrasonography for diagnosis of biliary atresia.

  17. Biliary atresia with hyaline cartilage at the porta hepatis: a novel ...

    African Journals Online (AJOL)

    Biliary atresia is an important cause of liver disease and morbidity in infants with ... hypoglycemia, nocturnal feed, constipation, or previous hospitalization was present. ... A clinical diagnosis of neonatal cholestasis (BA) was considered.

  18. Value of sup(99m)Tc-diethyl-IDA scintigraphy for the diagnosis of biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Bourdelat, D.; Gruel, Y.; Guibert, L.; Babut, J.M. (Hopital Pontchaillou 35 - Rennes (France)); Bourguet, P.; Herry, J.Y. (Centre Eugene Marquis, CHR Pontchaillou, 35 - Rennes (France))

    1983-04-01

    With reference to three cases, the value of cholescintigraphy for the diagnosis of biliary atresia is underscored. With this procedure, surgical indications can be determined in neonates with jaundice persisting beyond the physiologic period. Clolescintigraphy can be repeated as it is easy to perform, safe and well tolerated. sup(99m)Tc-diethyl-IDA (technetium 99m labelled N-(2,6 diethyl-acetanilide)-iminodiacetic acid) scintigraphy was carried out in eight neonates. This procedure helped to outrule the diagnosis of biliary atresia in 5 cases (1 choledochal cyst, 1 alpha-1-antitrypsin deficiency and 3 neonatal hepatitis'). Scintigraphic images and time/activity curves generated simultaneously from equal surfaces over the heart and liver were analyzed separately. A good uptake by the liver (rapid decrease in the cardiac curve) with subsequent retention (no decrease in the hepatic curve) is suggestive of biliary atresia. No activity is detected in the biliary ducts or intestinal tract. Post-operatively, cholescintigraphy is a useful tool for controling the efficiency of the surgical procedure.

  19. Biliary atresia: lessons learned from the voluntary German registry.

    Science.gov (United States)

    Leonhardt, J; Kuebler, J F; Leute, P J; Turowski, C; Becker, T; Pfister, E-D; Ure, B; Petersen, C

    2011-03-01

    Aim of the study was to carry out a 5-year survey of German patients with biliary atresia (BA) and to launch a discussion regarding the feasibility of voluntary registries in unregulated healthcare systems. A retrospective analysis of German BA patients born between 2001 and 2005, based on data collected from the voluntary European Biliary Atresia Registry (EBAR), was carried out and supplemented by data from all BA patients who underwent liver transplantation at the only 4 pediatric transplantation centers (pLTx) in Germany which are so far not registered at EBAR. Survival rates were calculated using Kaplan-Meier analysis and compared by Cox regression to determine the predictive value of age at surgery and the influence of the center size (fewer or more than 5 patients/study period) on overall survival and survival with native liver. A critical review of the 148 German EBAR charts revealed that 11 patients (7.4%) had no biliary atresia. The remaining 137 patients from EBAR together with 46 BA patients who underwent LTx without prior registration at EBAR were evaluated with a median follow-up of 39 months (range: 25-85 months). 29 hospitals performed a total of 159 Kasai procedures, but only 7 centers treated 5 or more patients (116 patients, range: 5-68), and 22 hospitals performed less than 5 KP (43 patients, range: 1-4). Primary LTx was performed in 21 patients (11.5%) and 3 patients died without surgical intervention. 16 patients were lost to follow-up (8.7%). Overall survival after 2 years was 83.3% (139 patients), including 105 patients (63%) who had undergone LTx and 34 patients (20.3%) with native liver. 28 patients died (16.7%), 8 after LTx (5.8%). The experience of the center was the only factor with a significant predictive value for jaundice-free survival with native liver (p=0.001). 25% of all German BA patients were not registered at EBAR, and 29 clinics were involved in the surgical management of BA patients. Therefore a new approach consisting of

  20. Thirty-four years' experience with biliary atresia in Denmark

    DEFF Research Database (Denmark)

    Kvist, N; Davenport, M

    2011-01-01

    Biliary atresia (BA) is a rare disease in Denmark (population ∼ 5.5 million) and there has been some controversy on how smaller countries should manage such conditions to obtain the best possible outcomes. The aim of this study was to evaluate the efficacy of primary surgery (i. e., Kasai portoen...

  1. Long-term follow-up of patients with a clinically benign extrahepatic biliary stenosis and K-ras mutation in endobiliary brush cytology

    NARCIS (Netherlands)

    van Heek, N. Tjarda; Rauws, Erik A. J.; Caspers, Eric; Drillenburg, Paul; Gouma, Dirk J.; Offerhaus, G. Johan A.

    2002-01-01

    Background. K-ras mutations in endobiliary brush cytology are an early event in carcinogenesis and justify a suspicion of malignancy in patients with extrahepatic biliary stenosis. However, K-ras mutations have been detected in specimens obtained by brushing of clinically benign extrahepatic biliary

  2. Unilocular extrahepatic biliary cystadenoma mimicking choledochal cyst: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ju Hyun; Lee, Dong Ho; Kim, Hyoung Jung; Ko, Young Tae; Lim, Joo Won; Yang, Moon Ho [Kyunghee University Hospital, Seoul (Korea, Republic of)

    2004-12-15

    We report here on a case of extrahepatic biliary cystadenoma arising from the common hepatic duct. A 42-year-old woman was evaluated by us to find the cause of her jaundice. Ultrasonography and CT showed a cystic dilatation of the common hepatic duct and also marked dilatation of the intrahepatic duct. Direct cholangiography demonstrated a large filling defect between the left hepatic duct and the common hepatic duct; dilatation of the intrahepatic duct was also demonstrated. Following excision of the cystic mass, it was pathologically confirmed as a unilocular biliary mucinous cystadenoma arising from the common hepatic duct.

  3. Long survival ( 21 years) after portoenterostomy for biliary atresia: A ...

    African Journals Online (AJOL)

    Long term survival for decades after portoenterostomy (Kasai procedure) for biliary atresia is rare and the association of portoenterostomy with liver cirrhosis is well known. Not much attention was given in the evaluation of the imaging features of cirrhosis caused by portoenterostomy as received by other known usual ...

  4. [Laparoscopic Kasai portoenterostom: present and future of biliary atresia treatment].

    Science.gov (United States)

    Ayuso, L; Vila-Carbó, J J; Lluna, J; Hernández, E; Marco, A

    2008-01-01

    Kasai's operation has proved its value in surgical treatment of biliary atresia (BA). Its laparoscopic approach is a new challenge for pediatric surgeons, with all the potential advantages of minimally invasive surgery. The aim of the present study has been to report our experience in laparoscopic management of five patients with biliary atresia. The average of age of five patients with biliary atresia, three boys and two girls was 58 days (range 40-64). Pre and postoperative management included antibiotic prophylaxis and choleretic treatment. Laparoscopic procedure was accomplished using one umbilical 10-mm trocar and two additional 5-mm trocars. We carried out the same technique in all the patients except in one of them with a total situs inversus and who compelled us to modify the original procedure. All five patients underwent a laparoscopic procedure, conversion was not necessary. The mean surgical time was 3 hours and 40 minutes (range: 5:30 y 3:10). There were not intra operative complications and all of them had a satisfactory recovery, except for the patient with situs inversus, who suffered a small bowel volvulus 9 days after the operation, leading us to perform an extensive bowel resection. All the patients, except this one, showed signs of adequate bile flow, with disappearance of clinical cholestasis. Biochemistry test became normal. Besides the certain advantages compared with conventional surgical procedures (lower surgical damage, diminished post-operative recovery), laparoscopic management of BA, allows a better exposure of the porta hepatis without hepatic mobilization so it shows similar or better preliminary results than conventional techniques. The advantages of laparoscopic portoenterostomy are yet to be proved whenever liver transplantation is indicated.

  5. Atresia biliar: continuamos operando tarde Biliary atresia: we still operate too late

    Directory of Open Access Journals (Sweden)

    Carlos O. Kieling

    2008-10-01

    Full Text Available OBJETIVO: Analisar a idade na cirurgia de crianças com atresia biliar e a sobrevida sem necessidade de transplante de fígado. O estudo foi realizado no Hospital de Clínicas de Porto Alegre. MÉTODOS: Foram revisados os prontuários dos pacientes operados entre 1982 e 2007, residentes no Rio Grande do Sul. RESULTADOS: Dos 112 casos estudados de crianças com atresia biliar, 38 (33,9% ocorreram de 1982 a 1989, 46 (41,1% de 1990 a 1999 e 28 (25,0% a partir de 2000. Em 12 (10,7% casos, não foi realizada a portoenterostomia. A idade na cirurgia variou de 25 a 297 dias (mediana: 80,5; IIQ25-75: 61,3-109,0 dias; em 20,5% dos casos, a idade das crianças foi menor do que 60 dias. Não houve diferença na idade, no momento do diagnóstico, entre as 3 décadas. Os pacientes do interior do estado (mediana: 87,0; IIQ25-75: 69,0-115,0 dias foram encaminhados significativamente (p = 0,007 mais tarde do que os da região metropolitana de Porto Alegre (RS (mediana: 68,0; IIQ25-75: 55,5-98,0 dias. A proporção de pacientes com menos de 60 dias foi significativamente menor (p = 0,013 nos oriundos do interior. A sobrevida com fígado nativo do total dos pacientes foi de 46,2% em 2 anos, diminuindo progressivamente até 15,3% em 20 anos. Os pacientes operados com menos de 60 dias tiveram maior sobrevida com fígado nativo (log rank OBJECTIVE: To analyze the age at surgery for children with biliary atresia and their survival periods without need for liver transplantation. The study was performed at Hospital de Clínicas de Porto Alegre, in Porto Alegre, state of Rio Grande do Sul, Brazil. METHODS: The medical records of patients operated between 1982 and 2007 who were residents of the state of Rio Grande do Sul were reviewed. RESULTS: Of the 112 cases of children with biliary atresia studied, 38 (33.9% occurred between 1982 and 1989, 46 (41.1% between 1990 and 1999 and 28 (25.0% after 2000. Portoenterostomy was not performed for 12 cases (10.7%. Age at surgery

  6. Annular pancreas causing extrahepatic biliary obstruction

    International Nuclear Information System (INIS)

    Ogulin, M.; Jamar, B.

    2004-01-01

    Background. Annular pancreas is an uncommon congenital abnormality, consisting of a flat band of pancreatic tissue, which encircles duodenum or extrahepatic biliary duct. We present a case of obstructive jaundice, caused by annular pancreas. Case report. A 46 years old female was admitted because of a sudden onset of abdominal pain, vomiting and jaundice. For the last six years she occasionally noticed her skin was light yellow, in the last year she felt distension in the upper abdomen, especially after fatty meals. Conclusions. Two US examinations, the first one six months before the admission, showed dilated hepatic ducts. The reason of dilatation was unclear, even after the endoscopic US examination. At operation an almost complete obstruction of the common hepatic duct was found, caused by a narrow band of pancreatic tissue. (author)

  7. Multidetector Computed Tomography Assessment in Biliary Atresia for the Diagnosis of Portosystemic Collaterals before Liver Transplant

    International Nuclear Information System (INIS)

    Cadavid A, Lina; Barber, Ignasi; Bueno, Javier

    2011-01-01

    Introduction: Chronic liver disease increases portal vein pressure and modifies splanchnic circulation. This is particularly significant in infants with biliary atresia. Large collaterals steal portal flow and increase the risk of post transplant portal vein thrombosis. Objective: to describe different types of portosystemic collaterals prior to liver transplantation with low-dose multidetector CT (MDCT) in patients with biliary atresia. Material and methods: 13 patients with severe liver dysfunction due to biliary atresia underwent low-dose 64-MDCT before liver transplantation (effective tube current ranged from 20 to 120 mAs according to weight, with a kilo voltage of 80-120 for all CT). Hepatic arterial and portal venous phases were performed after IV contrast administration [1.5-2 ml/kg]. The mean age of the study group was1 year (range, 4 months to 3.6 years). Two radiologists reviewed the CT images to determine the grade and types of the portosystemic collaterals. Results: A total of 16 CT scans were obtained.the most common portosystemic collaterals found were esophageal (11), gastric submucosal (8), gastric adventitial (7, splenic (7), hemorrhoidal (10), mesenteric [dilated or tortuous branches of the inferior mesenteric vein (8)], retroperitoneal varices [gastro renal shunt (10), splenorenal shunt (4)] and dilated or tortuous left gastric vein (13). Conclusion: MDCT provides important information on venous system patency, presence of varices, and location of venous shunts in pediatric patients with biliary atresia going to liver transplant. in addition, it is critical to detect collaterals that are not evident on ultrasound in order to avoid the steal phenomenon that may lead to portal vein thrombosis and graft failure.

  8. Scintigraphic hepatobiliary function studies in newborn infants to diagnose biliary hypoplasia or atresia

    International Nuclear Information System (INIS)

    Askari-Sabi, Z.

    1987-01-01

    The results obtained from scintigraphic hepatobiliary function studies, intraoperative cholangiography and histological examinations in a total of 17 infants suspected of having biliary atresia were compared and analysed with reference to the clinical signs and symptoms observed. In most cases, the individual diagnostic procedures led to consistent findings, even though there were some variations in the clinical picture. Patient outcome is largely determined by the site of atresia, due to which fact surgical correction should be carried out as soon as possible, in any case before the 8th week post partum. (TRV) [de

  9. Application of RI hepatogram to evaluate liver cirrhosis in biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Ogawa, Tomio; Suruga, Keijiro; Miyano, Takeshi; Arai, Takeo; Shimomura, Hiroshi; Nagase, Katsuya; Iida, Susumu; Arakawa, Yoshiya

    1985-01-01

    RI hepatgram with sup(99m)TcO/sub 4/ was developed for the evaluation of the changes in hepatic circulation in liver cirrhosis in 41 cases of pediatric liver diseases including 25 cases of biliary atresia. After bolus intravenous injection of 1-5 mCi of sup(99m)TcO/sub 4/ radioactive count over the abdomen was measured by a scinticamera for ten minutes. Time activity curve of a ROI on the liver right lobe was drawn for ten minutes. The count reached to plateau about one minute after injection and gradually decreased thereafter. The ratio of the counts of eight minutes to the plateau was calculated. It ranged from 49% to 98%, and this ratio correlated well with the physical and laboratory findings of liver cirrhosis. There is a significant correlation between the ratio and liver fibrosis. Most of the cases (4/5) that showed over 80% were accompanied by gastrointestinal bleeding due to portal hypertension. This method can be used for postoperative follow-up of liver cirrhosis of biliary atresia to predict G-I bleeding as it is easily performed, almost noninvasive and easily repeated. (author).

  10. Biliary fibrosis in microsurgical extrahepatic cholestasis in the rat.

    Science.gov (United States)

    Sánchez-Patán, Fernando; Anchuelo, Raquel; Corcuera, María-Teresa; Casado, Isabel; Gómez-Aguado, Fernando; Aller, María-Angeles; Cruz, Arturo; Alonso, María-José; Arias, Jaime

    2008-01-01

    A new model of extrahepatic cholestasis, using a microsurgical technique, is performed as an alternative to the traditional model of the bile duct ligated-rat, in order to study the stage of fibrosis in the long-term. Male Wistar rats were divided into two groups: I (Sham-operated, n = 9) and II [Microsurgical Cholestasis (MC), n = 10]. After 4 weeks, portal pressure, types of portosystemic collateral circulation, mesenteric venous vasculopathy, hepatic function test, and liver histopathology were studied by using the Knodell index and fibrosis was determined by reticulin and Sirius red stains. The animals with MC presented portal hypertension with extrahepatic portosistemic collateral circulation, associated with mesenteric venous vasculopathy and increased plasma levels of bilirubin (6.30 +/- 1.80 vs. 0.22 +/- 0.37 mg/dL; P = 0.0001), alkaline phosphatase (293.00 +/- 82.40 vs. 126.30 +/- 33.42 U/L; P = 0.001), AST (380.00 +/- 78.50 vs. 68.33 +/- 11.74 IU/L; P = 0.0001), ALT (87.60 +/- 22.32 vs. 42.22 +/- 7.89 IU/L; P = 0.0001), and LDH (697.76 +/- 75.13 vs. 384.80 +/- 100.03 IU/L; P = 0.0001). On the contrary, plasma levels of albumin decreased (2.72 +/- 0.12 mg/dl vs. 2.99 +/- 0.10; P = 0.001). The microsurgical resection of the extrahepatic biliary tract in the rat produces an experimental model of hepatic inflammation, characterized by a high Knodell hepatic activity index (4), bile proliferation, and fibrosis.

  11. Magnetic resonance cholangiopancreatography (MRCP) using new negative per-oral contrast agent based on superparamagnetic iron oxide nanoparticles for extrahepatic biliary duct visualization in liver cirrhosis.

    Science.gov (United States)

    Polakova, Katerina; Mocikova, Ingrid; Purova, Dana; Tucek, Pavel; Novak, Pavel; Novotna, Katerina; Izak, Niko; Bielik, Radoslav; Zboril, Radek; Miroslav, Herman

    2016-12-01

    Magnetic resonance cholangiopancreatography (MRCP) is often used for imaging of the biliary tree and is required by surgeons before liver transplantation. Advanced liver cirrhosis and ascites in patients however present diagnostic problems for MRCP. The aim of this study was to find out if the use of our negative per-oral contrast agent containing superparamagnetic iron oxide nanoparticles (SPIO) in MRCP is helpful for imaging of hepatobiliary tree in patients with liver cirrhosis. Forty patients with liver cirrhosis were examined on a 1.5 T MR unit using standard MRCP protocol. Twenty patients (group A) underwent MRCP after administration of per-oral SPIO contrast agent 30 min before examination. In group B, twenty patients were examined without per-oral bowel preparation. Ascites was present in eleven patients from group A and in thirteen patients in group B. Four radiologists analyzed MR images for visibility and delineation of the biliary tree. χ 2 tests were used for comparison of the visibility of intrahepatic and extrahepatic biliary ducts in patients with and without ascites. Better extrahepatic biliary duct visualization and visibility of extraluminal pathologies in patients with ascites was proved after administration of SPIO contrast agent. No statistically significant difference between group A and B was found for visualization of extrahepatic biliary ducts in patients without ascites. Delineation of intrahepatic biliary ducts was independent on bowel preparation. Application of our negative per-oral SPIO contrast agent before MRCP improves the visualization of extrahepatic biliary ducts in patients with ascites which is helpful during the liver surgery, mainly in liver transplantation.

  12. Endotoxin and CD14 in the progression of biliary atresia

    Directory of Open Access Journals (Sweden)

    Chen Ching-Mei

    2010-12-01

    Full Text Available Abstract Background Biliary atresia (BA is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/or extra-hepatic bile ducts. However, the mechanisms contributing to the pathogenesis of BA remain uncertain. Because of decreased bile flow, infectious complications and damaging endotoxemia occur frequently in patients with BA. The aim of this study was to investigate endotoxin levels in patients with BA and the relation of these levels with the expression of the endotoxin receptor, CD14. Methods The plasma levels of endotoxin and soluble CD14 were measured with a pyrochrome Limulus amebocyte lysate assay and enzyme-linked immunosorbent assay in patients with early-stage BA when they received the Kasai procedure (KP, in patients who were jaundice-free post-KP and followed-up at the outpatient department, in patients with late-stage BA when they received liver transplantation, and in patients with choledochal cysts. The correlation of CD14 expression with endotoxin levels in rats following common bile duct ligation was investigated. Results The results demonstrated a significantly higher hepatic CD14 mRNA and soluble CD14 plasma levels in patients with early-stage BA relative to those with late-stage BA. However, plasma endotoxin levels were significantly higher in both the early and late stages of BA relative to controls. In rat model, the results demonstrated that both endotoxin and CD14 levels were significantly increased in liver tissues of rats following bile duct ligation. Conclusions The significant increase in plasma endotoxin and soluble CD14 levels during BA implies a possible involvement of endotoxin stimulated CD14 production by hepatocytes in the early stage of BA for removal of endotoxin; whereas, endotoxin signaling likely induced liver injury and impaired soluble CD14 synthesis in the late stages of BA.

  13. Radiation therapy for patients with obstructive jaundice caused by carcinoma of the extrahepatic biliary system

    International Nuclear Information System (INIS)

    Kawamura, Masashi; Nakagawa, Hirofumi; Kataoka, Masaaki

    1992-01-01

    From February 1980 through September 1990, 92 patients with obstructive jaundice resulting from biliary tract cancer were registered at Shikoku Cancer Center Hospital or Ehime University Hospital. Radiation therapy (RT) was used to treat 38 of these patients (30 with carcinoma of the extrahepatic bile duct, excluding ampulla of Vater, and eight patients with carcinoma of the gallbladder). Of 38 patients, 11 underwent intraoperative radiation therapy (IORT), and 27 were treated by external radiation therapy (ERT) alone. In contrast, 54 patients (39 with carcinoma of the extrahepatic bile duct and eight with carcinoma of the gallbladder) were not treated by RT. All jaundiced patients received external and/or internal biliary drainage of some kind. Among patients undergoing biliary drainage with a catheter, 21 patients who underwent RT (four with IORT) survived significantly longer than 19 patients who did not (generalized Wilcoxon test: p<0.05). There were no significant differences in survival between 7 patients with recanalization and 11 patients with no recanalization. Concerning the survival of laparotomized patients, excluding those with complete resection or perioperative death, eight patients treated with postoperative ERT survived longer than 12 patients who did not have postoperative ERT (not significant). Eleven patients underwent IORT. A patient with unresectable carcinoma of the hilar bile duct survived 2 years and 3 months after a combination treatment of ERT and IOTR. In four of eight autopsied patients, radiation effects of Grade II were observed (Oboshi and Shimosato's evaluation system for the histological effects of radiation therapy). Our experience suggests that RT is effective in patients with obstructive jaundice caused by carcinoma of the biliary system. (author)

  14. Simultaneous /sup 99m/Tc-p-butyl-IDA and 131I-rose bengal scintigraphy in neonatal jaundice

    International Nuclear Information System (INIS)

    Collier, B.D.; Treves, S.; Davis, M.A.; Heyman, S.; Subramanian, G.; McAfee, J.G.

    1980-01-01

    Eight neonates with jaundice were studied simultaneously with /sup 99m/Tc-p-butyl-IDA and 131 I-rose bengal. Due to physical decay, /sup 99m/Tc-p-butyl-IDA failed to demonstrate delayed excretion through the patent extrahepatic biliary tract in 3 of 5 patients with concomitant hepatitis; 131 I-rose bengal showed small-bowel activity in all 5. Neither agent demonstrated small-bowel activity in 3 neonates with extrahepatic biliary atresia. Based on this clinical trial, 131 I-rose bengal remains the radiopharmaceutical of choice for distinguishing between hepatitis and biliary atresia in these patients

  15. Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016

    DEFF Research Database (Denmark)

    Pakarinen, Mikko P; Johansen, Lars Søndergaard; Svensson, Jan F

    2017-01-01

    therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >3 patients and diagnostic protocol without routine liver biopsy...

  16. Prevention of vitamin K deficiency bleeding in breastfed infants: lessons from the Dutch and Danish biliary atresia registries

    DEFF Research Database (Denmark)

    Hasselt, P.M. van; Koning, T.J. de; Vries, E. de

    2008-01-01

    OBJECTIVE: Newborns routinely receive vitamin K to prevent vitamin K deficiency bleeding. The efficacy of oral vitamin K administration may be compromised in infants with unrecognized cholestasis. We aimed to compare the risk of vitamin K deficiency bleeding under different prophylactic regimens...... in infants with biliary atresia. PATIENTS AND METHODS: From Dutch and Danish national biliary atresia registries, we retrieved infants who were either breastfed and received 1 mg of oral vitamin K at birth followed by 25 microg of daily oral vitamin K prophylaxis (Netherlands, 1991-2003), 2 mg of oral...... vitamin K at birth followed by 1 mg of weekly oral prophylaxis (Denmark, 1994 to May 2000), or 2 mg of intramuscular prophylaxis at birth (Denmark, June 2000-2005) or were fed by formula. We determined the absolute and relative risk of severe vitamin K deficiency and vitamin K deficiency bleeding...

  17. The Triangular Sign, a Useful Diagnostic Marker for Biliary Atresia: A Case Series of Three Irish Infants

    LENUS (Irish Health Repository)

    Smith, A

    2018-06-01

    The triangular cord (TC) sign is the appearance of a triangular shaped echogenic density visualised immediately cranial to the portal vein bifurcation on ultrasonographic examination. Several studies have reported that this ultrasonographic sign is a reliable and helpful marker in identifying Biliary Atresia (BA).

  18. Occupational exposure to endocrine-disrupting compounds and biliary tract cancer among men

    DEFF Research Database (Denmark)

    Ahrens, Wolfgang; Mambetova, Chinara; Bourdon-Raverdy, Nicole

    2007-01-01

    OBJECTIVES: This study investigated the association between cancer of the extrahepatic biliary tract and exposure to endocrine-disrupting compounds. METHODS: Altogether 183 men with histologically confirmed carcinoma of the extrahepatic biliary tract and 1938 matched controls were interviewed bet......-disrupting compounds in the workplace and the risk for cancer of the extrahepatic biliary tract among men, particularly for the extrahepatic bile duct and ampulla of Vater. Polychlorinated biphenyls could possibly be a strong risk factor. Udgivelsesdato: 2007-Oct......OBJECTIVES: This study investigated the association between cancer of the extrahepatic biliary tract and exposure to endocrine-disrupting compounds. METHODS: Altogether 183 men with histologically confirmed carcinoma of the extrahepatic biliary tract and 1938 matched controls were interviewed...

  19. Ultrasonography of Neonatal Cholestasis

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Jung Eun [Seoul National University Hospital, Seoul (Korea, Republic of)

    2012-06-15

    Ultrasonography (US) is as an important tool for differentiation of obstructive and non-obstructive causes of jaundice in infants and children. Beyond two weeks of age, extrahepatic biliary atresia and neonatal hepatitis are the two most common causes of persistent neonatal jaundice: differentiation of extrahepatic biliary atresia, which requires early surgical intervention, is very important. Meticulous analysis should focus on size and configuration of the gallbladder and anatomical changes of the portahepatis. In order to narrow the differential diagnosis, combined approaches using hepatic scintigraphy, MR cholangiography, and, at times, percutaneous liver biopsy are necessary. US is useful for demonstrating choledochal cyst, bile plug syndrome, and spontaneous perforation of the extrahepatic bile duct

  20. Combined liver and extrahepatic bile duct resection for biliary invasion of colorectal metastasis: a case-cohort analysis and systematic review

    NARCIS (Netherlands)

    Wiggers, Jimme K.; te Riele, Wouter W.; van Dongen, Tristan H.; Verheij, Joanne; Busch, Olivier R. C.; van Gulik, Thomas M.

    2016-01-01

    Background: Colorectal liver metastases (CRLMs) with biliary invasion can be treated with a combined liver and extrahepatic bile duct resection. The aim of this study was to analyze outcomes of this procedure in a case-cohort analysis and systematic review. Methods: Consecutive patients who

  1. External Beam Radiotherapy for Carcinoma of the Extrahepatic Biliary System

    International Nuclear Information System (INIS)

    Chun, Ha Chung; Lee, Myung Za

    1996-01-01

    Purpose : To evaluate the effectiveness and tolerance of patients of external beam radiotherapy of carcinoma of the extrahepatic biliary system (EHBS) including gall bladder (GB) and extrahepatic bile ducts (EHBD) and to define the role of radiotherapy for these tumors. Methods and Materials : We retrospectively analyzed the records of 43 patients with carcinoma of the EHBS treated with external beam radiotherapy at our institution between April, 1986 and July, 1994. Twenty three patients had GB cancers and remaining 20 patients did EHBD cancers. Of those 23 GB cancers, 2 had Stage II, 12 did Stage III and 9 did Stage IV disease, respectively. Male to female ratio was 11 to 12. Fifteen patients underwent radical surgery with curative intent and 8 patients did biopsy and bypass surgery alone. Postoperatively 16 patients were irradiated with 4500 cGy or higher doses and 4 patients with 3180 to 4140 cGy. Follow up periods ranged from 8 to 34 months. Results : overall median survival time of patients with GB cancer was 11 months. Median survival time for patients with Stage III and IV disease were 14 months and 5 months, respectively. Corresponding two year survival rates were 36%(4/11) and 13%(1/8), respectively. Those who underwent surgery with curative intent showed significantly better survival at 12 months than those who underwent bypass surgery alone(67% vs 13%). None of the patients died of treatment related complications. Median survival time for entire group of 20 EHBD patients was 10 months. Median survivals of 10 Stage III and 7 Stage IV disease were 10 and 8 months, respectively. Two patients who underwent Whipple's procedure had 11 and 14 month survival and those treated with resection and drainage showed median survival of 10 month. Conclusion : Postoperative external beam radiotherapy for carcinoma of the extrahepatic billary system is well tolerated and might improve survival of patients. especially those with respectable lesions with microscopic or

  2. Plastic or metal stents for benign extrahepatic biliary strictures: a systematic review

    Directory of Open Access Journals (Sweden)

    Vleggaar Frank P

    2009-12-01

    Full Text Available Abstract Background Benign biliary strictures may be a consequence of surgical procedures, chronic pancreatitis or iatrogenic injuries to the ampulla. Stents are increasingly being used for this indication, however it is not completely clear which stent type should be preferred. Methods A systematic review on stent placement for benign extrahepatic biliary strictures was performed after searching PubMed and EMBASE databases. Data were pooled and evaluated for technical success, clinical success and complications. Results In total, 47 studies (1116 patients on outcome of stent placement were identified. No randomized controlled trials (RCTs, one non-randomized comparative studies and 46 case series were found. Technical success was 98,9% for uncovered self-expandable metal stents (uSEMS, 94,8% for single plastic stents and 94,0% for multiple plastic stents. Overall clinical success rate was highest for placement of multiple plastic stents (94,3% followed by uSEMS (79,5% and single plastic stents (59.6%. Complications occurred more frequently with uSEMS (39.5% compared with single plastic stents (36.0% and multiple plastic stents (20,3%. Conclusion Based on clinical success and risk of complications, placement of multiple plastic stents is currently the best choice. The evolving role of cSEMS placement as a more patient friendly and cost effective treatment for benign biliary strictures needs further elucidation. There is a need for RCTs comparing different stent types for this indication.

  3. The impact of caudate lobe resection on margin status and outcomes in patients with hilar cholangiocarcinoma: a multi-institutional analysis from the US Extrahepatic Biliary Malignancy Consortium.

    Science.gov (United States)

    Bhutiani, Neal; Scoggins, Charles R; McMasters, Kelly M; Ethun, Cecilia G; Poultsides, George A; Pawlik, Timothy M; Weber, Sharon M; Schmidt, Carl R; Fields, Ryan C; Idrees, Kamran; Hatzaras, Ioannis; Shen, Perry; Maithel, Shishir K; Martin, Robert C G

    2018-04-01

    The objective of this study was to determine the impact of caudate resection on margin status and outcomes during resection of extrahepatic hilar cholangiocarcinoma. A database of 1,092 patients treated for biliary malignancies at institutions of the Extrahepatic Biliary Malignancy Consortium was queried for individuals undergoing curative-intent resection for extrahepatic hilar cholangiocarcinoma. Patients who did versus did not undergo concomitant caudate resection were compared with regard to demographic, baseline, and tumor characteristics as well as perioperative outcomes. A total of 241 patients underwent resection for a hilar cholangiocarcinoma, of whom 85 underwent caudate resection. Patients undergoing caudate resection were less likely to have a final positive margin (P = .01). Kaplan-Meier curve of overall survival for patients undergoing caudate resection indicated no improvement over patients not undergoing caudate resection (P = .16). On multivariable analysis, caudate resection was not associated with improved overall survival or recurrence-free survival, although lymph node positivity was associated with worse overall survival and recurrence-free survival, and adjuvant chemoradiotherapy was associated with improved overall survival and recurrence-free survival. Caudate resection is associated with a greater likelihood of margin-negative resection in patients with extrahepatic hilar cholangiocarcinoma. Precise preoperative imaging is critical to assess the extent of biliary involvement, so that all degrees of hepatic resections are possible at the time of the initial operation. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Comparison of the novel quantitative ARMS assay and an enriched PCR-ASO assay for K-ras mutations with conventional cytology on endobiliary brush cytology from 312 consecutive extrahepatic biliary stenoses

    NARCIS (Netherlands)

    van Heek, N. T.; Clayton, S. J.; Sturm, P. D. J.; Walker, J.; Gouma, D. J.; Noorduyn, L. A.; Offerhaus, G. J. A.; Fox, J. C.

    2005-01-01

    Background: Extrahepatic biliary stenosis (EBS) has malignant and benign causes. Patients with EBS are at risk of having or developing malignancy. Accurate diagnostic tests for early detection and surveillance are needed. The sensitivity of biliary cytology for malignancy is low. K-ras mutation

  5. Comparison of the novel quantitative ARMS assay and an enriched PCR-ASO assay for K-ras mutations with conventional cytology on endobiliary brush cytology from 312 consecutive extrahepatic biliary stenoses.

    NARCIS (Netherlands)

    Heek, N.T. van; Clayton, S.J.; Sturm, P.D.J.; Walker, J.; Gouma, D.J.; Noorduyn, L.A.; Offerhaus, G.J.; Fox, J.C.

    2005-01-01

    BACKGROUND: Extrahepatic biliary stenosis (EBS) has malignant and benign causes. Patients with EBS are at risk of having or developing malignancy. Accurate diagnostic tests for early detection and surveillance are needed. The sensitivity of biliary cytology for malignancy is low. K-ras mutation

  6. Transjugular Intrahepatic Portosystemic Shunts in Children with Biliary Atresia

    International Nuclear Information System (INIS)

    Huppert, Peter E.; Goffette, Pierre; Astfalk, Wolfgang; Sokal, Emil M.; Brambs, Hans-Juergen; Schott, Ullrich; Duda, Stephan H.; Schweizer, Paul; Claussen, Claus D.

    2002-01-01

    Purpose: We retrospectively evaluated the technical and long-term clinical results of transjugularintrahepatic portosystemic shunts (TIPS) in children with portal hypertension and biliary atresia (BA). Methods: Nine children with BA and recurrent bleeding from esophagogastric and/or intestinal varices were treated by TIPS at the age of 34-156 months and followed-up in two centers. Different types of stents were used. Results: Shunt insertion succeeded in all patients, but in two a second procedure was necessary. Seven procedures lasted more than 3 hr, mainly due to difficult portal vein puncture.Variceal bleeding ceased in all patients; however, 16 reinterventions were performed in eight patients for clinical reasons (n =11) and sonographically suspected restenosis (n =5). Four patients underwent successful liver transplantation 4-51 months after TIPS and five are in good clinical conditions 64-75 months after TIPS. Conclusions: TIPS in children with BA is technically difficult, mainly due to periportal fibrosis and small portal veins. Frequency of reinterventions seems to be higher compared with adults

  7. Near-infrared fluorescence cholangiography with indocyanine green for biliary atresia. Real-time imaging during the Kasai procedure: a pilot study.

    Science.gov (United States)

    Hirayama, Yutaka; Iinuma, Yasushi; Yokoyama, Naoyuki; Otani, Tetsuya; Masui, Daisuke; Komatsuzaki, Naoko; Higashidate, Naruki; Tsuruhisa, Shiori; Iida, Hisataka; Nakaya, Kengo; Naito, Shinichi; Nitta, Koju; Yagi, Minoru

    2015-12-01

    Hepatoportoenterostomy (HPE) with the Kasai procedure is the treatment of choice for biliary atresia (BA) as the initial surgery. However, the appropriate level of dissection level of the fibrous cone (FC) of the porta hepatis (PH) is frequently unclear, and the procedure sometimes results in unsuccessful outcomes. Recently, indocyanine green near-infrared fluorescence imaging (ICG-FCG) has been developed as a form of real-time cholangiography. We applied this technique in five patients with BA to visualize the biliary flow at the PH intraoperatively. ICG was injected intravenously the day before surgery as the liver function test, and the liver was observed with a near-infrared camera system during the operation while the patient's feces was also observed. In all patients, the whole liver fluoresced diffusely with ICG-containing stagnant bile, whereas no extrahepatic structures fluoresced. The findings of the ICG fluorescence pattern of the PH after dissection of the FC were classified into three types: spotty fluorescence, one patient; diffuse weak fluorescence, three patients; and diffuse strong fluorescence, one patient. In all five patients, the feces evacuated after HPE showed distinct fluorescent spots, although that obtained before surgery showed no fluorescence. One patient with diffuse strong fluorescence who did not achieve JF underwent living related liver transplantation six months after the initial HPE procedure. Four patients, including three cases involving diffuse weak fluorescence and one case involving spotty fluorescence showed weak fluorescence compared to that of the surrounding liver surface. We were able to detect the presence of bile excretion at the time of HPE intraoperatively and successfully evaluated the extent of bile excretion using this new technique. Furthermore, the ICG-FCG findings may provide information leading to a new classification and potentially function as an indicator predicting the clinical outcomes after HPE.

  8. Congenital extrahepatic portosystemic shunts

    Energy Technology Data Exchange (ETDEWEB)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S. [Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, M5G 1X8, Toronto, Ontario (Canada)

    2003-09-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  9. Congenital extrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S.

    2003-01-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  10. Current concept of the treatment of biliary atresia.

    Science.gov (United States)

    Miyano, T; Fujimoto, T; Ohya, T; Shimomura, H

    1993-01-01

    Hepatic portoenterostomy (Kasai operation) for the patient with biliary atresia (BA) can restore the bile flow in approximately 80% of children operated on before 60 days of life [1]. However, in terms of long-term survival, according to a recent nationwide survey among the major pediatric centers in Japan, only 325 of 2013 patients had more than 10 years' survival, and only 157 patients (7.8%) remained jaundice-free with normal liver function [2]. About 20% of BA cases without jaundice are generally able to survive for long periods; and most of those patients have portal hypertension or abnormal liver function [3-5]. As the results of liver transplantation have improved, controversy has arisen over the optimal care of these children [4, 6, 7]. Some investigators have claimed that transplantation is the favored primary therapy for most patients with BA [8]. We are thus at a turning point concerning the primary therapy of BA, which makes it necessary to determine the exact indications for the Kasai portoenterostomy and the timing of liver transplantation. This paper describes our strategy for the optimal treatment of BA patients based on our 117 patients who have had various form of portoenterostomy.

  11. Effect of phenobarbital on 99mTc-IDA scintigraphy in the evaluation of neonatal jaundice

    International Nuclear Information System (INIS)

    Majd, M.; Reba, R.C.; Altman, R.P.

    1981-01-01

    Hepatobiliary scintigraphy with 99mTc-IDA derivatives was used to evaluate 40 neonates with mixed jaundice. Fourteen patients proved to have biliary atresia. The remaining 26 patients had intrahepatic cholestasis with patent extrahepatic ducts. Sixteen of the 40 patients underwent examinations without phenobarbital stimulation. Sixteen patients had two examinations, one before and one after 3-7 days of phenobarbital therapy. The remaining 8 patients had their initial examinations after phenobarbital therapy. The results of this study show that administration of phenobarbital in a dose of 5 mg/kg/day for at least 5 days prior to the examination enhances and accelerates biliary excretion of IDA compounds and thereby significantly increases the accuracy of 99mTc-IDA scintigraphy in differentiating extrahepatic biliary atresia from neonatal hepatitis. Its routine use in the evaluation of neonatal jaundice is therefore highly recommended

  12. Metabonomics reveals metabolite changes in biliary atresia infants.

    Science.gov (United States)

    Zhou, Kejun; Xie, Guoxiang; Wang, Jun; Zhao, Aihua; Liu, Jiajian; Su, Mingming; Ni, Yan; Zhou, Ying; Pan, Weihua; Che, Yanran; Zhang, Ting; Xiao, Yongtao; Wang, Yang; Wen, Jie; Jia, Wei; Cai, Wei

    2015-06-05

    Biliary atresia (BA) is a rare neonatal cholestatic disorder caused by obstruction of extra- and intra-hepatic bile ducts. If untreated, progressive liver cirrhosis will lead to death within 2 years. Early diagnosis and operation improve the outcome significantly. Infants with neonatal hepatitis syndrome (NHS) present similar symptoms, confounding the early diagnosis of BA. The lack of noninvasive diagnostic methods to differentiate BA from NHS greatly delays the surgery of BA infants, thus deteriorating the outcome. Here we performed a metabolomics study in plasma of BA, NHS, and healthy infants using gas chromatography-time-of-flight mass spectrometry. Scores plots of orthogonal partial least-squares discriminant analysis clearly separated BA from NHS and healthy infants. Eighteen metabolites were found to be differentially expressed between BA and NHS, among which seven (l-glutamic acid, l-ornithine, l-isoleucine, l-lysine, l-valine, l-tryptophan, and l-serine) were amino acids. The altered amino acids were quantitatively verified using ultraperformance liquid chromatography-tandem mass spectrometry. Ingenuity pathway analysis revealed the network of "Cellular Function and Maintenance, Hepatic System Development and Function, Neurological Disease" was altered most significantly. This study suggests that plasma metabolic profiling has great potential in differentiating BA from NHS, and amino acid metabolism is significantly different between the two diseases.

  13. Incidence of hepatotropic viruses in biliary atresia.

    Science.gov (United States)

    Rauschenfels, Stefan; Krassmann, Miriam; Al-Masri, Ahmed N; Verhagen, Willem; Leonhardt, Johannes; Kuebler, Joachim F; Petersen, Claus

    2009-04-01

    Biliary atresia (BA) is the most frequent indication for paediatric liver transplantation. We tested the hypothesis of a viral aetiology of this disease by screening liver samples of a large number of BA patients for the common human hepatotropic viruses. Moreover, we correlated our findings to the expression of Mx protein, which has been shown to be significantly up-regulated during viral infections. Seventy-four liver biopsies (taken during Kasai portoenterostomy) were tested by polymerase chain reaction (PCR) for DNA viruses (herpes simplex virus [HSV], Epstein-Barr virus [EBV], varicella zoster virus [VZV], cytomegalovirus [CMV], adenovirus, parvovirus B19 and polyoma BK) and RNA viruses (enteroviruses, rotavirus and reovirus 3). Mx protein expression was assessed by immunohistochemistry. Virus DNA/RNA was found in less than half of the biopsies (8/74 CMV, 1/74 adenovirus; 21/64 reovirus, 1/64 enterovirus). A limited number presented with double infection. Patients that had detectable viral RNA/DNA in their liver biopsies were significantly older than virus-free patients (P = 0.037). The majority (54/59) of the liver biopsies showed expression of Mx proteins in hepatocytes, bile ducts and epithelium. Our data suggest that the known hepatotropic viruses do not play a major role in the aetiology and progression of BA. Their incidence appears to be, rather, a secondary phenomenon. Nonetheless, the inflammatory response in the livers of BA patients mimics that observed during viral infections.

  14. “Parenteral Nutrition Supplementation in Biliary Atresia Patients Listed for Liver Transplantation”

    Science.gov (United States)

    Sullivan, Jillian S; Sundaram, Shikha S; Pan, Zhaoxing; Sokol, Ronald J

    2011-01-01

    Objective To determine the impact of parenteral nutrition (PN) on outcomes in biliary atresia (BA) patients listed for liver transplantation (LTx). Study Design We retrospectively reviewed charts of all BA patients from 1990 through 2010 at our institution, s/p hepatoportoenterostomy, ≤ 36 months old, and listed for LTx. Initiation of PN was based on clinical indications. Results 25 PN and 22 non-PN subjects (74% female) were studied. Median PN initiation age was 7.7 months, mean duration 86 days, and mean PN energy supplied 77 kcal/kg/day. Prior to PN, triceps skinfold thickness (TSF) and mid-arm circumference (MAC) Z-scores were decreasing. After PN, TSF (p=0.003) and MAC (pnutritional status in malnourished BA patients awaiting LTx, which is associated with post-LTx outcomes comparable to those not requiring PN. PMID:21987426

  15. Innervation of Extrahepatic Biliary Tract, With Special Reference to the Direct Bidirectional Neural Connections of the Gall Bladder, Sphincter of Oddi and Duodenum in Suncus murinus, in Whole-Mount Immunohistochemical Study.

    Science.gov (United States)

    Yi, S-Q; Ren, K; Kinoshita, M; Takano, N; Itoh, M; Ozaki, N

    2016-06-01

    Sphincter of Oddi dysfunction is one of the most important symptoms in post-cholecystectomy syndrome. Using either electrical or mechanical stimulation and retrogradely transported neuronal dyes, it has been demonstrated that there are direct neural pathways connecting gall bladder and the sphincter of Oddi in the Australian opossum and the golden hamster. In the present study, we employed whole-mount immunohistochemistry staining to observe and verify that there are two different plexuses of the extrahepatic biliary tract in Suncus murinus. One, named Pathway One, showed a fine, irregular but dense network plexus that ran adhesively and resided on/in the extrahepatic biliary tract wall, and the plexus extended into the intrahepatic area. On the other hand, named Pathway Two, exhibiting simple, thicker and straight neural bundles, ran parallel to the surface of the extrahepatic biliary tract and passed between the gall bladder and duodenum, but did not give off any branches to the liver. Pathway Two was considered to involve direct bidirectional neural connections between the duodenum and the biliary tract system. For the first time, morphologically, we demonstrated direct neural connections between gall bladder and duodenum in S. murinus. Malfunction of the sphincter of Oddi may be caused by injury of the direct neural pathways between gall bladder and duodenum by cholecystectomy. From the viewpoint of preserving the function of the major duodenal papilla and common bile duct, we emphasize the importance of avoiding kocherization of the common bile duct so as to preserve the direct neural connections between gall bladder and sphincter of Oddi. © 2015 Blackwell Verlag GmbH.

  16. Correlation between hepatobiliary scintigraphy and surgery or postmortem examination findings in dogs and cats with extrahepatic biliary obstruction, partial obstruction, or patency of the biliary system: 18 cases (1995-2004).

    Science.gov (United States)

    Head, Laurie L; Daniel, Gregory B

    2005-11-15

    To evaluate the usefulness of serum biochemical variables and scintigraphic study results for differentiating between dogs and cats with complete extrahepatic biliary obstruction (EHO) and those with partial EHO or patent bile ducts. Retrospective case series. 17 dogs and 1 cat. Animals that underwent hepatobiliary scintigraphy and had either surgical or postmortem confirmation of the degree of bile duct patency were included. Scintigraphic images were evaluated and biliary tracts were classified as patent, partially obstructed but patent, or obstructed. Surgery or postmortem examination was considered the gold standard for diagnosis, and compared with those findings, sensitivity and specificity of scintigraphy were calculated. With absence of radioactivity in the intestinal tract as the diagnostic criterion for EHO, the sensitivity and specificity of scintigraphic diagnosis were both 83% when final images were acquired at 19 to 24 hours, compared with 100% and 33%, respectively, when 180 minutes was used as the cutoff time. Animals with partial biliary obstruction had less intestinal radioactivity that arrived later than that observed in animals with patent biliary tracts. Animals in which intestinal radioactivity has not been observed after the standard 3 to 4 hours should undergo additional scintigraphic imaging. Findings in animals with partial biliary obstruction include delayed arrival of radioactivity and less radioactivity in the intestine. Distinguishing between complete and partial biliary tract obstruction is important because animals with partial obstruction may respond favorably to medical management and should not be given an erroneous diagnosis of complete obstruction.

  17. Biliary Innate Immunity: Function and Modulation

    Directory of Open Access Journals (Sweden)

    Kenichi Harada

    2010-01-01

    Full Text Available Biliary innate immunity is involved in the pathogenesis of cholangiopathies in patients with primary biliary cirrhosis (PBC and biliary atresia. Biliary epithelial cells possess an innate immune system consisting of the Toll-like receptor (TLR family and recognize pathogen-associated molecular patterns (PAMPs. Tolerance to bacterial PAMPs such as lipopolysaccharides is also important to maintain homeostasis in the biliary tree, but tolerance to double-stranded RNA (dsRNA is not found. In PBC, CD4-positive Th17 cells characterized by the secretion of IL-17 are implicated in the chronic inflammation of bile ducts and the presence of Th17 cells around bile ducts is causally associated with the biliary innate immune responses to PAMPs. Moreover, a negative regulator of intracellular TLR signaling, peroxisome proliferator-activated receptor-γ (PPARγ, is involved in the pathogenesis of cholangitis. Immunosuppression using PPARγ ligands may help to attenuate the bile duct damage in PBC patients. In biliary atresia characterized by a progressive, inflammatory, and sclerosing cholangiopathy, dsRNA viruses are speculated to be an etiological agent and to directly induce enhanced biliary apoptosis via the expression of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL. Moreover, the epithelial-mesenchymal transition (EMT of biliary epithelial cells is also evoked by the biliary innate immune response to dsRNA.

  18. Colloid Carcinoma of the Extrahepatic Biliary Tract with Metastatic Lymphadenopathy Mimicking Cystic Neoplasm: A Case Report

    International Nuclear Information System (INIS)

    Han, Na Yeon; Park, Beom Jin; Sung, Deuk Jae; Kim, Min Ju; Cho, Sung Bum; Kim, Dong Sik; Lee, Jeong Hyeon

    2013-01-01

    The patient is a previously healthy 52-year-old woman who presented with dyspepsia for two months. Multiple imaging modalities including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) showed diffuse bile duct dilatation with an obstructive lesion of the distal extrahepatic biliary duct (EHD) as well as two masses in the peripancreatic area. The peripancreatic masses appeared cystic with posterior acoustic enhancement on ultrasound, low density on CT imaging, and high signal intensity on T2-weighted MRI. The lesion in the distal EHD exhibited similar characteristics on CT and MRI. A Whipple procedure was performed and histological specimens showed malignant cells with large mucin pools that was consistent with a diagnosis of colloid carcinoma of the EHD with metastatic lymphadenopathies. Colloid carcinoma, also called mucinous carcinoma, is classified as a histologic variant of adenocarcinoma. Because the colloid carcinoma of the biliary tree is exceedingly rare, the imaging characteristics and the clinical features of colloid carcinoma remain relatively unknown. We report a case of colloid carcinoma of the common bile duct and its accompanied metastatic lymphadenopathies with characteristic imaging findings reflecting abundant intratumoral mucin pools

  19. Scottish outcomes for extra hepatic biliary atresia post-rationalisation of services.

    Science.gov (United States)

    Tayler, Rachel; Barclay, Andrew R; Rogers, Pam; Mcintyre, Karen; Russell, Richard K; Devadason, David; Bisset, W Mike; Ling, Simon C; McGrogan, Paraic

    2013-05-01

    To evaluate the outcome of Scottish children with extra hepatic biliary atresia (EHBA) since rationalisation of Kasai services to three English centres in 2002 (The 'Group A' centres). All Scottish children with EHBA diagnosed between 2002 and 2009 were identified via the Scottish Society of Paediatric Gastroenterology, Hepatology and Nutrition (SSPGHAN) clinicians. A case-note review was conducted with demographics, presentation and outcome data recorded. These data were compared with historical Scottish data and data published previously by the supraregional liver units. 25 patients were identified, of whom 22 were referred for Kasai in the group A centres, and of whom 19 had a Kasai. 2 year transplant-free survival (TFS) was significantly lower in the SSPGHAN 2002-2009 group than the group A centres in (1) (6/18 (33%) vs 36/57 (63%), p=0.023). These postrationalisation data are disappointing. The emphasis for care will now focus on improved communication between, primary care, general paediatricians and surgical centres through regional and national managed clinical networks, aiming to improve future outcomes for Scottish children with BA.

  20. Ultrasonography and surgery of canine biliary diseases.

    Science.gov (United States)

    Vörös, K; Németh, T; Vrabély, T; Manczur, F; Tóth, J; Magdus, M; Perge, E

    2001-01-01

    Findings of hepatic and gallbladder ultrasonography were analyzed in 12 dogs with gallbladder and/or extrahepatic biliary tract obstruction and compared with the results of exploratory laparotomy. Hepatic ultrasonography demonstrated normal liver in 2 dogs and hepatic abnormalities in 10 animals. The following ultrasonographic diagnoses were established compared to surgical findings: gallbladder obstruction caused by bile sludge (correct/incorrect: 1/2, surgical diagnosis: choleliths in one case), gallbladder obstruction caused by neoplasm (0/1, surgical diagnosis: mucocele), gallbladder and extrahepatic biliary tract obstruction due to choleliths (3/3), extrahepatic biliary tract obstruction caused by pancreatic mass (1/1) and small intestinal volvulus (1/1). Bile peritonitis caused by gallbladder rupture (4/4) was correctly diagnosed by ultrasound, aided with ultrasonographically-guided abdominocentesis and peritoneal fluid analysis. Rupture of the gallbladder should be suspected in the presence of a small, echogenic gallbladder or in the absence of the organ together with free abdominal fluid during ultrasonography. Laparotomy was correctly indicated by ultrasonography in all cases. However, the direct cause of obstruction could not be determined in 2 of the 12 dogs by ultrasonography alone.

  1. Molecular Mechanisms of Bile Duct Development

    OpenAIRE

    Zong, Yiwei; Stanger, Ben Z.

    2010-01-01

    The mammalian biliary system, consisting of the intrahepatic and extrahepatic bile ducts, is responsible for transporting bile from the liver to the intestine. Bile duct dysfunction, as is seen in some congenital biliary diseases such as Alagille syndrome and biliary atresia, can lead to the accumulation of bile in the liver, preventing the excretion of detoxification products and ultimately leading to liver damage. Bile duct formation requires coordinated cell-cell interactions, resulting in...

  2. Paediatric infant presenting with an atypical spontaneous biliary ...

    African Journals Online (AJOL)

    Spontaneous biliary perforation (SBP) is a life-threatening condition. Although rare, it is the second most common surgical cause of jaundice in infancy after biliary atresia. SBP is theorized to occur due to a localized injury to the embryological developing biliary tree, predominantly at the junction of the cystic and the ...

  3. Akut, svær leverinsufficiens forårsaget af binyretumor hos nyfødt

    DEFF Research Database (Denmark)

    Maestri Ditlevsen, Jane; Kvist, Nina; Johansen, Lars Søndergaard

    2013-01-01

    A newborn female was hospitalized due to metabolic acidosis and conjugated hyperbilirubinaemia. Extrahepatic biliary atresia (EHBA) was suspected why a (99m)Tc-mebrofenin cholescintigraphy was performed. It showed poor hepatocyte tracer uptake and no drainage to the gut. The hepatocyte dysfunction...

  4. IL-6, TNF-α, IL-10, and nutritional status in pediatric patients with biliary atresia,

    Directory of Open Access Journals (Sweden)

    Maria Ines de Albuquerque Wilasco

    Full Text Available Abstract Objectives: The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. Methods: The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child–Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry. Results: IL-6, TNF-α, and IL-10 were significantly higher in the cirrhosis group when compared with the control group (2.4 vs. 0.24 (p < 0.001, 0.21 vs. 0.14 (p = 0.007, and 0.65 vs. 0.36 (p = 0.004, respectively. IL-6 and IL-10 were positively correlated with disease severity (0.450 [p = 0.001] and 0.410; [p = 0.002], respectively. TNF-α did not show a significant correlation with disease severity (0.100; p = 0.478. Regarding nutritional evaluation, IL-6 was negatively correlated with the standard deviation score of height-for-age (−0.493; p < 0.001 and of triceps skinfold thickness-for-age (−0.503; p < 0.001, respectively. IL-10 exhibited a negative correlation with the standard deviation score of height-for-age (−0.476; p < 0.001 and the standard deviation score of triceps skinfold thickness-for-age (−0.388; p = 0.004. TNF-α did not show any significance in both anthropometric parameters (−0.083 (p = 0.555 and −0.161 (p = 0.253. Conclusion: The authors suggest that, in patients with cirrhosis secondary to biliary atresia, IL-6 could be used as a possible supporting biomarker of deficient nutritional status and elevated IL-10 levels could be used as a possible early-stage supporting biomarker of deteriorating nutritional status.

  5. Factors Influencing Time-to-diagnosis of Biliary Atresia.

    Science.gov (United States)

    Harpavat, Sanjiv; Lupo, Philip J; Liwanag, Loriel; Hollier, John; Brandt, Mary L; Finegold, Milton J; Shneider, Benjamin L

    2018-06-01

    Diagnosing biliary atresia (BA) quickly is critical, because earlier treatment correlates with delayed or reduced need for liver transplantation. However, diagnosing BA quickly is also difficult, with infants usually treated after 60 days of life. In this study, we aim to accelerate BA diagnosis and treatment, by better understanding factors influencing the diagnostic timeline. Infants born between 2007 and 2014 and diagnosed with BA at our institution were included (n = 65). Two periods were examined retrospectively: P1, the time from birth to specialist referral, and P2, the time from specialist referral to treatment. How sociodemographic factors associate with P1 and P2 were analyzed with Kaplan-Meier curves and Cox proportional hazard models. In addition, to better characterize P2, laboratory results and early tissue histology were studied. P1 associated with race/ethnicity, with shorter times in non-Hispanic white infants compared to non-Hispanic black and Hispanic infants (P = 0.007 and P = 0.004, respectively). P2 associated with referral age, with shorter times in infants referred after 30, 45, or 60 days of life (P P2 in infants referred ≤30 days is that aminotransferase levels were normal or near-normal. However, despite reassuring laboratory values, tissue histology in early cases showed key features of BA. Our findings suggest 2 opportunities to accelerate BA diagnosis and treatment. First, to achieve prompt referrals for all races/ethnicities, universal screening strategies should be considered. Second, to ensure efficient evaluations independent of age, algorithms designed to detect early features of BA can be developed.

  6. Bone Health in a Nonjaundiced Population of Children with Biliary Atresia

    Directory of Open Access Journals (Sweden)

    Rachel A. Kramer

    2009-01-01

    Full Text Available Objectives. To assess bone health in a cohort of nonjaundiced children with biliary atresia (BA and the effect of growth and development on bone outcomes. Methods. Children ages one to eighteen years receiving care from Children's Hospital of Philadelphia were recruited. Each child was seen once and assessed for growth, pubertal development, concurrent medications, bilirubin, ALT, albumin, vitamin D status, bone mineral density (BMD, and bone mineral content (BMC of the lumbar spine and whole body. Results. BMD declined significantly with age, and upon further analysis with a well-phenotyped control cohort, it was found that BMC was significantly decreased for both lumbar spine and whole body, even after adjustment for confounding variables. An age interaction was identified, with older subjects having a significantly greater impairment in BMC. Conclusions. These preliminary results demonstrate that children with BA, including those without jaundice, are likely to have compromised bone health even when accounting for height and puberty, which are common confounding factors in chronic disease. Further investigation is needed to identify the determinants of poor bone mineral status and to develop strategies to prevent osteoporosis later in life.

  7. Hepaticocystic duct and a rare extra-hepatic "cruciate" arterial anastomosis: a case report

    Directory of Open Access Journals (Sweden)

    Abeysuriya Vasitha

    2008-02-01

    Full Text Available Abstract Introduction The variations in the morphological characteristics of the extra-hepatic biliary system are interesting. Case presentation During the dissection of cadavers to study the morphological characteristics of the extra-hepatic biliary system, a 46-year-old male cadaver was found to have drainage of the common hepatic duct drains directly into the gall bladder neck. The right and left hepatic ducts were not seen extra-hepatically. Further drainage of the bile away from the gallbladder and into the duodenum was provided by the cystic duct. Formation of the common bile duct by the union of the common hepatic duct and cystic duct was absent. Further more the right hepatic artery was found to be communicating with the left hepatic artery by a "bridging artery" after giving rise to the cystic artery. An accessory hepatic artery originated from the "bridging artery" forming a "cruciate" hepatic arterial anastomosis. Conclusion Combination of a Hepaticocystic duct and an aberrant variation in the extra-hepatic arterial system is extremely rare.

  8. Percutaneous treatment of extrahepatic bile duct stones assisted by balloon sphincteroplasty and occlusion balloon

    Energy Technology Data Exchange (ETDEWEB)

    Park, Yong Sung; Kim, Ji Hyung; Choi, Young Woo; Lee, Tae Hee; Hwang, Cheol Mog; Cho, Young Jun; Kim, Keum Won [Konyang University Hospital, Daejeon (Korea, Republic of)

    2005-12-15

    To describe the technical feasibility and usefulness of extrahepatic biliary stone removal by balloon sphincteroplasty and occlusion balloon pushing. Fifteen patients with extrahepatic bile duct stones were included in this study. Endoscopic stone removal was not successful in 13 patients, and two patients refused the procedure due to endoscopy phobia. At first, all patients underwent percutaneous transhepatic biliary drainage (PTBD). A few days later, through the PTBD route, balloon assisted dilatation for common bile duct (CBD) sphincter was performed, and then the stones were pushed into the duodenum using an 11.5 mm occlusion balloon. Success rate, reason for failure, and complications associated with the procedure were evaluated. Eight patients had one stone, five patients had two stones, and two patients had more than five stones. The procedure was successful in 13 patients (13/15). In 12 of the patients, all stones were removed in the first trial. In one patients, residual stones were discovered on follow-up cholangiography, and were subsequently removed in the second trial. Technical failure occurred in two patients. Both of these patients had severely dilated CBD and multiple stones with various sizes. Ten patients complained of pain in the right upper quadrant and epigastrium of the abdomen immediately following the procedure, but there were no significant procedure-related complications such as bleeding or pancreatitis. Percutaneous extrahepatic biliary stone removal by balloon sphincteroplasty and subsequent stone pushing with occlusion balloon is an effective, safe, and technically feasible procedure which can be used as an alternative method in patients when endoscopic extrahepatic biliary stone removal was not successful.

  9. Percutaneous treatment of extrahepatic bile duct stones assisted by balloon sphincteroplasty and occlusion balloon

    International Nuclear Information System (INIS)

    Park, Yong Sung; Kim, Ji Hyung; Choi, Young Woo; Lee, Tae Hee; Hwang, Cheol Mog; Cho, Young Jun; Kim, Keum Won

    2005-01-01

    To describe the technical feasibility and usefulness of extrahepatic biliary stone removal by balloon sphincteroplasty and occlusion balloon pushing. Fifteen patients with extrahepatic bile duct stones were included in this study. Endoscopic stone removal was not successful in 13 patients, and two patients refused the procedure due to endoscopy phobia. At first, all patients underwent percutaneous transhepatic biliary drainage (PTBD). A few days later, through the PTBD route, balloon assisted dilatation for common bile duct (CBD) sphincter was performed, and then the stones were pushed into the duodenum using an 11.5 mm occlusion balloon. Success rate, reason for failure, and complications associated with the procedure were evaluated. Eight patients had one stone, five patients had two stones, and two patients had more than five stones. The procedure was successful in 13 patients (13/15). In 12 of the patients, all stones were removed in the first trial. In one patients, residual stones were discovered on follow-up cholangiography, and were subsequently removed in the second trial. Technical failure occurred in two patients. Both of these patients had severely dilated CBD and multiple stones with various sizes. Ten patients complained of pain in the right upper quadrant and epigastrium of the abdomen immediately following the procedure, but there were no significant procedure-related complications such as bleeding or pancreatitis. Percutaneous extrahepatic biliary stone removal by balloon sphincteroplasty and subsequent stone pushing with occlusion balloon is an effective, safe, and technically feasible procedure which can be used as an alternative method in patients when endoscopic extrahepatic biliary stone removal was not successful

  10. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras

    OpenAIRE

    Carvalho,Elisa de; Ivantes,Cláudia Alexandra Pontes; Bezerra,Jorge A.

    2007-01-01

    OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed) pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal in...

  11. Custom-made covered transjugular intrahepatic portosystemic shunt (TIPS) in an infant with trisomy 22 and biliary atresia

    International Nuclear Information System (INIS)

    Chlapoutaki, Chrysanthi Emmanouil; Franchi-Abella, Stephanie; Pariente, Daniele; Habes, Dalila

    2009-01-01

    We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results. (orig.)

  12. Custom-made covered transjugular intrahepatic portosystemic shunt (TIPS) in an infant with trisomy 22 and biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Chlapoutaki, Chrysanthi Emmanouil; Franchi-Abella, Stephanie; Pariente, Daniele [Bicetre Hospital University Paris XI, Assistance Publique Hopitaux de Paris, Department of Paediatric Radiology, Paris (France); Habes, Dalila [Bicetre Hospital University Paris XI, Assistance Publique Hopitaux de Paris, Pediatric Hepatology and National Reference Center for Biliary Atresia, Paris (France)

    2009-07-15

    We report an 8-month-old girl with portal hypertension secondary to biliary atresia. The decision to treat with TIPS was made at the age of 8 months due to recurrent variceal bleeding. The procedure was carried out with a 6-mm bare stent due to her small size. Radiological follow-up with Doppler US showed gradual stenosis and finally occlusion of the stent 80 days after implantation. Revision was performed with placement of an additional 6-mm expanded polytetrafluoroethylene (e-PTFE) stent-graft that had remained patent for 9 months, proving that in small children with a portal vein diameter less than 8 mm, the combination of a bare stent and stent-graft can provide excellent results. (orig.)

  13. Clinical benefit of liver stiffness measurement at 3 months after Kasai hepatoportoenterostomy to predict the liver related events in biliary atresia.

    Directory of Open Access Journals (Sweden)

    Seung Min Hahn

    Full Text Available BACKGROUND: The progression of hepatic fibrosis may result in decompensated hepatic failure with cirrhosis, liver related events (LRE such as ascites, variceal bleeding, and death after successful and timely Kasai hepatoportoenterostomy (HPE in biliary atresia. The aim of this study is to suggest clinical benefit of the liver stiffness measurement (LSM using transient elastography at 3 months after the Kasai operation to predict LRE. METHODS: Between January 2007 and December 2011, 69 eligible biliary atresia patients who underwent Kasai HPE and performed transient elastography before and 3 months after HPE were included. The occurrences of LRE were analyzed for all patients. All patients were divided into 2 groups (with and without LRE for comparison. Multivariate analysis was used to detect the independent risk factors of LRE. The area under the receiver operation characteristics curve (AUROC was used to establish the LSM optimal cutoff value of 3 months after Kasai operation in predicting LRE. RESULTS: LSM value, aminotransferase, albumin, bilirubin, and PT-INR significantly differed among the two groups. Multivariate analysis demonstrated LSM value as the most powerful independent factor of the development of LRE. The cut-off value of 19.9 kPa was calculated to be optimal for predicting LRE development with total sensitivity and specificity of 1.804. AUROC resulted in 0.943, with sensitivity of 85.3% and specificity of 95.2%. CONCLUSIONS: The LSM value of 3 months after Kasai HPE can be a useful predictor of LRE development.

  14. Prognostic impact of hepatobiliary scintigraphy in diagnosis and postoperative follow-up of newborns with biliary atresia

    International Nuclear Information System (INIS)

    Rossmueller, B.; Porn, U.; Dresel, S.; Hahn, K.; Schuster, T.; Lang, T.

    2000-01-01

    Aim: To investigate the prognostic relevance of hepatobiliary scintigraphy (HBS) in newborns suffering from biliary atresia (BA) for establishing the primary diagnosis and in the postoperative follow-up after portoenterostomy (Kasai). Methods: Twenty newborns with direct hyperbilirubinemia and 6 children after operative treatment of BA (Kasai) underwent HBS with Tc-99m-DEIDA. In patients without intestinal drainage, hepatocellular extraction was estimated visually and calculated semiquantitatively by means of liver/heart-ratio 5 min p.i. Results: 10/20 patients with hyperbilirubinemia did not display biliary drainage; 6 had BA, 3 intrahepatic hypoplasia, and one showed a bile plug syndrom. 4/6 with BA but none of the 4 children with diagnoses other than BA presented with a good extraction. All of the 4 children with BA, who had either pre- or postoperatively a bad extraction, needed liver transplantation due to liver failure. Both of the two newborns with BA and favourable outcome after Kasai had a good extraction in the preoperative HBS and demonstrated good intestinal drainage in the postoperative scan. Conclusion: HBS rules out BA with high accuracy by demonstrating drainage of bile into the intestine. In newborns without drainage a good extraction favours the diagnosis of BA. In newborns with BA a bad extraction seems to indicate a poor postoperative prognosis after Kasai operation. HBS might therefore help to select those children who will not benefit from portoenterostomy. Postoperatively, HBS can easily and quickly confirm the successful hepatobiliary anastomosis by demonstrating biliary drainage into the intestine. (orig.) [de

  15. A new clarification method to visualize biliary degeneration during liver metamorphosis in sea lamprey (Petromyzon marinus)

    Science.gov (United States)

    Chung-Davidson, Yu-Wen; Davidson, Peter J.; Scott, Anne M.; Walaszczyk, Erin J.; Brant, Cory O.; Buchinger, Tyler; Johnson, Nicholas S.; Li, Weiming

    2014-01-01

    Biliary atresia is a rare disease of infancy, with an estimated 1 in 15,000 frequency in the southeast United States, but more common in East Asian countries, with a reported frequency of 1 in 5,000 in Taiwan. Although much is known about the management of biliary atresia, its pathogenesis is still elusive. The sea lamprey (Petromyzon marinus) provides a unique opportunity to examine the mechanism and progression of biliary degeneration. Sea lamprey develop through three distinct life stages: larval, parasitic, and adult. During the transition from larvae to parasitic juvenile, sea lamprey undergo metamorphosis with dramatic reorganization and remodeling in external morphology and internal organs. In the liver, the entire biliary system is lost, including the gall bladder and the biliary tree. A newly-developed method called “CLARITY” was modified to clarify the entire liver and the junction with the intestine in metamorphic sea lamprey. The process of biliary degeneration was visualized and discerned during sea lamprey metamorphosis by using laser scanning confocal microscopy. This method provides a powerful tool to study biliary atresia in a unique animal model.

  16. Neurodevelopmental Outcome of Young Children with Biliary Atresia and Native Liver: Results from the ChiLDReN Study.

    Science.gov (United States)

    Ng, Vicky L; Sorensen, Lisa G; Alonso, Estella M; Fredericks, Emily M; Ye, Wen; Moore, Jeff; Karpen, Saul J; Shneider, Benjamin L; Molleston, Jean P; Bezerra, Jorge A; Murray, Karen F; Loomes, Kathleen M; Rosenthal, Philip; Squires, Robert H; Wang, Kasper; Arnon, Ronen; Schwarz, Kathleen B; Turmelle, Yumirle P; Haber, Barbara H; Sherker, Averell H; Magee, John C; Sokol, Ronald J

    2018-05-01

    To assess neurodevelopmental outcomes among participants with biliary atresia with their native liver at ages 12 months (group 1) and 24 months (group 2), and to evaluate variables predictive of neurodevelopmental impairment. Participants enrolled in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with either the Bayley Scales of Infant Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition. Scores (normative mean = 100 ± 15) were categorized as ≥100, 85-99, and Development, 2nd edition, or Bayley Scales of Infant and Toddler Development, 3rd edition, scales) was analyzed using logistic regression. There were 148 children who completed 217 Bayley Scales of Infant and Toddler Development, 3rd edition, examinations (group 1, n = 132; group 2, n = 85). Neurodevelopmental score distributions significantly shifted downward compared with test norms at 1 and 2 years of age. Multivariate analysis identified ascites (OR, 3.17; P = .01) and low length z-scores at time of testing (OR, 0.70; P cognitive/language impairment at 1 year of age. An unsuccessful hepatoportoenterostomy was predictive of both physical/motor (OR, 4.88; P cognitive/language impairment (OR, 4.76; P = .02) at 2 years of age. Participants with biliary atresia surviving with native livers after hepatoportoenterostomy are at increased risk for neurodevelopmental delays at 12 and 24 months of age. Those with unsuccessful hepatoportoenterostomy are >4 times more likely to have neurodevelopmental impairment compared with those with successful hepatoportoenterostomy. Growth delays and/or complications indicating advanced liver disease should alert clinicians to the risk for neurodevelopmental delays, and expedite appropriate interventions. Clinicaltrials.gov: NCT00061828 and NCT00294684. Copyright © 2018 Elsevier Inc. All rights reserved.

  17. Management of blunt and penetrating biliary tract trauma.

    Science.gov (United States)

    Thomson, Benjamin N J; Nardino, Benson; Gumm, Kellie; Robertson, Amanda J; Knowles, Brett P; Collier, Neil A; Judson, Rodney

    2012-06-01

    Penetrating or blunt injury to the biliary tree remains a rare complication of trauma occurring in 0.1% of trauma admissions. Because of the different presentations, sites of biliary tract injury, and associated organ injury, there are many possible management pathways to be considered. A retrospective analysis of prospectively gathered data was performed for all gallbladder and biliary tract injuries presenting to the trauma service or hepatobiliary unit of the Royal Melbourne Hospital between January 1, 1999, and March 30, 2011. There were 33 biliary injuries in 30 patients (0.1%) among 26,014 trauma admissions. Three of the 30 patients (10%) died. Of 10 gallbladder injuries, 8 were managed with cholecystectomy. There were 23 injuries to the biliary tree. Fourteen patients had injuries to the intrahepatic biliary tree of which seven involved segmental ducts. Of these, four segmental duct injuries required hepatic resection or debridement. Nine patients had injury to the extrahepatic biliary tree of which five required T-tube placement ± bilioenteric anastomosis and one a pancreaticoduodenectomy. Biliary injury is a rare but important consequence of abdominal trauma, and good outcomes are possible when a major trauma center and hepatopancreaticobiliary service coexist. Cholecystectomy remains the gold standard for gallbladder injury. Drainage with or without endoscopic stenting will resolve the majority of intrahepatic and partial biliary injuries. Hepaticojejunostomy remains the gold standard for complete extrahepatic biliary disruption. Hepatic and pancreatic resection are only required in the circumstances of unreconstructable biliary injury. Therapeutic study, level V. Copyright © 2012 by Lippincott Williams & Wilkins.

  18. Embryonal rhabdomyosarcoma of the biliary tree: a case report

    International Nuclear Information System (INIS)

    Oh, Jong Young; Nam, Kyung Jin; Choi, Jong Chul; Park, Byung Ho; Lee, Ki Nam; Chung, Duck Hwan

    1995-01-01

    Rhabdomyosarcoma are reportedly the most common soft tissue sarcoma occurring in childhood, but the biliary tree is a rare site of origin for this tumor. Recently we experienced a case of embryonal rhabdomyosarcoma of the biliary tree in a 30-month-old child. Ultrasonography showed hypoechoic mass filling the dilated left intrahepatic and extrahepatic bile ducts, and CT showed hypodense mass with heterogeneous enhancement after contrast infusion. Intraoperative cholangiography showed filling defects within the dilated left intrahepatic and extrahepatic bile ducts. Postoperative MRI showed residual mass within the left intrahepatic duct which was hypointense on T1WI and hyperintense on T2WI

  19. [Bile duct atresia: outline for a solution].

    Science.gov (United States)

    Broto, J; Asensio, M; Gil Vernet, J M; Marhuenda, C; Boix Ochoa, J

    2000-07-01

    Biliary atresia continues to be a serious and relatively rare disease (1/50,000 newborns) and whose long-term prognosis has changed drastically since the appearance of liver transplant (LT) as a therapeutic weapon. The combination of two factors, early diagnosis and correct application of Kasai's surgical technique, is essential to obtain acceptable results and sufficient biliary drainage allowing the children to overcome the critical 7 kg barrier and place them in the lesser morbi-mortality range in relation to a possible LT. But we must keep in mind that despite its critics, Kasai's technique can guarantee, both in our own experience and in the literature, ten years survival percentages over 50% with correct hepatic function, as well as clinical normality and a quality of life clearly superior to first years post-LT. We present the evolution of a group of 20 patients affected with biliary atresia, diagnosed in our center since 1985, the year when pediatric LT began to be used as a therapeutic procedure in this country. We valued the age of intervention, technique, immediate and long-term results and the evolution and necessity of LT. All 20 patients were analyzed individually, and they currently have an age range from 2-14 years and were all operated by Kasai's technique. We classified the patients as having good, regular or poor results with regards to biliary flow, normalization of billirubin levels and clinical evolution. Sixteen patients presented biliary flow of such an extent that 14 of them, classified as good, completely normalized the billirubin levels. Two others, presently aged 14 and 8 years respectively, present average levels of 2.5-5.5 mg/100 ml and are classified as regular in a situation of advisable transplant, although with an acceptable hepatic function. Only one case, the first in the poor group, did not initially present biliary elimination and died at age six months while on the waiting list. Three other cases in the same group presented

  20. Cholangiopathy in extrahepatic portal venous obstruction: radiological appearances

    International Nuclear Information System (INIS)

    Nagi, B.; Kochhar, R.; Bhasin, D.; Singh, K.

    2000-01-01

    To evaluate cholangiographic abnormalities resulting from extrahepatic portal venous obstruction (EHPVO) by sonography and endoscopic retrograde cholangiopancreaticography (ERCP). Material and Methods: Forty-three patients with an established diagnosis of EHPVO were subjected to duplex Doppler sonography and ERCP. Of these, 8 patients had obstructive jaundice. Results: Dilated common bile duct with pericholedochal varices showing a continuous type of flow pattern was seen in 5 EHPVO patients with obstructive jaundice. ERCP revealed cholangiographic abnormalities in 40 patients (93%). Extrahepatic bile ducts were involved in 100% of cases compared to intrahepatic bile ducts (57%). Abnormalities noted were contour irregularity with indentations, displacement and angulation, strictures and filling defects in the extrahepatic ductal system. Intrahepatic bile ducts showed dilatation with areas of narrowing and filling defects. Conclusion: Cholangiographic abnormalities are very common in patients with EHPVO, even without clinical manifestations of biliary disease. Extrahepatic bile ducts are far more often involved compared to intrahepatic bile ducts. Extrinsic compression with contour irregularity is the most common cholangiographic finding. Sonographic findings are not diagnostic. Dilated common bile duct with pericholedochal varices was seen in only 5 patients

  1. Preoperative intraluminal irradiation of the extrahepatic bile duct tumor

    International Nuclear Information System (INIS)

    Kamada, Tadashi; Tsujii, Hirohiko; Arimoto, Takuro; Irie, Goro.

    1991-01-01

    From 1984 through 1986, six patients with extrahepatic bile duct tumor were treated preoperatively with intraluminal irradiation of the bile duct. There were no unresectable cases and pathological examination of the surgical specimens showed moderate to remarkable tumor regression in all cases. Postoperative biliary tract hemorrhage occurred in 2 of 3 patients who received 60 Gy at a point 7.5 mm from the center of the source. With accurate preoperative diagnosis of the tumor extent and careful setting of the target area of intraluminal irradiation, improved local tumor control of extrahepatic bile duct tumor can be expected with this method. (author)

  2. The accumulation of regulatory T cells in the hepatic hilar lymph nodes in biliary atresia.

    Science.gov (United States)

    Sakamoto, Naoya; Muraji, Toshihiro; Ohtani, Haruo; Masumoto, Kouji

    2017-10-01

    A proposed etiopathogenesis of biliary atresia (BA) involves T-cell-mediated inflammatory bile duct damage and progressive hepatic fibrosis. Pediatric surgeons often observe swelling of the hepatic hilar lymph nodes during the Kasai procedure. Given the importance of regulatory mechanisms in immune responses, the present study was designed to analyze the quantitative changes of regulatory T cells (T reg cells) in the hepatic hilar lymph nodes (hepatic hilar LNs) and peripheral blood (PB) in BA. The hepatic hilar LNs and PB obtained during the Kasai procedure were analyzed by flow cytometry. The ratios of total and active Tregs to the total CD4 + cells in the PB and the hepatic hilar LNs were compared. In patients with BA, the ratios of both the total and active T reg cells in the hepatic hilar LNs were higher than those in the PB (total T reg cells: PB vs. LN; P hilar lymph nodes of BA patients. This finding could shed light on the pathogenesis of BA.

  3. IL-6, TNF-α, IL-10, and nutritional status in pediatric patients with biliary atresia.

    Science.gov (United States)

    Wilasco, Maria Ines de Albuquerque; Uribe-Cruz, Carolina; Santetti, Daniele; Fries, Gabriel Rodrigo; Dornelles, Cristina Toscani Leal; Silveira, Themis Reverbel da

    The objective of the present study is to evaluate whether IL-6, TNF-α, IL-10 are associated with nutritional status in patients with cirrhosis secondary to biliary atresia and compare to healthy controls. The parameters used for nutritional assessment were the standard deviation scores of height-for-age and of triceps skinfold thickness-for-age. The severity of cirrhosis was evaluated using the Child-Pugh score and PELD/MELD. Serum cytokines were measured using Cytometric Bead Array flow cytometry. IL-6, TNF-α, and IL-10 were significantly higher in the cirrhosis group when compared with the control group (2.4 vs. 0.24 (patresia, IL-6 could be used as a possible supporting biomarker of deficient nutritional status and elevated IL-10 levels could be used as a possible early-stage supporting biomarker of deteriorating nutritional status. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  4. {sup 99m}Tc sestamibi imaging. Can it be a useful substitute for hepatobiliary scintigraphy in infantile jaundice?

    Energy Technology Data Exchange (ETDEWEB)

    Sadeghi, R.; Kakhki, V.R.D.; Zakavi, R. [Mashhad Univ. of Medical Sciences (Iran). Nuclear Medicine Dept.; Kianifar, H.R. [Mashhad Univ. of Medical Sciences (Iran). Paediatric Dept.; Ansari, K. [Tehran Univ. of Medical Sciences (Iran). Nuclear Medicine Dept.

    2009-07-01

    Hepatobiliary scintigraphy is an integral part in the diagnostic work-up of the neonatal cholestasis syndrome. However, less than optimal specificity is its major disadvantage. Differentiation between biliary atresia and neonatal hepatitis is nearly impossible in some cases with poor hepatocellular function. {sup 99m}Tc sestamibi (MIBI) is a cationic lipophilic agent which is a substrate of P-glycoprotein. This glycoprotein is normally expressed in biliary canalicular surfaces of hepatocytes. This property provides a hepatic excretory mechanism which is different from bilirubin excretion. In this study we evaluated the value of {sup 99m}Tc MIBI in differential diagnosis of neonatal cholestasis. 20 infants with a mean age of 2.41 months (range, 0.1-5 months) were included in the study. Ten infants turned out to have extrahepatic biliary atresia and the other ten had neonatal hepatitis. Hepatobiliary (with {sup 99m}Tc BrIDA) and {sup 99m}Tc MIBI scintigraphy were performed for all the patients. {sup 99m}Tc MIBI scintigraphy has shown bowel activity in all patients, including the patients with biliary atresia. Hepatobiliary scintigraphy revealed bowel activity only in five patients with neonatal hepatitis. Bowel visualization with {sup 99m}Tc MIBI may be seen in patients with biliary atresia and {sup 99m}Tc MIBI has limited value in differential diagnosis of neonatal cholestasis. (orig.)

  5. Diet and biliary tract cancer risk in Shanghai, China.

    Directory of Open Access Journals (Sweden)

    Shakira M Nelson

    Full Text Available Trends in biliary tract cancer incidence rates have increased in Shanghai, China. These trends have coincided with economic and developmental growth, as well as a shift in dietary patterns to a more Westernized diet. To examine the effect of dietary changes on incident disease, we evaluated associations between diet and biliary tract cancers amongst men and women from a population-based case-control study in Shanghai, China. Biliary tract cancer cases were recruited from 42 collaborating hospitals in urban Shanghai, and population-based controls were randomly selected from the Shanghai Household Registry. Food frequency questionnaire data were available for 225 gallbladder, 190 extrahepatic bile duct, and 68 ampulla of Vater cancer cases. A total of 39 food groups were created and examined for associations with biliary tract cancer. Interestingly, only four food groups demonstrated a suggested association with gallbladder, extrahepatic bile duct, or ampulla of Vater cancers. The allium food group, consisting of onions, garlic, and shallots showed an inverse association with gallbladder cancer (OR: 0.81, 95% CI: 0.68-0.97. Similar trends were seen in the food group containing seaweed and kelp (OR: 0.79, 95% CI: 0.67-0.96. In contrast, both preserved vegetables and salted meats food groups showed positive associations with gallbladder cancer (OR:1.27, 95% CI: 1.06-1.52; OR: 1.18, 95% CI: 1.02-1.37, respectively. Each of these four food groups showed similar trends for extrahepatic bile duct and ampulla of Vater cancers. The results of our analysis suggest intake of foods with greater anti-inflammatory properties may play a role in decreasing the risk of biliary tract cancers. Future studies should be done to better understand effects of cultural changes on diet, and to further examine the impact diet and inflammation have on biliary tract cancer incidence.

  6. The role of splenectomy before liver transplantation in biliary atresia patients.

    Science.gov (United States)

    Takahashi, Yoshiaki; Matsuura, Toshiharu; Yanagi, Yusuke; Yoshimaru, Koichiro; Taguchi, Tomoaki

    2016-12-01

    There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score. The mean age was 17.5±7.0years (range 11-31years), and the male-to-female ratio was 1:1. The platelet and leukocyte levels increased after splenectomy and returned to normal levels one month postoperatively. The mean MELD score after splenectomy was significantly decreased after splenectomy: 10±2.1 vs 7.6±1.8. In particular, PT-INR improved. Five patients underwent liver transplantation because of hepatopulmonary syndrome and repeated bouts of cholangitis, whereas the remaining five patients did not undergo liver transplantation because of improvements in the liver function (the mean follow-up period was 56months). The postoperative complications included portal vein thrombosis and intestinal perforation, but the patient survival rates remained at 100%. After splenectomy, both pancytopenia and the liver function clearly improved. Splenectomy should therefore be a treatment option for patients with hypersplenism before liver transplantation. Retrospective Comparative Study - Level III. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Therapeutic percutaneous ultrasound-guided cholecystocentesis in three dogs with extrahepatic biliary obstruction and pancreatitis.

    Science.gov (United States)

    Herman, Beth A; Brawer, Robert S; Murtaugh, Robert J; Hackner, Susan G

    2005-12-01

    Three dogs were examined because of acute pancreatitis. In all 3, distension of the gallbladder was seen ultrasonographically, and extrahepatic biliary tract obstruction (EHBO) was diagnosed on the basis of ultrasonographic findings and serum biochemical abnormalities (i.e., high serum bilirubin and cholesterol concentrations and increased hepatic enzyme activities). In all 3 dogs, percutaneous ultrasound-guided cholecystocentesis (PUCC) was used to decompress the gallbladder, with cholecystocentesis performed multiple times in 1 dog. Serum bilirubin concentration was substantially decreased following the procedure in all 3 dogs. Two of the 3 dogs did not require surgery to resolve the obstruction. In the third dog, an exploratory laparotomy was performed because of concerns about development of abdominal effusion following the procedure. Bile staining of the mesenteric fat was seen during the laparotomy, but no defect in the gallbladder wall could be identified. In most dogs with EHBO secondary to pancreatitis, the obstruction resolves spontaneously as the acute pancreatitis improves so that surgery is not required. In those few dogs in which EHBO does not resolve or in which EHBO results in complications, therapeutic PUCC may be useful in relieving gallbladder distension.

  8. Preterm Infants With Biliary Atresia: A Nationwide Cohort Analysis From The Netherlands.

    Science.gov (United States)

    van Wessel, Daan B E; Boere, Thomas; Hulzebos, Christian V; de Kleine, Ruben H J; Verkade, Henkjan J; Hulscher, Jan B F

    2017-10-01

    Biliary atresia (BA) occurs in 0.54 of 10.000 of overall live births in the Netherlands. BA has an unfavorable prognosis: Netherlands. Retrospective study including Dutch preterm infants treated for BA. Parameters included gestational age, congenital anomalies, age at KPE, days between first symptoms, and KPE and referral interval (first hospital to KPE). Outcome parameters were clearance of jaundice (COJ) and (transplant-free) survival. Data are presented as medians (ranges). Included 28 preterm infants (13 boys/15 girls) between March 1988 and December 2015. The incidence of BA was 1.06 of 10.000 preterm live births. Gestational age was 34.8 (27.3-36.9) weeks. Congenital anomalies were present in 11 of 28 (39%) infants. Time between first symptoms and KPE was 57 (9-138) days. Referral interval was 28 (8-86) days. Age at KPE was 70 (35-145) days. COJ was achieved in 23% of cases. Four-year transplant-free survival rate was 21%. Four-year overall survival was 61%. BA has a higher incidence in the preterm population compared to the overall BA population. The outcome of BA in preterm infants is poor, regarding COJ and (transplant-free) survival. We speculate that timely recognition of BA-related signs and symptoms in preterm infants will improve prognosis.

  9. Cooperative study with sup(99m)Tc diisopropil-IDA on biliary tract diseases

    International Nuclear Information System (INIS)

    Mitta, A.E.A.; Mollerach, F; Almeida, C.A.

    1982-01-01

    The results of a cooperative study on the investigation of the biliary tract with sup(99m)Tc diisopropyl-IDA are presented. 15 normal volunteers and 75 patients with acute cholecystitis (AC), chronic cholecystitis (CC), hepatocellular jaundice (HCJ), incomplete extrahepatic biliary obstruction (IEBO) and complete extrahepatic biliary obstruction (CEBO) were studied. The biological behaviour of the radiopharmaceutical in the two groups was determined through the measurement of the uptake times and of the times of arrival in the hepatic parenchyma, in the intra and extrahepatic bile ducts, in the gallbladder, in the duodenum and in the kidneys. The pathophysiologic features found in the AC, CC, HCJ, IEBO and CEBO are described. It is concluded that the procedure has a selective indication in the AC, that its value is limited in the CC, and that in jaundice, except in very special situations, the method gives very little information of clinic value. The possibility of performing cooperative scientific studies between Latin-American Departments, increasing their efficiency, is emphasized. (author) [es

  10. Extrahepatic Pseudoaneurysms and Ruptures of the Hepatic Artery in Liver Transplant Recipients: Endovascular Management and a New Iatrogenic Etiology

    International Nuclear Information System (INIS)

    Saad, Wael E. A.; Dasgupta, Niloy; Lippert, Allison J.; Turba, Ulku C.; Davies, Mark G.; Kumer, Sean; Gardenier, Jason C.; Sabri, Saher S.; Park, Auh-Whan; Waldman, David L.; Schmitt, Timothy; Matsumoto, Alan H.; Angle, John F.

    2013-01-01

    To characterize extrahepatic pseudoaneurysm regarding incidence and etiology and determine the effectiveness of endovascular management. A retrospective audit of 1,857 liver transplants in two institutions was performed (1996–2009). Recipients’ demographics, clinical presentation, transplant type, biliary anastomosis, and presence of biliary endoprostheses were noted. Pseudoaneurysms were classified into iatrogenic (associated with biliary endoprosthesis or angioplasty) or spontaneous extrahepatic pseudoaneurysms. Spontaneous and iatrogenic pseudoaneurysms were compared for time from transplant, presenting symptoms, location in the arterial anatomy, and 3-month graft survival. Arterial patency and 6-month graft survival were calculated. Twenty pseudoaneurysms were found (1.1 %, 20/1,857): 9 (0.5 % of transplants, 9/1,857) were spontaneous and 11 (0.6 % of transplants, 11/1,857) were “iatrogenic” (due to minimally invasive procedures: 4 angioplasty and 7 biliary endoprostheses). Sixty percent (12/20) underwent endovascular management (4 coil embolization and 8 stent-grafts). Technical success was 83 % (10/12) with a mean arterial patency of 70 % (follow-up mean, 4.9; range, 0–18 months). The 1-, 3-, and 6-month graft survival was 70, 40, and 35 %, respectively. Due to minimally invasive procedures, posttransplant extrahepatic pseudoaneurysms are no longer an exclusive complication of the transplant surgery itself. Endovascular management is effective to stabilize patients but has not improved historic postsurgical graft survival.

  11. Extrahepatic Pseudoaneurysms and Ruptures of the Hepatic Artery in Liver Transplant Recipients: Endovascular Management and a New Iatrogenic Etiology

    Energy Technology Data Exchange (ETDEWEB)

    Saad, Wael E. A., E-mail: wspikes@yahoo.com; Dasgupta, Niloy; Lippert, Allison J.; Turba, Ulku C.; Davies, Mark G. [University of Virginia Health System, Division of Vascular Interventional Radiology, Department of Radiology (United States); Kumer, Sean [University of Virginia Health System, Division of Solid Organ Transplantation, Department of Surgery (United States); Gardenier, Jason C.; Sabri, Saher S.; Park, Auh-Whan [University of Virginia Health System, Division of Vascular Interventional Radiology, Department of Radiology (United States); Waldman, David L. [University of Rochester Medical Center, Department of Imaging Sciences (United States); Schmitt, Timothy [University of Virginia Health System, Division of Solid Organ Transplantation, Department of Surgery (United States); Matsumoto, Alan H.; Angle, John F. [University of Virginia Health System, Division of Vascular Interventional Radiology, Department of Radiology (United States)

    2013-02-15

    To characterize extrahepatic pseudoaneurysm regarding incidence and etiology and determine the effectiveness of endovascular management. A retrospective audit of 1,857 liver transplants in two institutions was performed (1996-2009). Recipients' demographics, clinical presentation, transplant type, biliary anastomosis, and presence of biliary endoprostheses were noted. Pseudoaneurysms were classified into iatrogenic (associated with biliary endoprosthesis or angioplasty) or spontaneous extrahepatic pseudoaneurysms. Spontaneous and iatrogenic pseudoaneurysms were compared for time from transplant, presenting symptoms, location in the arterial anatomy, and 3-month graft survival. Arterial patency and 6-month graft survival were calculated. Twenty pseudoaneurysms were found (1.1 %, 20/1,857): 9 (0.5 % of transplants, 9/1,857) were spontaneous and 11 (0.6 % of transplants, 11/1,857) were 'iatrogenic' (due to minimally invasive procedures: 4 angioplasty and 7 biliary endoprostheses). Sixty percent (12/20) underwent endovascular management (4 coil embolization and 8 stent-grafts). Technical success was 83 % (10/12) with a mean arterial patency of 70 % (follow-up mean, 4.9; range, 0-18 months). The 1-, 3-, and 6-month graft survival was 70, 40, and 35 %, respectively. Due to minimally invasive procedures, posttransplant extrahepatic pseudoaneurysms are no longer an exclusive complication of the transplant surgery itself. Endovascular management is effective to stabilize patients but has not improved historic postsurgical graft survival.

  12. Pattern and Survival of Biliary Atresia Patients; Experience in ...

    African Journals Online (AJOL)

    experience of the teams managing these patients. There may be need to establish regional biliary surgery centers where all babies suspected of BA are referred and managed by teams specially dedicated to management of BA. This concept of regional biliary centers has been put into practice in some advanced countries.

  13. Percutaneous placement of self-expandable metallic biliary stents in malignant extrahepatic strictures: indications of transpapillary and suprapapillary methods

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Deok Hee; Yu, Jeong Sik; Kim, Ki Hwang [Yonsei University College of Medicine, Seoul (Korea, Republic of); Hwang, Jae Cheol [Ulsan University Hospital, Seoul (Korea, Republic of)

    2000-06-01

    To compare the efficacy of suprapapillary and transpapillary methods of transhepatic biliary metallic stent placement in malignant biliary strictures and to specify the indications of each method applied. Stents were placed in 59 patients. Strictures were categorized as type A (within 3 cm of the ampulla, n = 27), type B (over 3 cm from ampulla, n = 7), type C (within 3 cm of the bending portion, n = 9), or type D (over 3 cm above the bending portion, n=16). The stenting method was suprapapillary in 34 cases and transpapillary in 25. The rates of initial and long-term patency and of early recurrence were compared. Initial patency rates for the suprapapillary and transpapillary methods were 1/7 (14.3%) and 20/20 (100%) respectively for type A (p < 0.0001), 4/5 (80.0%) and 2/2 for type B, 3/7 (42.9%) and 2/2 for type C, and 15/16 (93.8%) and 0/0 for type D. Early recurrence rates were 7/30 (23.3%) using the suprapapillary method and 4/29 (13.8%) using the transpapillary method (p = 0.51). The long-term patency rate did not differ significantly according to either type (p = 0.37) or method (p = 0 . 6 2 ). For good initial patency, the transpapillary method is recommended for strictures of the distal extrahepatic duct near the ampulla and just above the bending portion. Long-term patency is not influenced by the stenting method employed.

  14. Clinical value of K-ras codon 12 analysis and endobiliary brush cytology for the diagnosis of malignant extrahepatic bile duct stenosis

    NARCIS (Netherlands)

    Sturm, P. D.; Rauws, E. A.; Hruban, R. H.; Caspers, E.; Ramsoekh, T. B.; Huibregtse, K.; Noorduyn, L. A.; Offerhaus, G. J.

    1999-01-01

    Extrahepatic biliary stenosis can be caused by benign and malignant disorders. In most cases, a tissue diagnosis is needed for optimal management of patients, but the sensitivity of biliary cytology for the diagnosis of a malignancy is relatively low. The additional diagnostic value of K-ras

  15. Biliary scintigraphy in neonatal cytomegalovirus cholestasis

    International Nuclear Information System (INIS)

    Tadzher, I.S.; Grujovska, S.; Todorovski, G.; Josifovska, T.; Arsovska, S.

    1996-01-01

    Diagnostic value of hepatobiliary scintigraphy using mebrofenin-Te-99m was assessed in three newborns with cytomegalovirus (CMV) hepatitis and one baby with hepatitis B jaundice. All cases were affected by persistent jaundice with predominately conjugated bilirubin, alcoholic stools, anemia. One of this newborns (case number 1) was suspected of having biliary atresia due to the absence of intestinal excretion of the tracer. After three weeks intestinal passage was seen in scintiscan late after 24 h. Hepatobiliary scintigraphy represents a non-invasive diagnostic procedure which enables the detection of permeability of the biliary tract. (Author)

  16. Pattern and survival of biliary atresia patients; Experience in Southern Nigeria

    Directory of Open Access Journals (Sweden)

    Philemon E Okoro

    2013-01-01

    Full Text Available Background: Biliary atresia (BA has been a challenge to surgeons worldwide. Beyond the revolutionary surgical technique popularised by Kasai, liver transplantation has added renewed hope in the long-term outcome. In Nigeria, where late presentation is very common, there is need to assess the long-term results of the treatment options available to us. Aim: We aimed to evaluate the presentation and management outcome of BA and the long-term survival of BA patients seen in our practice. Materials and Methods: Cases of BA seen between January 2007 and December 2011 in three tertiary health facilities in South East Nigeria were included. Data obtained included age at presentation, clinical features, treatment offered and age at the time of death. Analysis was with the SPSS 17.0. Results: Twenty four patients comprising 10 (41.7% males and 14 (58.3% females were included in the study. The mean age of presentation was 4.02 (±214 months; range 1.75-11.0 months. Fifteen (62.5% patients had surgery while 9 (37.5% received medical treatment only. The mean age at death was 14.2 (±8.1 months; range 2.5-30 months. Conclusion: BA poses a daunting challenge in our practice. Outcome of treatment is still discouraging. We identified late presentation, lack of facilities to make early diagnosis, lack of adequately trained manpower to manage these children and lack of post-operative care and support for patients as the major challenges in the management of BA children in our region.

  17. Influence of distal ileum exclusion on hepatic and renal functions in presence of extrahepatic cholestasis

    Directory of Open Access Journals (Sweden)

    Evandro Luis de Oliveira Costa

    Full Text Available OBJECTIVE: To verify whether the ileal exclusion interferes with liver and kidney functional changes secondary to extrahepatic cholestasis.METHODS: We studied 24 rats, divided into three groups with eight individuals each: Group 1 (control, Group 2 (ligation of the hepatic duct combined with internal biliary drainage, and Group 3 (bile duct ligation combined with internal biliary drainage and exclusion of the terminal ileum. Animals in Group 1 (control underwent sham laparotomy. The animals of groups 2 and 3 underwent ligation and section of the hepatic duct and were kept in cholestasis for four weeks. Next, they underwent an internal biliary bypass. In Group 3, besides the biliary-enteric bypass, we associated the exclusion of the last ten centimeters of the terminal ileum and carried out an ileocolic anastomosis. After four weeks of monitoring, blood was collected from all animals of the three groups for liver and kidney biochemical evaluation (albumin, ALT, AST, direct and indirect bilirubin, alkaline phosphatase, cGT, creatinine and urea.RESULTS: there were increased values of ALT, AST, direct bilirubin, cGT, creatinine and urea in rats from Group 3 (p < 0.05.CONCLUSION: ileal exclusion worsened liver and kidney functions in the murine model of extrahepatic cholestasis, being disadvantageous as therapeutic procedure for cholestatic disorders.

  18. Radioisotopic splenoportography in pediatric patients with portal hypertension

    International Nuclear Information System (INIS)

    Ohshima, Koji; Miyamoto, Kazutoshi; Yokoyama, Yasuhiro; Kubo, Yoshihiko; Samejima, Natsuki

    1990-01-01

    Radioisotopic splenoportography (RI-splenoportography) was performed in 11 children (6 patients with biliary atresia, 3 with extrahepatic portal obstruction, 1 with congenital hepatic fibrosis and 1 with pediatric liver cirrhosis). Collateral pathways were found in 9 patients by RI-imaging, of whom 6 patients had ascending pathways alone, 1 had both ascending and descending pathways, and 2 had only descending pathways. Esophageal varices were found endoscopically in all 7 patients with ascending collateral pathways, but were not found in 2 patients with the descending collateral pathways alone or in 2 patients without collateral pathway images. Endoscopic injection sclerotherapy (EST) was performed in one patient with biliary atresia who had had episodes of rupture of esophageal varices. Ascending collateral pathways were recognized by RI-splenoportography before EST, but they disappeared completely after three sessions of EST. In two patients with extrahepatic portal obstruction and a patient with congenital hepatic fibrosis who underwent meso-caval shunt operations, shunt patency and disappearance of ascending collateral pathways were proved clearly by RI-splenoportography after the operations. RI-splenoportography is very useful for investigation of portal venous collateral circulation in portal hypertension or for confirming patency of portosystemic shunts. (author)

  19. Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome.

    Science.gov (United States)

    Wu, E T; Chen, H L; Ni, Y H; Lee, P I; Hsu, H Y; Lai, H S; Chang, M H

    2001-07-01

    Bacterial cholangitis (BC) is a common complication in patients with biliary atresia (BA) and is characterized by fever, acholic stools and positive blood cultures. The diagnosis is often empirical because the yield of blood cultures is low. It is difficult to differentiate BC from other febrile episodes. In order to characterize the clinical and laboratory features of BC in patients with BA, identify risk factors, and correlate cholangitis with outcome, 37 patients with BA from 1993 to 1998 who underwent a Kasai operation in our hospital were studied. The follow-up period ranged from 6 to 59 months. A total of 107 febrile episodes were documented in these patients. The diagnostic criteria for cholangitis were fever, increased jaundice, or acholic stools. The clinical features, laboratory data, results of bacterial cultures, and outcomes were analyzed retrospectively. A total of 107 febrile episodes, including 78 bouts of cholangitis and 29 non-cholangitis infections, were found in 34 patients. Patients with BC had higher postoperative bilirubin levels (P = 0.02) and less frequent use of prophylactic antibiotics (P = 0.05) than those with non-cholangitis infections. Abnormal white blood cell counts (> 12,000 or Acinetobacter baumanni, and Salmonella typhi. The sensitivity tests justified empirical therapy with ceftriaxone. The effectiveness of prophylactic trimethoprim-sulfamethoxazole or neomycin warrants further studies. BC was a highly prevalent postoperative complication in patients with BA, especially those with inadequate bile drainage. It significantly affected early mortality. Aggressive and complete treatment with empirical ceftriaxone was appropriate.

  20. Intra-biliary contrast-enhanced ultrasound for evaluating biliary obstruction during percutaneous transhepatic biliary drainage: A preliminary study

    Energy Technology Data Exchange (ETDEWEB)

    Xu, Er-jiao [Department of Medical Ultrasonics, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou 510630 (China); Zheng, Rong-qin, E-mail: zhengrq@mail.sysu.edu.cn [Department of Medical Ultrasonics, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou 510630 (China); Su, Zhong-zhen; Li, Kai; Ren, Jie; Guo, Huan-yi [Department of Medical Ultrasonics, The Third Affiliated Hospital of Sun Yat-sen University, No. 600 Tianhe Road, Guangzhou 510630 (China)

    2012-12-15

    Objectives: The aimed of this study was to investigate the value of intra-biliary contrast-enhanced ultrasound (IB-CEUS) for evaluating biliary obstruction during percutaneous transhepatic biliary drainage (PTBD). Materials and methods: 80 patients with obstructive jaundice who underwent IB-CEUS during PTBD were enrolled. The diluted ultrasound contrast agent was injected via the drainage catheter to perform IB-CEUS. Both conventional ultrasound and IB-CEUS were used to detect the tips of the drainage catheters and to compare the detection rates of the tips. The obstructive level and degree of biliary tract were evaluated by IB-CEUS. Fluoroscopic cholangiography (FC) and computer tomography cholangiography (CTC) were taken as standard reference for comparison. Results: Conventional ultrasound displayed only 43 tips (43/80, 53.8%) of the drainage catheters within the bile ducts while IB-CEUS identified all 80 tips (80/80, 100%) of the drainage catheters including 4 of them out of the bile duct (P < 0.001). IB-CEUS made correct diagnosis in 44 patients with intrahepatic and 36 patients with extrahepatic biliary obstructions. IB-CEUS accurately demonstrated complete obstruction in 56 patients and incomplete obstruction in 21 patients. There were 3 patients with incomplete obstruction misdiagnosed to be complete obstruction by IB-CEUS. The diagnostic accuracy of biliary obstruction degree was 96.3% (77/80). Conclusion: IB-CEUS could improve the visualization of the drainage catheters and evaluate the biliary obstructive level and degree during PTBD. IB-CEUS may be the potential substitute to FC in the PTBD procedure.

  1. Medical image of the week: ascending cholangitis from biliary obstruction

    Directory of Open Access Journals (Sweden)

    Wong C

    2013-04-01

    Full Text Available A 79 year old man with a history of quadriplegia presented to an outside hospital in septic shock. He was found to have an elevated total bilirubin of 10 mg/dL, direct bilirubin of 7 mg/dL, alkaline phosphatase of 405 U/L, and lipase of 370 U/L. Imaging showed cholelithiasis with likely intra- and extrahepatic biliary duct dilatation. The patient underwent placement of a biliary drain with clinical improvement. Additional imaging was requested prior to endoscopic retrograde cholangiopancreatography (ERCP, but magnetic resonance cholangiopancreatography (MRCP was unavailable due to metallic implants. Interventional radiology performed a cholangiogram using the biliary drain which confirmed biliary obstruction. ERCP was then performed, with significant biliary sludge found and two stents placed.

  2. [Colorimetric card use for early detection visual biliary atresia].

    Science.gov (United States)

    Reyes-Cerecedo, Alicia; Flores-Calderón, Judith; Villasis-Keever, Miguel Á; Chávez-Barrera, José A; Delgado-González, Elba E

    2018-01-01

    Bile duct atresia (BVA) is a condition that causes obstruction to biliary flow, not corrected surgically, causes cirrhosis and death before 2 years of age. In Mexico from 2013 the visual colorimetric card (VVC) was incorporated for the timely detection of BVA to the National Health Card (NHC). The aim of this study was to evaluate the impact of VCT for the detection of BVA before and after the use of NHC incorporation. Ambispective, analytical observational study. We included patients with AVB treated in two pediatric hospitals of third level care. We compared the age of reference, diagnosis and surgery before and after incorporation of the TCV. In addition, a questionnaire was made to the parents to know their perception about the TCV. In 59 children, there were no differences in age at diagnosis (75 vs 70 days) and age at surgery (84 vs 90 days) between the pre and post-implementation period of the VVC. The questionnaire showed that 10 (30%) of the parents received information about the use of the VVC and 13 (38%) identified the abnormal evacuations. This study did not show changes in time for the timely detection of BVA by using VVC. Therefore, it is necessary to reinforce the program in the three levels of care in our country. La atresia de vías biliares (AVB) es una condición que provoca obstrucción al flujo biliar, y de no corregirse quirúrgicamente, provoca cirrosis y la muerte antes de los 2 años de edad. En México, a partir del año 2013 se incorporó la tarjeta colorimétrica visual (TCV) para la detección oportuna de la AVB a la Cartilla Nacional de Salud (CNS). El objetivo de este estudio fue evaluar el impacto de la TCV para la detección de AVB antes y después de su incorporación a la CNS. Estudio ambispectivo, observacional y analítico. Se incluyeron pacientes con AVB atendidos en dos hospitales pediátricos de tercer nivel de atención. Se compararon la edad de referencia, el diagnóstico y la cirugía antes y después de la incorporaci

  3. Nutritional status of patients with biliary atresia and autoimmune hepatitis related to serum levels of vitamins A, D and E Estado nutricional de pacientes com atresia biliar e hepatite autoimune e relação com os níveis séricos de vitaminas A, D e E

    Directory of Open Access Journals (Sweden)

    Margareth L. G. Saron

    2009-03-01

    Full Text Available CONTEXT: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients underwent anthropometric evaluation, alimentary inquiry and determination of serum levels of vitamins A, D and E by high performance liquid chromatography. The Mann-Whitney test, Spearman correlation coefficients and variance analysis (ANOVA were utilized for data treatment, regarding significant difference if PCONTEXTO: As doenças hepáticas crônicas podem induzir à má absorção de lipídios e vitaminas lipossolúveis e levar ao comprometimento do estado nutricional. OBJETIVOS: Avaliar o estado nutricional e relacionar com os níveis séricos de vitaminas (A, D e E e a gravidade da doença em pacientes com atresia biliar e hepatite autoimune na faixa etária pediátrica. MÉTODOS: O estudo foi transversal controlado e foram avaliados os pacientes com hepatite autoimune e atresia biliar e um grupo controle pareado por sexo e idade. Foi realizada avaliação antropométrica, aplicação do inquérito alimentar e determinação dos níveis séricos das vitaminas A, D e E pela técnica de cromatografia líquida de alta eficiência. Foram empregados os testes de Mann-Whitney, o coeficiente de correlação de Spearman e análise de variância (ANOVA, sendo considerada diferença significativa se P<0,05. RESULTADOS: O déficit nutricional mais grave foi observado nos pacientes com atresia biliar, principalmente com colestase. Em relação às vitaminas, no grupo controle, constatou-se que os níveis séricos das vitaminas A e E variaram com a idade. Os n

  4. Endoscopic Ultrasound Guided Rendezvous Drainage of Biliary Obstruction Using a New Flexible 19-Gauge Fine Needle Aspiration Needle.

    Science.gov (United States)

    Tang, Zhouwen; Igbinomwanhia, Efehi; Elhanafi, Sherif; Othman, Mohamed O

    2016-01-01

    Background and Aim. A successful endoscopic ultrasound guided rendezvous (EUS-RV) biliary drainage is dependent on accurate puncture of the bile duct and precise guide wire manipulation across the ampulla of Vater. We aim to study the feasibility of using a flexible 19-gauge fine aspiration needle in the performance of EUS-RV biliary drainage. Method. This is a retrospective case series of EUS-RV biliary drainage procedures at a single center. Patients who failed ERCP during the same session for benign or malignant biliary obstruction underwent EUS-RV using a flexible, nitinol covered, 19-gauge needle for biliary access and guide wire manipulation. Result. 24 patients underwent EUS-RV biliary drainage via extrahepatic access while 1 attempt was via intrahepatic access. The technical success rate was 80%, including 83.3% of cases via extrahepatic access. There was no significant difference in success rate of inpatient and outpatient procedures, benign or malignant indications, or type of guide wire used. Adverse events included mild pancreatitis (3 patients) and cholangitis (1 patient). Conclusion. A flexible 19-gauge needle for biliary access can be safe and effective when used to perform EUS-RV biliary drainage. Direct comparison between the nitinol needle and conventional metal needles in the performance of EUS guided biliary drainage is needed.

  5. Estado nutricional de pacientes com atresia biliar e hepatite autoimune e relação com os níveis séricos de vitaminas A, D e E

    OpenAIRE

    Saron, Margareth L. G.; Godoy, Helena T.; Hessel, Gabriel

    2009-01-01

    CONTEXT: Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES: To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS: This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients...

  6. Ultrasonographic diagnosis of biliary atresia based on a decision-making tree model

    Energy Technology Data Exchange (ETDEWEB)

    Lee, So Mi; Cheon, Jung Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun Hye; Kim, In One; You, Sun Kyoung [Dept. of Radiology, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2015-12-15

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.

  7. Ultrasonographic diagnosis of biliary atresia based on a decision-making tree model

    International Nuclear Information System (INIS)

    Lee, So Mi; Cheon, Jung Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun Hye; Kim, In One; You, Sun Kyoung

    2015-01-01

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology

  8. Ultrasonographic Diagnosis of Biliary Atresia Based on a Decision-Making Tree Model.

    Science.gov (United States)

    Lee, So Mi; Cheon, Jung-Eun; Choi, Young Hun; Kim, Woo Sun; Cho, Hyun-Hae; Cho, Hyun-Hye; Kim, In-One; You, Sun Kyoung

    2015-01-01

    To assess the diagnostic value of various ultrasound (US) findings and to make a decision-tree model for US diagnosis of biliary atresia (BA). From March 2008 to January 2014, the following US findings were retrospectively evaluated in 100 infants with cholestatic jaundice (BA, n = 46; non-BA, n = 54): length and morphology of the gallbladder, triangular cord thickness, hepatic artery and portal vein diameters, and visualization of the common bile duct. Logistic regression analyses were performed to determine the features that would be useful in predicting BA. Conditional inference tree analysis was used to generate a decision-making tree for classifying patients into the BA or non-BA groups. Multivariate logistic regression analysis showed that abnormal gallbladder morphology and greater triangular cord thickness were significant predictors of BA (p = 0.003 and 0.001; adjusted odds ratio: 345.6 and 65.6, respectively). In the decision-making tree using conditional inference tree analysis, gallbladder morphology and triangular cord thickness (optimal cutoff value of triangular cord thickness, 3.4 mm) were also selected as significant discriminators for differential diagnosis of BA, and gallbladder morphology was the first discriminator. The diagnostic performance of the decision-making tree was excellent, with sensitivity of 100% (46/46), specificity of 94.4% (51/54), and overall accuracy of 97% (97/100). Abnormal gallbladder morphology and greater triangular cord thickness (> 3.4 mm) were the most useful predictors of BA on US. We suggest that the gallbladder morphology should be evaluated first and that triangular cord thickness should be evaluated subsequently in cases with normal gallbladder morphology.

  9. An experimental study on microcholangiographic manifestation in extrahepatic bile duct obstruction

    International Nuclear Information System (INIS)

    Lee, Yul; Kang, Heung Sik; Park, Jae Hyung; Kim, Chu Wan

    1987-01-01

    Microcholangiographic using microbarium was done for radiological observation of the morphology of intrahepatic microbiliary system and its charge after extrahepatic bile duct obstruction. Regurgitation of microbarium into the systemic circulation was also observed. Extrahepatic bile ducts of 40 rabbits were ligated and microcholangiography was done just after ligation, after 1 day, 3 days and 5 days. Injection pressure of microbarium was 58 cm H 2 O in 20 rabbits and 93 cm H 2 O in another 20 rabbits. Histologic findings of the liver was compared with microcholangiographic findings. The results were as follows: 1. In microcholangiography, interlobular bile ducts and ductules were well noted, but bile canaliculi were not visible. 2. After extrahepatic bile duct ligation, ductules and small interlobular bile ducts were tortuously dilated and proliferated. These findings progressed according as the time after extrahepatic bile duct ligation especially between 1 and 2 days. 3. Microbarium was regurgitated into hepatic sinusoids from a portal tract due to rupture of interlobular bile ducts or ductules, and this was observed only in limited portions of sample tissue sections, but could be found in most of 40 rabbits. From the above results, the morphology of intrahepatic microbiliary system and its change after extrahepatic bile duct obstruction could be radiologically observed with microcholangiography, so this technique could be used for experimental study about the biliary system.

  10. Anomalous pancreatic ductal system allowing distal bowel gas with duodenal atresia

    Directory of Open Access Journals (Sweden)

    Shruti Sevak

    2017-11-01

    Bypass of the atretic duodenal segment through an anomalous pancreatic ductal system is a rare anomaly described in the literature in only a handful of cases. This case report highlights the importance of considering duodenal atresia and pancreaticobiliary enteric bypass in the differential diagnosis of neonates presenting with partial duodenal obstruction. On ultrasound, the presence of gas in the biliary tree or pancreatic duct should alert the physician to the possibility of duodenal atresia with congenital pancreaticobiliary duct anomalies that allow for bypass of enteric contents, including air, into more distal bowel, thereby creating a gas pattern aptly described as double bubble with distal gas.

  11. Hepatic ADC map as an adjunct to conventional abdominal MRI to evaluate hepatic fibrotic and clinical cirrhotic severity in biliary atresia patients

    International Nuclear Information System (INIS)

    Peng, Steven Shinn-Forng; Jeng, Yung-Ming; Hsu, Wen-Ming; Ho, Ming-Chih; Yang, Justin Cheng-Ta

    2015-01-01

    Hepatic apparent diffusion coefficient (ADC) values and ADC-related indices were correlated with the Mayo risk score for primary biliary cirrhosis (MRSPBC) and METAVIR scores of liver specimens to determine the clinical and pathological significance of diffusion-weighted magnetic resonance imaging (DWMRI). Thirty-two patients with biliary atresia (BA; mean age 461 days, range 11-4616 days) received magnetic resonance examinations from March 2009 to August 2013. A free-breathing DWMRI sequence was performed with the single-shot echo-planar imaging technique with b = 0 and 500 s/mm 2 in all 32 BA patients and 24 controls. We used the ordinal logistic regression test and Spearman rank correlation test to analyse the relationships between the MRSPBC and METAVIR fibrosis scores and right liver-to-psoas ADC ratios (LTPARs). BA patients had significantly lower LTPARs in both hepatic lobes than controls (p < 0.01). Right LTPARs, showing moderate intraobserver agreement (intraclass correlation coefficient = 0.736) and interobserver reliability (intraclass correlation coefficient = 0.659), were negatively correlated with MRSPBC and METAVIR fibrosis scores (R 2 = 0.398, p = 0.024 and R 2 = 0.628, p < 0.001, respectively). Right LTPARs may be used for long-term follow-up of cirrhosis severity in BA patients. (orig.)

  12. Hepatic ADC map as an adjunct to conventional abdominal MRI to evaluate hepatic fibrotic and clinical cirrhotic severity in biliary atresia patients

    Energy Technology Data Exchange (ETDEWEB)

    Peng, Steven Shinn-Forng [National Taiwan University, Department of Medical Imaging, Medical College and Hospital, Taipei (China); Jeng, Yung-Ming [National Taiwan University, Department of Pathology, Medical College and Hospital, Taipei (China); Hsu, Wen-Ming; Ho, Ming-Chih [National Taiwan University, Department of Surgery, Medical College and Hospital, Taipei, Taiwan (China); Yang, Justin Cheng-Ta [National Taiwan University, Department of Medical Imaging, Medical College and Hospital, Taipei (China); National Taiwan University, Institute of Biomedical Engineering, College of Engineering and College of Medicine, Taipei (China)

    2015-10-15

    Hepatic apparent diffusion coefficient (ADC) values and ADC-related indices were correlated with the Mayo risk score for primary biliary cirrhosis (MRSPBC) and METAVIR scores of liver specimens to determine the clinical and pathological significance of diffusion-weighted magnetic resonance imaging (DWMRI). Thirty-two patients with biliary atresia (BA; mean age 461 days, range 11-4616 days) received magnetic resonance examinations from March 2009 to August 2013. A free-breathing DWMRI sequence was performed with the single-shot echo-planar imaging technique with b = 0 and 500 s/mm{sup 2} in all 32 BA patients and 24 controls. We used the ordinal logistic regression test and Spearman rank correlation test to analyse the relationships between the MRSPBC and METAVIR fibrosis scores and right liver-to-psoas ADC ratios (LTPARs). BA patients had significantly lower LTPARs in both hepatic lobes than controls (p < 0.01). Right LTPARs, showing moderate intraobserver agreement (intraclass correlation coefficient = 0.736) and interobserver reliability (intraclass correlation coefficient = 0.659), were negatively correlated with MRSPBC and METAVIR fibrosis scores (R{sup 2} = 0.398, p = 0.024 and R{sup 2} = 0.628, p < 0.001, respectively). Right LTPARs may be used for long-term follow-up of cirrhosis severity in BA patients. (orig.)

  13. Endoscopic internal biliary drainage in a child with malignant obstructive jaundice caused by neuroblastoma

    International Nuclear Information System (INIS)

    Okada, Tadao; Yoshida, Hideo; Matsunaga, Tadashi; Kouchi, Katunori; Ohtsuka, Yasuhiro; Ohnuma, Naomi; Tsuyuguchi, Toshio; Yamaguchi, Taketo; Saisho, Hiromitsu

    2003-01-01

    We describe a 13-year-old girl who underwent insertion of a Flexima biliary stent for obstructive jaundice due to compression of the extrahepatic bile duct by an enlarged lymph node secondary to neuroblastoma. This novel endoscopic internal biliary drainage procedure was safe and effective even for a child, and improved her quality of life. We further review other treatment options available for malignant obstructive jaundice in children. (orig.)

  14. Endoscopic internal biliary drainage in a child with malignant obstructive jaundice caused by neuroblastoma

    Energy Technology Data Exchange (ETDEWEB)

    Okada, Tadao; Yoshida, Hideo; Matsunaga, Tadashi; Kouchi, Katunori; Ohtsuka, Yasuhiro; Ohnuma, Naomi [Department of Paediatric Surgery, Chiba University, School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba 260-8677 (Japan); Tsuyuguchi, Toshio; Yamaguchi, Taketo; Saisho, Hiromitsu [First Department of Internal Medicine, Chiba University School of Medicine, Chiba (Japan)

    2003-02-01

    We describe a 13-year-old girl who underwent insertion of a Flexima biliary stent for obstructive jaundice due to compression of the extrahepatic bile duct by an enlarged lymph node secondary to neuroblastoma. This novel endoscopic internal biliary drainage procedure was safe and effective even for a child, and improved her quality of life. We further review other treatment options available for malignant obstructive jaundice in children. (orig.)

  15. Association between shift work and the risk of death from biliary tract cancer in Japanese men.

    Science.gov (United States)

    Lin, Yingsong; Nishiyama, Takeshi; Kurosawa, Michiko; Tamakoshi, Akiko; Kubo, Tatsuhiko; Fujino, Yoshihisa; Kikuchi, Shogo

    2015-10-21

    There is increasing evidence suggesting that shift work involving night work may increase cancer risk. We examined the association between working rotating shifts and the risk of death from biliary tract cancer among Japanese men who participated in the Japan Collaborative Cohort Study. Of the 46,395 men recruited, 22,224 men aged 40-65 at baseline (1988-1990) who reported working full-time or were self-employed were included in the present analysis. The study subjects were followed through December 31, 2009. Information regarding occupation and lifestyle factors was collected using a self-administered questionnaire. Cox proportional hazard models were used to estimate the hazard ratio (HR) and 95 % confidence interval (CI) for the risk of death from biliary tract cancer in relation to shift work. During a mean 17-year follow-up, we observed 94 biliary tract cancer deaths, including 23 deaths from gallbladder cancer and 71 deaths from extrahepatic bile duct cancer. Overall, shift work was associated with a statistically non-significant increase in the risk of biliary tract cancer, with an HR of 1.50 (95 % CI: 0.81-2.77), among rotating shift workers. When the analysis was limited to extrahepatic bile duct cancer, a significant association appeared, with a multivariable-adjusted HR of 1.93 (95 % CI: 1.00-3.72) for rotating shift workers. Our data indicate that shift work may be associated with increased risk of death from extrahepatic bile duct cancer in this cohort of Japanese men. The association with gallbladder cancer remains unclear because of the small number of deaths.

  16. Ischemic Cholangitis Caused by Transcatheter Hepatic Arterial Chemoembolization 10 Months After Resection of the Extrahepatic Bile Duct

    International Nuclear Information System (INIS)

    Hasegawa, Kiyoshi; Kubota, Keiichi; Aoki, Taku; Hirai, Ichiro; Miyazawa, Masashi; Ohtomo, Kuni; Makuuchi, Masatoshi

    2000-01-01

    We report a case of ischemic cholangitis that occurred after transcatheter hepatic arterial chemoembolization (TAE). Ten months prior to TAE the patient had undergone central bisegmentectomy for hepatocellular carcinoma with resection of the extrahepatic bile duct. Eleven days after TAE, he developed suppurative cholangitis and multiple organ failure. Prior surgical ligation of the peribiliary arteries around the extrahepatic bile duct followed by TAE was considered to have played a crucial role in the development of ischemic cholangitis. This case demonstrates the importance of blood flow from the peribiliary arteries for the survival of the biliary epithelium

  17. Preoperative biliary drainage in hilar cholangiocarcinoma: When and how?

    Science.gov (United States)

    Paik, Woo Hyun; Loganathan, Nerenthran; Hwang, Jin-Hyeok

    2014-01-01

    Hilar cholangiocarcinoma is a tumor of the extrahepatic bile duct involving the left main hepatic duct, the right main hepatic duct, or their confluence. Biliary drainage in hilar cholangiocarcinoma is sometimes clinically challenging because of complexities associated with the level of biliary obstruction. This may result in some adverse events, especially acute cholangitis. Hence the decision on the indication and methods of biliary drainage in patients with hilar cholangiocarcinoma should be carefully evaluated. This review focuses on the optimal method and duration of preoperative biliary drainage (PBD) in resectable hilar cholangiocarcinoma. Under certain special indications such as right lobectomy for Bismuth type IIIA or IV hilar cholangiocarcinoma, or preoperative portal vein embolization with chemoradiation therapy, PBD should be strongly recommended. Generally, selective biliary drainage is enough before surgery, however, in the cases of development of cholangitis after unilateral drainage or slow resolving hyperbilirubinemia, total biliary drainage may be considered. Although the optimal preoperative bilirubin level is still a matter of debate, the shortest possible duration of PBD is recommended. Endoscopic nasobiliary drainage seems to be the most appropriate method of PBD in terms of minimizing the risks of tract seeding and inflammatory reactions. PMID:24634710

  18. Telomere Length in Peripheral Blood Leukocytes Is Associated with Severity of Biliary Atresia.

    Directory of Open Access Journals (Sweden)

    Wanvisa Udomsinprasert

    Full Text Available The purpose of this study was to investigate the association of telomere length in peripheral blood leukocytes with the severity of biliary atresia (BA.One hundred and fourteen BA patients and 114 age-matched healthy controls were enrolled. Relative telomere length (RTL was assessed using a quantitative real-time polymerase chain reaction. Multivariate regression analysis was used to estimate RTL as an independent risk factor of BA. Receiver operating characteristic curve analysis was used to calculate the accuracy of biomarkers in the prediction of liver cirrhosis.BA patients had significantly shorter telomeres than healthy controls (p < 0.0001. The RTL in BA patients with jaundice was considerably lower than that of patients without jaundice (p = 0.005. Moreover, RTL was markedly shorter in patients with cirrhosis (F4, as compared to patients with mild fibrosis (F2 and non-fibrosis (F0-F1, p < 0.0001. Logistic regression analysis indicated that short RTL was associated with a higher risk of liver cirrhosis in BA. Tertile analysis showed a dose-response effect for this association (p trend < 0.0001. Additionally, RTL in BA children revealed a negative correlation with age (r = -0.50, p < 0.001. We noted an association between reduction of RTL and liver stiffness scores, adjusted for age and gender (b = -0.01, p < 0.0001. Short RTL can be employed to distinguish cirrhosis patients from non-cirrhosis patients (AUC = 0.78. Further analysis showed a linear correlation between leukocyte RTL and liver RTL in BA patients (r = 0.83, p < 0.001.The findings of this study provide evidence that telomere shortening is associated with an elevated risk of liver cirrhosis in BA.

  19. Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

    Directory of Open Access Journals (Sweden)

    Tae Jun Park

    2014-03-01

    Full Text Available Hepatopulmonary syndrome (HPS is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE, and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%. Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

  20. Association between Promoter Hypomethylation and Overexpression of Autotaxin with Outcome Parameters in Biliary Atresia.

    Directory of Open Access Journals (Sweden)

    Wanvisa Udomsinprasert

    Full Text Available Biliary atresia (BA is a progressive fibroinflammatory liver disease. Autotaxin (ATX has a profibrotic effect resulting from lysophosphatidic acid activity. The purpose of this study was to examine ATX expression and ATX promoter methylation in peripheral blood leukocytes and liver tissues from BA patients and controls and investigate their associations with outcome parameters in BA patients.A total of 130 subjects (65 BA patients and 65 age-matched controls were enrolled. DNA was extracted from circulating leukocytes and liver tissues of BA patients and from and age-matched controls. ATX promoter methylation status was determined by bisulfite pyrosequencing. ATX expression was analyzed using quantitative real-time polymerase chain reaction and enzyme-linked immunosorbent assay.Decreased methylation of specific CpGs were observed at the ATX promoter in BA patients. Subsequent analysis revealed that BA patients with advanced stage had lower methylation levels of ATX promoter than those with early stage. ATX promoter methylation levels were found to be associated with hepatic dysfunction in BA. In addition, ATX expression was significantly elevated and correlated with a decrease in ATX promoter methylation in BA patients compared to the controls. Furthermore, promoter hypomethylation and overexpression of ATX were inversely associated with jaundice status, hepatic dysfunction, and liver stiffness in BA patients.Accordingly, it has been hypothesized that ATX promoter methylation and ATX expression in peripheral blood may serve as possible biomarkers reflecting the progression of liver fibrosis in postoperative BA. These findings suggest that the promoter hypomethylation and overexpression of ATX might play a contributory role in the pathogenesis of liver fibrosis in BA.

  1. Hepatic tumours in children with biliary atresia: Single-centre experience in 13 cases and review of the literature

    International Nuclear Information System (INIS)

    Yoon, H.J.; Jeon, T.Y.; Yoo, S.-Y.; Kim, J.H.; Eo, H.; Lee, S.-K.; Kim, J.S.

    2014-01-01

    Aim: To establish the risks of developing of hepatic tumours and to investigate their clinical and imaging findings in children with biliary atresia (BA) after Kasai portoenterostomy (Kasai). Materials and methods: Among 157 children who had undergone Kasai for BA over an 18 year period, patients who had newly developed hepatic tumours were identified. Patient demographics, clinical features, and imaging findings were retrospectively reviewed. Results: Three male and 10 female patients (mean age 3.9 years) all (8%, of 157) had single hepatic tumours, which were confirmed in 10 explanted and three non-explanted livers. Ten (77%) were benign and three (23%) were malignant. Of the benign hepatic tumours, focal nodular hyperplasia (FNH; n = 6) was the most common, followed by regenerative nodules (n = 3) and adenoma (n = 1). All FNH appeared in young children <1 year of age and showed a subcapsular location, bulging contour, and lack of central scar. Malignant tumours included two hepatocellular carcinomas and one cholangiocarcinoma. Conclusion: Hepatic tumours developed in approximately 8% of children with BA after Kasai. Although benign tumours, including FNHs and regenerative nodules, were more common than malignant tumours, screening with alpha-foetoprotein (AFP) levels and regular imaging studies are the mainstay of malignant tumour detection

  2. Reactivity of Biliatresone, a Natural Biliary Toxin, with Glutathione, Histamine, and Amino Acids.

    Science.gov (United States)

    Koo, Kyung A; Waisbourd-Zinman, Orith; Wells, Rebecca G; Pack, Michael; Porter, John R

    2016-02-15

    In our previous work, we identified a natural toxin, biliatresone, from Dysphania glomulifera and D. littoralis, endemic plants associated with outbreaks of biliary atresia in Australian neonatal livestock. Biliatresone is a very rare isoflavonoid with an α-methylene ketone between two phenyls, 1,2-diaryl-2-propenone, along with methylenedioxy, dimethoxyl, and hydroxyl functional groups, that causes extrahepatic biliary toxicity in zebrafish. The toxic core of biliatresone is a methylene in the α-position relative to the ketone of 1,2-diaryl-2-propenone that serves as an electrophilic Michael acceptor. The α-methylene of biliatresone spontaneously conjugated with water and methanol (MeOH), respectively, via Michael addition in a reverse phase high-performance liquid chromatography (RP-HPLC) analysis. We here report the reactivity of biliatresone toward glutathione (GSH), several amino acids, and other thiol- or imidazole-containing biomolecules. LC-MS and HPLC analysis of the conjugation reaction showed the reactivity of biliatresone to be in the order histidine > N-acetyl-d-cysteine (D-NAC) = N-acetyl-l-cysteine (L-NAC) > histamine > glutathione ≥ cysteine ≫ glycine > glutamate > phenylalanine, while serine and adenine had no reactivity due to intramolecular hydrogen bonding in the protic solvents. The reactivity of ethyl vinyl ketone (EVK, 1-penten-3-one), an example of a highly reactive α,ß-unsaturated ketone, toward GSH gave a 6.7-fold lower reaction rate constant than that of biliatresone. The reaction rate constant of synthetic 1,2-diaryl-2-propen-1-one (DP), a core structure of the toxic molecule, was 10-fold and 1.5-fold weaker in potency compared to the reaction rate constants of biliatresone and EVK, respectively. These results demostrated that the methylenedioxy, dimethoxyl, and hydroxyl functional groups of biliatresone contribute to the stronger reactivity of the Michael acceptor α-methylene ketone toward nucleophiles compared to that of DP

  3. Heterozygous deletion of FOXA2 segregates with disease in a family with heterotaxy, panhypopituitarism, and biliary atresia.

    Science.gov (United States)

    Tsai, Ellen A; Grochowski, Christopher M; Falsey, Alexandra M; Rajagopalan, Ramakrishnan; Wendel, Danielle; Devoto, Marcella; Krantz, Ian D; Loomes, Kathleen M; Spinner, Nancy B

    2015-06-01

    Biliary atresia (BA) is a pediatric cholangiopathy with unknown etiology occurring in isolated and syndromic forms. Laterality defects affecting the cardiovascular and gastrointestinal systems are the most common features present in syndromic BA. Most cases are sporadic, although reports of familial cases have led to the hypothesis of genetic susceptibility in some patients. We identified a child with BA, malrotation, and interrupted inferior vena cava whose father presented with situs inversus, polysplenia, panhypopituitarism, and mildly dysmorphic facial features. Chromosomal microarray analysis demonstrated a 277 kb heterozygous deletion on chromosome 20, which included a single gene, FOXA2, in the proband and her father. This deletion was confirmed to be de novo in the father. The proband and her father share a common diagnosis of heterotaxy, but they also each presented with a variety of other issues. Further genetic screening revealed that the proband carried an additional protein-altering polymorphism (rs1904589; p.His165Arg) in the NODAL gene that is not present in the father, and this variant has been shown to decrease expression of the gene. As FOXA2 can be a regulator of NODAL expression, we propose that haploinsufficiency for FOXA2 combined with a decreased expression of NODAL is the likely cause for syndromic BA in this proband. © 2015 WILEY PERIODICALS, INC.

  4. Floating tumor debris. A cause of intermittent biliary obstruction.

    Science.gov (United States)

    Roslyn, J J; Kuchenbecker, S; Longmire, W P; Tompkins, R K

    1984-11-01

    Tumor debris, free-floating in the major biliary ductal system, is a cause of intermittent biliary obstruction that has previously not been recognized. Six patients had hepatic neoplasms with episodic jaundice and/or cholangitis due to floating tumor debris. Diagnosis included metastatic adenocarcinoma of the colon (n = 3), cholangiocarcinoma (n = 1), hepatocellular carcinoma (n = 1), and cavernous hemangioma (n = 1). All patients underwent biliary exploration, with hepatic resection and transhepatic intubation in two and T-tube placement in four. One patient died in the early postoperative period, and the major complication rate in the five survivors was 0%. Four of the five survivors had no further episodes suggestive of major bile duct obstruction. Our experience emphasizes the importance of distinguishing extrahepatic obstruction secondary to tumor debris from the more common causes of jaundice in patients with tumors and suggests that safe and effective palliation can be achieved in these patients.

  5. Extrahepatic duct injury in blunt trauma: two case reports and a literature review.

    Science.gov (United States)

    Zago, Thiago Messias; Pereira, Bruno Monteiro Tavares; Calderan, Thiago Rodrigues Araujo; Hirano, Elcio Shiyoiti; Fraga, Gustavo Pereira

    2014-08-01

    Traumatic injuries of the extrahepatic biliary tract are rare. Associated injuries are usually responsible for immediate indication for surgical treatment, the time when an injury to the extrahepatic biliary ducts may be diagnosed. However, missed injuries are often common. The primary aim of this paper is to describe the clinical features, diagnosis, treatment, and outcome of two patients with left hepatic duct injury after blunt abdominal trauma. As a secondary objective, a literature review is presented. The two cases presented in this study are as follows: (1) A young male, involved in a motor vehicle crash, was admitted with blunt hepatic trauma in a general hospital. Endoscopic retrograde cholangiography was conducted 3 weeks later and revealed a large leakage at the left hepatic duct. Exploratory laparotomy was performed 26 days after the initial traumatic event and identified a complete section of the left hepatic duct, treated with anastomosis. (2) A male fell from a height. On exploratory laparotomy, a 30 % partial injury of the left hepatic duct was found in addition to hemoperitoneum, liver injury, gallbladder detachment together with cystic duct rupture, retroperitoneal hematoma to the right, and cecum hematoma. A high level of suspicion is necessary to identify injuries to the hepatic ducts. Early diagnosis that occurs during laparotomy due to associated injuries is important to reduce complications.

  6. A Multicenter, Prospective Study of a New Fully Covered Expandable Metal Biliary Stent for the Palliative Treatment of Malignant Bile Duct Obstruction

    Directory of Open Access Journals (Sweden)

    Bret T. Petersen

    2013-01-01

    Full Text Available Background and Study Aims. Endoscopic placement of self-expanding metal stents (SEMSs is indicated for palliation of inoperable malignant biliary obstruction. A fully covered biliary SEMS (WallFlex Biliary RX Boston Scientific, Natick, USA was assessed for palliation of extrahepatic malignant biliary obstruction. Patients and Methods. 58 patients were included in this prospective, multicenter series conducted under an FDA-approved IDE. Main outcome measurements included (1 absence of stent occlusion within six months or until death, whichever occurred first and (2 technical success, need for reintervention, bilirubin levels, stent patency, time to stent occlusion, and adverse events. Results. Technical success was achieved in 98% (57/58, with demonstrated acute removability in two patients. Adequate clinical palliation until completion of followup was achievedin 98% (54/55 of evaluable patients, with 1 reintervention due to stent obstruction after 142 days. Mean total bilirubin decreased from 8.9 mg/dL to 1.2 mg/dL at 1 month. Device-related adverse events were limited and included 2 cases of cholecystitis. One stent migrated following radiation therapy. Conclusions. The WallFlex Biliary fully covered stent yielded technically successful placement with uncomplicated acute removal where required, appropriate reduction in bilirubin levels, and low rates of stent migration and occlusion. This SEMS allows successful palliation of malignant extrahepatic biliary obstruction.

  7. Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation.

    Science.gov (United States)

    Lien, Tien-Hau; Bu, Ling-Nan; Wu, Jia-Feng; Chen, Huey-Ling; Chen, An-Chyi; Lai, Ming-Wei; Shih, Hsiang-Hung; Lee, I-Hsien; Hsu, Hong-Yuan; Ni, Yen-Hsuan; Chang, Mei-Hwei

    2015-05-01

    Recurrent cholangitis may aggravate cholestatic liver cirrhosis in biliary atresia (BA) after the Kasai operation. This pilot study aimed to investigate whether Lactobacillus casei rhamnosus has the prophylactic efficacy for recurrent cholangitis in comparison with the conventional neomycin prophylaxis. Twenty jaundice-free patients with BA ages 0 to 3 years who underwent a Kasai operation were enrolled and randomized into 2 groups with 10 patients each: neomycin (25 mg · kg · day for 4 days/wk) and L casei rhamnosus (8 × 10 colony-forming unit per day) groups. The treatment duration was 6 months. Bacterial stool cultures were performed before treatment and 1, 3, and 6 months after starting treatment. In addition, 10 patients with BA with similar status but without prophylaxis served as the historical control group. In the Lactobacillus group, 2 patients (20%, mean 0.03 ± 0.07 episodes per month) developed cholangitis during the study period, with the same frequency as in the neomycin group and significantly lower than that in the control group (80%, P = 0.005, mean 0.22 ± 0.16 episodes per month). The mean change in body weight z score during the 6 months in the Lactobacillus group was 0.97 ± 0.59, which was significantly better than that in the control group (-0.01 ± 0.79, P = 0.006). In bacterial stool cultures, the Lactobacillus and Escherichia coli populations significantly increased and decreased, respectively, in the Lactobacillus group. The use of L casei rhamnosus was as effective as neomycin in preventing cholangitis in patients with BA who underwent Kasai operation, and therefore could be considered as a potential alternative prophylactic regimen.

  8. An inguinal hernia sac tumor of extrahepatic cholangiocarcinoma origin

    Directory of Open Access Journals (Sweden)

    Yamazaki Hidehiro

    2006-03-01

    Full Text Available Abstract Background Metastatic hernia sac tumor from biliary malignancy is extremely rare with only one such case previously reported. We herein report an additional case of extrahepatic cholangiocarcinoma presenting as a hernia sac tumor. Case presentation A 78-year-old man presented with an irreducible right inguinal hernia associated with a firm tumor, 2.0 cm in diameter. A computed tomography scan demonstrated a soft tissue density mass with heterogeneous enhancement within the right inguinal canal. The patient underwent a hernia repair and the hernia sac tumor was resected. Histological examination of the tumor revealed a metastatic adenocarcinoma suggesting the tumor was of pancreato-biliary origin. Further investigation using imaging studies disclosed a primary tumor in the upper bile duct. The patient died of the disease nine months after the resection. Conclusion Hernia sac tumors should be considered when an irreducible, growing mass appears within an inguinal hernia. Computed tomography may be useful for the early detection of hernia sac tumors from undiagnosed intra-abdominal malignancies.

  9. Congenital biliary atresia: liver injury begins at birth

    DEFF Research Database (Denmark)

    Makin, Erica; Quaglia, Alberto; Kvist, Nina

    2009-01-01

    -note review for infants with definite BA who underwent laparotomy within first week of life. RESULTS: Three infants were identified who had occlusive BA evident on the first day of life. In all cases, their liver was grossly normal, and histologic changes were trivial. CONCLUSION: This suggests...... that the detrimental cholestatic liver injury, later characteristic of BA, only begins from the time of birth despite a prenatal occlusive biliary pathology. It may be that tissue injury only occurs with the onset of the perinatal bile surge initiating periductal bile leakage and the triggering of an inflammatory...

  10. Serum Mac-2 binding protein glycosylation isomer predicts grade F4 liver fibrosis in patients with biliary atresia.

    Science.gov (United States)

    Yamada, Naoya; Sanada, Yukihiro; Tashiro, Masahisa; Hirata, Yuta; Okada, Noriki; Ihara, Yoshiyuki; Urahashi, Taizen; Mizuta, Koichi

    2017-02-01

    Mac-2 Binding Protein Glycosylation Isomer (M2BPGi) is a novel fibrosis marker. We examined the ability of M2BPGi to predict liver fibrosis in patients with biliary atresia. Sixty-four patients who underwent living donor liver transplantation (LDLT) were included [median age, 1.1 years (range 0.4-16.0), male 16 patients (25.0 %)]. We examined M2BPGi levels in serum obtained the day before LDLT, and we compared the value of the preoperative M2BPGi levels with the histological evaluation of fibrosis using the METAVIR fibrosis score. Subsequently, we assessed the ability of M2BPGi levels to predict fibrosis. The median M2BPGi level in patients with BA was 6.02 (range, 0.36-20.0), and 0, 1, 1, 11, and 51 patients had METAVIR fibrosis scores of F0, F1, F2, F3, and F4, respectively. In patients with F4 fibrosis, the median M2BPGi level was 6.88 (quartile; 5.235, 12.10), significantly higher than that in patients with F3 fibrosis who had a median level of 2.42 (quartile; 1.93, 2.895, p F4 fibrosis. M2BPGi is a novel fibrosis marker for evaluating the status of the liver in patients with BA, especially when predicting grade F4 fibrosis.

  11. Invaded depth of extrahepatic bile duct cancer diagnosed on angiographic CT during late contrast enhancement

    International Nuclear Information System (INIS)

    Kikuyama, Masataka; Sasada, Yuzo; Koide, Shigeki; Hirai, Ritsuko; Oota, Yuji

    2006-01-01

    Thirteen patients with extrahepatic bile duct cancer were evaluated for enhancement pattern and tumor feature during late contrast enhancement on angiographic CT (AG-CT), performed before surgery. Eleven patients with late contrast enhancement had advanced cancers with invasion over the subserosal layer. Eight of these patients whose cancer had an irregular outer layer had invasion over the serosal layer (≥panc 1 b, General Rules for Surgical and Pathological Studies on Cancer of the Biliary Tract, Japanese Society of Biliary Surgery). Seven of the 13 patients had intra-pancreatic bile duct cancer. Among them, five had an irregular outer layer of the intra-pancreatic bile duct cancer; one of three with a wall thickness under 5 mm on AG-CT had panc 1 b invasion, and the other two had panc 2 (General Rules for Surgical and Pathological Studies on Cancer of the Biliary Tract, Japanese Society of Biliary Surgery) invasion. Two with a wall thickness over 5 mm on AG-CT had panc 2 invasion. These results suggest that late contrast enhancement on AG-CT is effective for diagnosing the depth of tumor invasion of the bile duct. (author)

  12. Unusual biliary scan appearance in a child with a transplanted liver with hepatic arterial thrombosis: a case report.

    Science.gov (United States)

    Porn, U; Howman-Giles, R; Shun, A; Dorney, S; Uren, R

    2000-02-01

    A 5-year-old girl with biliary atresia and a subsequent Kasai procedure is described. She had clinical symptoms suggestive of rejection after a recent orthotopic liver transplant A hepatobiliary scan showed partial hepatic infarction and a biloma in the infarcted area.

  13. Evaluation of the patient with suspected extrahepatic biliary obstruction

    International Nuclear Information System (INIS)

    Watson, C.G.

    1975-01-01

    In most patients, obstructive jaundice can be differentiated from intrahepatic cholestatic jaundice employing conventional clinical, radiologic, and laboratory techniques. Roughly 20 percent of these patients will remain without a diagnosis. During the past decade, several invasive and noninvasive techniques for visualizing the biliary tree have been developed, increasing the diagnostic yield to over 90 percent. The combination of selective visceral arteriography and percutaneous transhepatic cholangiography is currently the preferred approach. Duodenoscopy with retrograde choledochopancreatography, as an alternative approach, will become increasingly utilized in the near future. An exploratory laparotomy for jaundice of undetermined etiology should not be undertaken without first performing one or the other of the above procedures

  14. The Combination of Gastroschisis, Jejunal Atresia, and Colonic Atresia in a Newborn

    Directory of Open Access Journals (Sweden)

    Zachary Bauman

    2015-01-01

    Full Text Available We encountered a rare case of gastroschisis associated with jejunal atresia and colonic atresia. In our case, the jejunal atresia was not discovered for 27 days after the initial abdominal wall closure. The colonic atresia was not discovered for 48 days after initial repair of the gastroschisis secondary to the rarity of the disorder. Both types of atresia were repaired with primary hand-sewn anastomoses. Other than the prolonged parenteral nutrition and hyperbilirubinemia, our patient did very well throughout his hospital course. Based on our case presentation, small bowel atresia and colonic atresia must be considered in patients who undergo abdominal wall closure for gastroschisis with prolonged symptoms suggestive of bowel obstruction. Our case report also demonstrates primary enteric anastomosis as a safe, well-tolerated surgical option for patients with types of intestinal atresia.

  15. Concentration of sup(99m)Tc-Sn-N-pyridoxyl-5-methyltryptophan, a biliary agent, in distant metastases of hepatomas

    Energy Technology Data Exchange (ETDEWEB)

    Hasegawa, Y; Nakano, S; Ibuka, K; Hashizume, T; Sasaki, Y; Imaoka, S; Ishiguro, S; Tanaka, S; Kasugai, H; Okano, Y

    1985-03-01

    During the last 2 years, eight patients with hepatocellular carcinoma who were suspected of having distant metastases have been studied to determine whether a new biliary agent, sup(99m)Tc-Sn-N-pyridoxyl-5-methyl-tryptophan (sup(99m)Tc-PMT), is taken up by extrahepatic tumors. In all eight patients, scintigrams showed a clearly increased uptake of sup(99m)Tc-PMT radioactivity by the extrahepatic tumors. In contrast, an increased uptake by the tumors of gallium citrate Ga 67 was only detected in four of the seven patients examined. The results obtained in this study suggest that sup(99m)Tc-PMT is useful both for characterizing the nature of extrahepatic tumors in patients with hepatoma and for detecting the metastases.

  16. Contrast amplification of the liver parenchyma in the computer tomogram by using intravenous and peroral biliary contrast media

    International Nuclear Information System (INIS)

    Justich, E.; Sager, W.D.; Dietrich, G.; Fotter, R.; Nedden, D. zur; Innsbruck Univ.

    1980-01-01

    If intravenous, biliary contrast media are used, a slight albeit specific enhancement of contrast of the liver parenchyma occurs with the applied dosage, which can be utilised in individual cases, for example for identifying isodense lesions. Contrast amplification by the peroral cholegraphic agent under examination, is insufficient for use in computer tomography of the liver. The use of biliary contrast media usually enables very good visualisation of the extrahepatic bile ducts. Attention is drawn to the possibility of pharmakokinetic studies by means of computer tomography. (orig.) [de

  17. Susceptibility to experimental biliary atresia linked to different hepatic gene expression profiles in two mouse strains.

    Science.gov (United States)

    Leonhardt, Johannes; Kuebler, Joachim F; Turowski, Carmen; Tschernig, Thomas; Geffers, Robert; Petersen, Claus

    2010-02-01

    To compare hepatic gene expression during the development of experimental biliary atresia (BA) in two different mouse strains. Balb/c mice and C57Black/6 (Black/6) mice were infected with rhesus rotavirus (RRV) postpartum, clinical signs of BA and survival were noted. Liver sections were assessed for cluster of differentiation antigen (CD) 3, CD4 and CD8 expression, and the hepatic virus load was determined. Second, mice of both strains were sacrificed three days after infection. Isolated hepatic RNA was subjected to gene expression analysis using Affymetrix Gene Chip MOE 430 2.0. The incidence of BA was significantly lower in Black/6 mice compared to Balb/c mice (13.5% vs. 67%, P < 0.05). The mean virus titers were higher in mice with BA compared to mice without BA. Different gene profiles three days after virus infection were noted, with differential expression of 201 genes, including those regulating apoptosis, nucleic acid binding, transport function and particularly the immune response (chemokine C-C motif ligand 2, toll-like receptor 3, CD antigen 14, chemokine (C-X-C motif) ligands 10 and 11). This correlated with a significant increase of CD4 positive cells only in Balb/c mice with BA compared to healthy mice (13.5 vs. 5.0; P < 0.05). Black/6 mice did not exhibit any significant increase of CD3 or CD4 leukocytes despite cholestasis. The different susceptibility to experimental BA was associated with an increase of CD4 T-cells in the liver of Balb/c mice, which is linked to different gene profiles at the onset of bile duct obstruction.

  18. Expression of Her-2/neu in extrahepatic cholangiocarcinoma

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    Shamekh R

    2017-02-01

    Full Text Available Rania Shamekh,1,* Marilin Rosa,2,* Zena Sayegh,2 Masoumeh Ghayouri,2 Richard Kim,3 Mokenge P Malafa,3 Domenico Coppola2 1Department of Pathology, University of South Florida, 2Department of Anatomic Pathology, 3Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA *These authors contributed equally to this work Background: Receptor tyrosine-protein kinase erbB-2, which is also frequently called human epidermal growth factor receptor-2 (Her-2 or Her-2/neu, has been found to be overexpressed in various human cancers.Hypothesis: The aim of this pilot study was to explore the frequency of Her-2/neu gene amplification and protein expression in extrahepatic cholangiocarcinoma (EHBC. We used the World Health Organization classification criteria for EHBC.Materials and methods: This was a retrospective study using 88 tissue samples, including 45 samples from non-neoplastic biliary tissue (NNB and 43 samples of extrahepatic cholangiocarcinoma (EHBC. A tissue microarray including NNB and EHBC was constructed and analyzed by immunohistochemistry (IHC and dual in situ hybridization for Her-2/neu protein expression and amplification, respectively. The Her-2/neu expression was scored following the guidelines used for the ToGA study.Results: All NNB samples and all but one EHBC samples showed no expression of Her-2/neu by IHC. The one EHBC case immunohistochemically positive for Her-2/neu had an IHC score of 3+. Her-2/neu gene amplification was present in two EHBC samples only and included the case found to be positive by IHC.Conclusion: Our findings are similar to those reported in the literature. Although Her-2/neu overexpression has been documented in many types of cancer, Her-2/neu protein overexpression tends to have no role in the development and/or progression of EHBC. Keywords: extrahepatic, cholangiocarcinoma, Her-2/neu, ToGA, immunohistochemistry

  19. [Neonatal cholestasis

    Science.gov (United States)

    Roquete, M L

    2000-07-01

    OBJECTIVE: To warn pediatricians about the early recognition of cholestasis in newborns and infants. METHODS: A bibliographic research about cholestasis was performed using Medline, and emphasizing the most relevant publications of the last 30 years. RESULTS: The concept of cholestasis and the causes of cholestatic tendency in newborns and infants are described. Several causes of intra and extrahepatic cholestasis are reported as well. In this review, only the diseases with diagnostic, therapeutic or prognostic peculiarities are commented, including extrahepatic biliary atresia, idiopathic neonatal hepatitis, galactosemia, and Alagille s syndrome. Furthermore, several resources are discussed for the diagnosis of cholestasis. CONCLUSIONS: The establishment of the diagnosis of cholestasis through the detection of hyperbilirubinemia in newborns who present jaundice after 14 days of life is a goal that could change the prognosis of several diseases responsible for neonatal cholestasis.

  20. Biliary obstruction dissipates bioelectric sinusoidal-canalicular barrier without altering taurocholate uptake

    International Nuclear Information System (INIS)

    Cotting, J.; Zysset, T.; Reichen, J.

    1989-01-01

    To study immediate events during extrahepatic cholestasis, we investigated the effect of short-term biliary obstruction on the bioelectrical sinusoidal-canalicular barrier in the rat using molecular weight-matched uncharged and negatively charged inert solute pairs. The bioelectrical barrier averaged -22 +/- 5 and -18 +/- 4 mV (NS) using the pair carboxy-/methoxyinulin and ferrocyanide/sucrose, respectively. After a 20-min biliary obstruction both decreased by 61 and 11%, respectively, but only the large molecular weight pair (the inulins) returned to base line after release of the obstruction. Inert solute clearances were increased after short biliary obstruction depending on molecular size and negative charge (ferrocyanide greater than sucrose greater than carboxyinulin greater than inulin), suggesting that both permeability and bioelectrical barriers were affected by obstruction. The hepatic extraction in vivo of a passively transported drug not excreted into bile (D-propranolol) was not affected by obstruction, whereas that of an actively transported drug (glycocholate) decreased from 66 +/- 8 to 41 +/- 20% during biliary obstruction (P less than 0.01). Unidirectional transfer of glycocholate was not affected by short-term biliary obstruction in the situ perfused rat liver; however, 2 min after [14C]glycocholate administration, increased return was observed in hepatic venous effluent in obstructed animals. Our findings demonstrate a loss of the bioelectrical barrier immediately after short-term biliary obstruction. Decreased hepatic extraction in the view of unaltered sinusoidal uptake demonstrates regurgitation of bile into blood during short-term biliary obstruction

  1. The value of the hepatobiliaric function scintigraphy with Tc-99m-DAIDA for post-operative biliary tract function diagnosis

    International Nuclear Information System (INIS)

    Ast, G.

    1982-01-01

    The goal of this work was the examination of the value of biliary tract scintigraphy with 99m Tc DAIDA for post-operative supervision after biliary tract operations. The subjects were made up of 22 patients, who between 1969 and 1980 had undergone a choledochus revision with the insertion of a T drainage. In all cases with cholestase-specific laboratory findings the intra- and extrahepatic biliary tracts were expanded and in 71% of the cases were combined with an organically dependent drainage retardation which was scintigraphically proven. The proof of an organically dependent drainage retardation in scintigraphy coincided almost always with expanded biliary paths and in almost half the cases with blockage-specific changes in the laboratory values. Conclusion: By patients with operations on the biliary tracts the indication for biliary tract scintigraphy with 99m Tc DAIDA is always given, if with cholestase- oder liver-specifically changed laboratory values no concretions using sonography or preliminary radiographs could be proven. (TRV) [de

  2. Th-17 cells infiltrate the liver in human biliary atresia and are related to surgical outcome.

    Science.gov (United States)

    Hill, Richard; Quaglia, Alberto; Hussain, Munther; Hadzic, Nedim; Mieli-Vergani, Giorgina; Vergani, Diego; Davenport, Mark

    2015-08-01

    Biliary atresia (BA), a cholangiopathy of unknown etiology is associated with intrahepatic mononuclear cell infiltrate. An abnormal reaction to viral exposure has been hypothesized in some cases. We aimed to investigate the nature of the CD4+ hepatic infiltrate in defined clinical variants of BA by quantification of inflammatory cell components. Liver biopsies of infants obtained at Kasai portoenterostomy (KPE) were stained immunohistochemically using monoclonal antibodies to Tbet, GATA-3, FOXP3 and interleukin (IL) 17, identifying Th-1, Th-2, Tregs and Th-17 cells respectively. T cells were counted with the aid of a graticule. Data are reported as median (range) of cells per high-power-field (×400) and compared using nonparametric statistical tests with P≤0.05 regarded as significant. Liver biopsies from BA (n=37) and age-matched cholestatic controls (e.g. alpha-1-anti trypsin deficiency, Alagilles syndrome, n=12) were investigated. BA infants were divided into three groups: cytomegalovirus IgM +ve (CMV; n=9); BA splenic malformation (BASM; n=9) and isolated BA (IBA; n=19). All T-cell subsets were present in the portal tracts, with an overrepresentation of Th-1 (PTh-17 (PTh-2 (P=0.94) or Tregs (P=0.15), compared to controls. Th-1 cells predominated in the CMV group; (18 [7-37] vs. 3 [0-14] [BASM] and vs. 5 [3-23] [IBA]; PTh-17 cells. The degree of Th-1 cell infiltrate inversely correlated with platelet count (rS=-0.49; PTh-17 cells were fewer (6 [2-11] vs. 11 [8-20]; P=0.02) in infants who cleared their jaundice (n=15, Th-17 cells infiltrate the liver in BA and are associated with a worse surgical outcome; a Th-1 profile predominates in CMV-associated BA. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Prognos is of living donor liver transplantation performed during different periods in infants with biliary atresia: a single-center experience with 101 cases

    Directory of Open Access Journals (Sweden)

    HUA Xiangwei

    2014-01-01

    Full Text Available ObjectiveTo compared the prognosis between infants with biliary atresia (BA who underwent living donor liver transplantation (LDLT during 2006-2009 and 2010-2012 in the Department of Liver Surgery, Renji Hospital, Shanghai Jiaotong University School of Medicine and to evaluate the prognos is of LDLT performed during different periods. MethodsA retrospective analysis was performed on the clinical data of 101 infants with BA who underwent LDLT from October 2006 to December 2012. These patients were divided into 2006-2009 group and 2010-2012 group according to the year the procedure was performed. The two groups were compared in terms of preoperative general data, intraoperative status, and treatment outcome. The Kaplan-Meier method was used for survival analysis; measurement data were analyzed by t test, and enumeration data were analyzed by Fisher exact probability test and chi-square test. ResultsThe preoperative general data showed no significant difference between the two groups. Compared with the 2006-2009 group, the 2010-2012 group had significantly less intraoperative blood loss (t=2.05, P=0.04, a significantly shorter cold ischemia time of donor liver (t=3.25, P<0.01, and significantly lower incidence of postoperative biliary complications (χ2=4.27, P=0.04, pulmonary infection (χ2=4.47, P=0.03, and acute rejection (P=0.03. The 2010-2012 group had significantly improved survival compared with the 2006-2009 group; the 1 and 2 year cumulative survival rates for the 2010-2012 group were 884% and 88.4%, respectively, versus 84.4% and 75% for the 2006-2009 group. ConclusionThe development of surgical techniques, improvement in perioperative management, and standardized follow-up can significantly increase the success rate of surgery and survival rate and reduce postoperative complications in BA patients.

  4. A case of distal extrahepatic cholangiocarcinoma with two positive resection margins.

    Science.gov (United States)

    Warner, Wayne A; Ramcharan, Wesley; Harnanan, Dave; Umakanthan, Srikanth; Maharaj, Ravi

    2016-11-01

    Cholangiocarcinoma is an uncommon primary malignancy of the biliary tract that is challenging to diagnose and treat effectively due to its relatively silent and late clinical presentation. The present study reports a case of a 60-year-old male with distal extrahepatic cholangiocarcinoma with a 3-week history of painless obstructive jaundice symptoms and subjective weight loss. Imaging revealed an obstructing lesion in the common bile duct, just distal to the entrance of the cystic duct. Pathology revealed moderately differentiated cholangiocarcinoma with two positive proximal resection margins. The two positive resection margins presented a challenge during surgery and points to an urgent need for further studies to better illuminate diagnostic and therapeutic options for patients with similar clinicopathological presentation.

  5. Administration of biliary contrast media in computed tomography

    International Nuclear Information System (INIS)

    Huebener, K.-H.; Treugut, H.

    1981-01-01

    Biliary contrast media have 2 main uses in computed tomography (CT) of the liver and bile ducts: 1. Labelling of extrahepatic bile ducts in order to aid in the identification of the common bile ducts and the papilla of Vater, particularly in cases of complex, mostly postoperative situs. 2. Differentiation between normal and abnormal liver tissue in cases of focal nodular hyperplasia with proliferation of tumorous bile ducts. The applicability of biliary contrast media is rather limited as far as the improvement of spatial resolution by an increase of contrast is concerned, because the attainable enhancement today remains small. The possibility of interpretation of the liver function is likewise insufficient, because the standard deviation of the time-dependent enhancement is too great in the normal collective in order to register deviations reliably. In cases of liver cirrhosis, a rise of density of at least 40-60 Hounsfield Units (HU) would be desirable. (Auth.)

  6. Management of benign biliary strictures with a novel retrievable self-expandable metal stent.

    Science.gov (United States)

    Hu, Bing; Leung, Joseph W; Gao, Dao Jian; Wang, Tian Tian; Wu, Jun

    2014-03-01

    Endoscopic placement of covered self-expandable metal stent (SEMS) has gained popularity in the management of benign biliary strictures (BBS). The existing SEMS has been designed primarily to palliate malignant biliary obstruction and has a high frequency of stent migration, difficulty in retrieval and stricture recurrence after stent removal. This study aimed to design a novel retrievable SEMS dedicated to the treatment of extrahepatic BBS and evaluate its clinical efficacy and safety. A short fully covered SEMS (FCSEMS) with a retrieval lasso was designed for the specific treatment of BBS. A total of 45 patients with segmental extrahepatic BBS were included in this study. The stent was placed entirely inside the bile duct with only the retrieval lasso extending from the papilla. The stents were recommended to be in situ for 6 to 12 months before removal. The FCSEMS was successfully placed in all 45 patients. In all, 33 patients had their FCSEMS successfully removed after a mean period of 8.6 ± 3.7 (range 2-15.5) months. Stent migration occurred in 9.1% of the patients. During a mean follow-up of 18.9 months after stent removal, recurrent stricture was found in 2 (6.1%) patients and was successfully treated with a second FCSEMS. Overall, the strictures resolved in 30/33 (90.9%) patients. Intraductal placement of a short FCSEMS is suitable for the treatment of segmental extrahepatic BBS. This new removable design offered prolonged stenting and drainage for BBS for up to one year with minimal complications. © 2013 Chinese Medical Association Shanghai Branch, Chinese Society of Gastroenterology, Renji Hospital Affiliated to Shanghai Jiaotong University School of Medicine and Wiley Publishing Asia Pty Ltd.

  7. Biliary complications after liver transplantation: diagnosis with multi-slice CT

    International Nuclear Information System (INIS)

    Zhu Kangshun; Meng Xiaochun; Xu Changmou; Shen Min; Qian Jiesheng; Pang Pengfei; Guan Shouhai; Jiang Zaibo; Shan Hong

    2009-01-01

    Objective: To evaluate multislice CT in the diagnosis of biliary complications after liver transplantation. Methods: Eighty-three consecutive patients who had undergone orthotopic liver transplantation (OLT) presented with clinical or biochemical signs of biliary complications and underwent contrast-enhancement CT examination. Three experienced radiologists, who were blinded to patient's clinical data, assessed CT images for the detection of biliary complications in consensus. Diagnostic confirmation of biliary complications was obtained with direct cholangiography in 69 patients, histologic study in 11 patients and hepaticojejunostomy in 3 patients. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of CT for the detection of biliary complications were calculated. In addition, CT features of anastomotic biliary stricture (ABS) were compared with those of non-anastomotic biliary stricture (NABS) using χ 2 test. Results: A total of 62 biliary complications (74.7%) was eventually confirmed in the 83 patients, including ABS in 32 patients, NABS in 21 patients, biliary duct stones in 16 patients (of which 12 patients with biliary stricture) , anastomotic bile leakage in 5 patients, biloma in 4 patients with biliary stricture, and biliogenic abscess in 2 patients with biliary stricture. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of CT for the detection of biliary stricture were 90.6%, 86.7%, 89.2%, 92.3% and 83.9%, respectively. Other biliary complications, including biliary duct stones (16 cases), anastomotic bile leak (5 cases), biloma (4 cases), and biliogenic abscess (2 cases), were correctly diagnosed by CT; there was no false-positive or false-negative result. The incidence of irregular dilatation of bile duct was 71.4% (15/21), which was significantly higher in NABS cases than in ABS of 25.0% (8/32, P<0.01); whereas the incidence of extrahepatic biliary dilatation

  8. Evolving Role of Endoscopic Retrograde Cholangiopancreatography in Management of Extrahepatic Hepatic Ductal Injuries due to Blunt Trauma: Diagnostic and Treatment Algorithms

    Directory of Open Access Journals (Sweden)

    Nikhil P. Jaik

    2008-01-01

    Full Text Available Extrahepatic hepatic ductal injuries (EHDIs due to blunt abdominal trauma are rare. Given the rarity of these injuries and the insidious onset of symptoms, EHDI are commonly missed during the initial trauma evaluation, making their diagnosis difficult and frequently delayed. Diagnostic modalities useful in the setting of EHDI include computed tomography (CT, abdominal ultrasonography (AUS, nuclear imaging (HIDA scan, and cholangiography. Traditional options in management of EHDI include primary ductal repair with or without a T-tube, biliary-enteric anastomosis, ductal ligation, stenting, and drainage. Simple drainage and biliary decompression is often the most appropriate treatment in unstable patients. More recently, endoscopic retrograde cholangiopancreatography (ERCP allowed for diagnosis and potential treatment of these injuries via stenting and/or papillotomy. Our review of 53 cases of EHDI reported in the English-language literature has focused on the evolving role of ERCP in diagnosis and treatment of these injuries. Diagnostic and treatment algorithms incorporating ERCP have been designed to help systematize and simplify the management of EHDI. An illustrative case is reported of blunt traumatic injury involving both the extrahepatic portion of the left hepatic duct and its confluence with the right hepatic duct. This injury was successfully diagnosed and treated using ERCP.

  9. Extrahepatic manifestations of cholestasis

    NARCIS (Netherlands)

    Glasova, Helena; Beuers, Ulrich

    2002-01-01

    Pruritus, fatigue and metabolic bone disease represent three major extrahepatic manifestations of chronic cholestatic liver disease that considerably affect the patient's quality of life. The present article reviews pathogenetic aspects of and current therapeutic approaches to extrahepatic

  10. DMBT1 expression in biliary carcinogenesis with correlation of clinicopathological data

    DEFF Research Database (Denmark)

    Goeppert, Benjamin; Roessler, Stephanie; Becker, Natalia

    2017-01-01

    Aims: Deleted in malignant brain tumours 1 (DMBT1) exerts functions in the regulation of epithelial differentiation and inflammation and has been proposed as a tumour suppressor. Because chronic inflammation is a hallmark of cholangiocarcinogenesis, the aim of this study was to investigate...... the expression of DMBT1 in biliary tract cancer (BTC) and to correlate this expression with clinicopathological data. Methods and results: The expression of DMBT1 protein was examined immunohistochemically in 157 BTC patients [41 intrahepatic (ICC), 60 extrahepatic cholangiocarcinomas (ECC) and 56...

  11. Multicenter study of endoscopic preoperative biliary drainage for malignant hilar biliary obstruction: E-POD hilar study.

    Science.gov (United States)

    Nakai, Yousuke; Yamamoto, Ryuichi; Matsuyama, Masato; Sakai, Yuji; Takayama, Yukiko; Ushio, Jun; Ito, Yukiko; Kitamura, Katsuya; Ryozawa, Shomei; Imamura, Tsunao; Tsuchida, Kouhei; Hayama, Jo; Itoi, Takao; Kawaguchi, Yoshiaki; Yoshida, Yu; Sugimori, Kazuya; Shimura, Kenji; Mizuide, Masafumi; Iwai, Tomohisa; Nishikawa, Ko; Yagioka, Hiroshi; Nagahama, Masatsugu; Toda, Nobuo; Saito, Tomotaka; Yasuda, Ichiro; Hirano, Kenji; Togawa, Osamu; Nakamura, Kenji; Maetani, Iruru; Sasahira, Naoki; Isayama, Hiroyuki

    2018-05-01

    Endoscopic nasobiliary drainage (ENBD) is often recommended in preoperative biliary drainage (PBD) for hilar malignant biliary obstruction (MBO), but endoscopic biliary stent (EBS) is also used in the clinical practice. We conducted this large-scale multicenter study to compare ENBD and EBS in this setting. A total of 374 cases undergoing PBD including 281 ENBD and 76 EBS for hilar MBO in 29 centers were retrospectively studied. Extrahepatic cholangiocarcinoma (ECC) accounted for 69.8% and Bismuth-Corlette classification was III or more in 58.8% of the study population. Endoscopic PBD was technically successful in 94.6%, and adverse event rate was 21.9%. The rate of post-endoscopic retrograde cholangiopancreatography pancreatitis was 16.0%, and non-endoscopic sphincterotomy was the only risk factor (odds ratio [OR] 2.51). Preoperative re-intervention was performed in 61.5%: planned re-interventions in 48.4% and unplanned re-interventions in 31.0%. Percutaneous transhepatic biliary drainage was placed in 6.4% at the time of surgery. The risk factors for unplanned procedures were ECC (OR 2.64) and total bilirubin ≥ 10 mg/dL (OR 2.18). In surgically resected cases, prognostic factors were ECC (hazard ratio [HR] 0.57), predraiange magnetic resonance cholangiopancreatography (HR 1.62) and unplanned re-interventions (HR 1.81). EBS was not associated with increased adverse events, unplanned re-interventions, or a poor prognosis. Our retrospective analysis did not demonstrate the advantage of ENBD over EBS as the initial PBD for resectable hilar MBO. Although the technical success rate of endoscopic PBD was high, its re-intervention rate was not negligible, and unplanned re-intervention was associated with a poor prognosis in resected hilar MBO. © 2017 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

  12. Extrahepatic spread of hepatocellular carcinoma: a pictorial review

    International Nuclear Information System (INIS)

    Hong, Seong Sook; Kim, Tae Kyoung; Sung, Kyu-Bo; Kim, Pyo Nyun; Ha, Hyun Kwon; Kim, Ah Young; Lee, Moon-Gyu

    2003-01-01

    Although extrahepatic spread of hepatocellular carcinoma (HCC) is uncommon, it can be found anywhere in the body. Most extrahepatic metastases of HCC occur in patients with advanced-stage intrahepatic tumor, but incidental extrahepatic lesions have also occasionally been found in patients with early-stage intrahepatic HCC. The detection of extrahepatic metastatic disease is crucial when planning therapy for patients with HCC and should be used to avoid unnecessary surgical intervention. In this study we illustrate the radiologic findings of extrahepatic metastases of HCC involving various sites. The presumed mechanism of extrahepatic extension of HCC is also discussed. (orig.)

  13. Expression and localization of sterile alpha motif domain containing 5 is associated with cell type and malignancy of biliary tree.

    Directory of Open Access Journals (Sweden)

    Tomoki Yagai

    Full Text Available Cholangiocarcinoma (CC is a type of relatively rare neoplasm in adenocarcinoma. The characteristics of CCs as well as biliary epithelial cells are heterogeneous at the different portion of the biliary tree. There are two candidate stem/progenitor cells of the biliary tree, i.e., biliary tree stem/progenitor cell (BTSC at the peribiliary gland (PBG of large bile ducts and liver stem/progenitor cell (LPC at the canals of Hering of peripheral small bile duct. Although previous reports suggest that intrahepatic CC (ICC can arise from such stem/progenitor cells, the characteristic difference between BTSC and LPC in pathological process needs further investigation, and the etiology of CC remains poorly understood. Here we show that Sterile alpha motif domain containing 5 (SAMD5 is exclusively expressed in PBGs of large bile ducts in normal mice. Using a mouse model of cholestatic liver disease, we demonstrated that SAMD5 expression was upregulated in the large bile duct at the hepatic hilum, the extrahepatic bile duct and PBGs, but not in proliferating intrahepatic ductules, suggesting that SAMD5 is expressed in BTSC but not LPC. Intriguingly, human ICCs and extrahepatic CCs exhibited striking nuclear localization of SAMD5 while the normal hilar large bile duct displayed slight-to-moderate expression in cytoplasm. In vitro experiments using siRNA for SAMD5 revealed that SAMD5 expression was associated with the cell cycle regulation of CC cell lines.SAMD5 is a novel marker for PBG but not LPC in mice. In humans, the expression and location of SAMD5 could become a promising diagnostic marker for the cell type as well as malignancy of bile ducts and CCs.

  14. Familial atrophia maculosa varioliformis cutis.

    Science.gov (United States)

    Kalayciyan, Aylin; Kotogyan, Agop; Demirkesen, Cuyan; Tüzün, Yalçin

    2003-07-01

    Atrophia maculosa varioliformis cutis (AVMC) was first described by Heidingsfeld in 1918, as a rarely reported form of idiopathic macular atrophy on the cheek (1). It is characterized, clinically, by shallow, sharply demarcated depressions in various shapes. Extrahepatic biliary atresia (2) and pachydermodactyly (3) have been the only conditions associated with AMVC reported in the past 80 years. Although keratosis pilaris is a common skin disorder, it is related to other idiopathic atrophic conditions considered in the differential diagnosis of AMVC, namely keratosis pilaris atrophicans (4). However, the two associations may be coincidental. We observed a patient with keratosis pilaris, and her brother and an unrelated young man, whose findings led to a diagnosis of AMVC.

  15. Macrophage Migration Inhibitory Factor (MIF) Gene Promotor Polymorphism Is Associated with Increased Fibrosis in Biliary Atresia Patients, but Not with Disease Susceptibility.

    Science.gov (United States)

    Sadek, Khaled H; Ezzat, Sameera; Abdel-Aziz, Samira A; Alaraby, Hanaa; Mosbeh, Asmaa; Abdel-Rahman, Mohamed H

    2017-09-01

    Two polymorphisms, rs755622 and rs5844572, in the promoter region of the macrophage migration inhibitory factor (MIF) gene influence the basal and/or induced transcriptional activity and have been linked to several inflammatory and autoimmune diseases. The aim of this study was to investigate the association between these two polymorphisms and disease susceptibility in patients with biliary atresia (BA). Allele frequencies of rs755622 and rs5844572 were assessed in 60 Egyptian infants with a confirmed diagnosis of BA. DNA was extracted from archival material. For the rs755622, samples were tested using Taqman real-time PCR, and for the rs5844572, samples were tested using fluorescence-based genotyping. The allele frequency in the general population was assessed in 141 healthy adults from the same geographical location. No statistical differences were observed in the allele frequencies of either rs755622 or rs5844572 between BA patients and controls. The homozygous and heterozygous short repeats (5/5, or 5/X) of rs5844572 were observed more frequently (16/28, 57.1%) in BA patients with mild to moderate fibrosis compared with those with marked fibrosis (10/32, 31.3%). The difference was statistically significant (P  =  0.032). In conclusion, we observed no association between MIF rs755622 and rs5844572 polymorphisms and susceptibility to BA; however, the rs5844572 could be linked to the rate of progression of the disease and extent of fibrosis. © 2017 John Wiley & Sons Ltd/University College London.

  16. Tutorial Review for Understanding of Cholangiopathy

    Directory of Open Access Journals (Sweden)

    Yasuni Nakanuma

    2012-01-01

    Full Text Available The biliary tree consists of intrahepatic and extrahepatic bile ducts and is lined by biliary epithelial cells (or cholangiocytes. There are also peribiliary glands around the intrahepatic large bile ducts and extrahepatic bile ducts. The biliary tree is a conduit of bile secreted by hepatocytes and biliary epithelial cells and also of the peribiliary glands and has several physiological roles. A number of diseases affect mainly the intrahepatic and extrahepatic biliary tree, and, in this special issue, these cholangiopathies are reviewed in detail with respect to genetics, pathogenesis, and pathology. In this paper, the anatomy and physiology of the biliary tree, basic injuries to biliary epithelial cells from stress and bile duct damage, and representative cholangiopathies are briefly reviewed.

  17. Congenital pyloric atresia: clinical features, diagnosis, associated ...

    African Journals Online (AJOL)

    Epidermolysis bullosa was seen in eight (40%) patients and multiple intestinal atresias in five (25%). Three patients had associated esophageal atresia. Pyloric diaphragm was the most common and seen in 13 patients including double diaphragms in two followed by pyloric atresia with a gap in four and pyloric atresia ...

  18. Thread sign in biliary intraductal papillary mucinous neoplasm: a novel specific finding for MRI

    International Nuclear Information System (INIS)

    Hong, Gil-Sun; Byun, Jae Ho; Kim, Jin Hee; Kim, Hyoung Jung; Lee, Seung Soo; Lee, Moon-Gyu; Hong, Seung-Mo

    2016-01-01

    To evaluate thread sign of biliary intraductal papillary mucinous neoplasm (B-IPMN) on magnetic resonance imaging (MRI). Thread sign was defined as intraductal linear or curvilinear hypointense striations. Two radiologists independently evaluated the presence and location of thread sign on MR cholangiography (thin-slice, thick-slab and 3D MRC) and axial MR images (T2 TSE, T2 HASTE and DWI) in patients with B-IPMN (n = 38) and in matched control groups with benign (n = 36) or malignant (n = 35) biliary diseases. Sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV) of thread sign for diagnosing B-IPMN were evaluated. Thread sign was observed only in patients with B-IPMN on MRC (44.7-52.6 % [17/38-20/38], P < 0.001) and axial MR images (31.6 % [12/38], P < 0.001), except in one patient with recurrent pyogenic cholangitis on MRC (2.8 %, 1/36). The sensitivity, specificity, accuracy, PPV and NPV of thread sign for diagnosing B-IPMN on MRC were 0.53, 0.99, 0.83, 0.95 and 0.80, respectively (reader 1) and 0.45, 1.0, 0.81, 1.0 and 0.77, respectively (reader 2). Thread sign was detected mainly at the extrahepatic bile duct (52.6 %, 20/38). B-IPMN can manifest thread sign, a novel specific MR finding, mainly at the extrahepatic bile duct on MRI, especially on MRC. (orig.)

  19. Interleukin 17, Produced by γδ T Cells, Contributes to Hepatic Inflammation in a Mouse Model of Biliary Atresia and Is Increased in Livers of Patients.

    Science.gov (United States)

    Klemann, Christian; Schröder, Arne; Dreier, Anika; Möhn, Nora; Dippel, Stephanie; Winterberg, Thomas; Wilde, Anne; Yu, Yi; Thorenz, Anja; Gueler, Faikah; Jörns, Anne; Tolosa, Eva; Leonhardt, Johannes; Haas, Jan D; Prinz, Immo; Vieten, Gertrud; Petersen, Claus; Kuebler, Joachim F

    2016-01-01

    Biliary atresia (BA) is a rare disease in infants, with unknown mechanisms of pathogenesis. It is characterized by hepatobiliary inflammatory, progressive destruction of the biliary system leading to liver fibrosis, and deterioration of liver function. Interleukin (IL) 17A promotes inflammatory and autoimmune processes. We studied the role of IL17A and cells that produce this cytokine in a mouse model of BA and in hepatic biopsy samples from infants with BA. We obtained peripheral blood and liver tissue specimens from 20 patients with BA, collected at the time of Kasai portoenterostomy, along with liver biopsies from infants without BA (controls). The tissue samples were analyzed by reverse transcription quantitative polymerase chain reaction (PCR), in situ PCR, and flow cytometry analyses. BA was induced in balb/cAnNCrl mice by rhesus rotavirus infection; uninfected mice were used as controls. Liver tissues were collected from mice and analyzed histologically and by reverse transcriptase PCR; leukocytes were isolated, stimulated, and analyzed by flow cytometry and PCR analyses. Some mice were given 3 intraperitoneal injections of a monoclonal antibody against IL17 or an isotype antibody (control). Livers from rhesus rota virus-infected mice with BA had 7-fold more Il17a messenger RNA than control mice (P = .02). γδ T cells were the exclusive source of IL17; no T-helper 17 cells were detected in livers of mice with BA. The increased number of IL17a-positive γδ T cells liver tissues of mice with BA was associated with increased levels of IL17A, IL17F, retinoid-orphan-receptor C, C-C chemokine receptor 6, and the IL23 receptor. Mice that were developing BA and given antibodies against IL17 had lower levels of liver inflammation and mean serum levels of bilirubin than mice receiving control antibodies (191 μmol/L vs 78 μmol/L, P = .002). Liver tissues from patients with BA had 4.6-fold higher levels of IL17 messenger RNA than control liver tissues (P = .02

  20. Acute Abdomen Secondary to a Spontaneous Perforation of the Biliary Tract, a Rare Complication of Choledocholithiasis

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    G.A. Gómez-Torres

    Full Text Available Introduction: The spontaneous perforation of the biliary tract (SPBT is an extremely rare cause of peritonitis, which was first described by Freeland in 1982, to date only around 70 cases have been reported. Here we present a case of spontaneous perforation of the biliary tract, in a patient with choledocholithiasis, which was treated with ultrasound-guided drainage and ERCP. Case report: A 51-year-old male was admitted to the emergency room for 15-day evolution jaundice, localized pain in the right flank and hypochondrium of 3 days. He had a history of cholecystectomy 15 years ago and 4 episodes of cholangitis, the last one in 2015. A magnetic resonance imaging (MRI was performed, that showed evidence of choledocholithiasis, in addition to a possible biliary leakage. The patient was treated with ultrasound-guided drainage and ERCP successfully. Discussion: Spontaneous perforation of the biliary tract is a disease entity in which wall of the extrahepatic or intrahepatic duct is perforated without any traumatic or iatrogenic injury. The clinical presentation varies from nonspecific abdominal pain to biliary peritonitis, in most of the cases forming bilomas. Universal management involves decompression of the biliary tree and repair of the leak site. Conclusion: The spontaneous perforation of the biliary tract is a disease that represents a diagnostic challenge. The treatment in the patients with SPBT is not well established and has to be individualized for each case, depending on the history of the patient, the site of perforation, the time of evolution, the suspicion of infection, and the patient status. Keywords: Acute abdomen, Spontaneous perforation biliary tract, Biloma, Complication choledocholithiasis, Case report

  1. Grade IV fibrosis interferes in biliary drainage after Kasai procedure.

    Science.gov (United States)

    Salzedas-Netto, A A; Chinen, E; de Oliveira, D F; Pasquetti, A F; Azevedo, R A; da Silva Patricio, F F; Cury, E K; Gonzalez, A M; Vicentine, F P P; Martins, J L

    2014-01-01

    Biliary atresia (BA) is the most common cause of liver transplantation in children. The earlier the treatment is done, the better the prognosis. The aim is to evaluate the impact of late diagnosis in children with BA, including the histopathological findings and success rate of biliary drainage in patients submitted to hepatic portoenterostomy (HPE). A retrospective study of cases of BA in the Department of Pediatric Surgery, Federal University of São Paulo (UNIFESP) between 1998-2011. We found 63 cases of BA; of these, 42 underwent HPE and 21 were referred for liver transplantation. Clinic and pathologic data were evaluated. The HPE was performed with a mean age of 86.5 days, with 16.6% having the operation at 60 days or earlier; 59.2% between 61 and 90 days; and 23.8% after 90 days. Successful biliary drainage occurred in 31% of surgeries, Mean days when HPE drained was 69.1 days, and 94.3 days when the surgery did not drain (P = .05). All patients who were successfully drained, did not have grade IV fibrosis on histology. In cases in which surgery was performed after 60 days that had not drained, 25% had grade IV fibrosis on biopsy (P = .0469). The age of HPE relates to better prognosis of the disease. It was found that the rate of grade IV fibrosis is higher in no drainage patients. All patients with grade IV fibrosis had no biliary drainage. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Radiologic diagnostic criteria of sphincter of oddi dysfunction: analysis of five cases confirmed by biliary manometry

    International Nuclear Information System (INIS)

    Auh, Yong Ho; Kim, Myung Hwan; Lim, Hyun Chul; Baek, Seung Yeon; Lee, Moon Gyu; Cho, Kyoung Sik; Sung, Hyun Kyung

    1994-01-01

    Biliary dyskinesia was considered as a wastebasket of quasi-biliary disease which could not be clearly explained under the basis of morphologic pathology. This entity was a source of confusion because of misconception and poor understanding. Recent introduction of biliary manometric technique enlightened some of these disorders. We evaluated the cholangiographic morphology of these disorders to clarify and to characterize the some characteristic findings, subsequently in order to help the diagnosis. Five cases were confirmed by this technique as sphincter of Oddi dysfunction for last 13 months. All patients were female and age range was 53 to 75 years old. All patients were suffered from intermittent and recurrent biliary type pain. ERCP showed five common findings. The common bile duct was dilated over 12 mm in all patients. Different from recurrent pyogenic cholangiohepatitis, intrahepatic ducts were proportionally dilated as extrahepatic ducts, in four patients and they branched normally and ductal wall was smooth. Transient or persistent meniscus sign was observed in four patients. All patients showed delayed emptying of contrast media from the common bile duct into the duodenum. Following IV injection of cholecystokinin, persistent meniscus disappeared and contrast media inflowed into the duodenum. Identification of all or some characteristic cholangiographic findings may eliminate a cumbersome and painful biliary manometric test for the diagnosis of sphincter of Oddi dysfunction

  3. Biliary Fasciola gigantica infestation in a nonendemic area--An intraoperative surprise.

    Science.gov (United States)

    Menon, Prema; Sinha, Amit Kumar; Rao, Katragadda Lakshmi Narasimha; Khurana, Sumeeta; Lal, Sadhana; Thapa, Babu Ram

    2015-11-01

    A 7year old girl infected with the zoonotic trematode, Fasciola gigantica is reported because of the extreme rarity of this condition in our region. Because of the overlap in symptomatology and radiological features, the more common amebic/pyogenic liver abscess in the initial hepatic migratory phase and later choledochal cyst/biliary ascariasis when the parasite was finally located in the extrahepatic bile ducts, were thought of delaying effective treatment. The diagnosis was confirmed only by surgical exploration. The characteristic contrast enhanced computed tomography scan features retrospectively identified were multiple clustered hypodense lesions in the liver with peripheral enhancement in the acute hepatic migratory phase, and periportal tracking in the previously affected areas of the liver with biliary dilatation and a linear hypointense lesion within the common bile duct in the chronic phase. Although a known association, she did not have eosinophilia. This child, who became symptomatic at the age of 5.5years, also appears to be one of the youngest patients reported with Fasciola gigantica. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Spontaneous biliary peritonitis: Is bed side diagnosis possible?

    Directory of Open Access Journals (Sweden)

    Vijai Datta Upadhyaya

    2013-01-01

    Full Text Available Background: Spontaneous biliary peritonitis is a rare cause of acute abdomen. In spontaneous biliary peritonitis there is perforation in the wall of the extra-hepatic or intra-hepatic duct occurs without any traumatic or iatrogenic injury and have been described more often in neonates. The symptoms may be acute or insidious delaying the diagnosis. Present manuscript deals with diagnosis and management of these cases. Materials and Methods: This is a prospective study and all patients of suspected biliary peritonitis presented during Dec 2010 to Feb 2012 were included in the study. After preliminary investigations in all patients abdominal paracentesis was done and in cases where intra-abdominal fluid bilirubin level was several fold higher than serum bilirubin level were subjected to exploratory laparotomy. Further investigation like T-tube cholangiogram and magnetic resonance cholangiopancreatography (MRCP was done to rule out choledochal cyst before leveling these cases as SPBD. Results: A total of 6 patients were included in present series commonest presenting symptom was progressive abdominal distension without signs of overt peritonitis followed by progressive jaundice, fever and abdominal pain. On exploration site of perforation was observed in 50% of cases and in 50% of cases bile duct was not dilated. Second surgery was not required in 34% of cases. There was no mortality or significant morbidity in our series. Conclusion: Spontaneous perforation of bile duct is rare disease and high index of suspicion is required for diagnosis. Simple bed side test can help in diagnosis but T tube cholangiogram or MRCP are must to rule out choledochal cyst.

  5. Biliatresone, a Reactive Natural Toxin from Dysphania glomulifera and D. littoralis: Discovery of the Toxic Moiety 1,2-Diaryl-2-Propenone.

    Science.gov (United States)

    Koo, Kyung A; Lorent, Kristin; Gong, Weilong; Windsor, Peter; Whittaker, Stephen J; Pack, Michael; Wells, Rebecca G; Porter, John R

    2015-08-17

    We identified a reactive natural toxin, biliatresone, from Dysphania glomulifera and D. littoralis collected in Australia that produces extrahepatic biliary atresia in a zebrafish model. Three additional isoflavonoids, including the known isoflavone betavulgarin, were also isolated. Biliatresone is in the very rare 1,2-diaryl-2-propenone class of isoflavonoids. The α-methylene of the 1,2-diaryl-2-propenone of biliatresone spontaneously reacts via Michael addition in the formation of water and methanol adducts. The lethal dose of biliatresone in a zebrafish assay was 1 μg/mL, while the lethal dose of synthetic 1,2-diaryl-2-propen-1-one was 5 μg/mL, suggesting 1,2-diaryl-2-propenone as the toxic Michael acceptor.

  6. Needle-Knife Fistulotomy for the Rescue: An Unusual Cause of Iatrogenic Extrahepatic Biliary Obstruction

    Directory of Open Access Journals (Sweden)

    Laura L. Ulmer

    2018-01-01

    Full Text Available A 71-year-old male presented to our institution with cholestatic hepatitis after having recently undergone upper endoscopy for treatment of gastrointestinal bleeding. Further investigation with endoscopic retrograde cholangiopancreatography revealed a hemostatic clip on the ampulla of Vater. After initial attempts at cannulation of the common bile duct were unsuccessful, biliary decompression was achieved by use of needle-knife fistulotomy. A common bile duct stent was placed and the liver function tests improved prior to discharge.

  7. Surgery for congenital choanal atresia.

    Science.gov (United States)

    Cedin, Antonio C; Atallah, Alvaro N; Andriolo, Régis B; Cruz, Oswaldo L; Pignatari, Shirley N

    2012-02-15

    Congenital choanal atresia is a rare abnormality characterized by unilateral or bilateral lack of patency of the posterior end of the nasal cavity. With an incidence of 1:5000 to 1:8000 births, it is twice as prevalent in females as it is in males. Surgical procedures aim to provide adequate functional choanal patency and a low rate of restenosis, avoid harm to any structure in development, enable shorter surgery and hospitalization times, and minimize morbidity and mortality. To evaluate the effectiveness and safety of the available surgical techniques for the treatment of congenital choanal atresia in patients with unilateral and bilateral atresia. We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ISRCTN and additional sources for published and unpublished trials. The date of the search was 31 January 2011. We planned to include parallel randomized or quasi-randomized controlled trials testing surgical approaches for the treatment of congenital atresia (irrespective of gender and age) that evaluated normal/adequate respiratory function (self reported or preserved nasal airway) and restenosis as the main primary outcomes. We did not consider reoperation and non-congenital atresia (e.g. traumatic, iatrogenic atresias) for inclusion. Three review authors independently assessed the titles and abstracts of the identified articles to determine potential relevance. For dichotomous and continuous variables, we planned to calculate risk ratios (relative risks; RR) and mean differences (MD) with 95% confidence intervals (CI), respectively. We planned to use the random-effects model since we were expecting substantial clinical and methodological heterogeneity. No randomized controlled trials were identified. From the 120 reports retrieved using our search strategy, 46 primary studies had the

  8. Hepatobiliary scan in neonatal Jaundice

    International Nuclear Information System (INIS)

    Nahar, Nurun; Hasan, Mizanul; Karim, M.A.

    2002-01-01

    Jaundice is more or less common in newborn babies. Through physiological jaundice is most common cause of neonatal jaundice, possibility of obstructive jaundice especially biliary atresia should be kept in mind. Early diagnosis of biliary atresia followed by surgical treatment can save baby's life. Otherwise death is inevitable due to liver failure. Hepatobiliary scan is the imaging study of choice in neonatal jaundice especially when there is persistent conjugated hyperbilirubinaemia. Total 27 newborn babies of suspected biliary atresia, aged 14 days to 4 months were referred to Institute of Nuclear Medicine for Hepatobiliary scan. All of them had high serum bilirubin ranged from 6.0 mg/dl with an average of 9.35 ng/dl serum bilirubin level. Ultrasonography of hepatobiliary system was performed in 14 cases showing normal sized liver in 4 cases and hepatomegaly in 10 cases. Hepatobiliary scan was done with 99m Tc-Mebrofenin (Br IDA) after preparing the baby with phenobarbitone for 3-5 days. 20 (67%) cases were scan positive suggesting biliary atresia (BA) and 7(27%) cases were scan negative. In BA there will be increased hepatic uptake of the radionuclide without any significant excretion even in 24 hours delayed images. Presence of radiotracer in the bowel exclude the diagnosis of BA. Early diagnosis of biliary atresia is very important because in this condition surgery should be performed early (within 60 days of life). Studies suggest that hepatobiliary scan after hepatic stimulation with phenobarbitone for a period of 3-5 days is highly accurate for differentiating biliary atresia from other causes of neonatal jaundice. It is very important to perform hepatobiliary scan in a case of neonatal jaundice to exclude biliary atresia for the sake of baby's life.(author)

  9. A blinded prospective study comparing four current noninvasive approaches in the differential diagnosis of medical versus surgical jaundice

    International Nuclear Information System (INIS)

    O'Connor, K.W.; Snodgrass, P.J.; Swonder, J.E.; Mahoney, S.; Burt, R.; Cockerill, E.M.; Lumeng, L.

    1983-01-01

    A prospective study was undertaken to compare the diagnostic accuracy of clinical evaluation, ultrasound, computed tomography, and technetium 99m-HIDA or -PIPIDA biliary scans in distinguishing between intrahepatic and extrahepatic jaundice. A final diagnosis was established in each of the 50 patients who completed the study, among whom 29 had intrahepatic cholestasis and 21 had extrahepatic obstruction. In the diagnosis of extrahepatic obstruction, the sensitivities of clinical evaluation, ultrasound, computed tomography, and nuclear medicine biliary scan were 95%, 55%, 63%, and 41%, respectively; the specificities were 76%, 93%, 93%, and 88%; and the overall accuracies were 84%, 78%, 81%, and 68%. These data support the conclusion that when the clinical evaluation is carefully performed, it is the single most effective noninvasive means of detecting extrahepatic biliary obstruction in a jaundiced patient. Although ultrasound, computed tomography, and radionuclide biliary scan are less sensitive, they are highly reliable if they indicate that extrahepatic obstruction is present. A flow chart of invasive and noninvasive approaches for evaluation of the jaundiced patient is presented

  10. A Case of Adenomyomatous Hyperplasia of the Extrahepatic Bile Duct

    Directory of Open Access Journals (Sweden)

    Masakatsu Numata

    2011-08-01

    Full Text Available Adenomyomatous hyperplasia is rarely found in the extrahepatic bile duct. A 54-year-old man was referred to our center with a diagnosis of extrahepatic bile duct stenosis which had been detected by endoscopic retrograde choloangiopancreatography. Abdominal computed tomography revealed thickening of the wall of the middle extrahepatic bile duct, however no malignant cells were detected by cytology. Since bile duct carcinoma could not be ruled out, we performed resection of the extrahepatic duct accompanied by lymph node dissection. Histopathologically, the lesion was diagnosed as adenomyomatous hyperplasia of the extrahepatic bile duct. Present and previously reported cases showed the difficulty of making a diagnosis of adenomyomatous hyperplasia of the extrahepatic bile duct preoperatively or intraoperatively. Therefore, when adenomyomatous hyperplasia is suspected, a radical surgical procedure according to malignant disease may be necessary for definitive diagnosis.

  11. Percutaneous Intraductal Radiofrequency Ablation for Extrahepatic Distal Cholangiocarcinoma: A Method for Prolonging Stent Patency and Achieving Better Functional Status and Quality of Life

    International Nuclear Information System (INIS)

    Wu, Tian-tian; Li, Wei-min; Li, Hu-cheng; Ao, Guo-kun; Zheng, Fang; Lin, Hu

    2017-01-01

    PurposeThe clinical efficacy of intraductal radiofrequency ablation (RFA) with Habib™ EndoHPB catheter, a newly developed intervention for malignant extrahepatic biliary obstruction, remains uncertain. The aim of this study was to investigate the clinical efficacy of intraductal RFA.MethodsData from 71 patients with extrahepatic distal cholangiocarcinoma were retrospectively analyzed. The study patients were divided into RFA and control groups. The RFA group had undergone percutaneous transhepatic intraductal RFA with a Habib™ EndoHPB catheter, followed by placement of covered or uncovered biliary self-expandable metallic stents (SEMs) whereas the control group had undergone percutaneous transhepatic covered or uncovered SEMs placement. Procedure-related complications, stent patency, patient survival, and postoperative serum bilirubin concentrations were compared between the two groups. The Functional Assessment of Cancer Therapy-Hepatobiliary (FACT-Hep) questionnaire was administered to evaluate functional status, improvement in clinical manifestations, and quality of life.ResultsThe RFA group had a longer median stent patency than the control group (p = 0.001 for uncovered SEMs placement). Higher functional well-being, hepatobiliary-specific cancer subscale, Trial Outcome Index, and total FACT-Hep scores were observed during post-procedure follow-up in the RFA group. However, median survival did not differ significantly between the two groups (p > 0.05).ConclusionsProlongation of stent patency and better functional status and quality of life, which are all important clinical endpoints, were observed in patients treated with intraductal RFA. Prospective randomized controlled clinical trials are necessary to further investigate the clinical efficacy and long-term benefits of intraductal RFA.

  12. Percutaneous Intraductal Radiofrequency Ablation for Extrahepatic Distal Cholangiocarcinoma: A Method for Prolonging Stent Patency and Achieving Better Functional Status and Quality of Life

    Energy Technology Data Exchange (ETDEWEB)

    Wu, Tian-tian, E-mail: matthewwu1979@hotmail.com; Li, Wei-min, E-mail: weimin-li-surgery@126.com [The 309th Hospital of PLA, Hepatobiliary Surgery Department (China); Li, Hu-cheng, E-mail: hucheng-li-surgery@126.com [The 307th Hospital of PLA, General Surgery Department (China); Ao, Guo-kun, E-mail: guokun-ao-radiology@126.com [The 309th Hospital of PLA, Radiology Department (China); Zheng, Fang, E-mail: fang-zheng-surgery@126.com [The 309th Hospital of PLA, Hepatobiliary Surgery Department (China); Lin, Hu, E-mail: hu-lin-radiology@126.com [The 309th Hospital of PLA, Radiology Department (China)

    2017-02-15

    PurposeThe clinical efficacy of intraductal radiofrequency ablation (RFA) with Habib™ EndoHPB catheter, a newly developed intervention for malignant extrahepatic biliary obstruction, remains uncertain. The aim of this study was to investigate the clinical efficacy of intraductal RFA.MethodsData from 71 patients with extrahepatic distal cholangiocarcinoma were retrospectively analyzed. The study patients were divided into RFA and control groups. The RFA group had undergone percutaneous transhepatic intraductal RFA with a Habib™ EndoHPB catheter, followed by placement of covered or uncovered biliary self-expandable metallic stents (SEMs) whereas the control group had undergone percutaneous transhepatic covered or uncovered SEMs placement. Procedure-related complications, stent patency, patient survival, and postoperative serum bilirubin concentrations were compared between the two groups. The Functional Assessment of Cancer Therapy-Hepatobiliary (FACT-Hep) questionnaire was administered to evaluate functional status, improvement in clinical manifestations, and quality of life.ResultsThe RFA group had a longer median stent patency than the control group (p = 0.001 for uncovered SEMs placement). Higher functional well-being, hepatobiliary-specific cancer subscale, Trial Outcome Index, and total FACT-Hep scores were observed during post-procedure follow-up in the RFA group. However, median survival did not differ significantly between the two groups (p > 0.05).ConclusionsProlongation of stent patency and better functional status and quality of life, which are all important clinical endpoints, were observed in patients treated with intraductal RFA. Prospective randomized controlled clinical trials are necessary to further investigate the clinical efficacy and long-term benefits of intraductal RFA.

  13. The correlation between the dilated extent of bile duct and gallbladder and low bile duct obstructive jaundice diseases

    International Nuclear Information System (INIS)

    Wang Zhongqiu; Lu Guangming; Li Jieshou; Li Weiqin

    2005-01-01

    Objective: To evaluate the diagnostic value about the dilated extent of bile duct and gallbladder in low biliary obstructive diseases. Methods: CT and ERCP findings of 105 patients with low biliary obstructive disease were retrospectively analyzed. The dilated extent of intrahepatic and extra- hepatic bile duct and gallbladder were classified into seven types: Type I: severe dilatation of intrahepatic and extrahepatic bile duct and gallbladder; Type II: severe dilatation of extrahepatic bile duct and gallbladder and slight dilated intrahapetic bile duct; Type III: severe dilatation of intrahepatic and extrahepatic bile duct without or slight dilatation of gallbladder; Type IV: severe extrahepatic bile duct dilatation without or slight dilatation of intrahepatic bile duct and gallbladder; Type V: severe intrahepatic bile duct dilatation without or with slight dilatation of extrahepatic bile duct and gallbladder; Type VI: severe gallbladder dilatation without or with slight intrahepatic and extra- hepatic bile duct dilatation; Type VII: without or with slight dilatation of intrahepatic and extrahepatic bile duct and gallbladder. The biliary system dilated extent of low biliary obstructive disease on CT and ERCP were compared with results of clinical, operation, and pathology. Results: Thirty-three cases of tumor and 72 cases of non-tumor were proved by clinical and operation in 105 patients with low biliary obstructive disease. In 33 tumor patients, 16 patients were identified as Type I, 10 patients Type II, 4 patients Type III, 1 patient Type IV, 2 patients Type VII. In 72 non-tumor patients, 4 patients were identified as Type I, 4 patients Type II, 9 patients Type III, 33 patients Type IV, 2 patients Type V, 11 patients Type VI, 19 patients Type VII. A large difference between I, II type and III-VII type biliary dilatation existed in tumor and non-tumor group (χ 2 =47.33, P<0.01). Conclusion:Low obstructive biliary diseases are closely correlated with the dilated

  14. Differential diagnosis of persistent neonatal jaundice: Role of sonography and scintigraphy

    International Nuclear Information System (INIS)

    Lee, Sun Wha; Ko, Young Tae; Lim, Jae Hoon

    1993-01-01

    The most common causes of conjugated hyperbilirubinaemia after the first or second week of life are neonatal hepatitis and biliary atresia. Since these entities represent variable expressions of same pathologic process and have similar clinical, biochemical, and histologic features, differential diagnosis is extremely difficult. We prospectively studies 28 jaundiced infants by sonography and hepatobiliary scintigraphy. Final diagnoses included 12 biliary atresia and 16 neonatal hepatitis. Visualization of a normal sized common bile duct or gallbladder was compatible with the diagnosis of neonatal hepatitis. Non-visualized or atrophic gallbladder on sonography coupled with non-visualization of bowel activity on scintigraphy was highly suggestive of biliary atresia. We believe that sonography plays a valuable role in the initial evaluation of the infants with persistent neonatal jaundice. The combined use of sonography and hepatobiliary scintigraphy provides the most valuable information in suspected biliary atresia for prompt surgical treatment

  15. Biliary Atresia

    Science.gov (United States)

    ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ...

  16. Pulmonary atresia

    Science.gov (United States)

    ... another type of congenital heart defect called a patent ductus arteriosus (PDA). Pulmonary atresia may occur with ... known way to prevent this condition. All pregnant women should get routine prenatal care. Many congenital defects ...

  17. Interactions between Th1 cells and Tregs affect regulation of hepatic fibrosis in biliary atresia through the IFN-γ/STAT1 pathway.

    Science.gov (United States)

    Wen, Jie; Zhou, Ying; Wang, Jun; Chen, Jie; Yan, Wenbo; Wu, Jin; Yan, Junkai; Zhou, Kejun; Xiao, Yongtao; Wang, Yang; Xia, Qiang; Cai, Wei

    2017-06-01

    Regulatory T cells (Tregs) and CD4 + T helper (Th) cells have important roles in bile duct injury of biliary atresia (BA). However, their impacts on liver fibrosis are undefined. Between 2013 and 2016, 146 patients with various stages of BA were enrolled in this study. Peripheral blood, liver biopsy and lymph node samples were collected. Flow cytometry, magnetic cell sorting and immunostaining were used to characterize lymphocytes from BA patients. Deficiency of Tregs was observed along with increased Th1, Th2 and Th17 frequencies in the peripheral blood and livers of BA patients. The levels of peripheral and intrahepatic Th1 cells positively correlated with the stage of liver fibrosis. Furthermore, Th1 cells were located in close proximity to activated hepatic stellate cells (HSCs) and areas of fibrosis in BA livers. In culture, Th1 cells accelerated the proliferation and secretion of profibrogenic markers of HSCs through the IFN-γ/STAT1 pathway. Of note, Tregs blocked the Th1-stimulated effects on HSCs by inhibiting Th1-induced activation of STAT1. Consistent with the results of in vitro study, intrahepatic IFN-γ/STAT1 levels increased in relation to the severity of liver fibrosis in BA patients, and the altered balance between MMP2 and TIMP1 expressions in livers may contribute to increased deposition of extracellular matrix and fibrosis. Finally, to identify the effects of Th1 cells on Tregs, we demonstrated that Th1 cells upregulated the proportion of aTreg cells by secreting IFN-γ cytokine. Thus, aberrant Th1 immune responses in BA promote the proliferation and secretion of HSCs through the IFN-γ/STAT1 pathway. The regulation of HSCs by the interactions between Tregs and Th1 cells might be part of the mechanism underlying progressive liver fibrosis and may be a suitable target for therapy.

  18. Chronic Extrahepatic Bile Duct Dilatation: Sonographic Screening in the Patients with Opioid Addiction

    Energy Technology Data Exchange (ETDEWEB)

    Farahmand, H.; PourGholami, M.; Fathollah, Sheikh [Rafsanjan University of Medical Sciences, Rafsanjan (Iran, Islamic Republic of)

    2007-06-15

    One of the best known side effects of using opium is spasm of the sphincter of Oddi, which may increase the diameter of the extrahepatic bile ducts. Ultrasound is the first imaging modality used for evaluating the biliary system because it is commonly available and noninvasive. The principal objective of this study was to measure the common bile duct (CBD) diameter via ultrasonography in opium addicts and to evaluate the relation between the CBD diameter and the period of addiction. This research was an analytical-cross sectional study that was done on 110 opium addicts that were admitted to a drug treatment center. The diameter of the CBD in these cases was measured by ultrasonography and the results were analyzed with other factors like age, the period of addiction and the laboratory findings. According to the findings, there is a significant increase in the range of the CBD diameter in comparison with normal bile ducts. Also, the mean diameter of the CBD in the different age groups showed a significant difference (p < 0.0001) and there was a significant relation between the CBD diameter and the period of addiction (p < 0.001, r = 0.74); so, with the increased length of the addiction period, the mean CBD diameter increases. Opium addiction is one of the factors that causes extrahepatic bile duct dilatation, so in these cases, if no obstructing lesion was found on ultrasound examination and the serum bilirobine and alkaline phosphatase levels are normal, then further evaluation is not needed.

  19. Chronic Extrahepatic Bile Duct Dilatation: Sonographic Screening in the Patients with Opioid Addiction

    International Nuclear Information System (INIS)

    Farahmand, H.; PourGholami, M.; Fathollah, Sheikh

    2007-01-01

    One of the best known side effects of using opium is spasm of the sphincter of Oddi, which may increase the diameter of the extrahepatic bile ducts. Ultrasound is the first imaging modality used for evaluating the biliary system because it is commonly available and noninvasive. The principal objective of this study was to measure the common bile duct (CBD) diameter via ultrasonography in opium addicts and to evaluate the relation between the CBD diameter and the period of addiction. This research was an analytical-cross sectional study that was done on 110 opium addicts that were admitted to a drug treatment center. The diameter of the CBD in these cases was measured by ultrasonography and the results were analyzed with other factors like age, the period of addiction and the laboratory findings. According to the findings, there is a significant increase in the range of the CBD diameter in comparison with normal bile ducts. Also, the mean diameter of the CBD in the different age groups showed a significant difference (p < 0.0001) and there was a significant relation between the CBD diameter and the period of addiction (p < 0.001, r = 0.74); so, with the increased length of the addiction period, the mean CBD diameter increases. Opium addiction is one of the factors that causes extrahepatic bile duct dilatation, so in these cases, if no obstructing lesion was found on ultrasound examination and the serum bilirobine and alkaline phosphatase levels are normal, then further evaluation is not needed

  20. Biliary drainage by teflon endoprosthesis in obstructive jaundice - experiences in 69 patients treated by PTCD or ERCD

    Energy Technology Data Exchange (ETDEWEB)

    Rupp, N; Kramann, B; Gullotta, U; Reiser, M

    1983-02-01

    In 69 patients with extrahepatic biliary obstruction a specially designed teflon tube, the endoprosthesis, was inserted across the ductal stenosis either by percutaneous or by endoscopic route to reduce jaundice. After gaining experience our success rate of stent placement was more than 90%. Compared with catheter drainage the endoprosthesis worked faster and more efficiently, while in palliative treatment the quality of life of the patient improved and secondary cholangitis was prevented. The rate of significant long-term stent obstruction can be tolerated in view of the expected life span of four months as an average in our material.

  1. Pyloric atresia: a challenge in an underdeveloped country | Sagna ...

    African Journals Online (AJOL)

    Pyloric atresia is a rare congenital malformation. We report a case in a 5-day newborn with pyloric atresia type C. Authors emphasize the diagnostic difficulties and therapeutic challenges in a resource-limited country. Keywords: Pyloric atresia, newborn, rare congenital malformation ...

  2. Synchronous double primary cancers of the extrahepatic bile duct: A case report and literature review.

    Science.gov (United States)

    Nishi, Takeshi; Sato, Yoshitoshi; Hanaoka, Takuya; Takahashi, Takuya; Miura, Hiroshi; Takubo, Kenji

    2018-01-01

    Double cancers of the biliary tract system are rare. Most of these cancers are synchronous double cancers of the gall bladder and bile duct, associated with pancreaticobiliary maljunction (PBM). Synchronous double cancers of the extrahepatic bile duct without PBM are especially rare, and only 4 cases have been reported. A 78-year-old woman was admitted to our hospital for examination of hyperbilirubinemia and liver dysfunction. Contrast-enhanced abdominal computed tomography, Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography revealed 2 stenotic regions in the common bile duct: at its junction with the cystic duct and in the distal bile duct. No findings suggested PBM, such as a markedly long common channel. The diagnosis based on endoscopic brush cytology from both stricture portions was adenocarcinoma. The patient had a pylorus-preserving pancreaticoduodenectomy with regional lymph node resection. Macroscopically, there were 2 stenotic regions at the cystic duct junction and in the distal bile duct. Microscopically, the tumor at the junction of the cystic duct was a well-to-moderately differentiated adenocarcinoma. On the other hand, the tumor of the distal bile duct was a poorly differentiated adenocarcinoma. There was no evidence of communication between these 2 cancers. Double cancers of the extrahepatic bile duct without PBM are very rare. Therefore, an accurate diagnosis prior to surgery is necessary. Furthermore, this rare condition seems to be associated with a poor prognosis. Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.

  3. Extrahepatic portal hypertension in childhood

    International Nuclear Information System (INIS)

    Takehara, Hiroo; Komi, Nobuhiko; Goh, Masahiro; Yoshida, Sadahiro; Kameoka, Kazuhiro; Hino, Masao; Sui, Osamu

    1986-01-01

    Four pediatric patients with extrahepatic portal hypertension had undergone portography and computerized radionuclide angiography in order to examine shape of portal system and to evaluate hepatic blood flow before and aftersurgical treatment. In all patients, cavernous transformation of portal system was demonstrated by portography, and in one of them spontaneous splenorenal shunt occurred. In 3 of them, who underwent esophageal transsection combined with paraesophagogastric devascularization and splenectomy, so-called direct operation, increase of portal blood flow was revealed by computerized radionuclide angiography. It is suggested that direct operation increasing portal blood flow after surgery is effective in treating extrahepatic portal hypertension in childhood. (author)

  4. Wind sock deformity in rectal atresia

    International Nuclear Information System (INIS)

    Hosseini, Seyed M V; Ghahramani, Farhad; Shamsaeefar, Alireza; Razmi, Tannaz; Zarenezhad, Mohammad

    2009-01-01

    Rectal atresia is a rare anorectal deformity. It usually presents with neonatal obstruction and it is often a complete membrane or severe stenosis. Windsock deformity has not been reported in rectal atresia especially, having been missed for 2 years. A 2-year-old girl reported only a severe constipation despite having a 1.5-cm anal canal in rectal examination with scanty discharge. She underwent loop colostomy and loopogram, which showed a wind sock deformity of rectum with mega colon. The patient underwent abdominoperineal pull-through with good result and follow-up. This is the first case of the wind sock deformity in rectal atresia being reported after 2 years of age. (author)

  5. Influence of glutamine on morphological and functional changes of liver in the presence of extrahepatic biliary obstruction in rats Influência da glutamina em alterações funcionais e morfológicas do fígado na vigência de obstrução biliar extra-hepática em ratos

    Directory of Open Access Journals (Sweden)

    Manoel Camelo de Paiva Neto

    2010-08-01

    Full Text Available PURPOSE: To study the influence of glutamine on functional and morphological changes of liver in the extrahepatic biliary obstruction through an experimental model in rats. METHODS: Seventy Wistar rats were divided into four groups: control (group C fictitious operation, (group FO, submitted to laparotomy with handling of bile ducts, but without hepatic duct ligation, (group EBO submitted to laparotomy and hepatic duct ligation, one of them submitted to supplementation with glutamine 2% (group G. The control group consisted of 6 animals. The animals from groups FO, EBO and G were divided into three groups consisting of 6 animals each, being sacrificed at 7, 14 and 21 days after operation, respectively. Blood samples were collected for biochemical analysis and a fragment of liver tissue was collected from the middle lobe for histological analysis. RESULTS: Both for biochemical analysis (BT, aspartate and alanine aminotransferase AST, ALT and alkaline phosphatase FAL and for histopathological changes (fibrosis, portal inflammation, parenchymal inflammation, hepatocytic changes and duct proliferation, no statistical difference between groups submitted to extrahepatic biliary obstruction (EBO with and without treatment with glutamine (G was observed. CONCLUSION: Glutamine supplementation did not alter the prognosis of liver enzymes and histopathological changes in animals submitted to extrahepatic biliary obstruction.OBJETIVO: Estudar a influência da glutamina em alterações funcionais e morfológicas do fígado na obstrução biliar extra-hepática por meio de um modelo experimental desenvolvido em ratos. MÉTODOS: Setenta ratos Wistar distribuídos em quatro grupos: controle (grupo C; operação fictícia (grupo OF, submetido à laparotomia com manuseio das vias biliares, mas sem ligadura do ducto hepático; (grupo OBE, submetido à laparotomia exploradora e ligadura do ducto hepático, sendo um deles submetido à suplementação com glutamina a

  6. Role of 99mTc-mebrofenin in evaluation of neonatal cholestasis syndrome

    International Nuclear Information System (INIS)

    Maini, Atul; Khanduri, Arun; Gambhir, S.; Yacha, S.K.; Das, B.K.

    1997-01-01

    We prospectively evaluated 40 patients with neonatal cholestasis syndrome (NCS) over a period of 2.5 years. Main aim was to evaluate the efficacy of hepatobiliary scintigraphy using 99m Tc-mebrofenin, without phenobarbital induction, in differentiating Extrahepatic Biliary Atresia (EHBA) from Neonatal Hepatitis (NH). All infants were clinically examined. Liver function tests, ultrasonography and liver biopsy were carried out in each case. EHBA was diagnosed either by per-operative cholangiography or scintigraphy and liver biopsy. NH was diagnosed by liver histology and ultrasonography or by scintigraphy or by per operative cholangiography. Our sensitivity (100%) and specificity (80%) were found to be similar to results of previous studies using other 99m Tc-IDA agents with phenobarbital induction. Therefore, with 99m Tc-mebrofenin phenobarbital induction may not be needed in differentiating EHBA from NH, thus decreasing the time of diagnostic evaluation. (author)

  7. Estudo anatômico das vias bilíferas extra-hepáticas no suíno. Comparação entre a dissecção convencional e por videolaparoscopia Anatomic study of the extrahepatic biliary ducts in the suine. Comparition betwenn conventional and videolaparascopic dissections

    Directory of Open Access Journals (Sweden)

    José Renan Escalante Hurtado

    1997-09-01

    Full Text Available Estudou-se no presente trabalho, através de dissecção de peças anatômicas constituidas de fígado e duodeno, a morfologia das vias bilíferas do suíno. Os animais foram separados em dois grupos iguais, sendo que o primeiro foi submetido a colecistectomia videolaparoscópica. O segundo grupo era constituído de suínos abatidos para consumo. Os animais do grupo da colecistectomia videolaparoscópica eram observados por um período curto de pós-operatório e depois abatidos para retirada de peças anatômicas, tendo sido realizado o estudo das vias bilíferas extra-hepáticas. No segundo grupo eram as peças retiradas após o abate dos animais, estas eram dissecadas, coradas com tinta guache e posteriormente submetidas a estudo radiológico com a injeção de Bário. Os dados obtidos foram analizados utilizando-se o teste de FISHER. Verificou-se que o ductus choledocus é constituído pela união do ductus cysticus e ductus hepaticus na grande maioria dos casos (84.3%. Verificou-se também que o ductus hepaticus é formado, em grande número dos casos, pela confluência do ductus principalis sinister e ductus principalis dexter. Os resultados encontrados permitem concluir que não existem diferenças significantes, no que se refere à conformação das vias bilíferas extra-hepáticas em ambos os grupos.The morphology of the biliary extrahepatic ducts of 32 Landrace pigs were studied through the dissection of anatomical specimens composed by liver and duodenum. The pigs were separated in two groups of 16 animals each. The first group was initially submited to a laparoscopic cholecystectomy, than observed during a brief postoperative period, and eventually killed for attainment of the anatomical specimens; while the second group was destined to slaughter and the anatomical specimens obtained after their death. The study of the anatomical specimens was performed by the dissection of the bilary extrahepatic ducts when they were painted with

  8. Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice

    International Nuclear Information System (INIS)

    Johnson, K.; Alton, H.M.; Chapman, S.

    1998-01-01

    Background. The prognosis of infants with prolonged neonatal jaundice is dependent on early diagnosis because of the need for prompt surgical management of biliary atresia. Objective. To evaluate the usefulness of 99 m Tc m -trimethylbromo-iminodiacetic acid (TBIDA, mebrofenin) in the investigation of infantile jaundice. Materials and methods. A retrospective study was undertaken of 58 patients with unexplained prolonged neonatal jaundice. Sixty-eight scans were reviewed. Results. Mebrofenin scintigraphy confirmed the presence of a choledochal cyst in three of the four cases with that diagnosis. There were no false negative results in the nine patients with extrahepatic biliary atresia (EHBA). Three further infants had an incorrect histological diagnosis of EHBA. A gall bladder was identified by US in each case and in one of these, scintigraphy showed gut excretion. In the 16 patients with no gut excretion by 24 h, the final diagnoses were intrahepatic cholestasis (n = 7), Alagille's syndrome (n = 3), neonatal hepatitis (n = 3), alpha-1-antitrypsin deficiency (n = 2) and juvenile xanthogranuloma (n = 1). Seven infants had repeat scintigraphy after the administration of ursodeoxycholic acid (URSO). This changed five non-excretors with hepatitis into excretors. Two infants with hepatitis continued to show non-excretion after URSO, but a gallbladder was identified by US in both. Conclusions. Mebrofenin scintigraphy is accurate in confirming the presence of a choledochal cyst and in refuting the diagnosis of EHBA. While histology and scintigraphy are each 100 % sensitive for the diagnosis of EHBA, neither, individually, is accurate and the investigation of prolonged neonatal jaundice requires a multi-modality imaging strategy. (orig.)

  9. Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence

    DEFF Research Database (Denmark)

    Bjørsum-Meyer, Thomas; Herlin, Morten; Qvist, Niels

    2016-01-01

    Background: The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac...... defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases. Furthermore, we aimed to collect existing knowledge...... in the embryopathogenesis and genetics in order to discuss a possible link between the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome. Case presentation: Our first case was a white girl...

  10. Radiation therapy in extrahepatic bile duct carcinoma

    International Nuclear Information System (INIS)

    Mahe, Marc; Romestaing, Pascale; Talon, Bernard; Ardiet, J.M.; Salerno, Nathalie; Sentenac, Irenee; Gerard, J.P.

    1991-01-01

    Fifty-one patients with carcinoma of the extrahepatic bile ducts (EHBD) received radiation therapy between Jan 1980-Dec 1988. The location of the tumors was: proximal third, 20 patients; middle third, 23; distal third, 3; diffuse, 5 patients. Thirty-six patients underwent surgery with complete gross resection in 14 (10/14 with positive margins), incomplete gross resection in 12 and only biopsy in 10. Fifteen patients had only biliary drainage without laparotomy after cytologic diagnosis of malignancy in 11/15. Radiation therapy was done with curative intent after complete or incomplete resection (n=26) and it was palliative in patients who had no resection or only biliary drainage (n=25). Twenty-five patients received external radiation-therapy (ERT) alone to the tumor and lymph nodes (mean dose 45 Gy/2Gy per fraction for cure, 35 Gy/10 fractions for palliation), 8 patients had only iridium-192 ( 192 Ir) implant (50-60 Gy at a 1 cm radius for cure, 30 Gy for palliation), 17 patients had both ERT + 192 Ir(ERT 42.5 Gy + 192 Ir 10-15 Gy for cure; ERT 20 Gy/5 fractions + 192 Ir 20-30 Gy for palliation) and one intra-operative irradiation + ERT. The overall survival rate for the entire group was 55, 28.5 and 15 percent at 12, 24, 36 months and median survival 12 months. Median survival was 22 months in patients treated with curative intent and only 10 months after palliative treatment (p0.03). Among patients who had curative treatment, median survival was 27.5 months after complete gross resection and 13 months after incomplete gross resection (p0.045). After complete gross resection 5/14 patients were alive without evolutive disease at 11, 19, 20, 23 and 41 months, 2 were alive with metastases at 25 and 27 months and 7/14 died of cancer from 7 to 59 months. The rate of complications was low: 3 cholangitis responsive to antibiotics, 1 hemobilia and 2 gastric ulcers. These results are encouraging especially for patients with complete gross resection but they must be

  11. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

    Directory of Open Access Journals (Sweden)

    Go Miyano

    2015-01-01

    Full Text Available We describe herein a case of unilateral pulmonary agenesis (PA with oesophageal atresia (EA/tracheoesophageal fistula (TEF that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  12. Trans-anastomotic porto-portal varices in patients with gastrointestinal haemorrhage

    International Nuclear Information System (INIS)

    Mitchell, A.W.M.; Jackson, J.E.

    2000-01-01

    AIM: Porto-portal varices are commonly seen in patients with segmental extra-hepatic portal hypertension and develop to provide a collateral circulation around an area of portal venous obstruction. It is not well recognized that such communications may also develop across surgical anastomoses and be the source of gastrointestinal haemorrhage. The possible mode of development of such communications has not been previously discussed. MATERIALS AND METHODS: Over a 3-year period between 1995 and 1998, porto-portal varices were demonstrated across surgical anastomoses in four patients who were referred for the investigation of acute (two), acute-on-chronic (one) and chronic gastrointestinal bleeding (one). Their medical notes and the findings at angiography were reviewed. RESULTS: Three patients had segmental portal hypertension due to extra-hepatic portal vein (one) or superior mesenteric vein (two) stenosis/occlusion. One patient had mild portal hypertension due to hepatic fibrosis secondary to congenital biliary atresia. At angiography all patients were shown to have varices crossing previous surgical anastomoses. These varices were presumed to be the cause of bleeding in three of the four patients; the site of bleeding in the fourth individual was not determined. CONCLUSIONS: Trans-anastomotic porto-portal varices are rare. They develop in the presence of extra-hepatic portal hypertension and presumably arise within peri-anastomotic inflammatory tissue. Such varices may be difficult to manage and their prognosis is poor when bleeding occurs. Mitchell, A.W.M., Jackson, J.E. (2000)

  13. Prenatal diagnosis of horseshoe lung and esophageal atresia

    International Nuclear Information System (INIS)

    Goldberg, Shlomit; Ringertz, Hans; Barth, Richard A.

    2006-01-01

    We present a case of horseshoe lung (HL) and esophageal atresia suspected prenatally on US imaging and confirmed with fetal MRI. Prenatal diagnosis of HL and esophageal atresia allowed for prenatal counseling and informed parental decisions. (orig.)

  14. Prenatal diagnosis of horseshoe lung and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Goldberg, Shlomit; Ringertz, Hans [Stanford University School of Medicine, Radiology Department, Stanford, CA (United States); Barth, Richard A. [Stanford University School of Medicine, Radiology Department, Stanford, CA (United States); Lucile Packard Children' s Hospital, Radiology, Palo Alto, CA (United States)

    2006-09-15

    We present a case of horseshoe lung (HL) and esophageal atresia suspected prenatally on US imaging and confirmed with fetal MRI. Prenatal diagnosis of HL and esophageal atresia allowed for prenatal counseling and informed parental decisions. (orig.)

  15. Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct

    Directory of Open Access Journals (Sweden)

    John Wysocki

    2014-01-01

    Full Text Available Mixed adenoneuroendocrine carcinomas, spindle cell carcinomas, and clear cell carcinomas are all rare tumors in the biliary tract. We present the first case, to our knowledge, of an extrahepatic bile duct carcinoma composed of all three types. A 65-year-old man with prior cholecystectomy presented with painless jaundice, vomiting, and weight loss. CA19-9 and alpha-fetoprotein (AFP were elevated. Cholangioscopy revealed a friable mass extending from the middle of the common bile duct to the common hepatic duct. A bile duct excision was performed. Gross examination revealed a 3.6 cm intraluminal polypoid tumor. Microscopically, the tumor had foci of conventional adenocarcinoma (CK7-positive and CA19-9-postive surrounded by malignant-appearing spindle cells that were positive for cytokeratins and vimentin. Additionally, there were separate areas of large cell neuroendocrine carcinoma (LCNEC. Foci of clear cell carcinoma merged into both the LCNEC and the adenocarcinoma. Tumor invaded through the bile duct wall with extensive perineural and vascular invasion. Circumferential margins were positive. The patient’s poor performance status precluded adjuvant therapy and he died with recurrent and metastatic disease 5 months after surgery. This is consistent with the reported poor survival rates of biliary mixed adenoneuroendocrine carcinomas.

  16. Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

    International Nuclear Information System (INIS)

    Githu, Tangayi; Merrow, Arnold C.; Lee, Jason K.; Garrison, Aaron P.; Brown, Rebeccah L.

    2014-01-01

    Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination. (orig.)

  17. Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation

    Energy Technology Data Exchange (ETDEWEB)

    Githu, Tangayi [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Radiology of Huntsville, P.C., Huntsville, AL (United States); Merrow, Arnold C.; Lee, Jason K. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Garrison, Aaron P. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States); Akron Children' s Hospital, Pediatric Surgery, Akron, OH (United States); Brown, Rebeccah L. [Cincinnati Children' s Hospital Medical Center, Department of Surgical Services, Division of Pediatric General and Thoracic Surgery, Cincinnati, OH (United States)

    2014-03-15

    Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination. (orig.)

  18. Percutaneous biliary drainage in acute suppurative cholangitis with biliary sepsis

    International Nuclear Information System (INIS)

    Kim, Hyung Lyul; Cho, June Sik; Kwon, Soon Tae; Lee, Sang Jin; Rhee, Byung Chull

    1993-01-01

    Acute suppurative cholangitis is a severe inflammatory process of the bile duct occurred as result of partial or complete obstruction of the bile duct, and may manifest clinically severe form of disease, rapidly deteriorating to life-threatening condition. We analyzed emergency percutaneous transhepatic biliary drainage in 20 patients of acute suppurative cholangitis with biliary sepsis to evaluate the therapeutic effect and complication of the procedure. The underlying cause were 12 benign disease(stones) and eight malignant tumors and among eight malignant tumors, bile duct stones(n=4) and clonorchiasis(n=1) were combined. Percutaneous transhepatic biliary drainage was performed successfully in 17 of 20 patients resulting in improvement of general condition and failed in three patients. The procedure were preterminated due to the patient's condition in two and biliary-proto fistula was developed in one. After biliary decompression by percutaneous transhepatic biliary drainage, effective and successful elective surgery was performed in nine cases, which were seven biliary stones and two biliary cancer with stones. Our experience suggest that emergency percutaneous transhepatic biliary drainage is an initial and effective treatment of choice for acute suppurative cholangitis with sepsis and a safe alternative for nonsurgical treatment

  19. Profile of preoperative fecal organic acids closely predicts the incidence of postoperative infectious complications after major hepatectomy with extrahepatic bile duct resection: Importance of fecal acetic acid plus butyric acid minus lactic acid gap.

    Science.gov (United States)

    Yokoyama, Yukihiro; Mizuno, Takashi; Sugawara, Gen; Asahara, Takashi; Nomoto, Koji; Igami, Tsuyoshi; Ebata, Tomoki; Nagino, Masato

    2017-10-01

    To investigate the association between preoperative fecal organic acid concentrations and the incidence of postoperative infectious complications in patients undergoing major hepatectomy with extrahepatic bile duct resection for biliary malignancies. The fecal samples of 44 patients were collected before undergoing hepatectomy with bile duct resection for biliary malignancies. The concentrations of fecal organic acids, including acetic acid, butyric acid, and lactic acid, and representative fecal bacteria were measured. The perioperative clinical characteristics and the concentrations of fecal organic acids were compared between patients with and without postoperative infectious complications. Among 44 patients, 13 (30%) developed postoperative infectious complications. Patient age and intraoperative bleeding were significantly greater in patients with postoperative infectious complications compared with those without postoperative infectious complications. The concentrations of fecal acetic acid and butyric acid were significantly less, whereas the concentration of fecal lactic acid tended to be greater in the patients with postoperative infectious complications. The calculated gap between the concentrations of fecal acetic acid plus butyric acid minus lactic acid gap was less in the patients with postoperative infectious complications (median 43.5 vs 76.1 μmol/g of feces, P = .011). Multivariate analysis revealed that an acetic acid plus butyric acid minus lactic acid gap acid profile (especially low acetic acid, low butyric acid, and high lactic acid) had a clinically important impact on the incidence of postoperative infectious complications in patients undergoing major hepatectomy with extrahepatic bile duct resection. Copyright © 2017. Published by Elsevier Inc.

  20. Influence of the Biliary System on Biliary Bacteria Revealed by Bacterial Communities of the Human Biliary and Upper Digestive Tracts.

    Directory of Open Access Journals (Sweden)

    Fuqiang Ye

    Full Text Available Biliary bacteria have been implicated in gallstone pathogenesis, though a clear understanding of their composition and source is lacking. Moreover, the effects of the biliary environment, which is known to be generally hostile to most bacteria, on biliary bacteria are unclear. Here, we investigated the bacterial communities of the biliary tract, duodenum, stomach, and oral cavity from six gallstone patients by using 16S rRNA amplicon sequencing. We found that all observed biliary bacteria were detectable in the upper digestive tract. The biliary microbiota had a comparatively higher similarity with the duodenal microbiota, versus those of the other regions, but with a reduced diversity. Although the majority of identified bacteria were greatly diminished in bile samples, three Enterobacteriaceae genera (Escherichia, Klebsiella, and an unclassified genus and Pyramidobacter were abundant in bile. Predictive functional analysis indicated enhanced abilities of environmental information processing and cell motility of biliary bacteria. Our study provides evidence for the potential source of biliary bacteria, and illustrates the influence of the biliary system on biliary bacterial communities.

  1. Extrahepatic Manifestations and Autoantibodies in Patients with Hepatitis C Virus Infection

    Directory of Open Access Journals (Sweden)

    Takashi Himoto

    2012-01-01

    Full Text Available Patients with chronic hepatitis C virus (HCV infection frequently have many extrahepatic manifestations, as persistent HCV infection often triggers lymphoproliferative disorders and metabolic abnormalities. These manifestations primarily include autoimmune disorders such as cryoglobulinemia, Sjögren’s syndrome, and autoimmune thyroid disorders. It has been well established that chronic HCV infection plays important roles in the production of non-organ-specific autoantibodies, including antinuclear antibodies and smooth muscle antibodies, and organ-specific autoantibodies such as thyroid autoantibodies. However, the clinical significance of autoantibodies associated with the extrahepatic manifestations caused by HCV infection has not been fully recognized. In this paper, we mainly focus on the relationship between extrahepatic manifestations and the emergence of autoantibodies in patients with HCV infection and discuss the clinical relevance of the autoantibodies in the extrahepatic disorders.

  2. Sirenomelia with oesophageal atresia: a rare association.

    Science.gov (United States)

    Sathe, Pragati Aditya; Ghodke, Ratnaprabha Kundlikrao; Kandalkar, Bhuvaneshwari Mahendra

    2014-02-01

    We are reporting a rare case of sirenomelia with oesophageal atresia. Sirenomelia is a lethal sporadic defect of which lower gastrointestinal tract anomalies are characteristic findings. Respiratory and upper gastrointestinal tract malformations like oesophageal atresia occur in about 20-35% of cases. Though its occurrence has been described, it has been reported only rarely. This report aims at describing this uncommon association along with its histological features.

  3. Apple-peel atresia presenting as foetal intestinal obstruction ...

    African Journals Online (AJOL)

    Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA) is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed ...

  4. Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, K.; Alton, H.M.; Chapman, S. [Radiology Department, Birmingham Children`s Hospital, (United Kingdom)

    1998-12-01

    Background. The prognosis of infants with prolonged neonatal jaundice is dependent on early diagnosis because of the need for prompt surgical management of biliary atresia. Objective. To evaluate the usefulness of {sup 99} {sup m}Tc {sup m}-trimethylbromo-iminodiacetic acid (TBIDA, mebrofenin) in the investigation of infantile jaundice. Materials and methods. A retrospective study was undertaken of 58 patients with unexplained prolonged neonatal jaundice. Sixty-eight scans were reviewed. Results. Mebrofenin scintigraphy confirmed the presence of a choledochal cyst in three of the four cases with that diagnosis. There were no false negative results in the nine patients with extrahepatic biliary atresia (EHBA). Three further infants had an incorrect histological diagnosis of EHBA. A gall bladder was identified by US in each case and in one of these, scintigraphy showed gut excretion. In the 16 patients with no gut excretion by 24 h, the final diagnoses were intrahepatic cholestasis (n = 7), Alagille`s syndrome (n = 3), neonatal hepatitis (n = 3), alpha-1-antitrypsin deficiency (n = 2) and juvenile xanthogranuloma (n = 1). Seven infants had repeat scintigraphy after the administration of ursodeoxycholic acid (URSO). This changed five non-excretors with hepatitis into excretors. Two infants with hepatitis continued to show non-excretion after URSO, but a gallbladder was identified by US in both. Conclusions. Mebrofenin scintigraphy is accurate in confirming the presence of a choledochal cyst and in refuting the diagnosis of EHBA. While histology and scintigraphy are each 100 % sensitive for the diagnosis of EHBA, neither, individually, is accurate and the investigation of prolonged neonatal jaundice requires a multi-modality imaging strategy. (orig.) With 2 tabs., 12 refs.

  5. Prognostic impact of hepatobiliary scintigraphy in diagnosis and postoperative follow-up of newborns with biliary atresia; Prognostische Wertigkeit der hepatobiliaeren Funktionszintigraphie in Diagnostik und Nachsorge der Gallengangsatresie

    Energy Technology Data Exchange (ETDEWEB)

    Rossmueller, B.; Porn, U.; Dresel, S.; Hahn, K. [Muenchen Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin; Schuster, T. [Muenchen Univ. (Germany). Kinderchirurgische Klinik; Lang, T. [Muenchen Univ. (Germany). Kinderklinik

    2000-01-01

    Aim: To investigate the prognostic relevance of hepatobiliary scintigraphy (HBS) in newborns suffering from biliary atresia (BA) for establishing the primary diagnosis and in the postoperative follow-up after portoenterostomy (Kasai). Methods: Twenty newborns with direct hyperbilirubinemia and 6 children after operative treatment of BA (Kasai) underwent HBS with Tc-99m-DEIDA. In patients without intestinal drainage, hepatocellular extraction was estimated visually and calculated semiquantitatively by means of liver/heart-ratio 5 min p.i. Results: 10/20 patients with hyperbilirubinemia did not display biliary drainage; 6 had BA, 3 intrahepatic hypoplasia, and one showed a bile plug syndrom. 4/6 with BA but none of the 4 children with diagnoses other than BA presented with a good extraction. All of the 4 children with BA, who had either pre- or postoperatively a bad extraction, needed liver transplantation due to liver failure. Both of the two newborns with BA and favourable outcome after Kasai had a good extraction in the preoperative HBS and demonstrated good intestinal drainage in the postoperative scan. Conclusion: HBS rules out BA with high accuracy by demonstrating drainage of bile into the intestine. In newborns without drainage a good extraction favours the diagnosis of BA. In newborns with BA a bad extraction seems to indicate a poor postoperative prognosis after Kasai operation. HBS might therefore help to select those children who will not benefit from portoenterostomy. Postoperatively, HBS can easily and quickly confirm the successful hepatobiliary anastomosis by demonstrating biliary drainage into the intestine. (orig.) [German] Ziel der Studie war es, die diagnostische Wertigkeit und die prognostische Aussagekraft der hepatobiliaeren Funktionsszintigraphie (HB-FS) in der Primaerdiagnostik der Gallengangsatresie (GG-Atresie) und bei postoperativen Kontrollen nach Portoenterostomie (Kasai-OP) zu ueberpruefen. Methoden: 20 Neugeborene (Alter: 3-119 d) mit

  6. Mammalian follicular development and atresia: role of apoptosis.

    Science.gov (United States)

    Asselin, E; Xiao, C W; Wang, Y F; Tsang, B K

    2000-01-01

    The regulation of follicular development and atresia is a complex process and involves interactions between endocrine factors (gonadotropins) and intraovarian regulators (sex steroids, growth factors and cytokines) in the control of follicular cell fate (i.e. proliferation, differentiation and programmed cell death). Granulosa and theca cells are key players in this fascinating process. As atresia is the fate of most follicles, understanding of how these physiological regulators participate in determining the destiny of the follicle (to degenerate or to ovulate) at cellular and subcellular levels is fundamental. This short review summarizes the role of intraovarian modulators of programmed cell death in the induction of atresia during follicular development. Copyright 2000 S. Karger AG, Basel

  7. Strategy for use of biliary scintigraphy in non-iatrogenic biliary trauma

    International Nuclear Information System (INIS)

    Zeman, R.K.; Lee, C.H.; Stahl, R.; Viscomi, G.N.; Baker, C.; Cahow, C.E.; Dobbins, J.; Neumann, R.; Burrell, M.I.

    1984-01-01

    Biliary scintigraphy was used to examine 21 patients who had suspected non-iatrogenic biliary trauma. Seven patients (33%) had scintigraphic evidence of biliary leakage. Ultimately, surgical biliary repair was required for only three of these patients. Visualization of the gallbladder did not occur in eight trauma patients, but only one patient was shown to have cholecystitis. In this series, 16 patients had Tc-99m sulfur colloid scans that offered no significant advantage over cholescintigraphy in the detection of hepatic parenchymal defects. Biliary scintigraphy provides clinically useful information in cases both of blunt and penetrating trauma

  8. Normothermic machine perfusion reduces bile duct injury and improves biliary epithelial function in rat donor livers.

    Science.gov (United States)

    Op den Dries, Sanna; Karimian, Negin; Westerkamp, Andrie C; Sutton, Michael E; Kuipers, Michiel; Wiersema-Buist, Janneke; Ottens, Petra J; Kuipers, Jeroen; Giepmans, Ben N; Leuvenink, Henri G D; Lisman, Ton; Porte, Robert J

    2016-07-01

    Bile duct injury may occur during liver procurement and transplantation, especially in livers from donation after circulatory death (DCD) donors. Normothermic machine perfusion (NMP) has been shown to reduce hepatic injury compared to static cold storage (SCS). However, it is unknown whether NMP provides better preservation of bile ducts. The aim of this study was to determine the impact of NMP on bile duct preservation in both DCD and non-DCD livers. DCD and non-DCD livers obtained from Lewis rats were preserved for 3 hours using either SCS or NMP, followed by 2 hours ex vivo reperfusion. Biomarkers of bile duct injury (gamma-glutamyltransferase and lactate dehydrogenase in bile) were lower in NMP-preserved livers compared to SCS-preserved livers. Biliary bicarbonate concentration, reflecting biliary epithelial function, was 2-fold higher in NMP-preserved livers (P bile was significantly higher in NMP-preserved livers (7.63 ± 0.02 and 7.74 ± 0.05 for non-DCD and DCD livers, respectively) compared with SCS-preserved livers (7.46 ± 0.02 and 7.49 ± 0.04 for non-DCD and DCD livers, respectively). Scanning and transmission electron microscopy of donor extrahepatic bile ducts demonstrated significantly decreased injury of the biliary epithelium of NMP-preserved donor livers (including the loss of lateral interdigitations and mitochondrial injury). Differences between NMP and SCS were most prominent in DCD livers. Compared to conventional SCS, NMP provides superior preservation of bile duct epithelial cell function and morphology, especially in DCD donor livers. By reducing biliary injury, NMP could have an important impact on the utilization of DCD livers and outcome after transplantation. Liver Transplantation 22 994-1005 2016 AASLD. © 2016 American Association for the Study of Liver Diseases.

  9. The extrahepatic manifestations of hepatitis B virus.

    Science.gov (United States)

    Baig, Saeeda; Alamgir, Mohiuddin

    2008-07-01

    Hepatitis B Virus (HBV) leads to a number of hepatic complications, from acute to chronic hepatitis, cirrhosis and hepatocellular carcinoma, is a well-established fact. Upcoming clinical research, over the years, associates numerous extrahepatic manifestations during the acute and chronic episodes of hepatitis B with significant morbidity and mortality. A causal relationship between HBV and serious autoimmune disorders has also been observed among certain susceptible vaccine recipients in a defined temporal period following immunization. The cause of these extrahepatic manifestations is generally believed to be immune mediated. The most commonly described include skin rash, arthritis, arthralgia, glomerulonephritis, polyarteritis nodosa, and papular acrodermatitis etc. The serum-sickness like "arthritis-dermatitis" prodrome has also been observed in approximately one-third of patients acquiring HBV infections. Skin manifestations of HBV infection typically present as palpable purpura reported to be caused by chronic HBV, although this association remains controversial. To consider the relationship between HBV and other clinically significant disorders as well as serious autoimmune disorders among certain vaccine recipients is the topic of this review. Variable factors that influence extrahepatic manifestation are discussed, including possible synergy between hepatitis B virus and the immune system.

  10. Treatment of hepatic neoplasm through extrahepatic collaterals

    Energy Technology Data Exchange (ETDEWEB)

    Soo, C.S.; Chuang, V.P.; Wallace, S.; Charnsangavej, C.; Carrasco, H.

    1983-04-01

    Twenty-nine patients with hepatic artery occlusion were treated with additional hepatic infusion or embolization through extrahepatic collaterals. Seventeen courses of hepatic infusion were performed in 13 patients through the inferior pancreaticoduodenal artery, left gastric artery, or right gastric artery. Twenty-five hepatic embolization procedures were performed in 16 patients through the right and left phrenic arteries, left and right gastric arteries, pancreaticoduodenal artery, gastroduodenal artery, or omentoepiploic artery. In one patient gastric ulcers developed following left gastric artery infusion. No complication related to the embolization procedure was observed in the embolization group. The extrahepatic collaterals are important alternative routes for continuous transcatheter management of hepatic neoplasms following hepatic artery occlusion.

  11. Treatment of hepatic neoplasm through extrahepatic collaterals

    International Nuclear Information System (INIS)

    Soo, C.S.; Chuang, V.P.; Wallace, S.; Charnsangavej, C.; Carrasco, H.

    1983-01-01

    Twenty-nine patients with hepatic artery occlusion were treated with additional hepatic infusion or embolization through extrahepatic collaterals. Seventeen courses of hepatic infusion were performed in 13 patients through the inferior pancreaticoduodenal artery, left gastric artery, or right gastric artery. Twenty-five hepatic embolization procedures were performed in 16 patients through the right and left phrenic arteries, left and right gastric arteries, pancreaticoduodenal artery, gastroduodenal artery, or omentoepiploic artery. In one patient gastric ulcers developed following left gastric artery infusion. No complication related to the embolization procedure was observed in the embolization group. The extrahepatic collaterals are important alternative routes for continuous transcatheter management of hepatic neoplasms following hepatic artery occlusion

  12. Biliary-duodenal anastomosis using magnetic compression following massive resection of small intestine due to strangulated ileus after living donor liver transplantation: a case report.

    Science.gov (United States)

    Saito, Ryusuke; Tahara, Hiroyuki; Shimizu, Seiichi; Ohira, Masahiro; Ide, Kentaro; Ishiyama, Kohei; Kobayashi, Tsuyoshi; Ohdan, Hideki

    2017-12-01

    Despite the improvements of surgical techniques and postoperative management of patients with liver transplantation, biliary complications are one of the most common and important adverse events. We present a first case of choledochoduodenostomy using magnetic compression following a massive resection of the small intestine due to strangulated ileus after living donor liver transplantation. The 54-year-old female patient had end-stage liver disease, secondary to liver cirrhosis, due to primary sclerosing cholangitis with ulcerative colitis. Five years earlier, she had received living donor liver transplantation using a left lobe graft, with resection of the extrahepatic bile duct and Roux-en-Y anastomosis. The patient experienced sudden onset of intense abdominal pain. An emergency surgery was performed, and the diagnosis was confirmed as strangulated ileus due to twisting of the mesentery. Resection of the massive small intestine, including choledochojejunostomy, was performed. Only 70 cm of the small intestine remained. She was transferred to our hospital with an external drainage tube from the biliary cavity and jejunostomy. We initiated total parenteral nutrition, and percutaneous transhepatic biliary drainage was established to treat the cholangitis. Computed tomography revealed that the biliary duct was close to the duodenum; hence, we planned magnetic compression anastomosis of the biliary duct and the duodenum. The daughter magnet was placed in the biliary drainage tube, and the parent magnet was positioned in the bulbus duodeni using a fiberscope. Anastomosis between the left hepatic duct and the duodenum was accomplished after 25 days, and the biliary drainage stent was placed over the anastomosis to prevent re-stenosis. Contributions to the successful withdrawal of parenteral nutrition were closure of the ileostomy in the adaptive period, preservation of the ileocecal valve, internal drainage of bile, and side-to-side anastomosis

  13. Biliary ascariasis

    International Nuclear Information System (INIS)

    Mensing, M.; Cruz y Rivero, M.A.; Alarcon Hernandez, C.; Garcia Himmelstine, L.; Vogel, H.

    1986-01-01

    Biliary ascariasis is a complication of intestinal ascariasis. This results in characteristic findings in the intravenous cholangiocholecystogram and in the sonogram. Characteristic signs of biliary ascariasis are, in the longitudinal section, the 'strip sign', 'spaghetti sign', 'inner tube sign', and in transverse section 'a bull's eye in the triple O'. The helminth can travel from out of the biliary duct system back into the intestinum, so that control examinations can even be negative. (orig.) [de

  14. Choanal atresia in siblings; Case report | Kaitesi | East and Central ...

    African Journals Online (AJOL)

    Choanal atresia is an uncommon and often poorly recognized cause of unilateral or bilateral nasal obstruction. This report describes the case of bilateral choanal atresia in two consecutive siblings and describes the methods of treatment offered.

  15. Congenital aplasia of the optic chiasm and esophageal atresia: a case report

    Directory of Open Access Journals (Sweden)

    Madonia Maurizio

    2011-08-01

    Full Text Available Abstract Introduction The complete absence of the chiasm (chiasmal aplasia is a rare clinical condition. Hypoplasia of the optic nerve and congenital nystagmus are almost invariably associated characteristics. Microphthalmos or anophthalmos are common features in chiasmal aplasia, while central nervous system abnormalities are less frequent. Esophageal atresia can be isolated or syndromic. In syndromic cases, it is frequently associated with cardiac, limb, renal or vertebral malformations and anal atresia. More rarely, esophageal atresia can be part of anophthalmia-esophageal-genital syndrome, which comprises anophthalmia or microphthalmia, genital abnormalities, vertebral defects and cerebral malformations. Here, a previously unreported case of chiasmal aplasia presenting without microphthalmos and associated with esophageal atresia is described. Case presentation Aplasia of the optic chiasm was identified in a Caucasian Italian 8-month-old boy with esophageal atresia. An ultrasound examination carried out at 21 weeks' gestation revealed polyhydramnios. Intrauterine growth retardation, esophageal atresia and a small atrial-septal defect were subsequently detected at 28 weeks' gestation. Repair of the esophageal atresia was carried out shortly after birth. A jejunostomy was carried out at four months to facilitate enteral feeding. The child was subsequently noted to be visually inattentive and to be neurodevelopmentally delayed. Magnetic resonance imaging revealed chiasmal aplasia. No other midline brain defects were found. His karyotype was normal. Conclusion If achiasmia is a spectrum, our patient seems to depict the most severe form, since he appears to have an extremely severe visual impairment. This is in contrast to most of the cases described in the literature, where patients maintain good--or at least useful-- visual function. To the best of our knowledge, the association of optic nerve hypoplasia, complete chiasmal aplasia, esophageal

  16. Etiologies of chronic liver disease in children

    Directory of Open Access Journals (Sweden)

    Farahmand F

    2001-11-01

    Full Text Available Chronic Liver diseases in children is the result of many different diseases including: metabolic, genetic, infectious, toxic and idiopathic causes. This was a case series study on 133 infants and children with age range 6 month to 12 years old, who presented clinically with manifestation of chronic liver disease and were admitted to Children Hospital Medical Center from year 1999 to 2000. In this study, 32 (24.5 percent patients had autoimmune chronic hepatitis, 15 (11.3 percent Glycogen storage diseases, 12 (9 percent extrahepatic biliary atresia, 11 (8.2 percent willson disease, 10 (7.5 percent cryptogenic cirrhosis, 6 (4.5 percent chronic hepatitis C, 5 (3.8 percen chronic hepatitic B, 5 (3.8 percent galactosemia 3 (2.25 percent congenital hepatic fibrosis, 3 (3.8 percent histiocytosis X, 3 (2.25 percent sclerosing cholangitis, 2 (1.5 percent byler’s disease 2 (1.5 percent primary tuberculosis, 1 (0.75 percent choledocalcyst, 1 (0.75 percent Alagyle syndrome. According to our data, chronic liver disease should be considered in infants and children. In our study, the most common causes are found to be: metabolic and genetic diseases (37.5 percent, chronic autoimmune hepatitis (24 percent and biliary disorders (14 percent, that encompass 86 percent of the patients.

  17. The Modified Kimura's Technique for the Treatment of Duodenal Atresia

    Directory of Open Access Journals (Sweden)

    Biagio Zuccarello

    2009-01-01

    Full Text Available Background/Purpose. Kimura's diamond-shaped-duodenoduodenostomy (DSD is a known technique for the correction of congenital intrinsic duodenal obstruction. We present a modification of the technique and review the advantages of this new technique. Methods. From 1992 to 2006, 14 newborns were treated for duodenal atresia. We inverted the direction of the duodenal incisions: a longitudinal incision was made in the proximal duodenum while the distal was opened by transverse incision. Results. Our “inverted-diamond-shaped-duodenoduodenostomy” (i-DSD allowed postoperative oral feeding to start on days 2 to 3, peripheral intravenous fluids discontinuity on days 3 to 8 (median values 3.6; time to achieve full oral feeds on days 8 to 12 (median values 9.4; the length of hospitalisation ranged from 10 and 14 days (median value 11.2. No complications related to the anastomosis, by Viz leakage, dehiscence, biliary stasis, or stenosis were observed. Conclusions. The i-DSD provides a safe procedure to protect the ampulla of Vater from injury and avoids any formation of a blind loop. The results show that patients who have i-DSD achieve full oral feeds in a very short time period and, consequently, the length of hospitalisation is also significantly reduced.

  18. α7-nAChR Knockout Mice Decreases Biliary Hyperplasia and Liver Fibrosis in Cholestatic Bile-Duct Ligated Mice.

    Science.gov (United States)

    Ehrlich, Laurent; O'Brien, April; Hall, Chad; White, Tori; Chen, Lixian; Wu, Nan; Venter, Julie; Scrushy, Marinda; Mubarak, Muhammad; Meng, Fanyin; Dostal, David; Wu, Chaodong; Lairmore, Terry C; Alpini, Gianfranco; Glaser, Shannon

    2018-03-26

    α7-nAChR is a nicotinic acetylcholine receptor (specifically expressed on hepatic stellate cells, Kupffer cells, and cholangiocytes) that regulates inflammation and apoptosis in the liver. Thus, targeting α7-nAChR may be therapeutic in biliary diseases. Bile-duct ligation (BDL) was performed on wild-type (WT) and α7-nAChR-/- mice. We first evaluated the expression of α7-nAChR by immunohistochemistry (IHC) in liver sections. IHC was also performed to assess intrahepatic bile-duct mass (IBDM), and Sirius Red staining was performed to quantify the amount of collagen deposition. Immunofluorescence was performed to assess co-localization of α7-nAChR with bile ducts (co-stained with CK-19) and hepatic stellate cells (HSCs) (co-stained with desmin). The mRNA expression of α7-nAChR, Ki67/PCNA (proliferation), fibrosis genes (TGF-β1, Fibronectin-1, Col1α1, and α-SMA), and inflammatory markers (IL-6, IL-1β, and TNFα) was measured by real-time PCR. Biliary TGF-β1 and hepatic CD68 (Kupffer cell marker) expression was assessed using IHC. α7-nAChR immunoreactivity was observed in both bile ducts and HSCs and increased following BDL. α7-nAChR-/- BDL mice exhibited decreased: (i) bile duct mass, liver fibrosis, and inflammation; and (ii) immunoreactivity of TGF-1 as well as expression of fibrosis genes compared to WT BDL mice. α7-nAChR activation triggers biliary proliferation and liver fibrosis and may be a therapeutic target in managing extra-hepatic biliary obstruction.

  19. Strategy for the use of biliary scintigraphy in non-iatrogenic biliary trauma

    International Nuclear Information System (INIS)

    Zeman, R.K.; Lee, C.H.; Stahl, R.; Viscomi, G.N.; Baker, C.; Cahow, C.E.; Dobbins, J.; Neumann, R.; Burrell, M.I.

    1984-01-01

    Biliary scintigraphy was used to examine 21 patients who had suspected non-iatrogenic biliary trauma. Seven patients (33%) had scintigraphic evidence of biliary leakage. Ultimately, surgical biliary repair was required for only three of these patients. Visualization of the gallbladder did not occur in eight trauma patients, but only one patient was shown to have cholecystitis. In this series, 16 patients had Tc-99m sulfur colloid scans that offered no significant advantage over cholescintigraphy in the detection of hepatic parenchymal defects. Biliary scintigraphy provides clinically useful information in cases both of blunt and penetrating trauma

  20. Biliary ascariasis

    Energy Technology Data Exchange (ETDEWEB)

    Mensing, M.; Cruz y Rivero, M.A.; Alarcon Hernandez, C.; Garcia Himmelstine, L.; Vogel, H.

    1986-06-01

    Biliary ascariasis is a complication of intestinal ascariasis. This results in characteristic findings in the intravenous cholangiocholecystogram and in the sonogram. Characteristic signs of biliary ascariasis are, in the longitudinal section, the 'strip sign', 'spaghetti sign', 'inner tube sign', and in transverse section 'a bull's eye in the triple O'. The helminth can travel from out of the biliary duct system back into the intestinum, so that control examinations can even be negative.

  1. Risk factors for recurrent symptomatic pigmented biliary stones after percutaneous transhepatic biliary extraction.

    Science.gov (United States)

    Kim, Dong Won; Lee, Sang Yun; Cho, Jin-Han; Kang, Myong Jin; Noh, Myung Hwan; Park, Byeong-Ho

    2010-07-01

    To evaluate risk factors for the recurrence of biliary stones after a percutaneous transhepatic biliary stone extraction. The procedures were performed on 339 patients between July 2004 and December 2008 (54 months). Medical records and images were retrospectively reviewed for 135 patients (mean age, 66.4 years; 83 men and 52 women) who had undergone follow-up for a mean of 13.2 months (range, 3-37 months). To evaluate risk factors for the recurrence of biliary stones, variables were evaluated with univariate and multivariate analyses. Variables included sex, age, stone location, number of stones, stone size, presence of a peripapillary diverticulum, application of antegrade sphincteroplasty, presence of a biliary stricture, largest biliary diameter before the procedure, and gallbladder status. Thirty-three of the 135 patients (24%) had recurrent symptomatic biliary stones and underwent an additional extraction. The mean time to recurrence was 17.2 months +/- 8.7. Univariate analysis of risk factors for recurrence of biliary stones demonstrated that location, number of stones, stone size, application of antegrade sphincteroplasty, presence of a biliary stricture, and biliary diameter were significant factors (P or =6; relative risk, 64.8; 95% confidence interval: 5.8, 717.6) and stone size (> or =14 mm; relative risk, 3.8; 95% confidence interval: 1.138, 13.231) were determined to be significant risk factors. The independent risk factors for recurrence of symptomatic biliary stones after percutaneous transhepatic biliary stone extraction were a stone size of at least 14 mm and the presence of at least six stones. Copyright 2010 SIR. Published by Elsevier Inc. All rights reserved.

  2. Intestinal smooth muscle response to chronic obstruction : possible applications in jejunoileal atresia.

    Science.gov (United States)

    Cloutier, R

    1975-02-01

    Hyperplasia is the main change occurring in intestinal smooth muscle above a chronic obstruction and explains the functional obstruction seen in the proximal bowel of a jejunoileal atresia. With an experimental model in dogs, this hyperplasia has been shown to be reversible. However, changes are extreme in atresia, and experiments in animals with induced atresia will best evaluate various kinds of treatment.

  3. The "boomerang" malleus-incus complex in congenital aural atresia.

    Science.gov (United States)

    Mukherjee, S; Kesser, B W; Raghavan, P

    2014-01-01

    "Boomerang" malleus-incus fusion deformity is identified on axial high-resolution CT in a subset of patients with congenital aural atresia, and it is associated with an absent incudostapedial joint and stapes capitulum and attachment of the incus to the tympanic segment of the facial nerve canal. Twelve patients with this deformity were identified on a retrospective review of imaging from a cohort of 673 patients with congenital aural atresia, with surgical confirmation in 9 of these patients. Eight of 9 patients underwent partial ossicular replacement prosthesis reconstruction with improvement in hearing outcome. We hypothesize that the boomerang anomaly represents a more severe ossicular anomaly than is normally seen in congenital aural atresia, arising from an arrest earlier in the embryonic development of the first and second branchial arch. This has potentially important implications for surgical planning because hearing outcomes with placement of prosthesis may not be as good as with conventional atresia surgery, in which reconstruction is performed with the patient's native ossicular chain. © 2014 by American Journal of Neuroradiology.

  4. Angiography-based C-arm CT for the assessment of extrahepatic shunting before radioembolization

    International Nuclear Information System (INIS)

    Heusner, Till Alexander; Hahn, S.; Forsting, M.; Antoch, G.; Hamami, M.E.; Poeppel, T.; Bockisch, A.; Ertle, J.; Hilgard, P.

    2010-01-01

    Purpose: to retrospectively assess the accuracy of angiography-based C-arm CT for the detection of extrahepatic shunting before SIRT. Materials and methods: 30 patients (mean age: 64 ± 12 years) with hypervascularized hepatic tumors underwent hepatic angiography, coil embolization of gastrointestinal collaterals and 99mTc-macroaggregated albumin (MAA) SPECT/CT before SIRT. Before MAA injection via a microcatheter from the intended treatment position, an angiography and angiography-based C-arm CT (XperCT trademark, Philips Healthcare) were acquired. Angiographies and XperCT trademark were performed from 48 microcatheter positions followed by MAA injections and MAA-SPECT/CT. MAA-SPECT/CT served as the reference standard for determining the accuracy of hepatic arteriography and C-arm CT for the detection of extrahepatic shunting. Results: MAA-SPECT/CT revealed extrahepatic shunting in 5 patients (17%). Hepatic arteriography yielded a true negative in 22 (73%), a false negative in 5 (17%), and an unclear result in 3 patients (10%). C-arm CT yielded a true positive in 3 (10%), true negative in 24 (80%), false positive in 1 (3%), and false negative in 2 patients (7%). The specificity and the NPV of hepatic arteriography for the detection of extrahepatic shunting were 88% and 81%, respectively. For C-arm CT the sensitivity, specificity, PPV, NPV, and accuracy for the detection of extrahepatic shunting were 60%, 96%, 75%, 92%, and 90%, respectively. Conclusion: C-arm CT offers additional information to angiography when assessing SIRT patients for extrahepatic shunting. More accurate detection of extrahepatic shunting may optimize the workflow in SIRT preparations by avoiding unnecessary repeat angiographies. (orig.)

  5. Diffusion-weighted MR imaging for detection of extrahepatic cholangiocarcinoma

    International Nuclear Information System (INIS)

    Cui, Xing-Yu; Chen, Hong-Wei; Cai, Song; Bao, Jian; Tang, Qun-Feng; Wu, Li-Yuan; Fang, Xiang-Ming

    2012-01-01

    Objectives: To measure the sensitivity of diffusion-weighted imaging (DWI) and determine the most appropriate b value for DWI; to explore the correlation between the apparent diffusion coefficient (ADC) value and the degree of extrahepatic cholangiocarcinoma differentiation. Methods: Preoperative diffusion-weighted imaging and magnetic resonance examinations were performed for 31 patients with extrahepatic cholangiocarcinoma. Tumor ADC values were measured, and the signal-to-noise ratio, contrast-to-noise ratio, and signal-intensity ratio between the diffusion-weighted images with various b values as well as the T2-weighted images were calculated. Pathologically confirmed patients were pathologically graded to compare the ADC value with different b values of tumor at different degrees of differentiation, and the results were statistically analyzed by using the Friedman test. Results: A total of 29 cases of extrahepatic cholangiocarcinoma were detected by DWI. As the b value increased, tumor signal-to-noise ratio and contrast-to-noise ratio between the tumor and normal liver gradually decreased, but the tumor signal-intensity ratio gradually increased. When b = 800 s/mm 2 , contrast-to-noise ratio between tumor and normal liver, tumor signal-intensity ratio, and tumor signal-to-noise ratio of diffusion-weighted images were all higher than those of T2-weighted images; the differences were statistically significant (P 2 was the best in DWI of extrahepatic cholangiocarcinoma; the lesion ADC value declined as the degree of cancerous tissue differentiation decreased.

  6. Hepatobiliary scintigraphy in the assessment of biliary obstruction after hepatic resection with biliary-enteric anastomosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, J.S.; Moon, D.H.; Shin, J.W.; Ryu, J.S.; Lee, H.K. [Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea); Lee, S.G.; Lee, Y.J.; Park, K.M. [Department of General Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea)

    2000-02-01

    We investigated the usefulness of hepatobiliary scintigraphy (HBS) for diagnosing biliary obstruction after curative hepatic resection with biliary-enteric anastomosis. The study population consisted of 54 patients who underwent surgery for benign (n=18) or malignant (n=36) biliary disease. We analysed 68 technetium-99m DISIDA scintigrams which were performed at least 1 month after the surgery (median: 9 months). Final diagnosis was made by operative exploration, other invasive radiological studies or clinical and radiological follow-up for at least 6 months after the surgery. Diagnostic accuracy was analysed according to the pretest likelihood of biliary obstruction. There were two total and 15 segmental biliary obstructions. In patients with symptoms of biliary obstruction and abnormal liver function, HBS always allowed correct diagnosis (two instances of total obstruction, seven of segmental obstruction and seven of non-obstruction). Among the patients with non-specific symptoms or isolated elevation of serum alkaline phosphatase, HBS diagnosed segmental biliary obstruction in seven of the eight instances, and non-obstruction in 22 of 23 instances. There were no cases of biliary obstruction and no false-positive results of HBS in 21 instances with no clinical signs or symptoms of biliary obstruction. The diagnostic sensitivity and specificity of HBS for biliary obstruction were 94% (16/17) and 97% (50/51), respectively. In conclusion, HBS is a highly accurate modality for the diagnosis of segmental biliary obstruction during long-term follow-up after hepatic resection with biliary-enteric anastomosis. (orig.)

  7. Hepatobiliary scintigraphy in the assessment of biliary obstruction after hepatic resection with biliary-enteric anastomosis

    International Nuclear Information System (INIS)

    Kim, J.S.; Moon, D.H.; Shin, J.W.; Ryu, J.S.; Lee, H.K.; Lee, S.G.; Lee, Y.J.; Park, K.M.

    2000-01-01

    We investigated the usefulness of hepatobiliary scintigraphy (HBS) for diagnosing biliary obstruction after curative hepatic resection with biliary-enteric anastomosis. The study population consisted of 54 patients who underwent surgery for benign (n=18) or malignant (n=36) biliary disease. We analysed 68 technetium-99m DISIDA scintigrams which were performed at least 1 month after the surgery (median: 9 months). Final diagnosis was made by operative exploration, other invasive radiological studies or clinical and radiological follow-up for at least 6 months after the surgery. Diagnostic accuracy was analysed according to the pretest likelihood of biliary obstruction. There were two total and 15 segmental biliary obstructions. In patients with symptoms of biliary obstruction and abnormal liver function, HBS always allowed correct diagnosis (two instances of total obstruction, seven of segmental obstruction and seven of non-obstruction). Among the patients with non-specific symptoms or isolated elevation of serum alkaline phosphatase, HBS diagnosed segmental biliary obstruction in seven of the eight instances, and non-obstruction in 22 of 23 instances. There were no cases of biliary obstruction and no false-positive results of HBS in 21 instances with no clinical signs or symptoms of biliary obstruction. The diagnostic sensitivity and specificity of HBS for biliary obstruction were 94% (16/17) and 97% (50/51), respectively. In conclusion, HBS is a highly accurate modality for the diagnosis of segmental biliary obstruction during long-term follow-up after hepatic resection with biliary-enteric anastomosis. (orig.)

  8. Carcinosarcoma of the Extrahepatic Bile Duct Presenting with Stone-like Radiological Findings.

    Science.gov (United States)

    Kumei, Shinsuke; Onishi, Yutaka; Ogura, Takeshi; Kusumoto, Chosei; Matsuno, Yasuko; Nishigami, Takashi; Maeda, Mitsuo; Harada, Masaru

    2015-01-01

    A 73-year-old woman was referred to our hospital due to epigastralgia and jaundice. The radiological findings showed a stone-like tumor in the extrahepatic bile duct. The patient was initially thought to have adenocarcinoma of the bile duct based on the findings of a pathological examination of the bile duct biopsy specimen and underwent pancreaticoduodenectomy; the final diagnosis of the lesion was so-called carcinosarcoma of the extrahepatic bile duct. She died of liver metastasis six months after the surgery. This case suggests that surgical resection is not adequate for achieving a radical cure, and the optimal treatment for extrahepatic bile duct carcinosarcoma should be established immediately.

  9. Duodenal atresia with 'apple-peel configuration' of the ileum and ...

    African Journals Online (AJOL)

    According to the current understanding, duodenal atresia is considered to be a primary malformation resulting from the errors in recanalisation in early gestation. We report a rare case of duodenal atresia with apple-peel configuration of remaining small bowel with absent superior mesenteric artery in a preterm child, which ...

  10. Galdevejsatresi. Hepatoportoenteroanastomose og/eller levertransplantation

    DEFF Research Database (Denmark)

    Kvist, N E; Thorup, Jørgen Mogens

    1993-01-01

    Long-term survival of biliary atresia patients after Kasai hepatoportoenterostomy is being increasingly reported. Favorable prognostic factors are: 1) Young age at operation (max. 60 days). 2) Few incidents of postoperative cholangitis. Jaundice-free long-term survival is achieved in one-fourth t......Long-term survival of biliary atresia patients after Kasai hepatoportoenterostomy is being increasingly reported. Favorable prognostic factors are: 1) Young age at operation (max. 60 days). 2) Few incidents of postoperative cholangitis. Jaundice-free long-term survival is achieved in one...

  11. Novel extrahepatic cytochrome P450s

    International Nuclear Information System (INIS)

    Karlgren, Maria; Miura, Shin-ichi; Ingelman-Sundberg, Magnus

    2005-01-01

    The cytochrome P450 enzymes are highly expressed in the liver and are involved in the metabolism of xenobiotics. Because of the initiatives associated with the Human Genome Project, a great progress has recently been seen in the identification and characterization of novel extrahepatic P450s, including CYP2S1, CYP2R1, CYP2U1 and CYP2W1. Like the hepatic enzymes, these P450s may play a role in the tissue-specific metabolism of foreign compounds, but they may also have important endogenous functions. CYP2S1 has been shown to metabolize all-trans retinoic acid and CYP2R1 is a major vitamin D 25-hydroxylase. Regarding their metabolism of xenobiotics, much remains to be established, but CYP2S1 metabolizes naphthalene and it is likely that these P450s are responsible for metabolic activation of several different kinds of xenobiotic chemicals and contribute to extrahepatic toxicity and carcinogenesis

  12. Comparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma

    International Nuclear Information System (INIS)

    Yata, M.; Suzuki, K.; Furuhashi, N.; Kawakami, K.; Kawai, Y.; Naganawa, S.

    2016-01-01

    Aim: To compare the multidetector-row computed tomography (MDCT) findings of IgG4-related sclerosing cholangitis (IgG4-SC) and extrahepatic cholangiocarcinoma (EH-CCA). Materials and methods: Two radiologists who had no knowledge of the patients' clinical information retrospectively evaluated the CT findings of patients with IgG4-SC (n=33) and EH-CCA (n=39) on a consensus basis. Another radiologist measured the biliary lesions. IgG4-SC was diagnosed using the Japan Biliary Association criteria (2012) or the Mayo Clinic's HISORt criteria. EH-CCA was diagnosed based on surgical findings. Results: Compared with EH-CCA, IgG4-SC exhibited the following findings significantly more frequently: (a) wall thickening alone, (b) concentric wall thickening, (c) smooth inner margins, (d) homogeneous attenuation in the arterial phase, (e) a lesion involving the intrapancreatic bile duct, (f) smooth outer margins, (g) fully visible lumen, (h) a funnel-shaped proximal bile duct, (i) skip lesions, and (j) abnormal pancreatic findings. Conversely, (k) dual-layered attenuation in all phases was significantly more common in EH-CCA. The specificity values of parameters (e–k) were >80%. Regarding dimensions, (l) the biliary lesions were longer in IgG4-SC than in EH-CCA. (m) The diameters of the dilated proximal common bile duct and (n) the dilated proximal intrahepatic bile duct were smaller in IgG4-SC than in EH-CCA. Conclusion: A number of CT findings are useful for differentiating between IgG4-SC and EH-CCA. CT findings (e–k) are particularly useful for this purpose. - Highlights: • Some CT findings are useful for differentiating between IgG4-SC and EH-CCA. • Homogeneous attenuation in all phases was more common in IgG4-SC than in EH-CCA. • Abnormal pancreatic findings showed high sensitivity and specificity values. • Dual-layered attenuation in all phases was more common in EH-CCA than in IgG4-SC.

  13. Gastric diverticulum causing gastric outlet obstruction in the setting of duodenal atresia

    Directory of Open Access Journals (Sweden)

    Devashis Mukherjee

    2018-04-01

    Full Text Available Duodenal obstruction due to duodenal atresia occurs in 1 in 10,000 live births and is the most common type of intestinal obstruction in neonates [1–3]. Gastric outlet obstruction in the newborn period from causes other than hypertrophic pyloric stenosis is very uncommon [3]. Potential etiologies include gastric volvulus, antral web, and duplication cysts. Gastric diverticula in the infant is even more rare, with only a few case reports published, and only one describes a gastric diverticulum in the presence of a duodenal atresia [4–8]. In this report, we describe the first case of a gastric outlet obstruction due to a gastric diverticulum in the presence of duodenal atresia. Keywords: Duodenal atresia, Gastric diverticulum, Gastric outlet obstruction

  14. Endoscopic Ultrasound-Guided Rendezvous Technique for Failed Biliary Cannulation in Benign and Resectable Malignant Biliary Disorders.

    Science.gov (United States)

    Shiomi, Hideyuki; Yamao, Kentaro; Hoki, Noriyuki; Hisa, Takeshi; Ogura, Takeshi; Minaga, Kosuke; Masuda, Atsuhiro; Matsumoto, Kazuya; Kato, Hironari; Kamada, Hideki; Goto, Daisuke; Imai, Hajime; Takenaka, Mamoru; Noguchi, Chishio; Nishikiori, Hidefumi; Chiba, Yasutaka; Kutsumi, Hiromu; Kitano, Masayuki

    2018-03-01

    Endoscopic ultrasound-guided rendezvous technique (EUS-RV) has emerged as an effective salvage method for unsuccessful biliary cannulation. However, its application for benign and resectable malignant biliary disorders has not been fully evaluated. To assess the efficacy and safety of EUS-RV for benign and resectable malignant biliary disorders. This was a multicenter prospective study from 12 Japanese referral centers. Patients who underwent EUS-RV after failed biliary cannulation for biliary disorder were candidates for this study. Inclusion criteria were unsuccessful biliary cannulation for therapeutic endoscopic retrograde cholangiopancreatography with benign and potentially resectable malignant biliary obstruction. Exclusion criteria included unresectable malignant biliary obstruction, inaccessible papillae due to surgically altered upper gastrointestinal anatomy or duodenal stricture, and previous sphincterotomy and/or biliary stent placement. The primary outcome was the technical success rate of biliary cannulation; procedure time, adverse events, and clinical outcomes were secondary outcomes. Twenty patients were prospectively enrolled. The overall technical success rate and median procedure time were 85% and 33 min, respectively. Guidewire manipulation using a 4-Fr tapered tip catheter contributed to the success in advancing the guidewire into the duodenum. Adverse events were identified in 15% patients, including 2 with biliary peritonitis and 1 mild pancreatitis. EUS-RV did not affect surgical maneuvers or complications associated with surgery, or postoperative course. EUS-RV may be a safe and feasible salvage method for unsuccessful biliary cannulation for benign or resectable malignant biliary disorders. Use of a 4-Fr tapered tip catheter may improve the overall EUS-RV success rate.

  15. BIRTH INJURY RELATED UNILATERAL ANTERIOR NARES PARTIAL FIBROUS ATRESIA: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Jayagar

    2016-02-01

    Full Text Available Congenital atresia of anterior nares has been rarely reported and it may co-exist along with posterior choanal atresia. (1,2 In our case, birth injury caused by forceps delivery has caused unilateral anterior nares partial atresia. Twenty eight years old male patient came with the complaints of left nostril blocked sensation since birth, aggravated with attacks of upper respiratory tract infections. On examination he had left anterior nares partial atresia caused by fibrous bands as a result of birth injury due to instrumental delivery. Rhinoplasty performed to open-up left nostril and patient relieved of his symptoms and also on cosmetic appearance

  16. Apple-peel atresia presenting as foetal intestinal obstruction

    Directory of Open Access Journals (Sweden)

    Ashok Yadavrao Kshirsagar

    2011-01-01

    Full Text Available Apple-peel atresia or Type 3 jejuno-ileal atresia (JIA is an uncommon cause of foetal intestinal obstruction. Bowel obstruction in the foetus is diagnosed on the prenatal ultrasonography only in 50% cases. We report a case in which foetal intestinal obstruction was diagnosed on prenatal ultrasonography. The child showed signs of intestinal obstruction on day one after birth, for which an exploratory laparotomy was performed. Type 3 JIA was found for which resection of atretic segments with jejuno-ascending colon anastomosis was preformed.

  17. Immediate primary anastomosis for isolated oesophageal atresia: A single-centre experience

    Directory of Open Access Journals (Sweden)

    Ibrahim Uygun

    2015-01-01

    Full Text Available Background: Isolated oesophageal atresia without tracheo-oesophageal fistula represents a major challenge for most paediatric surgeons. Here, we present our experience with six neonates with isolated oesophageal atresia who successfully underwent immediate primary anastomosis using multiple Livaditis circular myotomy. Materials and Methods: All six neonates were gross type A isolated oesophageal atresia (6%, from among 102 neonates with oesophageal atresia, treated between January 2009 and December 2013. Five neonates were female; one was male. The mean birth weight was 2300 (range 1700-3100 g. Results: All six neonates successfully underwent immediate primary anastomosis using multiple myotomies (mean 3; range 2-4 within 10 (median 3 days after birth. The gap under traction ranged from 6 to 7 cm. One neonate died of a major cardiac anomaly. Another neonate was lost to follow-up after being well for 3 months. Three anastomotic strictures were treated with balloon dilatation, and four anastomotic leaks were treated conservatively. The mean duration of follow-up was 33 months. Conclusions: To treat isolated oesophageal atresia, an immediate primary anastomosis can be achieved using multiple myotomies. Although, this approach is associated with high complication rates, as are other similar approaches, these complications can be overcome.

  18. Biliary fascioliasis--an uncommon cause of recurrent biliary colics: report of a case and brief review.

    Science.gov (United States)

    Al Qurashi, Hesham; Masoodi, Ibrahim; Al Sofiyani, Mohammad; Al Musharaf, Hisham; Shaqhan, Mohammed; All, Gamal Nasr Ahmed Abdel

    2012-01-01

    Biliary parasitosis is one of the important causes of biliary obstruction in endemic areas, however due to migration and travel the disease is known to occur in non endemic zones as well. The spectrum of biliary fascioliasis ranges from recurrent biliary colics to acute cholangitis. The long term complications are gall stones, sclerosing cholangitis and biliary cirrhosis. We describe fascioliasis as a cause of recurrent biliary colics in a young male necessitating multiple hospitalizations over a period of four years. Investigative profile had been non-contributory every time he was hospitalized for his abdominal pain prior to the current presentation. He never had cholangitis due to the worm in the common bile duct. It was only at endoscopic retrograde cholangiopancreatography (ERCP) biliary fascioliasis was discovered to be the cause of his recurrent biliary colics. After removal of the live Fasciola hepatica from the common bile duct he became symptom free and is attending our clinic for last 11 months now. Clinical spectrum of biliary fascioliasis is discussed in this report.

  19. Histopathologic profile of esophageal atresia and ...

    African Journals Online (AJOL)

    dUMAss Memorial Pathology, UMass Memorial Medical Center, Worcester, ..... 2 Yokoi A, Nishijima E. Long-term complications of esophageal atresia. Nihon ... long upper esophageal pouch and short gap, associated with left congenital.

  20. Spiral CT biliary virtual endoscopy: preliminary clinical applications in the detection of biliary calculus

    International Nuclear Information System (INIS)

    Xiong Minghui; Wang Dong; Song Yunlong; Zhang Wanshi; Xu Jiaxing

    2000-01-01

    Objective: To evaluate imaging features and clinical value of CT biliary virtual endoscopy in the detection of biliary calculus. Methods: Eighteen patients with biliary calculi underwent volume scanning using spiral CT (Hispeed Advantage CT/i GE ). All data were transferred to computer workstation, and CT biliary virtual endoscopy images with pseudocolor encoding were generated from the volumetric data using the Navigator Smooth soft-ware. All cases were proved by ultrasound, axial CT or operation. Results: Among 18 cases, gallstones were found 8 in cases, common bile duct stones in 2 cases, gallstones and bile duct stones in 6 cases. The stones were 0.3-3.2 cm in size. CT biliary virtual endoscopy correctly demonstrated the surface details of stones which were viewed from extra- or intraluminal orientation in a 3D fashion. The findings were consistent with those of US, CT or operation. Conclusion: The CT virtual biliary endoscopy is a further development of virtual endoscopy for observing biliary calculus from intra- and extra-luminal views and providing three dimensional information of stone

  1. Aortic atresia with normal sized left ventricle

    Directory of Open Access Journals (Sweden)

    Priya Jagia

    2016-01-01

    Full Text Available Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best of our knowledge, use of multi-detector computed tomography in aortic atresia with well developed left ventricle has not been reported in literature till date.

  2. Dysphagia in Children with Esophageal Atresia: Current Diagnostic Options

    OpenAIRE

    Rayyan, Maissa; Allegaert, Karel; Omari, Taher; Rommel, Nathalie

    2015-01-01

    Dysphagia or swallowing disorder is very common (range, 15-52%) in patients with esophageal atresia. Children present with a wide range of symptoms. The most common diagnostic tools to evaluate esophageal dysphagia, such as upper barium study and manometry, aim to characterize anatomy and function of the esophageal body and the esophagogastric junction (EGJ). Using these technologies, a variety of pathological motor patterns have been identified in children with esophageal atresia. However, t...

  3. Fluorescence versus X-ray cholangiography during laparoscopic cholecystectomy

    DEFF Research Database (Denmark)

    Lehrskov, Lars Lang; Larsen, Søren S; Kristensen, Billy B

    2016-01-01

    INTRODUCTION: Intraoperative fluorescent cholangiography is a novel non-invasive imaging technique to visualise the extrahepatic biliary tract during laparoscopic cholecystectomy. It has been proven feasible, fast and cost effective. Never-theless, there is only sparse data on the capacity...... of fluorescent cholangiography to visualise the biliary anatomy. METHODS: Based on a non-inferiority design, patients with complicated gallstone disease are randomised to either -intraoperative conventional X-ray cholangiography (reference group, n = 60) or intraoperative fluorescent cholangiography (n = 60......). The primary outcome is visualisation of the junction between the cystic duct, the common hepatic duct and the common bile duct. CONCLUSION: The present study may show that fluorescent cholangiography is as valid for visualisation of important structures of the extrahepatic biliary tract as conventional X...

  4. New diagnosis and therapy model for ischemic-type biliary lesions following liver transplantation--a retrospective cohort study.

    Directory of Open Access Journals (Sweden)

    Ying-cai Zhang

    Full Text Available Ischemic-type biliary lesions (ITBLs are a major cause of graft loss and mortality after orthotopic liver transplantation (OLT. Impaired blood supply to the bile ducts may cause focal or extensive damage, resulting in intra- or extrahepatic bile duct strictures or dilatations that can be detected by ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and cholangiography. However, the radiographic changes occur at an advanced stage, after the optimal period for therapeutic intervention. Endoscopic retrograde cholangio-pancreatography (ERCP and percutaneous transhepatic cholangiodrainage (PTCD are the gold standard methods of detecting ITBLs, but these procedures cannot be used for continuous monitoring. Traditional methods of follow-up and diagnosis result in delayed diagnosis and treatment of ITBLs. Our center has used the early diagnosis and intervention model (EDIM for the diagnosis and treatment of ITBLs since February 2008. This model mainly involves preventive medication to protect the epithelial cellular membrane of the bile ducts, regular testing of liver function, and weekly monitor of contrast-enhanced ultrasonography (CEUS to detect ischemic changes to the bile ducts. If the liver enzyme levels become abnormal or CEUS shows low or no enhancement of the wall of the hilar bile duct during the arterial phase, early ERCP and PTCD are performed to confirm the diagnosis and to maintain biliary drainage. Compared with patients treated by the traditional model used prior to February 2008, patients in the EDIM group had a lower incidence of biliary tract infection (28.6% vs. 48.6%, P = 0.04, longer survival time of liver grafts (24±9.6 months vs. 17±12.3 months, P = 0.02, and better outcomes after treatment of ITBLs.

  5. A new method of three-dimensional computer assisted reconstruction of the developing biliary tract.

    Science.gov (United States)

    Prudhomme, M; Gaubert-Cristol, R; Jaeger, M; De Reffye, P; Godlewski, G

    1999-01-01

    A three-dimensional (3-D) computer assisted reconstruction of the biliary tract was performed in human and rat embryos at Carnegie stage 23 to describe and compare the biliary structures and to point out the anatomic relations between the structures of the hepatic pedicle. Light micrograph images from consecutive serial sagittal sections (diameter 7 mm) of one human and 16 rat embryos were directly digitalized with a CCD camera. The serial views were aligned automatically by software. The data were analysed following segmentation and thresholding, allowing automatic reconstruction. The main bile ducts ascended in the mesoderm of the hepatoduodenal ligament. The extrahepatic bile ducts: common bile duct (CD), cystic duct and gallbladder in the human, formed a compound system which could not be shown so clearly in histologic sections. The hepato-pancreatic ampulla was studied as visualised through the duodenum. The course of the CD was like a chicane. The gallbladder diameter and length were similar to those of the CD. Computer-assisted reconstruction permitted easy acquisition of the data by direct examination of the sections through the microscope. This method showed the relationships between the different structures of the hepatic pedicle and allowed estimation of the volume of the bile duct. These findings were not obvious in two-dimensional (2-D) views from histologic sections. Each embryonic stage could be rebuilt in 3-D, which could introduce the time as a fourth dimension, fundamental for the study of organogenesis.

  6. Endoscopic Biliary Stenting Versus Percutaneous Transhepatic Biliary Stenting in Advanced Malignant Biliary Obstruction: Cost-effectiveness Analysis.

    Science.gov (United States)

    Sun, Xin Rong; Tang, Cheng Wu; Lu, Wen Ming; Xu, Yong Qiang; Feng, Wen Ming; Bao, Yin; Zheng, Yin Yuan

    2014-05-01

    This study aims to compare the clinical outcomes and costs between endoscopic biliary stenting (EBS) and percutaneous transhepatic biliary stenting (PTBS). We randomly assigned 112 patients with unresectable malignant biliary obstruction 2006 and 2011 to receive EBS or PTBS with self-expandable metal stent (SEMS) as palliative treatment. PTBS was successfully performed in 55 patients who formed the PTBS group (failed in 2 patients). EBS was successfully performed in 52 patients who formed the EBS group (failed in 3 patients). The effectiveness of biliary drainage, hospital stay, complications, cost, survival time and mortality were compared. Patients in PTBS group had shorter hospital stay and lower initial and overall expense than the BBS group (P PTBS group was significantly lower than in EBS group (3/55 vs 11/52, P = 0.0343). Late complications in the EBS group did not differ significantly from PTBS group (7/55 vs 9/52, P = 0.6922). The survival curves in the two groups showed no significant difference (P = 0.5294). Conclusions: 3.

  7. Bonebridge Implantation for Conductive Hearing Loss in a Patient with Oval Window Atresia.

    Science.gov (United States)

    Kim, Minbum

    2015-08-01

    The occurrence of oval window atresia is a rare anomaly with conductive hearing loss. Traditional atresia surgeries involve challenging surgical techniques with risks of irreversible inner ear damage. Recent reports on Bonebridge (Medel, Innsbruck, Austria), a novel implantable bone conduction hearing aid system, assert that the device is safe and effective for conductive hearing loss. We present a case of Bonebridge implantation in an eight-year-old girl with bilateral oval window atresia.

  8. Association of Nasal Nostril Stenosis with Bilateral Choanal Atresia: A Case Report

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    Shahin Abdollahifakhim

    2014-01-01

    Full Text Available Introduction: Neonatal nasal airway obstruction induces various degrees of respiratory distress. The management of this disease, including surgical repair, will depend on the severity and location of the obstruction. We describe here a case of congenital nasal nostril stenosis that required surgical repair for stenting of both nares after coanal atresia repair.   Case Report: A 2 days old female newborn referred to neonatal department of Tabriz Children’s Hospital affiliated to the University of Medical Sciences of Tabriz, Iran on the 3rd of December, 2011 immediately after birth with respiratory distress due to bilateral coanal atresia and nasal hypoplasia with very small nostrils. CT scan showed normal brain and bilateral choanal atresia with normal size Pyriform apertures.   Conclusion: Nasal obstruction can lead to airway compromise and respiratory distress. Congenital bony nasal deformities are being recognized as an important cause of newborn airway obstruction. Nasal hypoplasia is seen in many craniofacial syndromes. Although our patient had hypoplastic nostrils with respiratory distress due to bilateral coanal atresia, correction of hypoplastic nostrils was necessary for completing the operation of choanal atresia.

  9. MRI in distal vaginal atresia

    International Nuclear Information System (INIS)

    Hugosson, C.; Jorulf, H.; Bakri, Y.

    1991-01-01

    Magnetric resonance imaging in two young females with abdominal pain revealed vaginal atresia with massive hematocolpos but a normal cervix and uterine body. Information obtained with MRI was superior to ultrasound and CT and is suggested as the examination of choice prior to surgical correction. (orig.)

  10. Chronic Pulmonary Aspergillosis Complicating Bronchial Atresia

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    Mazen O. Al-Qadi

    2014-01-01

    Full Text Available Bronchial atresia is a rare pulmonary developmental anomaly characterized by the presence of a focal obliteration of a segmental or lobar bronchial lumen. The lung distal to the atretic bronchus is typically emphysematous along with the presence of mucus filled ectatic bronchi (mucoceles. BA is usually asymptomatic but pulmonary infections can rarely develop in the emphysematous lung distal to the atretic bronchus. We present a unique case of chronic pulmonary aspergillosis (CPA in a patient with BA with no evidence of immune dysfunction. The patient was treated initially with voriconazole and subsequently underwent surgical excision of the involved area. On follow-up, she has done extremely well with no evidence for recurrence. In summary, we describe the first case of chronic pulmonary aspergillosis in an immunocompetent patient with bronchial atresia.

  11. Extrahepatic complications to cirrhosis and portal hypertension

    DEFF Research Database (Denmark)

    Møller, Søren; Henriksen, Jens H; Bendtsen, Flemming

    2014-01-01

    In addition to complications relating to the liver, patients with cirrhosis and portal hypertension develop extrahepatic functional disturbances of multiple organ systems. This can be considered a multiple organ failure that involves the heart, lungs, kidneys, the immune systems, and other organ...

  12. Role of Multislice Computed Tomography and Local Contrast in the Diagnosis and Characterization of Choanal Atresia

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    Khaled Al-Noury

    2011-01-01

    Full Text Available Objective. To illustrate the role of multislice computed tomography and local contrast instillation in the diagnosis and characterization of choanal atresia. To review the common associated radiological findings. Methods. We analyzed 9 pediatric patients (5 males and 4 females with suspected choanal atresia by multislice computed tomography. We recorded the type of atresia plate and other congenital malformations of the skull. Results. Multislice computed tomography with local contrast installed delineated the posterior choanae. Three patients had unilateral mixed membranous and bony atresia. Three patients had unilateral pure bony atresia. Only 1 of 7 patients have bilateral bony atresia. It also showed other congenital anomalies in the head region. One patient is with an ear abnormality. One patient had congenital nasal pyriform aperture stenosis. One of these patients had several congenital abnormalities, including cardiac and renal deformities and a hypoplastic lateral semicircular canal. Of the 6 patients diagnosed to have choanal atresia, 1 patient had esophageal atresia and a tracheoesophageal fistula. The remaining patients had no other CHARGE syndrome lesions. Conclusions. Local Contrast medium with the application of the low-dose technique helps to delineate the cause of the nasal obstruction avoiding a high radiation dose to the child.

  13. Identification and treatment of variation of extrahepatic bile duct in laparoscopic cholecystectomy

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    PENG Lei

    2015-10-01

    Full Text Available ObjectiveTo investigate the identification and treatment of variation of extrahepatic bile duct in laparoscopic cholecystectomy (LC, and to reduce the occurrence of bile duct injury. MethodsThis study included 60 patients who received LC in the People′s Hospital of Caidian District in Wuhan and had structural variation of extrahepatic bile duct found during the operation from January 2012 to January 2014. The clinical data were retrospectively analyzed, and the intraoperative and postoperative conditions were summarized. ResultsDuring operation, cystic duct variation was found in 32 cases, abnormal position of the point where the cystic duct joins the extrahepatic bile duct in 20 cases, the cystic duct and the common hepatic duct having the common wall before joining the common bile duct in 2 cases, aberrant bile duct in the gallbladder bed in 2 cases, and accessory hepatic duct in 4 cases. Fifty-one patients (85% successfully underwent LC; 9 patients (15% were converted to open surgery. All patients finished surgery successfully. There were 2 cases of postoperative complications; one patient developed residual stones in the bile duct, and bile leakage occurred in the other patient at one week after LC, who recovered after reoperation. All patients were cured and discharged, without severe complications such as intraperitoneal hemorrhage, infection, and intestinal injury. ConclusionIdentifying the structural variation of extrahepatic bile duct, dissecting the Calot′s triangle meticulously, and determining the type of variation of extrahepatic bile duct play important roles in LC and significantly reduce the incidence of bile duct injury.

  14. Duodenal Derotation and Extent Tapering Jejunoplasty as Primary Repair for Neonates With High Jejunal Atresia

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    Chih-Cheng Luo

    2010-10-01

    Conclusion: In very proximal high atresia, the extent of tapering is limited by the proximity of the ligament of Treitz. Duodenal derotation provides better access to the high atresia. The results of this limited experience suggest that the DDETJ procedure could provide an alternative therapy in patients with high jejunal atresia.

  15. Future developments in biliary stenting

    Directory of Open Access Journals (Sweden)

    Hair CD

    2013-06-01

    Full Text Available Clark D Hair,1 Divyesh V Sejpal21Department of Medicine, Section of Gastroenterology and Hepatology, Baylor College of Medicine, Houston, TX, USA; 2Department of Medicine, Section of Gastroenterology, Hofstra North Shore-LIJ School of Medicine, North Shore University Hospital, Manhasset, NY, USAAbstract: Biliary stenting has evolved dramatically over the past 30 years. Advancements in stent design have led to prolonged patency and improved efficacy. However, biliary stenting is still affected by occlusion, migration, anatomical difficulties, and the need for repeat procedures. Multiple novel plastic biliary stent designs have recently been introduced with the primary goals of reduced migration and improved ease of placement. Self-expandable bioabsorbable stents are currently being investigated in animal models. Although not US Food and Drug Administration approved for benign disease, fully covered self-expandable metal stents are increasingly being used in a variety of benign biliary conditions. In malignant disease, developments are being made to improve ease of placement and stent patency for both hilar and distal biliary strictures. The purpose of this review is to describe recent developments and future directions of biliary stenting.Keywords: plastic stents, self-expandable metal stents, drug eluting stents, bioabsorbable stents, malignant biliary strictures, benign biliary strictures

  16. Future developments in biliary stenting

    Science.gov (United States)

    Hair, Clark D; Sejpal, Divyesh V

    2013-01-01

    Biliary stenting has evolved dramatically over the past 30 years. Advancements in stent design have led to prolonged patency and improved efficacy. However, biliary stenting is still affected by occlusion, migration, anatomical difficulties, and the need for repeat procedures. Multiple novel plastic biliary stent designs have recently been introduced with the primary goals of reduced migration and improved ease of placement. Self-expandable bioabsorbable stents are currently being investigated in animal models. Although not US Food and Drug Administration approved for benign disease, fully covered self-expandable metal stents are increasingly being used in a variety of benign biliary conditions. In malignant disease, developments are being made to improve ease of placement and stent patency for both hilar and distal biliary strictures. The purpose of this review is to describe recent developments and future directions of biliary stenting. PMID:23837001

  17. Inaccurate preoperative imaging assessment on biliary anatomy not increases biliary complications after living donor liver transplantation

    International Nuclear Information System (INIS)

    Xu Xiao; Wei Xuyong; Ling Qi; Wang Kai; Bao Haiwei; Xie Haiyang; Zhou Lin; Zheng Shusen

    2012-01-01

    Backgrounds and aims: Accurate assessment of graft bile duct is important to plan surgical procedure. Magnetic resonance cholangiopancreatography (MRCP) has become an important diagnostic procedure in evaluation of pancreaticobiliary ductal abnormalities and has been reported as highly accurate. We aim to estimate the efficacy of preoperative MRCP on depicting biliary anatomy in living donor liver transplantation (LDLT), and to determine whether inaccurate preoperative imaging assessment would increase the biliary complications after LDLT. Methods: The data of 118 cases LDLT were recorded. Information from preoperative MRCP was assessed using intraoperative cholangiography (IOC) as the gold standard. The possible risk factors of recipient biliary complications were analyzed. Results: Of 118 donors, 84 had normal anatomy (type A) and 34 had anatomic variants (19 cases of type B, 9 cases of type C, 1 case of type E, 2 cases of type F and 3 cases of type I) confirmed by IOC. MRCP correctly predicted all 84 normal cases and 17 of 34 variant cases, and showed an accuracy of 85.6% (101/118). The incidence of biliary complications was comparable between cases with accurate and inaccurate classification of biliary tree from MRCP, and between cases with normal and variant anatomy of bile duct. While cases with graft duct opening ≤5 mm showed a significant higher incidence of total biliary complications (21.1% vs. 6.6%, P = 0.028) and biliary stricture (10.5% vs. 1.6%, P = 0.041) compared with cases with large duct opening >5 mm. Conclusion: MRCP could correctly predict normal but not variant biliary anatomy. Inaccurate assessment of biliary anatomy from MRCP not increases the rate of biliary complications, while small-sized graft duct may cause an increase in biliary complications particularly biliary stricture after LDLT.

  18. Gallbladder varices in extrahepatic portal venous obstruction: demonstration by intravenous

    International Nuclear Information System (INIS)

    Gulati, M.

    2002-01-01

    Full text: We performed a prospective study to determine frequency of presence of gallbladder varices (GBV) by intravenous CT portography (CTP) in patients with extrahepatic portal venous obstruction (EHPVO). 90 patients (age range: 2-55 years) with EHPVO (initially diagnosed on abdominal sonography) underwent CTP using a subsecond helical CT scanner. Axial overlapping sections of 2mm were obtained with collimation 3mm and table speed 4.5mm/sec (pitch 1.5). Presence and patterns of GBV were studied. CTP demonstrated GBV in 54 (60%) of 90 patients.GBV were said to be present when one or more of the following findings were seen: diffuse wall enhancement (26/90), pinpoint areas of enhancement in GB wall (33/90), obvious large collaterals in GB wall (8/90) and pericholecystic collaterals (49/90). Presence of GBV did not correlate with the site and extent of EHPVO. Contiguous intrahepatic collaterals extending from GB bed to intrahepatic portal vein branches were seen in 41 of 54 (76%) of patients with GBV, suggesting the role of GBV serving as bridging portoportal collaterals. Hepatic perfusion defects were seen in 5/54 patients with GBV and were not seen in remaining 36/90 patients of EHPVO. GB calculi were seen in only 4/54 cases with GBV (as determined on sonography) suggesting no increase in risk for cholelithiasis. GBV commonly develop as bridging collaterals in patients with EHPVO. CTP is very useful in detecting these varices and planning biliary surgery, given the frequency of iatrogenic surgical bleeding in these patients. Copyright (2002) Blackwell Science Pty Ltd

  19. Determining the optimal portal blood volume in a shunt before surgery in extrahepatic portal hypertension

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    Yurchuk Vladimir A

    2016-04-01

    Full Text Available The aim of the study: To determine the necessary shunt diameter and assess the optimal portal blood volume in a shunt in children with extrahepatic portal hypertension before the portosystemic shunt surgery. Changes in the liver hemodynamics were studied in 81 children aged from 4 to 7 years with extrahepatic portal hypertension. We established that it is necessary to calculate the shunt diameter and the blood volume in a shunt in patients with extrahepatic portal hypertension before the portosystemic shunt surgery. It allows us to preserve the hepatic portal blood flow and effectively decrease the pressure in the portal system. Portosystemic shunt surgery in patients with extrahepatic portal hypertension performed in accordance with the individualized shunt volume significantly decreases portal pressure, preserves stable hepatic hemodynamics and prevents gastro-esophageal hemorrhage.

  20. Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence: two case reports and a review of the literature.

    Science.gov (United States)

    Bjørsum-Meyer, Thomas; Herlin, Morten; Qvist, Niels; Petersen, Michael B

    2016-12-21

    The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases. Furthermore, we aimed to collect existing knowledge in the embryopathogenesis and genetics in order to discuss a possible link between the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome. Our first case was a white girl delivered by caesarean section at 37 weeks of gestation; our second case was a white girl born at a gestational age of 40 weeks. A co-occurrence of vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome was diagnosed in both cases. We performed a systematic literature search in PubMed ((VACTERL) OR (VATER)) AND ((MRKH) OR (Mayer-Rokitansky-Küster-Hauser) OR (mullerian agenesis) OR (mullerian aplasia) OR (MURCS)) without limitations. A similar search was performed in Embase and the Cochrane library. We added two cases from our local center. All cases (n = 9) presented with anal atresia and renal defect. Vertebral defects were present in eight patients. Rectovestibular fistula was confirmed in seven patients. Along with the uterovaginal agenesis, fallopian tube aplasia appeared in five of nine cases and in two cases ovarian involvement also existed. The co-occurrence of the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal

  1. Supra-transumbilical laparotomy (STL approach for small bowel atresia repair: Our experience and review of the literature

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    Ernesto Leva

    2013-01-01

    Full Text Available Background: Supra-Transumbilical Laparotomy (STL has been used in paediatric surgery for a broad spectrum of abdominal procedures. We report our experience with STL approach for small bowel atresia repair in newborns and review previous published series on the topic. Patients and Methods: Fourteen patients with small bowel atresia were treated via STL approach at our Institution over a 5-year period and their charts were retrospectively reviewed. Results: STL procedure was performed at mean age of 3.1 day. No malrotation disorders were detected with pre-operative contrast enema. Eight patients (54.1% presented jejunal atresia, five (35.7% ileal atresia, and one (7.1% multiple ileal and jejunal atresias. Standard repair with primary end-to-back anastomosis was performed in all but one patient. In the newborn with multiple atresia, STL incision was converted in supra-umbilical transverse incision due to difficulty of exposition. After surgery, one patient developed anastomotic stricture, and another developed occlusion due to adhesions: Both infants required second laparotomy. No infections of the umbilical site were recorded, and cosmetic results were excellent in all patients. Conclusions: Increasing evidence suggests that STL approach for small bowel atresia is feasible, safe and provides adequate exposure for small bowel atresia surgery. When malrotation and colonic/multiple atresia are pre-operatively ruled out, STL procedure can be choosen as first approach.

  2. Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida Sacrococcygeal agenesis association and anal atresia in mixed breed cats

    Directory of Open Access Journals (Sweden)

    Felipe Purcell de Araújo

    2009-09-01

    Full Text Available O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

  3. A US Multicenter Study of Safety and Efficacy of Fully Covered Self-Expandable Metallic Stents in Benign Extrahepatic Biliary Strictures.

    Science.gov (United States)

    Saxena, Payal; Diehl, David L; Kumbhari, Vivek; Shieh, Frederick; Buscaglia, Jonathan M; Sze, Wilson; Kapoor, Sumit; Komanduri, Srinadh; Nasr, John; Shin, Eun Ji; Singh, Vikesh; Lennon, Anne Marie; Kalloo, Anthony N; Khashab, Mouen A

    2015-11-01

    Endoscopic therapy is considered first line for management of benign biliary strictures (BBSs). Placement of plastic stents has been effective but limited by their short-term patency and need for repeated procedures. Fully covered self-expandable metallic stents (FCSEMSs) offer longer-lasting biliary drainage without the need for frequent exchanges. The aim of this study was to assess the efficacy and safety of FCSEMS in patients with BBS. A retrospective review of all patients who underwent ERCP and FCSEMS placement at five tertiary referral US hospitals was performed. Stricture resolution and adverse events related to ERCP and/or stenting were recorded. A total of 123 patients underwent FCSEMS placement for BBS and 112 underwent a subsequent follow-up ERCP. The mean age was 62 years (±15.6), and 57% were males. Stricture resolution occurred in 81% of patients after a mean of 1.2 stenting procedures (mean stent dwell time 24.4 ± 2.3 weeks), with a mean follow-up of 18.5 months. Stricture recurrence occurred in 5 patients, and 3 patients required surgery for treatment of refractory strictures. Stent migration (9.7%) was the most common complication, followed by stent occlusion (4.9%), cholangitis (4.1%), and pancreatitis (3.3%). There was one case of stent fracture during removal, and one stent could not be removed. There was one death due to cholangitis. Majority of BBS can be successfully managed with 1-2 consecutive FCSEMS with stent dwell time of 6 months.

  4. Evaluation of the angiographic findings for extrahepatic arterial supply to primary hepatic cancer and interventional therapy

    International Nuclear Information System (INIS)

    Wang Weiyu; Lv Weifu; Hou Changlong; Zhang Xingming; Zhang Zhengfeng; Lu Dong; Gao Zonggen

    2007-01-01

    Objective: To study the angiographic characteristics of extrahepatic arterial supply for primary hepatic cancer (PHC)and the significance of interventional therapy. Methods: 32 cases of primary. hepatic cancer were undertaken routine celiac arterial angiography and explored the extrahepatic arterial supply for the tumor, then followed by superselective transcatheter arterial chemoembolization (TACE). Results: 37 extrahepatic feeding arteries to hepatic cancers in 32 cases were found including 12 from superior mesenteric arteries(SMA), 9 right inferior phrenic arteries (RIPA), 1 left inferior phrenic arteries (LIPA), 2 pancreatic arterial arch, 1 right internal thoracic artery(RITA), 1 right intercostal artery(RICA), 6 left gastric arteries (LGA), 1 splenic artery, 2 omental arteries (OTA), 2 gastroduodenal arteries. The most common extrahepatic feeding arteries were originated from SMA and RIPA. The rest 33 were performed with superselective transcatheter arterial chemoembolization and the other 4 with only transcatheter arterial chemotherapeutic perfusion due to failure of superselective catheterization. Conclusion: The extrahepatic feeding artery is commonly seen with various kinds and also necessary for interventional treatment same as the primary ones for hepatic cancers. (authors)

  5. A study on ultrasonographic analysis of jaundiced patients

    International Nuclear Information System (INIS)

    Lee, Kyung Hi; Yeon, Kyung Mo; Kim, Chu Wan

    1981-01-01

    In 101 jaundiced patients, gray scale ultrasonography of longitudinal scan in RAO position demonstrated the measurable extrahepatic biliary system in 73 patients; 17 cases (50%) of those with nonobstructive jaundice and 56 cases (84%) of those with obstructive jaundice. The size of the internal diameter of extrahepatic biliary system indicated that obstructive jaundice was best differentiated from non-obstructive jaundice when diameter above 7mm served as abnormal extrahepatic duct in jaundiced patients, giving sensitivity 85.5% specificity 97% and diagnostic accuracy 89.5% by decision matrix analysis. The overall etiological diagnostic accuracy in obstructive jaundice was 40% which had higher one in choledocholithiasis and pancreas head carcinoma than other diseases. The sonography should be imaging procedure of choice in differential diagnosis of jaundiced patients, which is simple, safe, noninvasive and has high diagnostic accuracy in differentiation between the two

  6. I-131 rose bengal excretion test is not dead

    International Nuclear Information System (INIS)

    Antico, V.F.; Denhartog, P.; Ash, J.M.; Gilday, D.L.; Houle, S.

    1985-01-01

    One hundred and thirty I-131 Rose Bengal Excretion Studies (RBI) were performed on 84 patients over nine years. In 90% (56/60) of cases with biliary atresia, the 72-hour RBI was less than or equal to 7%. In only 12.5% (3/24) of cases with neonatal hepatitis was the 72-hour RBI less than or equal to 7%. The accuracy of the test was 91% with a specificity of 88%. Thirty patients later were studied following a Kasai procedure. The RBI test reliably predicted the patency of the anastomosis. The authors conclude that the 72-hour RBI is a reliable test in the diagnosis of biliary atresia and in the documentation of biliary patency following surgery, provided adequate care is taken in stool collection and measurement

  7. Etiological study on isolated esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Szendrey, T; Danyi, G; Czeizel, A

    1985-01-01

    A study group of 160 index patients with isolated esophageal atresia, a control group of 160 matched healthy controls, and the first-degree relatives of patients and controls were examined; epidemiological, family planning, teratological, and genetic data were obtained by personal interview in the study and control groups. One half of the index patients were male. Intrauterine growth retardation, a higher proportion of mothers under 19 or over 30 years of age, and less skilled professions of the parents were found in the study group. There were more extramarital conceptions, more pregnancies in spite of the use of contraceptive pills, and more delayed conceptions in index patients mothers. The teratogens studied did not have an obvious pathological effect here. The sib occurrence of isolated esophageal atresia was 0.43%, which did not correspond well to the expected figure of 1.34% based on the polygenic model.

  8. Biodegradable biliary stent implantation in the treatment of benign bilioplastic-refractory biliary strictures: preliminary experience.

    Science.gov (United States)

    Mauri, Giovanni; Michelozzi, Caterina; Melchiorre, Fabio; Poretti, Dario; Tramarin, Marco; Pedicini, Vittorio; Solbiati, Luigi; Cornalba, Gianpaolo; Sconfienza, Luca Maria

    2013-12-01

    To evaluate feasibility, safety, and outcome of patients treated with biodegradable biliary stents for benign biliary stenosis refractory to other treatments. Between March 2011 and September 2012, ten patients (seven men, three women; age 59 ± 7 years) with recurrent cholangitis due to postsurgical biliary stricture, previous multiple unsuccessful (two to five) bilioplasties, and unsuitability for surgical/endoscopic repair underwent percutaneous implantation of a biodegradable biliary stent. Patients were followed-up clinically and with ultrasound at 1, 3 and 6 months, and then at 6-month intervals. Stent implantation was always feasible. No immediate major or minor complications occurred. In all patients, 48-h cholangiographic control demonstrated optimal stent positioning and stenosis resolution. In a median follow-up time of 16.5 months (25th-75th percentiles = 11-20.25 months) no further invasive treatment was needed. Three patients experienced transient episodes of cholangitis. Neither re-stenosis nor dilatation of the biliary tree was documented during follow-up. No stent was visible at the 6-month follow-up. Percutaneous placement of biodegradable biliary stents represents a new option in treating benign biliary stenoses refractory to treatment with bilioplasty. This technique seems to be feasible, effective and free from major complications. Further investigations are warranted to confirm our preliminary results.

  9. Imaging findings of bronchial atresia in fetuses, neonates and infants

    Energy Technology Data Exchange (ETDEWEB)

    Alamo, Leonor; Meuli, Reto [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Diagnostic and Interventional Radiology, Lausanne (Switzerland); Vial, Yvan [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Obstetrics and Gynecology, Lausanne (Switzerland); Gengler, Carole [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Pathology, Lausanne (Switzerland)

    2016-03-15

    Congenital lung malformations are increasingly detected before birth. However, bronchial atresia is rarely identified in utero and not always recognized in neonates. There are two types of atresia: (1) proximal, located at the level of the mainstem or the proximal lobar bronchi, which is extremely rare and usually lethal during pregnancy, causing a tremendous volume increase of the distal involved lung with secondary hypoplasia of the normal lung, and (2) peripheral, located at the segmental/subsegmental bronchial level, which may present as an isolated lesion or as part of a complex congenital malformation. Prenatal findings are mostly nonspecific. Postnatal exams show overinflated lung areas and focal bronchial dilations. The typical fluid-filled bronchoceles are not always observed in neonates but develop progressively in the first months of life. This pictorial essay describes the spectrum of imaging findings of bronchial atresia in fetuses, neonates and infants. (orig.)

  10. Pancreaticobiliary maljunction and biliary cancer.

    Science.gov (United States)

    Kamisawa, Terumi; Kuruma, Sawako; Tabata, Taku; Chiba, Kazuro; Iwasaki, Susumu; Koizumi, Satomi; Kurata, Masanao; Honda, Goro; Itoi, Takao

    2015-03-01

    Pancreaticobiliary maljunction (PBM) is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall. Japanese clinical practice guidelines on how to deal with PBM were made in 2012, representing a world first. According to the 2013 revision to the diagnostic criteria for PBM, in addition to direct cholangiography, diagnosis can be made by magnetic resonance cholangiopancreatography (MRCP), 3-dimensional drip infusion cholangiography computed tomography, endoscopic ultrasonography (US), or multiplanar reconstruction images by multidetector row computed tomography. In PBM, the common channel is so long that sphincter action does not affect the pancreaticobiliary junction, and pancreatic juice frequently refluxes into the biliary tract. Persistence of refluxed pancreatic juice injures epithelium of the biliary tract and promotes cancer development, resulting in higher rates of carcinogenesis in the biliary tract. In a nationwide survey, biliary cancer was detected in 21.6% of adult patients with congenital biliary dilatation (bile duct cancer, 32.1% vs. gallbladder cancer, 62.3%) and in 42.4% of PBM patients without biliary dilatation (bile duct cancer, 7.3% vs. gallbladder cancer, 88.1%). Pathophysiological conditions due to pancreatobiliary reflux occur in patients with high confluence of pancreaticobiliary ducts, a common channel ≥6 mm long, and occlusion of communication during contraction of the sphincter. Once the diagnosis of PBM is established, immediate prophylactic surgery is recommended. However, the surgical strategy for PBM without biliary dilatation remains controversial. To detect PBM without biliary dilatation early, MRCP is recommended for patients showing gallbladder wall thickening on screening US under suspicion of PBM.

  11. Congenital external auditory canal atresia and stenosis: temporal bone CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Hoon; Kim, Bum Soo; Jung, So Lyung; Kim, Young Joo; Chun, Ho Jong; Choi, Kyu Ho; Park, Shi Nae [College of Medicine, Catholic Univ. of Korea, Seoul (Korea, Republic of)

    2002-04-01

    To determine the computed tomographic (CT) findings of atresia and stenosis of the external auditory canal (EAC), and to describe associated abnormalities in surrounding structures. We retrospectively reviewed the axial and coronal CT images of the temporal bone in 15 patients (M:F=8:7;mean age, 15.8 years) with 16 cases of EAC atresia (unilateral n=11, bilateral n=1) and EAC stenosis (unilateral n=3). Associated abnormalities of the EAC, tympanic cavity, ossicles, mastoid air cells, eustachian tube, facial nerve course, mandibular condyle and condylar fossa, sigmoid sinus and jugular bulb, and the base of the middle cranial fossa were evaluated. Thirteen cases of bony EAC atresia (one bilateral), with an atretic bony plate, were noted, and one case of unilateral membranous atresia, in which a soft tissue the EAC. A unilateral lesion occurred more frequently on the right temporal bone (n=8, 73%). Associated abnormalities included a small tympanic cavity (n=8, 62%), decreased mastoid pneumatization (n=8, 62%), displacement of the mandibular condyle and the posterior wall of the condylar fossa (n=7, 54%), dilatation of the Eustachian tube (n=7, 54%), and inferior displacement of the temporal fossa base (n=8, 62%). Abnormalities of ossicles were noted in the malleolus (n=12, 92%), incus (n=10, 77%) and stapes (n=6, 46%). The course of the facial nerve was abnormal in four cases, and abnormality of the auditory canal was noted in one. Among three cases of EAC stenosis, ossicular aplasia was observed in one, and in another the location of the mandibular condyle and condylar fossa was abnormal. In the remaining case there was no associated abnormality. Atresia of the EAC is frequently accompanied by abnormalities of the middle ear cavity, ossicles, and adjacent structures other than the inner ear. For patients with atresia and stenosis of this canal, CT of the temporal bone is essentially helpful in evaluating these associated abnormalities.

  12. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo; Park, Sang-Hyub; Koo, Hyun Jung; Cho, Young Hoon; Lee, Eunsol [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea, Republic of)

    2014-08-15

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  13. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    International Nuclear Information System (INIS)

    Goo, Hyun Woo; Park, Sang-Hyub; Koo, Hyun Jung; Cho, Young Hoon; Lee, Eunsol

    2014-01-01

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  14. MicroRNAs: New Insight in Modulating Follicular Atresia: A Review

    Directory of Open Access Journals (Sweden)

    Tesfaye Worku

    2017-02-01

    Full Text Available Our understanding of the post-transcriptional mechanisms involved in follicular atresia is limited; however, an important development has been made in understanding the biological regulatory networks responsible for mediating follicular atresia. MicroRNAs have come to be seen as a key regulatory actor in determining cell fate in a wide range of tissues in normal and pathological processes. Profiling studies of miRNAs during follicular atresia and development have identified several putative miRNAs enriched in apoptosis signaling pathways. Subsequent in vitro and/or in vivo studies of granulosa cells have elucidated the functional role of some miRNAs along with their molecular pathways. In particular, the regulatory roles of some miRNAs have been consistently observed during studies of follicular cellular apoptosis. Continued work should gradually lead to better understanding of the role of miRNAs in this field. Ultimately, we expect this understanding will have substantial benefits for fertility management at both the in vivo or/and in vitro levels. The stable nature of miRNA holds remarkable promise in clinical use as a diagnostic tool and in reproductive medicine to solve the ever-increasing fertility problem. In this review, we summarize current knowledge of the involvement of miRNAs in follicular atresia, discuss the challenges for further work and pinpoint areas for future research.

  15. Seasonal prevalence and intensity of follicular atresia in Baltic cod Gadus morhua callarias L

    DEFF Research Database (Denmark)

    Kraus, Gerd; Tomkiewicz, Jonna; Diekmann, R.

    2008-01-01

    of the physical disector method and volume fraction (Delesse principle). Atretic oocytes were observed in 32% of the ovaries. Prevalence of atresia was independent of female size, but increased significantly with declining female condition from prespawning and through the spawning stages. The relative intensity...... of atresia, i.e. number of atretic oocytes in relation to normally developed vitellogenic oocytes, was low amounting to 1.4% on average. Similar to prevalence, relative intensity of atresia differed significantly between maturity stages and increased with decreasing female condition. The population egg loss......In the present study, 307 ovaries of eastern Baltic cod Gadus morhua callarias sampled during the prespawning and spawning season 2000 were analysed histologically to estimate the seasonal prevalence and intensity of atresia. The number of atretic oocytes per ovary was estimated using a combination...

  16. Extrahepatic Portal Hypertension following Liver Transplantation: a Rare but Challenging Problem

    Directory of Open Access Journals (Sweden)

    B. Malassagne

    1998-01-01

    Full Text Available This study reports our experience of 8 cases of extrahepatic portal hypertension after 273 orthotopic liver transplantations in 244 adult patients over a 10- year period. The main clinical feature was ascites, and the life-threatening complication was variceal bleeding. Extrahepatic portal hypertension was caused by portal vein stenosis in 6 patients, and left-sided portal hypertension in 2 patients after inadventent ligation of portal venous tributaries or portasystemic shunts. All patients with portal vein stenosis had complete relief of portal hypertension after percutaneous transhepatic venoplasty (n=4 or surgical reconstruction (n=2, after a median follow-up of 33 (range: 6–62 months. Of the 2 patients with left-sided portal hypertension, one died after splenectomy and one rebled 6 months after left colectomy. This study suggests that extrahepatic portal hypertension is a series complication after liver transplantation that could be prevented by meticulous portal anastomosis and closure of portal tributaries or portasystemic shunts to improve the portal venous flow. However, any ligation has to be performed under ultrasound guidance to avoid inadventent venous ligations.

  17. Magnetic resonance cholangiographic (MRCP) features of ischemic-type biliary lesions (ITBL). A case-control study; Magnetresonanz-Cholangiografie (MRCP) bei Patienten mit Ischemic-Type Biliary Lesions (ITBL). Eine Fall-Kontroll-Studie

    Energy Technology Data Exchange (ETDEWEB)

    Collettini, F.; Kroencke, T.J.; Renz, D.M.; Bucourt, M. de; Poellinger, A. [Charite, Berlin (Germany). Klinik fuer Radiologie; Heidenhain, C.; Neuhaus, P. [Charite, Berlin (Germany). Klinik fuer Allgemein-, Visceral- und Transplantationschirurgie CVK

    2011-08-15

    Purpose: To evaluate the spectrum of MR cholangiography (MRCP) features of ischemic-type biliary lesions (ITBL) after orthotopic liver transplantation (OLT). Materials and Methods: 30 patients (16 m, 14 f) with an average age of 52 years (9 - 69 y) were examined in two 1.5 MR units using breath-hold 2D-SS-FSE-sequences and 3D-MRCP sequences. 20 of the 30 patients had an established ITBL, and the remaining 10 patients were post-OLT controls. MRCPs were evaluated independently by two experienced radiologists that were blinded to the clinical history as well as the results of other imaging modalities. All images were analyzed for the presence of 16 different pathological features. Differences between ITBL patients and controls were analyzed using the Mann-Whitney-U Test. Inter-rater variability was tested using the Cohen's Kappa test. Results: Abnormal findings of bile ducts were seen in all patients. The most common findings were (in percentage for reader 1/2) intrahepatic bile duct dilatation (95 %/95 %) and extrahepatic bile duct stenoses (95 %/85 %), followed by intrahepatic main duct stenoses (90 %/95 %) and segmental duct stenoses (85 %/85 %). Differences between ITBL patients and controls were significant for most of the analyzed features (Mann-Whitney-U test, p < 0.05). For 12 of 16 features, there was substantial or almost perfect agreement (K = 0.61 - 1.00), for 2 of 16 features moderate agreement (K = 0.41- 0.60) and for 2 of 16 features fair agreement (K < 0.40). Conclusion: In patients with ITBL, MR cholangiography reveals characteristic features that may allow differentiation from other biliary complications after liver transplantation. (orig.)

  18. Epidemiology of small intestinal atresia in Europe

    DEFF Research Database (Denmark)

    Best, Kate E; Tennant, Peter W G; Addor, Marie-Claude

    2012-01-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe....

  19. EUS-guided rendezvous for difficult biliary cannulation using a standardized algorithm: a multicenter prospective pilot study (with videos).

    Science.gov (United States)

    Iwashita, Takuji; Yasuda, Ichiro; Mukai, Tsuyoshi; Iwata, Keisuke; Ando, Nobuhiro; Doi, Shinpei; Nakashima, Masanori; Uemura, Shinya; Mabuchi, Masatoshi; Shimizu, Masahito

    2016-02-01

    Biliary cannulation is necessary in therapeutic ERCP for biliary disorders. EUS-guided rendezvous (EUS-RV) can salvage failed cannulation. Our aim was to determine the safety and efficacy of EUS-RV by using a standardized algorithm with regard to the endoscope position in a prospective study. EUS-RV was attempted after failed cannulation in 20 patients. In a standardized approach, extrahepatic bile duct (EHBD) cannulation was preferentially attempted from the second portion of the duodenum (D2) followed by additional approaches to the EHBD from the duodenal bulb (D1) or to the intrahepatic bile duct from the stomach, if necessary. A guidewire was placed in an antegrade fashion into the duodenum. After the guidewire was placed, the endoscope was exchanged for a duodenoscope to complete the cannulation. The bile duct was accessed from the D2 in 10 patients, but from the D1 in 5 patients and the stomach in 4 patients because of no dilation or tumor invasion at the distal EHBD. In the remaining patient, biliary puncture was not attempted due to the presence of collateral vessels. The guidewire was successfully manipulated in 80% of patients: 100% (10/10) with the D2 approach and 66.7% (6/9) with other approaches. The overall success rate was 80% (16/20). Failed EUS-RV was salvaged with a percutaneous approach in 2 patients, repeat ERCP in 1 patient, and conservative management in 1 patient. Minor adverse events occurred in 15% of patients (3/20). EUS-RV is a safe and effective salvage method. Using EUS-RV to approach the EHBD from the D2 may improve success rates. Copyright © 2016 American Society for Gastrointestinal Endoscopy. Published by Elsevier Inc. All rights reserved.

  20. Impact of unilateral conductive hearing loss due to aural atresia on academic performance in children.

    Science.gov (United States)

    Kesser, Bradley W; Krook, Kaelyn; Gray, Lincoln C

    2013-09-01

    This study evaluates the effect of unilateral conductive hearing loss secondary to aural atresia on elementary school children's academic performance. Case control survey and review of audiometric data. One hundred thirty-two surveys were mailed to families of children with aural atresia, and 48 surveys were sent to families of children with unilateral sensorineural hearing loss (SNHL) to identify rates of grade retention, use of any resource, and behavioral problems. Audiometric data of the cohort were tabulated. Of the 40 atresia patients, none repeated a grade, but 65% needed some resources: 12.5% currently use a hearing aid, 32.5% use(d) a frequency-modulated system in school, 47.5% had an Individualized Education Plan, and 45% utilized speech therapy. Compared to the unilateral SNHL group and a cohort of children with unilateral SNHL in an earlier study, children with unilateral atresia were less likely to repeat a grade. Children in both unilateral atresia and SNHL groups were more likely to utilize some resource in the academic setting compared to the unilateral SNHL children in the prior study. Unilateral conductive hearing loss due to aural atresia has an impact on academic performance in children, although not as profound when compared to children with unilateral SNHL. The majority of these children with unilateral atresia utilize resources in the school setting. Parents, educators, and health care professionals should be aware of the impact of unilateral conductive hearing loss and offer appropriate habilitative services. Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  1. Readability and quality assessment of websites related to microtia and aural atresia.

    Science.gov (United States)

    Alamoudi, Uthman; Hong, Paul

    2015-02-01

    Many parents and children utilize the Internet for health-related information, but the quality of these websites can vary. The objective of this study was to assess the quality and readability of microtia and aural atresia related websites. The search engine Google was queried with the terms 'microtia' and 'aural atresia.' The first 30 results were evaluated, and those websites containing original information written in English were reviewed. Quality of content was assessed with the DISCERN instrument, and readability was assessed with the Flesch-Kincaid Reading Grade Level (FKGL) and the Flesch Reading Ease Score (FRES) tests. Each website was also reviewed for ownership and the date of last update. Sixteen microtia and 14 aural atresia websites were included for full review. The mean DISCERN score for microtia websites was 54.4 (SD=8.3), and for aural atresia websites it was 47.6 (SD=10.7), which indicates 'good' and 'fair' quality of content, respectively. Readability assessments showed an average reading level requiring a grade 10 education on FKGL, and only one microtia (6.3%) and one aural atresia (7.1%) websites were deemed to be at 'reasonable' reading level on FRES. High-quality websites that are considered easily comprehensible to the general public were lacking. Since parents and children may use websites when making treatment decisions, physicians should be aware of the quality of health information pertaining to their area of expertise available on the Internet. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  2. Postoperative Course and Complications after Pull-through Vaginoplasty for Distal Vaginal Atresia.

    Science.gov (United States)

    Mansouri, Roshanak; Dietrich, Jennifer E

    2015-12-01

    To report the usual postoperative course and complications after pull-through vaginoplasty for isolated distal vaginal atresia. Retrospective chart review at Texas Children's Hospital of all patients who were diagnosed with isolated distal vaginal atresia and underwent pull-through vaginoplasty during the study time frame. None. Postoperative complications such as vaginal stenosis or infection and postoperative vaginal diameter. Sixteen patients were identified and charts were reviewed. Patients were initially evaluated by pelvic magnetic resonance imaging and found to have distended hematometrocolpos with distal vaginal atresia. All patients underwent pull-through vaginoplasty with similar operative techniques. The average distance from the perineum to the level of the obstruction was 1.84 ± 1.2 cm. Two patients, both with obstructions at greater than 3 cm, experienced stricture formation postoperatively. Four patients (25%) experienced postoperative vaginitis. One patient (6.25%) experienced a postoperative urinary tract infection. Two groups (3 cm or less versus greater than 3 cm) were compared, and the presence of stricture was statistically different based on mean centimeters from perineum prior to pull-through vaginoplasty (P = .038). Distal vaginal atresia is managed with pull-through vaginoplasty. Atresias that extend greater than 3 cm from the perineum are at increased risk for vaginal stricture formation and should be followed to monitor for their formation. Other complications are infrequent and minor. Copyright © 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  3. Non-Alcoholic Fatty Liver Disease and Extra-Hepatic Cancers

    Directory of Open Access Journals (Sweden)

    Claudia Sanna

    2016-05-01

    Full Text Available Non-alcoholic fatty liver disease (NAFLD is a leading cause of chronic liver disease but the second cause of death among NAFLD patients are attributed to malignancies at both gastrointestinal (liver, colon, esophagus, stomach, and pancreas and extra-intestinal sites (kidney in men, and breast in women. Obesity and related metabolic abnormalities are associated with increased incidence or mortality for a number of cancers. NAFLD has an intertwined relationship with metabolic syndrome and significantly contributes to the risk of hepatocellular carcinoma (HCC, but recent evidence have fuelled concerns that NAFLD may be a new, and added, risk factor for extra-hepatic cancers, particularly in the gastrointestinal tract. In this review we critically appraise key studies on NAFLD-associated extra-hepatic cancers and speculate on how NAFLD may influence carcinogenesis at these sites.

  4. Successful extracorporeal shock wave lithotripsy (ESWL) treatment of a symptomatic massive biliary stone proximal to an anastomotic biliary stricture.

    Science.gov (United States)

    Muratori, Rosangela; Mandolesi, Daniele; Galaverni, Maria Cristina; Azzaroli, Francesco

    2017-06-01

    Postoperative benign biliary stricture in the anastomotic site is one of the most common complications of biliary-enteric anastomosis, with a rate of 6.87% after 2-13 years of follow-up. If untreated, biliary strictures can induce other complications such as recurrent cholangitis, intrahepatic stones, pancreatitis and secondary biliary cirrhosis. We report our experience with extracorporeal shock wave lithotripsy (ESWL) in a patient with a massive symptomatic stone proximal to an anastomotic biliary stricture.

  5. Hepatobiliary scintigraphy with 99mTc-PIPIDA in the evaluation of neonatal jaundice

    International Nuclear Information System (INIS)

    Majd, M.; Reba, R.C.; Altman, R.P.

    1981-01-01

    Hepatobiliary scintigraphy with technetium 99m-labeled p-isopropylacetanilido iminodiacetic acid (99mTc-PIPIDA) was used to evaluate 22 neonates with mixed jaundice. Ten patients were proved to have biliary atresia; ten others were diagnosed as having neonatal hepatitis. In the remaining two, jaundice was secondary to prolonged hyperalimentation. Initial studies in all ten patients with biliary atresia showed no evidence of excretion of the tracer into the intestinal tract. Following three to seven days of oral administration of phenobarbital, repeat studies were performed in six of the ten patients. None showed evidence of excretion. Initial studies of the 12 patients with intrahepatic cholestasis showed definite excretion in five, questionable evidence of excretion in two, and no demonstrable excretion in five. Studies after phenobarbital therapy in five of the seven patients with questionable or no excretion on the initial studies showed definite excretion in four. Only in one patient who had poor hepatic extraction did the phenobarbital therapy not change the scintigraphic pattern. The authors conclude that hepatobiliary scintigraphy with 99mTc-PIPIDA after three to seven days of phenobarbital therapy is a highly accurate test for differentiating biliary atresia from other causes of neonatal jaundice

  6. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar Angiographic study of pulmonary circulation in tetralogy of Fallot with pulmonary atresia

    Directory of Open Access Journals (Sweden)

    Marco Aurélio Santos

    2005-02-01

    Full Text Available OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1 veia pulmonar encunhada, 2 colaterais aortopulmonares, 3 aorta torácica e 4 ductus arteriosus e/ou shunt sistêmico pulmonar. RESULTADOS: Dos 56 pacientes, 15 tinham o suprimento sangüíneo pulmonar através de colaterais aortopulmonares, em 36 o suprimento sangüíneo pulmonar era feito isoladamente pelo ductus arteriosus e em 5 pelo ductus arteriosus e colaterais aortopulmonares. Conforme a presença ou ausência de estruturas vasculares que compõem a circulação pulmonar na tetralogia de Fallot com atresia pulmonar e do tipo de perfusão vascular pulmonar, os doentes foram classificados em 6 tipos. CONCLUSÃO: Em função da grande complexidade e extrema variabilidade do suprimento sangüíneo pulmonar na tetralogia de Fallot com atresia pulmonar torna-se possível, com este tipo de abordagem, a obtenção de informações, suficientemente necessárias, para o correto manuseio clínico-cirúrgico.OBJECTIVE: To identify the types of pulmonary vascular blood supply in tetralogy of Fallot with pulmonary atresia by use of hemodynamic study. METHODS: Fifty-six patients with tetralogy of Fallot and pulmonary atresia, and ages ranging from 20 days to 4 years, underwent cineangiocardiographic study with contrast medium injections in the following vascular structures: 1 wedged pulmonary vein; 2 aortopulmonary collaterals; 3 thoracic aorta; and 4 ductus arteriosus or systemic-pulmonary shunt. RESULTS: In the 56 patients studied, pulmonary blood was supplied as follows: in 15, by aortopulmonary collaterals; in 36, only by the ductus arteriosus; and in 5, by the ductus

  7. Reoperation of Anastomotic Stricture after Oesophageal Atresia Repair: An Uncommon Event

    Directory of Open Access Journals (Sweden)

    A L Azakpa

    2017-01-01

    Full Text Available Oesophageal atresia is a common malformation in which the survival rate in developed countries is around 90%, while its mortality remains very high in developing countries. Oesophageal stricture post-oesophageal atresia repair is traditionally treated by non-surgical approach. However, surgical resection of the oesophageal stricture may be necessary after the failure of dilations. We report one case of refractory oesophageal stricture post-EA repair in a 3-year-old girl, who underwent oesophageal atresia Type III repair at 11-day-old. We performed an end-to-end oesophageal anastomosis with tracheal oesophageal fistula closure by extra-pleural approach. The patient was lost to follow-up for 3 years. She was seen later for anastomotic oesophageal stricture with the failure of oesophageal dilatations. Surgical resection of oesophageal stricture was performed with end-to-end oesophageal anastomosis.

  8. Pre-operative biliary drainage for obstructive jaundice

    Science.gov (United States)

    Fang, Yuan; Gurusamy, Kurinchi Selvan; Wang, Qin; Davidson, Brian R; Lin, He; Xie, Xiaodong; Wang, Chaohua

    2014-01-01

    Background Patients with obstructive jaundice have various pathophysiological changes that affect the liver, kidney, heart, and the immune system. There is considerable controversy as to whether temporary relief of biliary obstruction prior to major definitive surgery (pre-operative biliary drainage) is of any benefit to the patient. Objectives To assess the benefits and harms of pre-operative biliary drainage versus no pre-operative biliary drainage (direct surgery) in patients with obstructive jaundice (irrespective of a benign or malignant cause). Search methods We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, Cochrane Central Register of Controlled Clinical Trials (CENTRAL) in The Cochrane Library, MEDLINE, EMBASE, and Science Citation Index Expanded until February 2012. Selection criteria We included all randomised clinical trials comparing biliary drainage followed by surgery versus direct surgery, performed for obstructive jaundice, irrespective of the sample size, language, and publication status. Data collection and analysis Two authors independently assessed trials for inclusion and extracted data. We calculated the risk ratio (RR), rate ratio (RaR), or mean difference (MD) with 95% confidence intervals (CI) based on the available patient analyses. We assessed the risk of bias (systematic overestimation of benefit or systematic underestimation of harm) with components of the Cochrane risk of bias tool. We assessed the risk of play of chance (random errors) with trial sequential analysis. Main results We included six trials with 520 patients comparing pre-operative biliary drainage (265 patients) versus no pre-operative biliary drainage (255 patients). Four trials used percutaneous transhepatic biliary drainage and two trials used endoscopic sphincterotomy and stenting as the method of pre-operative biliary drainage. The risk of bias was high in all trials. The proportion of patients with malignant obstruction varied between 60

  9. Management of biliary perforation in children

    Directory of Open Access Journals (Sweden)

    Mirza Bilal

    2010-01-01

    Full Text Available Background: To study the aetiology, management and outcome of biliary perforations in paediatric age group. Patients and Methods: In a retrospective study, the records of patients presented with biliary peritonitis due to biliary perforations, managed from March 2006 to July 2009, are reviewed. Results: Eight male patients with biliary peritonitis due to biliary perforation were managed. These patients were divided in two groups, A and B. Group A, (n = 3 patients, had common bile duct (CBD perforation, and Group B (n=5 patients had gallbladder perforation. The presenting features were abdominal pain, fever, abdominal distension, vomiting, constipation, jaundice and signs of peritonism. The management of CBD perforations in Group A was by draining the site of perforation and biliary diversion (tube cholecystostomy. In Group B, the gallbladder perforations were managed by tube cholecystostomy in four patients and cholecystectomy in one patient, however, one patient had to be re-explored and cholecystectomy performed due to complete necrosis of gall bladder. There was no mortality in our series. All patients were asymptomatic on regular follow-up. Conclusion: Early optimal management of biliary perforations remarkably improved the very high mortality and morbidity that characterised this condition in the past.

  10. Percutaneous transhepatic biliary drainage

    International Nuclear Information System (INIS)

    Park, Jae Hyung; Hong, Seong Mo; Han, Man Chung

    1982-01-01

    Percutaneous transhepatic biliary drainage was successfully made 20 times on 17 patients of obstructive jaundice for recent 1 year since June 1981 at Department of Radiology in Seoul National University Hospital. The causes of obstructive jaundice was CBD Ca in 13 cases, metastasis in 2 cases, pancreatic cancer in 1 case and CBD stone in 1 case. Percutaneous transhepatic biliary drainage is a relatively ease, safe and effective method which can be done after PTC by radiologist. It is expected that percutaneous transhepatic biliary drainage should be done as an essential procedure for transient permanent palliation of obstructive jaundice

  11. Newborn jaundice - discharge

    Science.gov (United States)

    ... Biliary atresia Bili lights Bilirubin blood test Bilirubin encephalopathy Exchange transfusion Jaundice and breastfeeding Newborn jaundice Premature infant Rh incompatibility Patient Instructions Newborn ...

  12. Cervicovaginal atresia with hematometra: restoring menstrual and sexual function by utero-coloneovaginoplasty.

    Science.gov (United States)

    Kisku, Sundeep; Varghese, Lilly; Kekre, Aruna; Sen, Sudipta; Karl, Sampath; Mathai, John; Thomas, Reju Joseph; Barla, Ravi Kishore

    2014-10-01

    Cervicovaginal atresia is a rare Mullerian anomaly. The management of cervicovaginal atresia has evolved from historical recommendations of hysterectomy to various reconstructive procedures more recently. The latter carries a risk of significant morbidity and unknown fertility. We present our experience in the management of this complex anomaly. Twenty patients with cervicovaginal atresia were operated in our hospital from January 2004 through December 2013. The details of their anatomical variations and functional outcomes were analyzed. Eighteen out of twenty patients had cervical agenesis. Two patients had cervical hypoplasia. All patients underwent utero-coloneovaginoplasty. Post operatively, all patients have regular menstrual cycles. One patient is married, sexually active and has satisfactory coital function. One patient had a bowel anastomotic leak that required a diversion ileostomy. Two patients developed mild stenosis. One patient has mild neovaginal mucosal prolapse. No patient has developed pyometra. Patients with cervicovaginal atresia need to be counselled about the various reconstructive options available and the potential risks. Social and economic factor play a significant role in determining the plan of management. For patients from conservative societies, utero-coloneovaginoplasty provides a safe conduit for the passage of menstrual flow and coitus, at the cost of permanent infertility.

  13. Double sigmoid atresia with meconium pseudocyst masquerading ...

    African Journals Online (AJOL)

    8В 6cm dark cystic lesion was present in the left iliac fossa, which was delivered intact ... of Pediatric Surgery. Unauthorized reproduction of this article is prohibited. .... sigmoid-colon atresia: the perforated web variety. APSP J Case Rep. 2010 ...

  14. miR-26b promotes granulosa cell apoptosis by targeting ATM during follicular atresia in porcine ovary.

    Directory of Open Access Journals (Sweden)

    Fei Lin

    Full Text Available More than 99% of ovarian follicles undergo atresia in mammals, but the mechanism of follicular atresia remains to be elucidated. In this study, we explored microRNA (miRNA regulation of follicular atresia in porcine ovary. A miRNA expression profile was constructed for healthy, early atretic, and progressively atretic follicles, and the differentially expressed miRNAs were selected and analyzed. We found that miR-26b, which was upregulated during follicular atresia, increased the number of DNA breaks and promoted granulosa cell apoptosis by targeting the ataxia telangiectasia mutated gene directly in vitro.

  15. Dysphagia among Adult Patients who Underwent Surgery for Esophageal Atresia at Birth

    Directory of Open Access Journals (Sweden)

    Valérie Huynh-Trudeau

    2015-01-01

    Full Text Available BACKGROUND: Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and/or anatomical anomalies.

  16. Coexistence of bronchial atresia and bronchogenic cyst: diagnostic criteria and embryologic considerations

    International Nuclear Information System (INIS)

    Kuhn, C.; Kuhn, J.P.

    1992-01-01

    We report a case in a neonate of concurrent bronchial atresia and bronchogenic cyst. An accurate, noninvasive, preoperative diagnosis of this unusual combination of anomalies was made by ultrafast computed tomography (UFCT). This case supports the hypothesis that bronchial atresia results from an event occurring in the 5th-6th week of embryological development, rather than after the 16th week as previously believed. (orig.)

  17. Unilateral pulmonary veins atresia: evaluation by computed tomography; Atresia unilateral das veias pulmonares: avaliacao por tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Eifer, Diego Andre; Arsego, Felipe Veras, E-mail: felipesoarestorres@gmail.com [Hospital de Clinicas de Porto Alegre (HCPA), RS (Brazil). Serv. de Radiologia; Torres, Felipe Soares [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Escola de Medicina

    2013-11-15

    Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient. (author)

  18. Extrahepatic manifestations of cholestatic liver diseases: pathogenesis and therapy

    NARCIS (Netherlands)

    Pusl, Thomas; Beuers, Ulrich

    2005-01-01

    Pruritus, fatigue, and metabolic bone disease are frequent complications of cholestatic liver diseases, which can be quite distressing for the patient and can considerably reduce the quality of life. The molecular pathogenesis of these extrahepatic manifestations of cholestasis is poorly understood,

  19. Evaluation of the angiographic findings in pulmonary atresia

    International Nuclear Information System (INIS)

    Choe, Kyu Ok; Sul, Jun Hee; Lee, Seung Kyu; Cho, Bum Koo; Hong, Pill Whoon

    1986-01-01

    We studied the angiographic findings in 65 patients with congenital pulmonary atresia, ages 4 days to 14 years (mean 3.3 yrs), from 1981 to 1986 at Severance Hospital Yonsei University. 1. 6 had pulmonary atresia with an intact interventricular septum, 38 had it with cardiac anomaly Renodynamically simulating TOF, and 21 associated with more complicated cardiac anomalies. 2. In the group with an intact ventricular septum, 5 showed confluent intrapericardial pulmonary artery, all segmental pulmonary arteries connected to intrapericardial artery. 3. In the group simulating TOF, aorta arose from RV with or without overriding in 35. In 27 patients with confluent intrapericardial pulmonary artery, 23 had more than 10 segmental pulmonary arteries connected to intrapericardial artery and 5 had severely hypoplastic hilar pulmonary arteries. In 11 with non confluent intrapericardial pulmonary artery, 4 had more than 10 segmental pulmonary arteries connected to central pulmonary artery and 9 had severely hypoplastic hilar pulmonary arteries. 4. In the group associated with more complicated cardiac anomaly, included 8 patients with atrioventricular discordance, 7 with univentricular heart and 6 with tricuspid atresia. In 17 patients with confluent intrapericardial artery, 16 had more than 10 segmental pulmonary arteries connected to intrapericardial artery, one showed severe hypoplasia of hilar pulmonary arteries. In another 4 with non confluence, no one showed more than 10 segmental arteries connected to intrapericardial or hilar pulmonary artery.

  20. Congenital esophageal atresia with tracheo-esophageal fistula

    International Nuclear Information System (INIS)

    Rhee, Chung Sik

    1970-01-01

    Three cases of esophageal atresia with tracheo-esophageal fistula. 1). Case 1: A female infant birth Wt. 1.95 kg , Apgar Score 10, Skeletal anomalies, was delivered after a pregnancy compeicated by hydroamnious on Aug. 17, 1970. The family history was not contributory. 2) Case 2: A male infant birth Wt. 2.8 kg , Apgar Score 8, was forcep delivered after a pregnancy on Feb. 8, 1970. This infant is twin. The family history was not contributory. 3) Case 3: A female infant birth Wt. 2.22 kg , Apgar Score 10, was C-section after a pregnancy on May, 16. 1970. The family history was not contributory. All cases: After 24 hours 5% glucose solution was given and immediately vomited and some of it regurgitating through the nose and mouth with associated cyanosis and dyspnea. A catheter was inserted through the nose into the esophagus under diagnosis of the esophageal atresia

  1. Percutaneous biliary drainage and stenting

    International Nuclear Information System (INIS)

    Totev, M.

    2012-01-01

    Full text: Percutaneous transhepatic cholangiography (PTC) is an X-ray or US guided procedure that involves the injection of a contrast material directly into the bile ducts inside the liver to produce pictures of them. If a blockage or narrowing is found, additional procedures may be performed: 1. insertion of a catheter to drain excess bile out of the body or both - internal and external; 2. plastic endoprothesis placement; 3. self-expandable metal stents placement to help open bile ducts or to bypass an obstruction and allow fluids to drain. Current percutaneous biliary interventions include percutaneous transhepatic cholangiography (PTC) and biliary drainage to manage benign and malignant obstructions. Internal biliary stents are either plastic or metallic, and various types of each kind are available. Internal biliary stents have several advantages. An external tube can be uncomfortable and have a psychological disadvantage. An internal stent prevents the problems related to external catheters, for example, pericatheter leakage of bile and the need for daily flushing. The disadvantages include having to perform endoscopic retrograde cholangiopancreatography (ERCP) or new PTC procedures to obtain access in case of stent obstruction. Better patency rates are reported with metallic than with plastic stents in cases of malignant obstruction, though no effect on survival is noted. Plastic internal stents are the cheapest but reportedly prone to migration. Metallic stents are generally not used in the treatment of benign disease because studies have shown poor long-term patency rates. Limited applications may include the treatment of patients who are poor surgical candidates or of those in whom surgical treatment fails. Most postoperative strictures are treated surgically, though endoscopic and (less commonly) percutaneous placement of nonmetallic stents has increasingly been used in the past few years. Now there are some reports about use of biodegradable biliary

  2. Percutaneous Biliary Drainage Using Open Cell Stents for Malignant Biliary Hilar Obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, Sun Jun; Bae, Jae Ik; Han, Tae Sun; Won, Je Hwan; Kim, Ji Dae; Kwack, Kyu Sung; Lee, Jae Hee; Kim, Young Chul [Dept. of Radiology, Ajou University School of Medicine, Suwon (Korea, Republic of)

    2012-11-15

    To evaluate the feasibility, safety and the effectiveness of the complex assembly of open cell nitinol stents for biliary hilar malignancy. During the 10 month period between January and October 2007, 26 consecutive patients with malignant biliary hilar obstruction underwent percutaneous insertion of open cell design nitinol stents. Four types of stent placement methods were used according to the patients' ductal anatomy of the hilum. We evaluated the technical feasibility of stent placement, complications, patient survival, and the duration of stent patency. Bilobar biliary stent placement was conducted in 26 patients with malignant biliary obstruction-T (n = 9), Y (n 7), crisscross (n = 6) and multiple intersecting types (n = 4). Primary technical success was obtained in 24 of 26 (93%) patients. The crushing of the 1st stent during insertion of the 2nd stent occurred in two cases. Major complications occurred in 2 of 26 patients (7.7%). One case of active bleeding from hepatic segmental artery and one case of sepsis after procedure occurred. Clinical success was achieved in 21 of 24 (87.5%) patients, who were followed for a mean of 141.5 days (range 25-354 days). The mean primary stent patency period was 191.8 days and the mean patient survival period was 299 days. Applying an open cell stent in the biliary system is feasible, and can be effective, especially in multiple intersecting stent insertions in the hepatic hilum.

  3. Percutaneous Biliary Drainage Using Open Cell Stents for Malignant Biliary Hilar Obstruction

    International Nuclear Information System (INIS)

    Ahn, Sun Jun; Bae, Jae Ik; Han, Tae Sun; Won, Je Hwan; Kim, Ji Dae; Kwack, Kyu Sung; Lee, Jae Hee; Kim, Young Chul

    2012-01-01

    To evaluate the feasibility, safety and the effectiveness of the complex assembly of open cell nitinol stents for biliary hilar malignancy. During the 10 month period between January and October 2007, 26 consecutive patients with malignant biliary hilar obstruction underwent percutaneous insertion of open cell design nitinol stents. Four types of stent placement methods were used according to the patients' ductal anatomy of the hilum. We evaluated the technical feasibility of stent placement, complications, patient survival, and the duration of stent patency. Bilobar biliary stent placement was conducted in 26 patients with malignant biliary obstruction-T (n = 9), Y (n 7), crisscross (n = 6) and multiple intersecting types (n = 4). Primary technical success was obtained in 24 of 26 (93%) patients. The crushing of the 1st stent during insertion of the 2nd stent occurred in two cases. Major complications occurred in 2 of 26 patients (7.7%). One case of active bleeding from hepatic segmental artery and one case of sepsis after procedure occurred. Clinical success was achieved in 21 of 24 (87.5%) patients, who were followed for a mean of 141.5 days (range 25-354 days). The mean primary stent patency period was 191.8 days and the mean patient survival period was 299 days. Applying an open cell stent in the biliary system is feasible, and can be effective, especially in multiple intersecting stent insertions in the hepatic hilum.

  4. New successful one-step surgical repair for apple peel atresia

    Directory of Open Access Journals (Sweden)

    Machmouchi M

    2011-11-01

    Full Text Available Mahmoud MachmouchiDepartment of Pediatrics, Royal Commission Hospital, Jubail, Saudi ArabiaAbstract: A new successful surgical approach in two identical twins delivered with intestinal atresia, “apple peel” type is reported. This technique consists of: (1 an end-to-end oblique primary anastomosis with single layer inverted 5/0 Vicryl® sutures (Ethicon, Inc, Somerville, NJ; (2 proximal tube jejunostomy using Foley catheter, size 10 French, inserted through a stab wound in the left upper quadrant and entering the proximal dilated loop at about 10 cm proximal from its blind end (site of anastomosis; and (3 transanastomotic stenting using feeding tube, size 6 French, exteriorized in conjunction with the Foley catheter and reaching the lumen of the distal loop for more than 20 cm. The postoperative course was uneventful and progressive oral feed became ad libitum around day 40 postoperative. This procedure is simple, performed in one stage, and responds to the most obligatory requirements of this congenital malformation.Keywords: proximal jejunal atresia, intestinal atresia, surgical repair, anastomosis

  5. Congenital Pyloric Atresia; a report of two cases

    International Nuclear Information System (INIS)

    Tayeb, Maaen; Khogeer, Suzie; Fallatah, Amna; Hamchou, Mustafa A.

    2005-01-01

    Congenital pyloric atresia (CPA) is a very rare malformation that constitutes less than 1% of all gas upper gastrointestinal atresias. It is a unique malformation that is commonly seen as an isolated lesion, but can also occur in association with other genetically determined conditions such as hereditary multiple intestinal atresias (HMIA). This is a report of two cases of isolated isolated CPA, outlining aspects of diagnosis and management. A 2-day-old female, a product of 35-weeks gestation via a low cesarean delivery due to a transverse lie to a 25-year-old mother who had gestational insulin dependent diabetes and polyhydraminos was referred because of non-bile stained vomiting. Her abdominal x-ray showed dilated stomach with no gas distally. Gastrograffin meal confirmed the diagnosis of gastric outlet obstruction. She was found to have pyloric artesia. This was excised via longitudinal incision in the pylorus, which was then closed transversely. Subsequently she did well. A 1-year -old female was evaluated because of persistent non-bile stained vomiting. Abdominal x-ray showed dilated stomach with no gas distally and barium meal confirmed the diagnosis of gastric outlet obstruction. She was operated and on during surgery was found to have congenital pyloric atresia. This was excised via longitudinal incsion in the pylorus. She did well. CPA is divided in three types: 1) Pyloric membrane 2) Pyloric artesia without a gap 3) Pyloric artesia with a gap. Both our patients have pyloric diaphagrams. The treatment of CPA is surgical and depends on the type of arteseia. For those with pyloric diaphagram or pyloric artesia without a gap the treatment is excision of diphagaram. This is also of importance in case there is more than one diaphagram. For those with pyloric artesia with a gap, if the gap is short, they should be treated with a Finny or Heineke-Mickulicz pyloroplasty, but if the gap is long then a gastroduodenostomy becomes the treatment of choice

  6. Percutaneous transhepatic biliary stenting vs. surgical bypass in advanced malignant biliary obstruction: cost- effectiveness analysis.

    Science.gov (United States)

    Yao, Li Qin; Tang, Cheng Wu; Zheng, Yin Yuan; Feng, Wen Ming; Huang, San Xiong; Bao, Ying

    2013-01-01

    This study aims to compare the clinical outcomes and costs between percutaneous transhepatic biliary stenting (PTBS) and surgical bypass. We randomly assigned 142 patients with unresectable malignant biliary obstruction between 2005 and 2010 to receive PTBS or surgical bypass as palliative treatment. PTBS was successfully performed in 70 patients who formed the PTBS group (failed in 7 patients). Sixty five patients underwent surgical bypass treatment. Additional gastrojejunostomy was performed in five patients. The effectiveness of biliary drainage, hospital stay, complications, cost, survival time and mortality were compared. Patients in PTBS group had shorter hospital stay and lower initial and overall expense than the surgical group (pPTBS group was significantly lower than surgical group (3/75 vs. 11/65, p=0.0342). Late complication in PTBS group did not differ significantly from surgical group (9/70 vs. 6/65, p=0.6823). The survival curves in the two groups showed no significant difference (p=0.1032). PTBD is a better palliative treatment than surgical bypass for unresectable malignant biliary obstruction for its high effectiveness of biliary drainage and acceptable expense and complication.

  7. Endoscopic Ultrasound-Guided Biliary Drainage

    International Nuclear Information System (INIS)

    Artifon, Everson L.A.; Ferreira, Fla'vio C.; Sakai, Paulo

    2012-01-01

    To demonstrate a comprehensive review of published articles regarding endoscopic ultrasound (EUS)-guided biliary drainage. Review of studies regarding EUS-guided biliary drainage including case reports, case series and previous reviews. EUS-guided hepaticogastrostomy, coledochoduodenostomy and choledoantrostomy are advanced biliary and pancreatic endoscopy procedures, and together make up the echo-guided biliary drainage. Hepaticogastrostomy is indicated in cases of hilar obstruction, while the procedure of choice is the coledochoduodenostomy or choledochoantrostomy in distal lesions. Both procedures must be performed only after unsuccessful ERCPs. The indication of these procedures must be made under a multidisciplinary view while sharing information with the patient or legal guardian. Hepaticogastrostomy and coledochoduodenostomy or choledochoantrostomy are feasible when performed by endoscopists with expertise in biliopancreatic endoscopy. Advanced echo-endoscopy should currently be performed under a rigorous protocol in educational institutions.

  8. Endoscopic Ultrasound-Guided Biliary Drainage

    Energy Technology Data Exchange (ETDEWEB)

    Artifon, Everson L.A.; Ferreira, Fla& #x27; vio C.; Sakai, Paulo [University of Saeo Paulo, Saeo Paulo (Brazil)

    2012-02-15

    To demonstrate a comprehensive review of published articles regarding endoscopic ultrasound (EUS)-guided biliary drainage. Review of studies regarding EUS-guided biliary drainage including case reports, case series and previous reviews. EUS-guided hepaticogastrostomy, coledochoduodenostomy and choledoantrostomy are advanced biliary and pancreatic endoscopy procedures, and together make up the echo-guided biliary drainage. Hepaticogastrostomy is indicated in cases of hilar obstruction, while the procedure of choice is the coledochoduodenostomy or choledochoantrostomy in distal lesions. Both procedures must be performed only after unsuccessful ERCPs. The indication of these procedures must be made under a multidisciplinary view while sharing information with the patient or legal guardian. Hepaticogastrostomy and coledochoduodenostomy or choledochoantrostomy are feasible when performed by endoscopists with expertise in biliopancreatic endoscopy. Advanced echo-endoscopy should currently be performed under a rigorous protocol in educational institutions.

  9. Potential Pitfalls on the (99m)Tc-Mebrofenin Hepatobiliary Scintigraphy in a Patient with Biliary Atresia Splenic Malformation Syndrome

    DEFF Research Database (Denmark)

    Maestri Brittain, Jane; Borgwardt, Lise

    2016-01-01

    the first years of life. Kasai portoenterostomy restores bile flow and delay the progressive liver damage thereby postponing liver transplantation. An early diagnosis is of most importance to ensure the effectiveness of the operation. The (99m)Tc-Mebrofenin hepatobiliary scintigraphy is part...... of BA. Laparotomy with antegrade cholangiography is then performed giving the final diagnosis when the remains of the obliterated biliary tree are revealed in the case of BA. We present a case demonstrating some of the challenges of interpreting the (99m)Tc-Mebrofenin hepatobiliary scintigraphy...... in an infant with BASM and stress the importance that the (99m)Tc-Mebrofenin hepatobiliary scintigraphy is part of a spectrum of imaging modalities in diagnosing BA....

  10. Development of infantile hypertrophic pyloric stenosis in patients treated for oesophageal atresia. A case report

    DEFF Research Database (Denmark)

    Qvist, N; Rasmussen, L; Hansen, L P

    1986-01-01

    Two cases of infantile hypertrophic pyloric stenosis (IHPS) developed in 74 patients treated for oesophageal atresia. Treatment of oesophageal atresia is frequently followed by vomiting and failure to thrive due to gastrooesophageal reflux or anastomotic stricture. The diagnose of IHPS must...

  11. Imaging approach to persistent neonatal jaundice

    International Nuclear Information System (INIS)

    Kirks, D.; Coleman, R.E.; Filston, H.C.; Rosenberg, E.R.; Merten, D.F.

    1984-01-01

    Fifteen patients with persistent neonatal jaundice were evaluated by sonography and radionuclide scintigraphy. The sonographic features of both neonatal hepatitis and biliary atresia are nonspecific. Hepatobiliary scintigraphy after phenobarbital pretreatment in patients with neonatal hepatitis demonstrates normal hepatic extraction and delayed tracer excretion into the gastrointestinal tract. If there is neonatal hepatitis with severe hepatocellular damage, the hepatic extraction of tracer activity is decreased and excretion may be delayed or absent. Patients under 3 months of age with biliary atresia have normal hepatic extraction of tracer with no excretion into the gastrointestinal tract. Sonography in patients with a choledochal cyst shows a cystic mass in the porta hepatis with associated bile-duct dilatation. Hepatobiliary scintigraphy confirms that the choledochal cyst communicates with the biliary system. Initial sonography demonstrates hepatobiliary anatomy; subsequent phenobarbital-enhanced radionuclide scintigraphy determines hepatobiliary function. An expedient diagnostic approach is recommended for the evaluation of persistent neonatal jaundice

  12. Outcomes in Patients with Obstructive Jaundice from Metastatic Colorectal Cancer and Implications for Management

    Science.gov (United States)

    Nichols, Shawnn D.; Albert, Scott; Shirley, Lawrence; Schmidt, Carl; Abdel-Misih, Sherif; El-Dika, Samer; Groce, J. Royce; Wu, Christina; Goldberg, Richard M.; Bekaii-Saab, Tanios; Bloomston, Mark

    2016-01-01

    Introduction Patients with metastatic colorectal cancer can develop jaundice from intrahepatic or extrahepatic causes. Currently, there is little data on the underlying causes and overall survival after onset of jaundice. The purpose of this study was to characterize the causes of jaundice and determine outcomes. Methods Six hundred twenty-nine patients treated for metastatic colorectal cancer between 2004 and 2010 were retrospectively reviewed. Those developing jaundice were grouped as having intrahepatic or extrahepatic obstruction. Demographics, clinicopathologic, and outcome data were analyzed. Results Sixty-two patients with metastatic colorectal cancer developed jaundice. Intrahepatic biliary obstruction was most common, occurring in younger patients. Time from metastatic diagnosis to presentation of jaundice was similar between groups, as was the mean number of prior lines of chemotherapy. Biliary decompression was successful 41.7 % of the time and was attempted more commonly for extrahepatic causes. Median overall survival after onset of jaundice was 1.5 months and it was similar between groups, but improved to 9.6 months in patients who were able to receive further chemotherapy. Conclusions Jaundice due to metastatic colorectal cancer is an ominous finding, representing aggressive tumor biology or exhaustion of therapies. Biliary decompression is often difficult and should only be pursued when additional treatment options are available. PMID:25300799

  13. Dysphagia in children with repaired oesophageal atresia

    NARCIS (Netherlands)

    Coppens, C.H.; Engel-Hoek, L. van den; Scharbatke, H.E.; Groot, S.A. de; Draaisma, J.M.T.

    2016-01-01

    Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral

  14. Anaesthesia for oesophageal atresia with or without tracheo ...

    African Journals Online (AJOL)

    and a reduced closing pressure of the cardiac sphincter.5 An abnormality of ... Tetralogy of Fallot, double outlet right ventricle, tricuspid atresia, atrial septal defect ..... prostaglandins in ductus arteriosus-dependent physiology and a measure of ...

  15. Dysphagia in Children with Esophageal Atresia: Current Diagnostic Options.

    Science.gov (United States)

    Rayyan, Maissa; Allegaert, Karel; Omari, Taher; Rommel, Nathalie

    2015-08-01

    Dysphagia or swallowing disorder is very common (range, 15-52%) in patients with esophageal atresia. Children present with a wide range of symptoms. The most common diagnostic tools to evaluate esophageal dysphagia, such as upper barium study and manometry, aim to characterize anatomy and function of the esophageal body and the esophagogastric junction (EGJ). Using these technologies, a variety of pathological motor patterns have been identified in children with esophageal atresia. However, the most challenging part of diagnosing patients with esophageal dysphagia lies in the fact that these methods fail to link functional symptoms such as dysphagia with the esophageal motor disorders observed. A recent method, called pressure-flow analysis (PFA), uses simultaneously acquired impedance and manometry measurements, and applies an integrated analysis of these recordings to derive quantitative pressure-flow metrics. These pressure-flow metrics allow detection of the interplay between bolus flow, motor patterns, and symptomatology by combining data on bolus transit and bolus flow resistance. Based on a dichotomous categorization, flow resistance at the EGJ and ineffective esophageal bolus transit can be determined. This method has the potential to guide therapeutic decisions for esophageal dysmotility in pediatric patients with esophageal atresia. Georg Thieme Verlag KG Stuttgart · New York.

  16. Oesophageal atresia: triumph and tragedy.

    Science.gov (United States)

    Rickham, P P; Stauffer, U G; Cheng, S K

    1977-04-01

    An enormous amount has been written about oesophageal atresia during the last 30 years. This is not surprising because it is not so long ago that the condition was uniformly fatal, and even today, a generation after the first successful operations, many problems associated with its management have not been completely solved. This lecture discusses past and present management, past and present results and future prospects of infants suffering from this malformation.

  17. Broncho-biliary fistula secondary to biliary obstruction and lung abscess in a patient with pancreatic neuro-endocrine tumor

    International Nuclear Information System (INIS)

    Panda, D.; Aggarwal, M.; Kumar, S.; Mukund, A.; Baghmar, S.; Yadav, V.

    2016-01-01

    We present a case report of broncho-biliary fistula that developed due to the blockage of biliary stent placed during the management of pancreatic neuroendocrine tumor (pNET); diagnosed on high clinical suspicion, percutaneous cholangiogram and contrast enhanced computed tomography (CECT); and successfully treated with percutaneous transhepatic biliary drainage (PTBD)

  18. Bezafibrate for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Rudic, Jelena S; Poropat, Goran; Krstic, Miodrag N

    2012-01-01

    Treatment of primary biliary cirrhosis is complicated. There are studies suggesting that bezafibrate, alone or in combination with ursodeoxycholic acid (UDCA), is effective in the treatment of primary biliary cirrhosis, but no systematic review has summarised the evidence yet....

  19. Percutaneous management of tumoral biliary obstruction in children

    Energy Technology Data Exchange (ETDEWEB)

    Akinci, Devrim; Gumus, Burcak; Ozkan, Orhan S.; Ozmen, Mustafa N.; Akhan, Okan [Hacettepe School of Medicine, Department of Radiology, Sihhiye, Ankara (Turkey); Ekinci, Saniye [Hacettepe School of Medicine, Department of Paediatric Surgery, Sihhiye, Ankara (Turkey); Akcoren, Zuhal [Hacettepe School of Medicine, Department of Paediatric Pathology, Sihhiye, Ankara (Turkey); Kutluk, Tezer [Hacettepe School of Medicine, Department of Paediatric Oncology, Sihhiye, Ankara (Turkey)

    2007-10-15

    There is limited experience of percutaneous biliary interventions in children although they are safe and effective procedures. To evaluate the efficacy and safety of percutaneous management of tumoral biliary obstruction in children. Percutaneous biliary interventions were performed in eight children (six boys, two girls) with a mean age of 10.5 years (range 4-17 years). The interventions included percutaneous biliary drainage (five patients), percutaneous biliary drainage and placement of a self-expanding metallic stent (two patients), and percutaneous cholecystostomy (one patient). All patients had signs of obstructive jaundice and two had cholangitis. All procedures were successful. No procedure-related mortality was observed. Bilirubin levels returned to normal in four of the eight patients. Findings of cholangitis resolved in the two affected patients after the procedure and antibiotic treatment. Two patients underwent surgery after percutaneous biliary drainage procedures. A self-expanding metallic stent was placed in two patients with malignancy and the stents remained patent until death. Percutaneous biliary interventions can be performed safely for the management of tumoral biliary obstruction in children. (orig.)

  20. Percutaneous management of tumoral biliary obstruction in children

    International Nuclear Information System (INIS)

    Akinci, Devrim; Gumus, Burcak; Ozkan, Orhan S.; Ozmen, Mustafa N.; Akhan, Okan; Ekinci, Saniye; Akcoren, Zuhal; Kutluk, Tezer

    2007-01-01

    There is limited experience of percutaneous biliary interventions in children although they are safe and effective procedures. To evaluate the efficacy and safety of percutaneous management of tumoral biliary obstruction in children. Percutaneous biliary interventions were performed in eight children (six boys, two girls) with a mean age of 10.5 years (range 4-17 years). The interventions included percutaneous biliary drainage (five patients), percutaneous biliary drainage and placement of a self-expanding metallic stent (two patients), and percutaneous cholecystostomy (one patient). All patients had signs of obstructive jaundice and two had cholangitis. All procedures were successful. No procedure-related mortality was observed. Bilirubin levels returned to normal in four of the eight patients. Findings of cholangitis resolved in the two affected patients after the procedure and antibiotic treatment. Two patients underwent surgery after percutaneous biliary drainage procedures. A self-expanding metallic stent was placed in two patients with malignancy and the stents remained patent until death. Percutaneous biliary interventions can be performed safely for the management of tumoral biliary obstruction in children. (orig.)

  1. MRCP and 3D LAVA imaging of extrahepatic cholangiocarcinoma at 3 T MRI

    Energy Technology Data Exchange (ETDEWEB)

    Li, N.; Liu, C.; Bi, W.; Lin, X.; Jiao, H. [Shandong Medical Imaging Research Institute, Shandong University, Jinan (China); Zhao, P., E-mail: Gavinsdu@163.com [Department of Radiology, Shandong Provincial Hospital, Shandong University, Jinan (China)

    2012-06-15

    Extrahepatic cholangiocarcinoma (CCA) is a primary bile duct malignant tumour with poor prognosis. Familiarity with their varied imaging characteristics can be helpful in developing a correct diagnosis and in optimal treatment planning, and thus contribute to a better prognosis. The purpose of this article is to illustrate the typical appearances of extrahepatic CCA on magnetic resonance cholangiopancreatography (MRCP) and three-dimensional (3D) LAVA (liver acquisition with volume acceleration) sequences at 3 T magnetic resonance imaging (MRI), and to discuss the superiority of the two techniques in the diagnosis of CCA.

  2. Broncho-biliary fistula secondary to biliary obstruction and lung abscess in a patient with pancreatic neuro-endocrine tumor

    Directory of Open Access Journals (Sweden)

    Dipanjan Panda

    2016-06-01

    Full Text Available We present a case report of broncho-biliary fistula that developed due to the blockage of biliary stent placed during the management of pancreatic neuroendocrine tumor (pNET; diagnosed on high clinical suspicion, percutaneous cholangiogram and contrast enhanced computed tomography (CECT; and successfully treated with percutaneous transhepatic biliary drainage (PTBD.

  3. Fertility, pregnancy, and delivery after biventricular repair for pulmonary atresia with an intact ventricular septum

    NARCIS (Netherlands)

    Drenthen, Willem; Pieper, Petronella G.; Roos-Hesselink, Jollen W.; Zoon, Nicole; Voors, Adrlaan A.; Mulder, Barbara J. M.; van Dijk, Arie P. J.; Vliegen, Hubert W.; Sollie, Krystyna M.; Ebels, Tjark; van Veldhuisen, Dirk J.

    2006-01-01

    The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34

  4. Fertility, pregnancy, and delivery after biventricular repair for pulmonary atresia with an intact ventricular septum.

    NARCIS (Netherlands)

    Drenthen, W.; Pieper, P.G.; Roos-Hesselink, J.W.; Zoon, N.; Voors, A.A.; Mulder, B.J.M.; Dijk, A.P.J. van; Vliegen, H.W.; Sollie, K.M.; Ebels, T.; Veldhuisen, D.J. van

    2006-01-01

    The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34

  5. TOKYO criteria 2014 for transpapillary biliary stenting.

    Science.gov (United States)

    Isayama, Hiroyuki; Hamada, Tsuyoshi; Yasuda, Ichiro; Itoi, Takao; Ryozawa, Shomei; Nakai, Yousuke; Kogure, Hirofumi; Koike, Kazuhiko

    2015-01-01

    It is difficult to carry out meta-analyses or to compare the results of different studies of biliary stents because there is no uniform evaluation method. Therefore, a standardized reporting system is required. We propose a new standardized system for reporting on biliary stents, the 'TOKYO criteria 2014', based on a consensus among Japanese pancreatobiliary endoscopists. Instead of stent occlusion, we use recurrent biliary obstruction, which includes occlusion and migration. The time to recurrent biliary obstruction was estimated using Kaplan-Meier analysis with the log-rank test. We can evaluate both plastic and self-expandable metallic stents (uncovered and covered). We also propose specification of the cause of recurrent biliary obstruction, identification of complications other than recurrent biliary obstruction, indication of severity, measures of technical and clinical success, and a standard for clinical care. Most importantly, the TOKYO criteria 2014 allow comparison of biliary stent quality across studies. Because blocked stents can be drained not only using transpapillary techniques but also by an endoscopic ultrasonography-guided transmural procedure, we should devise an evaluation method that includes transmural stenting in the near future. © 2014 The Authors. Digestive Endoscopy © 2014 Japan Gastroenterological Endoscopy Society.

  6. False Computed Tomography Findings in Bilateral Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Elsheikh, Ezzeddin

    2016-01-01

    Full Text Available Introduction Choanal atresia (CA is a challenging surgical problem defined as a failure in the development of communication between the nasal cavity and nasopharynx. Objective The objective of this study is to describe computed tomography (CT findings in cases with bilateral choanal atresia. Methods The study involved performing axial and coronal non-contrast CT scanning with 2–3 mm sections on14 neonates that had bilateral CA. We used fiberoptic nasal endoscopy to confirm the diagnosis. We evaluated coronal CT to study the skull base area in such neonates. Results This study included 14 neonates with bilateral CA; with mean age of 7 ± 3.5 days. Mixed atretic plates were found in 12 (85.7% cases while two (14.3% had pure bony atresia. Isolated CA was detected in 9 cases (64.3% and 5 (35.7% cases had associated anomalies. Coronal CT showed soft tissue density in the nasal cavity that appeared to extend through an apparent defect in the nasal roof (cribriform plate, falsely diagnosed by radiologists as associated encephalocele. At the time of surgical repair, all patients showed thick tenacious mucous secretions in both nasal cavities and revealed no encephalocele. Nasal roof remained intact in all cases. Conclusion The thick secretion of bilateral CA could give a false encephalocele appearance on the CT. It is highly recommended to perform proper suction of the nasal cavity of suspected CA cases just before CT scanning.

  7. Novel targeted approaches to treating biliary tract cancer: the dual epidermal growth factor receptor and ErbB-2 tyrosine kinase inhibitor NVP-AEE788 is more efficient than the epidermal growth factor receptor inhibitors gefitinib and erlotinib.

    Science.gov (United States)

    Wiedmann, Marcus; Feisthammel, Jürgen; Blüthner, Thilo; Tannapfel, Andrea; Kamenz, Thomas; Kluge, Annett; Mössner, Joachim; Caca, Karel

    2006-08-01

    Aberrant activation of the epidermal growth factor receptor is frequently observed in neoplasia, notably in tumors of epithelial origin. Attempts to treat such tumors with epidermal growth factor receptor antagonists resulted in remarkable success in recent studies. Little is known, however, about the efficacy of this therapy in biliary tract cancer. Protein expression of epidermal growth factor receptor, ErbB-2, and vascular endothelial growth factor receptor-2 was assessed in seven human biliary tract cancer cell lines by immunoblotting. In addition, histological sections from 19 patients with extrahepatic cholangiocarcinoma were analyzed for epidermal growth factor receptor, ErbB-2 and vascular endothelial growth factor receptor-2 expression by immunohistochemistry. Moreover, we sequenced the cDNA products representing the entire epidermal growth factor receptor coding region of the seven cell lines, and searched for genomic epidermal growth factor receptor amplifications and polysomy by fluorescence in-situ hybridization. Cell growth inhibition by gefitinib erlotinib and NVP-AEE788 was studied in vitro by automated cell counting. In addition, the anti-tumoral effect of erlotinib and NVP-AEE788 was studied in a chimeric mouse model. The anti-tumoral drug mechanism in this model was assessed by MIB-1 antibody staining, terminal deoxynucleotidyl transfer-mediated dUTP nick end-labelling assay, von Willebrand factor staining, and immunoblotting for p-p42/44 (p-Erk1/2, p-MAPK) and p-AKT. Immunoblotting revealed expression of epidermal growth factor receptor, ErbB-2, and vascular endothelial growth factor receptor-2 in all biliary tract cancer cell lines. EGFR was detectable in six of 19 (32%) extrahepatic human cholangiocarcinoma tissue samples, ErbB-2 in 16 of 19 (84%), and vascular endothelial growth factor receptor-2 in nine of 19 (47%). Neither epidermal growth factor receptor mutations nor amplifications or polysomy were found in the seven biliary tract cancer

  8. Extrahepatic Blood Supply to Hepatocellular Carcinoma: Angiographic Demonstration and Transcatheter Arterial Chemoembolization

    International Nuclear Information System (INIS)

    Miyayama, Shiro; Matsui, Osamu; Taki, Keiichi; Minami, Tetsuya; Ryu, Yasuji; Ito, Chiharu; Nakamura, Koichi; Inoue, Dai; Notsumata, Kazuo; Toya, Daisyu; Tanaka, Nobuyoshi; Mitsui, Takeshi

    2006-01-01

    Purpose. To evaluate the incidence of each extrahepatic collateral pathway to hepatocellular carcinoma (HCC) and to assess technical success rates and complications of transcatheter arterial chemoembolization (TACE) through each collateral. Methods. We retrospective evaluated extrahepatic collateral pathways to HCC on angiography in 386 procedures on 181 consecutive patients. One hundred and seventy patients had previously undergone TACE. TACE through extrahepatic collaterals using iodized oil and gelatin sponge particles was performed when a catheter was advanced into the tumor-feeding branch to avoid nontarget embolization. Results. A single collateral was revealed in 275 TACE procedures, two were revealed in 74, and three or more were revealed in 34. Incidences of collateral source to HCC were 83% from the right inferior phrenic artery (IPA), 24% from the cystic artery, 13% from the omental artery, 12% from the right renal capsular artery (RCA) and left IPA, 8% from the right internal mammary artery (IMA) and right intercostal artery (ICA), and 7% from the right inferior adrenal artery (IAA). Technical success rates of TACE were 53% in the right ICA, 70% in the cystic artery, 74% in the omental artery, 93% in the left IPA, 96% in the right IPA, and 100% in the right RCA, right IMA, and right IAA. Complications included skin necrosis after TACE through the right IMA (n = 1), cholecystitis after TACE through the cystic artery (n = 1), and ulcer formation after TACE through the right gastric artery (n = 1), in addition to pleural effusion and basal atelectasis after TACE through the IPA and IMA. Conclusion. Our study suggests that TACE through extrahepatic collaterals is possible with high success rates, and is also relatively safe

  9. Laser ablation of a biliary duct for treatment of a persistent biliary-cutaneous fistula.

    Science.gov (United States)

    Eicher, Chad A; Adelson, Anthony B; Himmelberg, Jeffrey A; Chintalapudi, Udaya

    2008-02-01

    A persistent biliary-cutaneous fistula detected after biliary drainage catheter removal could not be resolved with diversionary techniques and Gelfoam and fibrin glue administration in the fistulous tract. As an alternative approach for treatment of the fistula, obliteration of the contributing bile duct with laser ablation was performed.

  10. Extrahepatic manifestations associated with Chronic Hepatitis C Virus Infection

    Directory of Open Access Journals (Sweden)

    A. Flores-Chávez

    Full Text Available Summary Chronic hepatitis C virus (HCV infection has been associated with both organ-specific and systemic autoimmune diseases, with cryoglobulinemia being the most frequent associated disease. Experimental, virologic, and clinical evidence have demon-strated a close association between HCV infection and some systemic autoimmune diseases, especially Sjögren’s syndrome, but also rheumatoid arthritis and lupus. A higher prevalence of hematological processes has also been described in patients with HCV infection, including cytopenias and lymphoproliferative disorders (B-cell lymphoma. In addition, patients with chronic HCV infection have a higher frequency of other extrahepatic manifestations including endocrine, metabolic and cardiovascular disorders that may worse the prognosis of patients, along with neuropsychiatric manifestations and general symptoms that have a significant influence on the quality of life of the patient. Direct-acting antiviral therapies (DAAs that have recently begun to be used are providing the opportunity to effectively cure chronic HCV infection and reduce the burden of both hepatic and extrahepatic complications.

  11. Magnetic resonance cholangiographic (MRCP) features of ischemic-type biliary lesions (ITBL). A case-control study

    International Nuclear Information System (INIS)

    Collettini, F.; Kroencke, T.J.; Renz, D.M.; Bucourt, M. de; Poellinger, A.; Heidenhain, C.; Neuhaus, P.

    2011-01-01

    Purpose: To evaluate the spectrum of MR cholangiography (MRCP) features of ischemic-type biliary lesions (ITBL) after orthotopic liver transplantation (OLT). Materials and Methods: 30 patients (16 m, 14 f) with an average age of 52 years (9 - 69 y) were examined in two 1.5 MR units using breath-hold 2D-SS-FSE-sequences and 3D-MRCP sequences. 20 of the 30 patients had an established ITBL, and the remaining 10 patients were post-OLT controls. MRCPs were evaluated independently by two experienced radiologists that were blinded to the clinical history as well as the results of other imaging modalities. All images were analyzed for the presence of 16 different pathological features. Differences between ITBL patients and controls were analyzed using the Mann-Whitney-U Test. Inter-rater variability was tested using the Cohen's Kappa test. Results: Abnormal findings of bile ducts were seen in all patients. The most common findings were (in percentage for reader 1/2) intrahepatic bile duct dilatation (95 %/95 %) and extrahepatic bile duct stenoses (95 %/85 %), followed by intrahepatic main duct stenoses (90 %/95 %) and segmental duct stenoses (85 %/85 %). Differences between ITBL patients and controls were significant for most of the analyzed features (Mann-Whitney-U test, p < 0.05). For 12 of 16 features, there was substantial or almost perfect agreement (K = 0.61 - 1.00), for 2 of 16 features moderate agreement (K = 0.41- 0.60) and for 2 of 16 features fair agreement (K < 0.40). Conclusion: In patients with ITBL, MR cholangiography reveals characteristic features that may allow differentiation from other biliary complications after liver transplantation. (orig.)

  12. Spastic quadriplegia in Down syndrome with congenital duodenal stenosis/atresia.

    Science.gov (United States)

    Kurosawa, Kenji; Enomoto, Keisuke; Tominaga, Makiko; Furuya, Noritaka; Sameshima, Kiyoko; Iai, Mizue; Take, Hiroshi; Shinkai, Masato; Ishikawa, Hiroshi; Yamanaka, Michiko; Matsui, Kiyoshi; Masuno, Mitsuo

    2012-06-01

    Down syndrome is an autosomal chromosome disorder, characterized by intellectual disability and muscle hypotonia. Muscle hypotonia is observed from neonates to adulthood in Down syndrome patients, but muscle hypertonicity is extremely unusual in this syndrome. During a study period of nine years, we found three patients with severe spastic quadriplegia among 20 cases with Down syndrome and congenital duodenal stenosis/atresia (3/20). However, we could find no patient with spastic quadriplegia among 644 cases with Down syndrome without congenital duodenal stenosis/atresia during the same period (0/644, P quadriplegia among 17 patients with congenital duodenal stenosis/atresia without Down syndrome admitted during the same period to use as a control group (0/17, P quadriplegia in patients with Down syndrome. Long-term survival is improving, and the large majority of people with Down syndrome are expected to live well into adult life. Management and further study for the various problems, representing a low prevalence but serious and specific to patients with Down syndrome, are required to improve their quality of life. © 2012 The Authors. Congenital Anomalies © 2012 Japanese Teratology Society.

  13. Cryoglobulinemia and its correlation with clinical extrahepatic manifestations in chronic hepatitis C

    Directory of Open Access Journals (Sweden)

    Joanna Jabłońska

    2017-12-01

    Results: Cryoglobulins were found in 93 persons (37.8%. Type II cryoglobulinemia was detected in 28 persons. Patients with cryoglobulinemia were elder (p<0.0004. 54 patients (21.95% manifested clinical extrahepatic symptoms. Arthralgia was found in 27 cases (10.98%. Skin changes were found in 22 patients (8.94%. 12 persons had glomerulonephritis (4.88%. 11 patients (4.47% had sicca syndrome. 6 patients developed peripheral polyneuropathy (2.4%. 4 persons developed B cell lymphoma (1.63%. There was no correlation between presence of symptoms and grading, staging, age, HCV genotype, and the presence of autoantibodies. Extrahepatic manifestations were present more frequently in women (p<0.0008.

  14. Rationale diagnostic approach to biliary tract imaging

    International Nuclear Information System (INIS)

    Helmberger, H.; Huppertz, A.; Ruell, T.; Zillinger, C.; Ehrenberg, C.; Roesch, T.

    1998-01-01

    Since the introduction of MR cholangiography (MRC) diagnostic imaging of the biliary tract has been significantly improved. While percutaneous ultrasonography is still the primary examination, computed tomography (CT), conventional magnetic resonance imaging (MRI), as well as the direct imaging modalities of the biliary tract - iv cholangiography, endoscopic-retrograde-cholangiography (ERC), and percutaneous-transhepatic-cholangiography (PTC) are in use. This article discusses the clinical value of the different diagnostic techniques for the various biliary pathologies with special attention to recent developments in MRC techniques. An algorithm is presented offering a rational approach to biliary disorders. With further technical improvement shifts from ERC(P) to MRC(P) for biliary imaging could be envisioned, ERCP further concentrating on its role as a minimal invasive treatment option. (orig.) [de

  15. A newborn with duodenal atresia and a gastric perforation

    Directory of Open Access Journals (Sweden)

    Akcora Bulent

    2010-01-01

    Full Text Available Congenital duodenal atresia complicated by gastric perforation (GP is a very rare and a very mortal condition. Only three newborns could be cured in the reported 13 cases. We report a successfully treated newborn with this complicated disease. A 2-day-old male was hospitalized with prediagnosis of duodenal obstruction. Twelve hours later, significant abdominal distention occurred promptly. At laparotomy, GP and preampullary duodenal atresia were detected. Gastrorrhaphy and duodenoduodenostomy were performed in the same operation. The patient was discharged on the 15th postoperative day. This complicated disease can be treated by early diagnosis and surgical intervention. We choose one-stage operation because of the clean peritoneal cavity. However, generalized peritonitis may require two-stage operation in delayed cases.

  16. Biliary obstruction caused by intra-biliary tumor growth from recurred hepatocellular carcinoma after radiofrequency ablation: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Yi, Ji Hyun; Kim, Jae Won [Dept. of Radiology, Yeungnam University College of Medicine, Daegu (Korea, Republic of)

    2014-04-15

    A 59-year-old man with a known central hepatocellular carcinoma (HCC) underwent a trans-arterial-chemo-embolization (TACE) and a post-TACE percutaneous radiofrequency ablation (PRFA). Two months after the PRFA, the patient presented jaundice and an abdominal computed tomography was obtained. An arterial enhancing mass adjacent to the ablated necrotic lesion with a continuously coexisting mass inside the right hepatic duct, suggestive of a HCC recurrence with a direct extension to the biliary tract was found. Finally a biliary tumor obstruction has been developed and a percutaneous transhepatic biliary drainage was performed. This case of biliary obstruction caused by directly invaded recurred HCC after PRFA will be reported because of its rare occurrence.

  17. Biliary interventionism in benign disease

    International Nuclear Information System (INIS)

    Marin, Jorge H

    2003-01-01

    The interventionism non-surgical biliary begins with the description of the drainage biliary percutaneous that has presented in the last 20 years a special development with the improvement of the techniques and materials for percutaneous and endoscopic use. At the present time the echographic technique allows a quick diagnosis of the possible causes of the obstructive jaundice and in most of the cases to approach the level of the obstruction; however, for a complete morphologic definition of the biliary tree; the cholangiography retrogrades endoscopic is used like first line of diagnosis and therapeutic leaving the transhepatic percutaneous cholangiography for some patients with bankrupt endoscopy or previous surgery with alteration of the anatomy and impossibility for the endoscopic canulation. Additionally, with the continuous improvement of the resonance images in the biliary duct and the new techniques of multiplanar reconstruction of the TAC have taken to that these techniques are of first diagnostic line and that the percutaneous boarding, is carried out with therapeutic and non diagnostic intention

  18. Pattern of liver diseases among children attending the National ...

    African Journals Online (AJOL)

    2015-08-05

    Aug 5, 2015 ... The clinical features of liver dysfunction may include ... stools, bleeding, weakness, vomiting, anorexia, pallor, .... studied. Non- infective cases, namely, biliary atresia, cholestatic hepatitis, hepatoblastoma and galactosaemia.

  19. Liver transplantation in Greek children: 15 years experience

    Directory of Open Access Journals (Sweden)

    Dimitrios Takoudas

    2010-09-01

    Full Text Available Liver transplantation (LT is the only available live-saving procedure for children with irreversible liver failure. This paper reports our experience from the follow-up of 16 Greek children with end-stage liver failure who underwent a LT. Over a period of 15 years, 16 pediatric liver recipients received follow up after being subjected to OLT (orthotopic liver transplantation due to end-stage liver failure. Nine children initially presented with extrahepatic biliary atresia, 2 with acute liver failure after toxic mushroom ingestion, 2 with intrahepatic cholestasis, 2 with metabolic diseases and one with hepatoblastoma. Ten children received a liver transplant in the Organ Transplantation Unit of Aristotle University of Thessaloniki and the rest in other transplant centers. Three transplants came from a living-related donor and 13 from a deceased donor. Six children underwent immunosuppressive treatment with cyclo­sporine, mycophenolate mofetil and corticosteroids, and 7 with tacrolimus, mycophenolate mofetil and corticosteroids. Three out of 16 children died within the first month after the transplantation due to post-transplant complications. Three children presented with acute rejection and one with chronic organ rejection which was successfully managed. Five children presented with cytomegalovirus infection, 5 with Epstein-Barr virus, 2 with HSV1,2, 2 with ParvoB19 virus, 2 with varicella-zoster virus and one with C. Albicans infection. One child presented with upper gastrointestinal hemorrhage and one with small biliary paucity. A satisfying outcome was achieved in most cases, with good graft function, except for the patient with small biliary paucity who required re-transplantation. The long-term clinical course of liver transplanted children is good under the condition that they are attended in specialized centers.

  20. Ursodeoxycholic acid for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Gong, Y.; Huang, Z.B.; Christensen, Erik

    2008-01-01

    , and the references of identified studies. The last search was performed in January 2007. SELECTION CRITERIA: Randomised clinical trials evaluating UDCA versus placebo or no intervention in patients with primary biliary cirrhosis. DATA COLLECTION AND ANALYSIS: The primary outcomes were mortality and mortality......, trial duration, and patient's severity of primary biliary cirrhosis. We also used Bayesian meta-analytic approach to estimate the UDCA effect as sensitivity analysis. MAIN RESULTS: Sixteen randomised clinical trials evaluating UDCA against placebo or no intervention were identified. Data from three......BACKGROUND: Primary biliary cirrhosis is an uncommon autoimmune liver disease with unknown aetiology. Ursodeoxycholic acid (UDCA) has been used for primary biliary cirrhosis, but the effects remain controversial. OBJECTIVES: To evaluate the benefits and harms of UDCA on patients with primary...

  1. Stages of Extrahepatic Bile Duct Cancer

    Science.gov (United States)

    ... liver, along with some normal tissue around it. Whipple procedure : A surgical procedure in which the head of ... to remove the cancer , which may include a Whipple procedure . Stent placement or percutaneous transhepatic biliary drainage as ...

  2. [Resection of juxtahilar bile duct carcinoma instead of palliative drainage of the biliary tract].

    Science.gov (United States)

    Pichlmayr, R; Lehr, L; Ziegler, H

    1983-01-01

    Instead of the widely recommended approach of treating hilar carcinoma of the bile ducts by simple palliative biliary drainage, step by step a policy of primarily aiming at resection for cure has been adopted. So far in 11 out of 22 patients excision of the tumor was possible by resection of the hepatic duct confluence; in 4 cases a left hemihepatectomy had to be added because of carcinomatous infiltration of the left liver lobe or the left hepatic artery. The multiple bile duct openings remaining after resection of such tumors were reconstructed to one or two orifices and a bi- or unilateral Roux-en-Y cholangiojejunal anastomosis performed. In further 3 cases orthotopic liver transplantation was necessary to remove all visibly infiltrated tissue. In the remaining 8 patients because of documented extrahepatic carcinomatous spread palliative biliary drainage by a percutaneous U-tube or an endoprothesis was indeed considered the only reasonable measure. Despite the relatively high resectional rate of 60% and the extensive operations performed early mortality was confined to one patient who succumbed to septic endocarditis 6 weeks after the operation. At present the longest postoperative interval without recurrence amounts to 3 1/2 years. Nine patients free of recurrent disease are in perfect health; in 3 patients in whom a recurrence was observed after 1/2, 1 1/2 and 2 years meanwhile palliation was perfect. In contrast all patients with unresected tumors but carrying draining stents suffered from cholangitis and after 1 1/2 years all but one had died. In conclusion resectional therapy for hilar carcinoma seems possible with acceptable risk. Since only resection can provide potential cure and also palliation was better than that achieved by draining tubes a more aggressive attitude to the treatment of these lesions is advocated from our experience.

  3. Outcomes of surgical management of intestinal atresias

    African Journals Online (AJOL)

    2013-11-22

    Nov 22, 2013 ... nutrition and neonatal surgical intensive care services are the norm. Thus, outcome of management in ... Social Sciences (SPSS 15.0 version, SPSS Inc, Chicago Ill) was used for data entry and analysis. ..... Excellent long‑term outcome for survivors of apple peel atresia. J Pediatr Surg 2002;37:61‑5. 17.

  4. Intrahepatic ascariasis – Common parasite at an uncommon site

    Directory of Open Access Journals (Sweden)

    Udit Chauhan

    2016-08-01

    Full Text Available Bacterial infections of the biliary tree are common infections of the biliary system which frequently lead to life-threatening sepsis. Parasitic infections of the biliary tree like ascariasis are not uncommon. Most adult worms reside into the extrahepatic biliary system. Intrahepatic existence is not commonly described. Urgent recognition of the intrahepatic existence of this common parasite is of paramount importance in order to start timely treatment of this lifethreatening infection. Authors described a case of intrahepatic ascariasis in a young male who was diagnosed radiologically and thereafter managed with endoscopic retrograde cholangiopancreatography and antibiotics.

  5. [Tomato peel: rare cause of biliary tract obstruction].

    Science.gov (United States)

    Hagymási, Krisztina; Péter, Zoltán; Csöregh, Eva; Szabó, Emese; Tulassay, Zsolt

    2011-11-20

    Foreign bodies in the biliary tree are rare causes of obstructive jaundice. Food bezoars are infrequent as well. They can cause biliary obstruction after biliary tract interventions, or in the presence of biliary-bowel fistula or duodenum diverticulum. Food bezoars usually pass the gastrointestinal tract without any symptoms, but they can cause abdominal pain and obstructive jaundice in the case of biliary tract obstruction. Endoscopic retrograde cholangio-pancreatography has the major role in the diagnosis and the treatment of the disease. Authors summarize the medical history of a 91-year-old female patient, who developed vomiting and right subcostal pain due to the presence of tomato peel within the ductus choledochus.

  6. Cellophane banding for the gradual attenuation of single extrahepatic portosystemic shunts in eleven dogs.

    Science.gov (United States)

    Youmans, K R; Hunt, G B

    1998-08-01

    To evaluate the efficacy and short term effects of a cellophane banding technique for progressive attenuation of canine single extrahepatic portosystemic shunts. A prospective trial of 11 dogs with single congenital extrahepatic shunts. Rectal ammonia tolerance testing and routine biochemical tests were performed preoperatively on all dogs. In seven dogs, preoperative abdominal Doppler ultrasonography was also performed. Exploratory laparotomy revealed a single extrahepatic portocaval shunt in each animal, which was attenuated using a cellophane band with an internal diameter of 2 to 3 mm. The abdomen was closed routinely. Follow-up biochemical analysis and abdominal Doppler ultrasonography or splenoportography were performed postoperatively. The shunt was not amenable to total ligation in 11 dogs, based upon reported criteria. All dogs recovered uneventfully from surgery without evidence of portal hypertension, and showed clinical improvement thereafter. Shunt occlusion was deemed to have occurred in 10 dogs based on resolution of biochemical and/or sonographic abnormalities. One dog continued to have sonographic evidence of portosystemic shunting when evaluated 3 weeks after surgery, despite normal ammonia tolerance, but was lost to subsequent follow-up. Two dogs, in which 3 mm cellophane bands were placed, experienced delayed shunt occlusion. Cellophane banding is simple to perform, and causes progressive attenuation of single extrahepatic shunts in dogs. Further work is needed to determine the maximum diameter of a cellophane band which will produce total attenuation, and the long-term safety and reliability of the treatment.

  7. Consequences of extrahepatic manifestations of hepatitis C viral infection (HCV

    Directory of Open Access Journals (Sweden)

    Agnieszka Pawełczyk

    2016-04-01

    Full Text Available The hepatitis C virus (HCV is a primarily hepatotropic virus. However, numerous extrahepatic symptoms are observed in patients chronically infected with HCV, e.g. cryoglobulinemia, lymphoproliferative disorders, kidney diseases, disturbances of the central and peripheral nervous system, thyroid gland, pancreas, lymph nodes and pituitary gland, that develop at various times after the infection. Complex mechanisms underlie these processes, both molecular, related to direct effects of the virus on cells or tissues and indirect mechanisms, resulting from the response of the immune system to infection (via cytokines or oxidative stress, and from the antiviral treatment used. Understanding these mechanisms may contribute to the definition of new prognostic factors, important for the early diagnosis of the infection, which in turn may improve treatment efficacy.This paper is a review of the incidence of selected extrahepatic manifestations of HCV infection and their underlying pathogenetic mechanisms and risk factors.

  8. Ulcerative Colitis in Colonic Interposition for Esophageal Atresia

    OpenAIRE

    Arshad, Hafiz Muhammad Sharjeel; Tetangco, Eula; Elkhatib, Imad

    2016-01-01

    A 38-year-old male with a history of colonic interposition for esophageal atresia as an infant presented with dysphagia and abdominal pain. On the basis of endoscopy findings, pathology, and response to therapy, he was found to have ulcerative colitis of the colonic conduit.

  9. Gadoxate-enhanced T1-weighted MR cholangiography: comparison of 1.5 T and 3.0 T

    Energy Technology Data Exchange (ETDEWEB)

    Koelblinger, C.; Schima, W.; Weber, M.; Mang, T.; Nemec, S.; Kulinna-Cosentini, C.; Bastati, N.; Ba-Ssalamah, A. [Universitaetsklinik fuer Radiodiagnostik, Medizinische Univ. Wien (Austria)

    2009-06-15

    Purpose: to qualitatively and quantitatively compare gadoxate-enhanced T1-weighted MR cholangiography at magnetic field strengths of 1.5 T and 3.0 T. Materials and methods: a total of 40 patients with a non-dilated biliary system were retrospectively included in the study. T1-weighted MR cholangiography 20 min after IV administration of 0.025 mmol/kg gadoxate (Primovist trademark) was performed in 20 patients at 1.5 T and in another 20 patients at 3.0 T. Contrast-to-noise ratios (CNR) of the biliary system (common bile duct - CBD, right hepatic duct - RHD, left hepatic duct - LHD) compared to the periductal tissue were measured. Two radiologists also qualitatively assessed the visibility of the intrahepatic and extrahepatic biliary system using a six-point rating scale. The Mann-Whitney U-test and Pearson's correlation coefficient were used for statistical analysis. Results: the CNRs of the intrahepatic and extrahepatic hepatic bile ducts were significantly higher at 3.0 T. Qualitative analysis showed a significant superiority for 3.0 T in the delineation of the intrahepatic biliary system (RHD, LHD, segmental ducts). (orig.)

  10. Broncho-biliary fistula secondary to biliary obstruction and lung abscess in a patient with pancreatic neuro-endocrine tumor.

    Science.gov (United States)

    Panda, Dipanjan; Aggarwal, Mayank; Yadav, Vikas; Kumar, Sachin; Mukund, Amar; Baghmar, Saphalta

    2016-06-01

    We present a case report of broncho-biliary fistula that developed due to the blockage of biliary stent placed during the management of pancreatic neuroendocrine tumor (pNET); diagnosed on high clinical suspicion, percutaneous cholangiogram and contrast enhanced computed tomography (CECT); and successfully treated with percutaneous transhepatic biliary drainage (PTBD). Copyright © 2016 National Cancer Institute, Cairo University. Production and hosting by Elsevier B.V. All rights reserved.

  11. Primary Segmental Volvulus Mimicking Ileal Atresia

    Science.gov (United States)

    Rao, Sadashiva; B Shetty, Kishan

    2013-01-01

    Neonatal intestinal volvulus in the absence of malrotation is a rare occurrence and rarer still is the intestinal volvulus in absence of any other predisposing factors. Primary segmental volvulus in neonates is very rare entity, which can have catastrophic outcome if not intervened at appropriate time. We report two such cases, which were preoperatively diagnosed as ileal atresia and intraoperatively revealed to be primary segmental volvulus of the ileum. PMID:26023426

  12. Plastic biliary stents for malignant biliary diseases

    NARCIS (Netherlands)

    Huibregtse, Inge; Fockens, Paul

    2011-01-01

    Plastic biliary endoprostheses have not changed much since their introduction more than 3 decades ago. Although their use has been challenged by the introduction of metal stents, plastic stents still remain commonly used. Much work has been done to improve the problem of stent obstruction but

  13. Treatment strategies in the management of jejunoileal and colonic atresia

    Directory of Open Access Journals (Sweden)

    Chadha Rajiv

    2006-01-01

    Full Text Available BACKGROUND/PURPOSE: The purpose of this prospective study was to review the operative findings, treatment strategies, as well as the results of management of 46 consecutive cases of jejunoileal and colonic atresia, managed over a 2-year period. MATERIALS AND METHODS: There were 42 patients with jejunoileal atresia (JIA and 4 with colonic atresia (CA. The 4 group types were: type I-membranous (n=20, type II- blind ends separated by a fibrous cord (n=6, type IIIa- blind ends with a V-shaped mesenteric defect (n=10, type IIIb- apple-peel atresia (n=4 and type IV- multiple atresias (n=6. Primary surgery for JIA consisted of resection with a single anastomosis (n=37, anastomosis after tapering jejunoplasty (n=3, multiple anastomosis (n=1 and a Bishop-Koop ileostomy (n=1. For CA, resection with primary anastomosis was performed. A single end-to-oblique anastomosis after adequate resection of dilated proximal bowel, was the preferred surgical procedure. In the absence of facilities for administering TPN, early oral/nasogastric (NG tube feeding was encouraged. In patients with anastomotic dysfunction, conservative treatment of the obstruction followed after its resolution by gradually increased NG feeds, was the preferred treatment protocol. RESULTS: Late presentation or diagnosis with hypovolemia, electrolyte imbalance, unconjugated hyperbilirubinemia (n=25 and sepsis (n=6, were significant preoperative findings. After resection and anastomosis, significant shortening of bowel length was seen in 16 patients (34.7%. Postoperative complications included an anastomotic leak (n=3, a perforation proximal to the anastomosis in 1 and anastomotic dysfunction in 5 patients. Full oral or NG tube feeding was possible only by the 13th to 31st postoperative day (POD, after the primary surgery in patients with anastomotic dysfunction and those undergoing reoperation. Overall, 38 patients survived (82.6%. Mortality was highest in patients with type IIIb or type IV

  14. Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators

    Directory of Open Access Journals (Sweden)

    Gürkan Altun

    2013-01-01

    Full Text Available Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein′s anomaly, Uhl′s anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.

  15. First branchial cleft sinus presenting with cholesteatoma and external auditory canal atresia.

    Science.gov (United States)

    Yalçin, Sinasi; Karlidağ, Turgut; Kaygusuz, Irfan; Demirbağ, Erhan

    2003-07-01

    First branchial cleft abnormalities are rare. They may involve the external auditory canal and middle ear. We describe a 6-year-old girl with congenital external auditory canal atresia, microtia, and cholesteatoma of mastoid and middle ear in addition to the first branchial cleft abnormalities. Clinical features of the patient are briefly described and the embryological relationship between first branchial cleft anomaly and external auditory canal atresia is discussed. The surgical management of these lesions may be performed, both the complete excision of the sinus and reconstructive otologic surgery.

  16. Methotrexate for primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Giljaca, Vanja; Poropat, Goran; Stimac, Davor

    2010-01-01

    Methotrexate has been used to treat patients with primary biliary cirrhosis as it possesses immunosuppressive properties. The previously prepared version of this review from 2005 showed that methotrexate seemed to significantly increase mortality in patients with primary biliary cirrhosis. Since...... that last review version, follow-up data of the included trials have been published....

  17. Management of Benign Biliary Strictures

    International Nuclear Information System (INIS)

    Laasch, Hans-Ulrich; Martin, Derrick F.

    2002-01-01

    Benign biliary strictures are most commonly a consequence of injury at laparoscopic cholecystectomy or fibrosis after biliary-enteric anastomosis. These strictures are notoriously difficult to treat and traditionally are managed by resection and fashioning of acholedocho- or hepato-jejunostomy. Promising results are being achieved with newer minimally invasive techniques using endoscopic or percutaneous dilatation and/or stenting and these are likely to play an increasing role in the management. Even low-grade biliary obstruction carries the risks of stone formation, ascending cholangitis and hepatic cirrhosis and it is important to identify and treat this group of patients. There is currently no consensus on which patient should have what type of procedure, and the full range of techniques may not be available in all hospitals. Careful assessment of the risks and likely benefits have to be made on an individual basis. This article reviews the current literature and discusses the options available. The techniques of endoscopic and percutaneous dilatation and stenting are described with evaluation of the likely success and complication rates and compared to the gold standard of biliary-enteric anastomosis

  18. Hepatobiliary scintigraphy in the assessment of long-term complication after biliary-enteric anastomosis: role in the diagnosis of post-operative segmental or total biliary obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Seung; Moon, Dae Hyuk; Lee, Sung Gyu; Lee, Yung Joo; Park, Kwang Min; Shin, Jung Woo; Ryu, Jin Sook; Lee, Hee Kyung [Asan Medicial Center, Seoul (Korea, Republic of)

    1998-07-01

    The purpose of this study was to investigate the accuracy of hepatobiliary scintigraphy (HBS) in the diagnosis of segmental or total biliary obstruction during long-term follow-up period after curative radical surgery with biliary-enteric anastomosis. The study population included 80 patients who underwent biliary-enteric anastomoses for benign (n=33) or malignant (n=47) biliary disease. Fifty-six of these 80 patients also underwent curative hepatic resection. Ninety eight hepatobiliary scintigrams using {sup 99m}Tc-DISIDA were performed at least 1 month after surgery (median 9 month). The scintigraphic criteria of total biliary obstruction we used were intestinal excretion beyond one hour or delayed hepatobiliary washout despite the presence of intestinal excretion. Segmental biliary obstruction was defined as delayed segmental excretion. The accuracy for biliary obstruction was evaluated according to different clinical situations. There were 9 instances with total biliary obstruction and 23 with segmental bile duct obstruction. Diagnosis of biliary obstruction was confirmed by percutaneous transhepatic cholangiography or surgery in 13, and follow-up clinical data for at least 6 months in 19 instances. Among the 32 instances with biliary symptoms and abnormal liver function, HBS allowed correct diagnosis in all 32(9 total, 14 segmental obstruction and 9 non-obstruction). Of the 40 with nonspecific symptom or isolated elevation of serum alkaline phosphatase, HBS diagnosed 8 of the 9 segmental biliary obstruction and 30 of the 31 non-obstruction. There were no biliary obstruction and no false positive result of scintigraphy in 26 instances which had no clinical symptom or signs of biliary obstruction. Diagnostic sensitivity of HBS was 100% (9/9) for total biliary obstruction, and 96%(22/23) for segmental bile obstruction. Specificity was 98%(39/40) in patients who had abnormal symptom or sign. Hepatobiliary scintigraphy is a highly accurate modality in the

  19. Hepatobiliary scintigraphy in the assessment of long-term complication after biliary-enteric anastomosis: role in the diagnosis of post-operative segmental or total biliary obstruction

    International Nuclear Information System (INIS)

    Kim, Jae Seung; Moon, Dae Hyuk; Lee, Sung Gyu; Lee, Yung Joo; Park, Kwang Min; Shin, Jung Woo; Ryu, Jin Sook; Lee, Hee Kyung

    1998-01-01

    The purpose of this study was to investigate the accuracy of hepatobiliary scintigraphy (HBS) in the diagnosis of segmental or total biliary obstruction during long-term follow-up period after curative radical surgery with biliary-enteric anastomosis. The study population included 80 patients who underwent biliary-enteric anastomoses for benign (n=33) or malignant (n=47) biliary disease. Fifty-six of these 80 patients also underwent curative hepatic resection. Ninety eight hepatobiliary scintigrams using 99m Tc-DISIDA were performed at least 1 month after surgery (median 9 month). The scintigraphic criteria of total biliary obstruction we used were intestinal excretion beyond one hour or delayed hepatobiliary washout despite the presence of intestinal excretion. Segmental biliary obstruction was defined as delayed segmental excretion. The accuracy for biliary obstruction was evaluated according to different clinical situations. There were 9 instances with total biliary obstruction and 23 with segmental bile duct obstruction. Diagnosis of biliary obstruction was confirmed by percutaneous transhepatic cholangiography or surgery in 13, and follow-up clinical data for at least 6 months in 19 instances. Among the 32 instances with biliary symptoms and abnormal liver function, HBS allowed correct diagnosis in all 32(9 total, 14 segmental obstruction and 9 non-obstruction). Of the 40 with nonspecific symptom or isolated elevation of serum alkaline phosphatase, HBS diagnosed 8 of the 9 segmental biliary obstruction and 30 of the 31 non-obstruction. There were no biliary obstruction and no false positive result of scintigraphy in 26 instances which had no clinical symptom or signs of biliary obstruction. Diagnostic sensitivity of HBS was 100% (9/9) for total biliary obstruction, and 96%(22/23) for segmental bile obstruction. Specificity was 98%(39/40) in patients who had abnormal symptom or sign. Hepatobiliary scintigraphy is a highly accurate modality in the evaluation of

  20. Primary biliary cirrhosis--autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

    Science.gov (United States)

    Pamfil, Cristina; Candrea, Elisabeta; Berki, Emese; Popov, Horațiu I; Radu, Pompilia I; Rednic, Simona

    2015-03-01

    Autoimmune liver diseases may be associated with extrahepatic autoimmune pathology. We report the case of a 52-year old woman who initially presented to the gastroenterology department for extreme fatigue, pale stools, dark urine and pruritus. Laboratory tests showed significant cholestasis and elevation of aminotransferase levels. Immunological tests revealed positive antinuclear (ANA=1:320) and antimitochondrial antibodies (AMA=1:40) with negative anti-smooth muscle and liver kidney microsomal type 1 antibodies. The biopsy was compatible with overlap syndrome type 1. The patient was commenced on immunosuppressive therapy according to standard of care (azathioprine 50mg, ursodeoxycholic acid and prednisone 0.5mg/kg), with moderate biochemical improvement. She subsequently developed proximal symmetrical weakness and cutaneous involvement and was diagnosed with biopsy-proven dermatomyositis. The immunosuppressive regimen was intensified to 150 mg azathioprine. At the three-month follow-up, her symptoms subsided and aminotransferases and muscle enzymes normalized. Upon further investigation the patient was diagnosed with autoimmune thyroiditis and antiphospholipid syndrome. To our knowledge, this is the first case of primary biliary cirrhosis - autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

  1. A functional study on small intestinal smooth muscles in jejunal atresia

    Directory of Open Access Journals (Sweden)

    Preeti Tyagi

    2016-01-01

    Full Text Available Aim: The present study was aimed to assess the contractile status of neonatal small intestinal smooth muscle of dilated pre-atretic part of intestinal atresia to resolve debatable issues related to mechanisms of persistent dysmotility after surgical repair. Materials and Methods: A total of 34 longitudinally sectioned strips were prepared from pre-atretic dilated part of freshly excised 8 jejunal atresia type III a cases. Spontaneous as well as acetylcholine- and histamine-induced contractions were recorded in vitro by using organ bath preparations. Chemically evoked contractions were further evaluated after application of atropine (muscarinic blocker, pheniramine (H1 blocker, and lignocaine (neuronal blocker to ascertain receptors and neuronal involvement. Histological examinations of strips were made by using Masson trichrome stain to assess the fibrotic changes. Results: All 34 strips, except four showed spontaneous contractions with mean frequency and amplitude of 5.49 ± 0.26/min and 24.41 ± 5.26 g/g wet tissue respectively. The response to ACh was nearly twice as compared to histamine for equimolar concentrations (100 μM. ACh (100 μM induced contractions were attenuated (by 60% by atropine. Histamine (100 μM-induced contractions was blocked by pheniramine (0.32 μM and lignocaine (4 μM by 74% and 78%, respectively. Histopathological examination showed varying degree of fibrotic changes in muscle layers. Conclusions: Pre-atretic dilated part of jejunal atresia retains functional activity but with definitive histopathologic abnormalities. It is suggested that excision of a length of pre-atretic part and early stimulation of peristalsis by locally acting cholinomimetic or H1 agonist may help in reducing postoperative motility problems in atresia patients.

  2. A functional study on small intestinal smooth muscles in jejunal atresia.

    Science.gov (United States)

    Tyagi, Preeti; Mandal, Maloy B; Gangopadhyay, Ajay N; Patne, Shashikant C U

    2016-01-01

    The present study was aimed to assess the contractile status of neonatal small intestinal smooth muscle of dilated pre-atretic part of intestinal atresia to resolve debatable issues related to mechanisms of persistent dysmotility after surgical repair. A total of 34 longitudinally sectioned strips were prepared from pre-atretic dilated part of freshly excised 8 jejunal atresia type III a cases. Spontaneous as well as acetylcholine- and histamine-induced contractions were recorded in vitro by using organ bath preparations. Chemically evoked contractions were further evaluated after application of atropine (muscarinic blocker), pheniramine (H1 blocker), and lignocaine (neuronal blocker) to ascertain receptors and neuronal involvement. Histological examinations of strips were made by using Masson trichrome stain to assess the fibrotic changes. All 34 strips, except four showed spontaneous contractions with mean frequency and amplitude of 5.49 ± 0.26/min and 24.41 ± 5.26 g/g wet tissue respectively. The response to ACh was nearly twice as compared to histamine for equimolar concentrations (100 μM). ACh (100 μM) induced contractions were attenuated (by 60%) by atropine. Histamine (100 μM)-induced contractions was blocked by pheniramine (0.32 μM) and lignocaine (4 μM) by 74% and 78%, respectively. Histopathological examination showed varying degree of fibrotic changes in muscle layers. Pre-atretic dilated part of jejunal atresia retains functional activity but with definitive histopathologic abnormalities. It is suggested that excision of a length of pre-atretic part and early stimulation of peristalsis by locally acting cholinomimetic or H1 agonist may help in reducing postoperative motility problems in atresia patients.

  3. Pharmacokinetics and Biliary Excretion of Fisetin in Rats.

    Science.gov (United States)

    Huang, Miao-Chan; Hsueh, Thomas Y; Cheng, Yung-Yi; Lin, Lie-Chwen; Tsai, Tung-Hu

    2018-06-14

    The hypothesis of this study is that fisetin and phase II conjugated forms of fisetin may partly undergo biliary excretion. To investigate this hypothesis, male Sprague-Dawley rats were used for the experiment, and their bile ducts were cannulated with polyethylene tubes for bile sampling. The pharmacokinetic results demonstrated that the average area-under-the-curve (AUC) ratios ( k (%) = AUC conjugate /AUC free-form ) of fisetin, its glucuronides, and its sulfates were 1:6:21 in plasma and 1:4:75 in bile, respectively. Particularly, the sulfated metabolites were the main forms that underwent biliary excretion. The biliary excretion rate ( k BE (%) = AUC bile /AUC plasma ) indicates the amount of fisetin eliminated by biliary excretion. The biliary excretion rates of fisetin, its glucuronide conjugates, and its sulfate conjugates were approximately 144, 109, and 823%, respectively, after fisetin administration (30 mg/kg, iv). Furthermore, biliary excretion of fisetin is mediated by P-glycoprotein.

  4. Evaluation of late stage iatrogenic extrahepatic bile duct stricture by using magnetic resonance cholangiopancreagraphy

    International Nuclear Information System (INIS)

    Sun Changjin; Zhou Xiangping; Liu Rongbo; Song Bin; Yan Zhihan; Chen Xian; Wang Wentao; Xiong Yan; Xu Minsheng; Gu Jianping

    2003-01-01

    Objective: To assess the role of magnetic resonance cholangiopancreagraphy (MRCP) in the preoperative evaluation of late stage iatrogenic extrahepatic bile duct stricture. Methods: Eighteen cases, which were diagnosed as late stage iatrogenic extrahepatic bile duct strictures only by MRCP and proved by surgery and pathology, were classified according to Bismuth classification and the surgical operation strategy was planed on the basis of MR cholangiopancreagraphic findings before surgery. The results were compared with surgery. Results: Diagnostic MR Cholangiopancreagrams were acquired in 18 patients. Among all 18 patients, the level of stricture was classified as Bismuth I in 3 patients, Bismuth II in 7 patients, Bismuth III in 3 patients, and Bismuth IV in 3 patients. A Bismuth II stricture was incorrectly classified as a Bismuth III lesion. On the basis of MR cholangiopancreagraphic findings, a surgical operation strategy can be planed. The therapeutic plan anticipated with MRCP matched the actually used procedure in 16 of 18 patients. conclusion: MRCP plays an important role in the evaluation of late stage iatrogenic extrahepatic bile duct stricture

  5. Congenital bronchial atresia coexistent with intralobar pulmonary sequestration: a case report

    International Nuclear Information System (INIS)

    Han, Young Min; Ku, Ja Hong; Lee, Dong Keun; Chung, Kyung Ho; Kim, Chong Soo; Sohn, Myung Hee; Choi, Ki Chul

    1995-01-01

    Bronchial atresia coexistent with intralobar pulmonary sequestration is so rare that only two cases have been reported in the literature. We report a case of congential bronchial atresia coexistent with intralobar pulmonary sequestation in a 51-year-old woman. Computed tomography showed the branching mass with hyperinflation of adjacent pulmonary parenchyma in the medial segment of the right middle lobe and a large thin-walled cystic mass with air-fluid levels in the medial basal segment of the right lower lobe. Selective inferior phrenic arteriography showed two aberrant arteries supplying the large cystic mass in the right lower lobe. The venous drainage was through the right pulmonary vein

  6. Congenital bronchial atresia coexistent with intralobar pulmonary sequestration: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Han, Young Min; Ku, Ja Hong; Lee, Dong Keun; Chung, Kyung Ho; Kim, Chong Soo; Sohn, Myung Hee; Choi, Ki Chul [Chonbuk National University Medical School, Jeonju (Korea, Republic of)

    1995-02-15

    Bronchial atresia coexistent with intralobar pulmonary sequestration is so rare that only two cases have been reported in the literature. We report a case of congential bronchial atresia coexistent with intralobar pulmonary sequestation in a 51-year-old woman. Computed tomography showed the branching mass with hyperinflation of adjacent pulmonary parenchyma in the medial segment of the right middle lobe and a large thin-walled cystic mass with air-fluid levels in the medial basal segment of the right lower lobe. Selective inferior phrenic arteriography showed two aberrant arteries supplying the large cystic mass in the right lower lobe. The venous drainage was through the right pulmonary vein.

  7. Protein induced by vitamin K absence or antagonist-II production is a strong predictive marker for extrahepatic metastases in early hepatocellular carcinoma: a prospective evaluation

    International Nuclear Information System (INIS)

    Bae, Hyun-Mi; Lee, Jeong-Hoon; Yoon, Jung-Hwan; Kim, Yoon Jun; Heo, Dae Seog; Lee, Hyo-Suk

    2011-01-01

    Clinicians often experience extrahepatic metastases associated with hepatocellular carcinoma (HCC), even if no evidence of intrahepatic recurrence after treatment is observed. We investigated the pretreatment predictors of extrahepatic metastases in HCC patients. Patients diagnosed with HCC without evidence of extrahepatic metastases were prospectively enrolled. We evaluated the correlation between extrahepatic metastases and pretreatment clinical variables, including serum tumor markers. A total of 354 patients were included. Seventy-six patients (21%) had extrahepatic metastases during the observation period (median, 25.3 months; range, 0.6-51.3 months). Cox regression multivariate analysis showed that serum protein induced by vitamin K absence or antagonist-II (PIVKA-II) production levels, the intrahepatic tumor stage, platelet count, and portal vein thrombosis were independent risk factors for extrahepatic metastases. Patients with a PIVKA-II production ≥ 300 mAU/mL had a 2.7-fold (95% confidence interval; 1.5-4.8; P < 0.001) and 3.7-fold (95% confidence interval; 2.0-6.6; P < 0.001) increased risk for extrahepatic metastases after adjustment for stage, platelet count, alpha-fetoprotein ≥ 400 ng/mL, and portal vein thrombosis according to the AJCC and BCLC staging systems, respectively. PIVKA-II production levels might be a good candidate predictive marker for extrahepatic HCC metastases, especially in patients with smaller and/or fewer tumors in the liver with in stages regardless of serum alpha-fetoprotein

  8. Biliary complications after orthotopic liver transplantation

    NARCIS (Netherlands)

    Karimian, Negin; Westerkamp, Andrie C.; Porte, Robert J.

    Purpose of reviewThe incidence, pathogenesis and management of the most common biliary complications are summarized, with an emphasis on nonanastomotic biliary strictures (NAS) and potential strategies to prevent NAS after liver transplantation.Recent findingsNAS have variable presentations in time

  9. Definition of ACLF and inclusion criteria for extra-hepatic organ failure.

    Science.gov (United States)

    Wang, Xiaojing; Sarin, Shiv Kumar; Ning, Qin

    2015-07-01

    A prominent characteristic of ACLF is rapid hepatic disease progression with subsequent extra-hepatic organ failure, manifesting as either hepatic coma or hepatorenal syndrome, which is associated with a high mortality rate in a short time. The APASL definition mainly emphasizes recognizing patients with hepatic failure. These patients may subsequently develop extra-hepatic multisystem organ failure leading to high mortality. It is therefore worthwhile to identify the short interim period between the development of liver failure and the onset of extra-hepatic organ failure, the potential therapeutic 'golden window.' Interventions during this period may prevent the development of complications and eventually change the course of the illness. Organ failure is suggested to be a central component of ACLF and may behave differently from chronic decompensated liver disease. Clear and practical criteria for the inclusion of organ failure are urgently needed so that patients with these life-threatening complications can be treated in a timely and appropriate manner. Recent studies suggested that the scoring systems evaluating organ failure [acute physiology, age and chronic health evaluation (APACHE) and sequential organ failure assessment (SOFA) scores] work better than those addressing the severity of liver disease [Child-Pugh and model of end-stage liver disease (MELD) scores] in ACLF. However, a key problem remains that the former scoring systems are reflective of organ failure and not predictive, thus limiting their value as an early indication for intervention.

  10. Biliary tract duplication cyst with gastric heterotopia

    Energy Technology Data Exchange (ETDEWEB)

    Grumbach, K.; Baker, D.H.; Weigert, J.; Altman, R.P.

    1988-05-01

    Cystic duplications of the biliary tract are rare anomalies, easily mistaken for choledochal cysts. Surgical drainage is the preferred therapy for choledochal cyst, but cystic duplication necessitates surgical excision as duplications may contain heterotopic gastric mucosa leading to peptic ulceration of the biliary tract. We report a case of biliary tract duplication cyst containing heterotopic alimentary mucosa which had initially been diagnosed and surgically treated as a choledochal cyst.

  11. Biliary tract duplication cyst with gastric heterotopia

    International Nuclear Information System (INIS)

    Grumbach, K.; Baker, D.H.; Weigert, J.; Altman, R.P.

    1988-01-01

    Cystic duplications of the biliary tract are rare anomalies, easily mistaken for choledochal cysts. Surgical drainage is the preferred therapy for choledochal cyst, but cystic duplication necessitates surgical excision as duplications may contain heterotopic gastric mucosa leading to peptic ulceration of the biliary tract. We report a case of biliary tract duplication cyst containing heterotopic alimentary mucosa which had initially been diagnosed and surgically treated as a choledochal cyst. (orig.)

  12. Endoscopic management of biliary injuries and leaks

    Directory of Open Access Journals (Sweden)

    T S Chandrasekar

    2012-01-01

    Full Text Available Bile duct injuries and subsequent leaks can occur following laparoscopic and open cholecystectomies and also during other hepatobiliary surgeries. Various patient related and technical factors are implicated in the causation of biliary injuries. Over a period of twenty five years managing such patients of biliary injuries our team has found a practical approach to assess the cause of biliary injuries based on the symptoms, clinical examination and imaging. Bismuth classification is helpful in most of the cases. Immediate referral to a centre experienced in the management of bile duct injury and timely intervention is associated with improved outcomes. Resuscitation, correcting dyselectrolytemia, aspiration of undrained biloma and antibiotics take the priority in the management. The goal is to restore the bile conduit, and to prevent short and longterm complications such as biliary fistula, intra-abdominal abscess, biliary stricture, recurrent cholangitis and secondary biliary cirrhosis. Endoscopic therapy by reducing the transpapillary pressure gradient helps in reducing the leak. Endoscopic therapy with biliary sphincterotomy alone or with additional placement of a biliary stent/ nasobiliary drainage is advocated. In our tertiary care referral unit, we found endoscopic interventions are useful in situations where there is leak with associated CBD calculus or a foreign body, peripheral bile duct injury, cystic duct stump leak and partial bile duct injury with leak/ narrowing of the lumen. Endotherapy is not useful in case of complete transection (total cut off and complete stricture involving common hepatic or common bile ducts. In conclusion, endoscopic treatment can be considered a highly effective therapy and should be the first-line therapy in such patients. Though less successful, an endoscopic attempt is warranted in patients suffering from central bile duct leakages failing which surgical management is recommended.

  13. A case of biliary stones and anastomotic biliary stricture after liver transplant treated with the rendez - vous technique and electrokinetic lithotritor

    Institute of Scientific and Technical Information of China (English)

    Marta Di Pisa; Mario Traina; Roberto Miraglia; Luigi Maruzzelli; Riccardo Volpes; Salvatore Piazza; Angelo Luca; Bruno Gridelli

    2008-01-01

    The paper studies the combined radiologic and endoscopic approach (rendezvous technique) to the treatment of the biliary complications following liver transplant. The "rendez-vous" technique was used with an electrokinetic lithotripter, in the treatment of a biliary anastomotic stricture with multiple biliary stones in a patient who underwent orthotopic liver transplant. In this patient, endoscopic or percutaneous transhepatic management of the biliary complication failed. The combined approach, percutaneous transhepatic and endoscopic treatment (rendez-vous technique) with the use of an electrokinetic lithotritor, was used to solve the biliary stenosis and to remove the stones.Technical success, defined as disappearance of the biliary stenosis and stone removal, was obtained in just one session, which definitively solved the complications.The combined approach of percutaneous transhepatic and endoscopic (rendez-vous technique) treatment, in association with an electrokinetic lithotritor, is a safe and feasible alternative treatment, especially after the failure of endoscopic and/or percutaneous trans-hepatic isolated procedures.

  14. The role of radiotherapy in the management of extrahepatic bile duct cancer: an analysis of 145 consecutive patients treated with intraluminal and/or external beam radiotherapy

    International Nuclear Information System (INIS)

    Kamada, Tadashi; Saitou, Hiroya; Takamura, Akio; Nojima, Takayuki; Okushiba, Shun-Ichi

    1996-01-01

    Purpose: To determine the feasibility of high dose radiotherapy and to evaluate its role in the management of extrahepatic bile duct (EHBD) cancer. Methods and Materials: Between 1983 and 1991, 145 consecutive patients with EHBD cancer were treated by low dose rate intraluminal 192 Ir irradiation (ILRT) either alone or in combination with external beam radiotherapy (EBRT). Among the primarily irradiated, 77 patients unsuitable for surgical resection, 54 were enrolled in radical radiotherapy, and 23 received palliative radiotherapy. Fifty-nine received postoperative radiotherapy, and the remaining 9 preoperative radiotherapy. The mean radiation dose was 67.8 Gy, ranging from 10 to 135 Gy. Intraluminal 192 Ir irradiation was indicated in 103 patients, and 85 of them were combined with EBRT. Expandable metallic biliary endoprosthesis (EMBE) was used in 32 primarily irradiated patients (31 radical and 1 palliative radiotherapy) after the completion of radiotherapy. Results: The 1-, 3-, and 5-year actuarial survival rates for all 145 patients were 55%, 18%, and 10%, for the 54 patients treated by radical radiotherapy (mean 83.1 Gy), 56%, 13%, and 6% [median survival time (MST) 12.4 months], and for the 59 patients receiving postoperative radiotherapy (mean 61.6 Gy), 73%, 31%, and 18% (MST 21.5 months), respectively. Expandable metallic biliary endoprosthesis was useful for the early establishment of an internal bile passage in radically irradiated patients and MST of 14.9 months in these 31 patients was significantly longer than that of 9.3 months in the remaining 23 patients without EMBE placement (p < 0.05). Eighteen patients whose surgical margins were positive in the hepatic side bile duct(s) showed significantly better survival compared with 15 patients whose surgical margins were positive in the adjacent structure(s) (44% vs. 0% survival at 3 years, p < 0.001). No survival benefit was obtained in patients given palliative or preoperative radiotherapy

  15. Evidence-Based Decompression in Malignant Biliary Obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Ho, Chia Sing [University of Toronto, Toronto General Hospital, Toronto (Canada); Warkentin, Andrew E [University of Toronto, 1 King& #x27; s College Circle, Toronto (Canada)

    2012-02-15

    As recent advances in chemotherapy and surgical treatment have improved outcomes in patients with biliary cancers, the search for an optimal strategy for relief of their obstructive jaundice has become even more important. Without satisfactory relief of biliary obstruction, many patients would be ineligible for treatment. We review all prospective randomized trials and recent retrospective non-randomized studies for evidence that would support such a strategy. For distal malignant biliary obstruction, an optimal strategy would be insertion of metallic stents either endoscopically or percutaneously. Evidence shows that a metallic stent inserted percutaneously has better outcomes than plastic stents inserted endoscopically. For malignant hilar obstruction, percutaneous biliary drainage with or without metallic stents is preferred.

  16. Atresia biliar: una enfermedad grave

    OpenAIRE

    Ramonet, Margarita; Ciocca, Mirta; Alvarez, Fernando

    2014-01-01

    La atresia biliar es una grave enfermedad que se manifiesta en los recién nacidos, y se desconoce su causa. La inflamación y destrucciónprogresiva de los conductos biliares conducen a la aparición de ictericia, coluria y acolia entre la segunda y sexta semana de vida. Como existen múltiples causas de colestasis neonatal en esta etapa de la vida, es necesario realizar un diagnóstico y derivación precoz para ofrecer un tratamiento quirúrgico, con el fin de restablecer el flujo biliar. Alrededor...

  17. Preoperative biliary drainage for pancreatic cancer

    NARCIS (Netherlands)

    van Heek, N. T.; Busch, O. R.; van Gulik, T. M.; Gouma, D. J.

    2014-01-01

    This review is to summarize the current knowledge about preoperative biliary drainage (PBD) in patients with biliary obstruction caused by pancreatic cancer. Most patients with pancreatic carcinoma (85%) will present with obstructive jaundice. The presence of toxic substances as bilirubin and bile

  18. Characterization of the coagulation profile in children with liver disease and extrahepatic portal vein obstruction or shunt.

    Science.gov (United States)

    Beattie, William; Magnusson, Maria; Hardikar, Winita; Monagle, Paul; Ignjatovic, Vera

    2017-03-01

    Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children. Children with severe liver disease had coagulopathic changes, including either decreased concentration or activity of factor II, factor V, and factor VII. Nineteen percent (8/42) of the cohort had significant bleeding. Thrombophilic changes were also observed, including decreased concentration or activity of protein C, protein S, and antithrombin and increased concentration and activity of factor VIII and Von Willebrand factor. Similar coagulation factor changes were observed in children with extrahepatic portal vein obstruction or shunt. There was a trend toward greater changes in coagulation factor activity compared to concentration. This study provides a detailed description of the changes in both the concentration and activity of coagulation factors in pediatric liver disease and extrahepatic portal vein obstruction or shunt. Interestingly, procoagulant and anticoagulant factors were deranged in portal vein obstruction or shunt to a similar degree as in liver disease. An improved understanding of the coagulation profile in the pediatric setting will contribute to the improved management of liver disease and extrahepatic portal obstruction or shunt. PELD: pediatric end-stage liver disease score; MELD: model for end-stage liver disease score; ELISA: enzyme-linked immunosorbent assay; MCRI: Murdoch Childrens

  19. Diurnal variation in the biliary excretion of flomoxef in patients with percutaneous transhepatic biliary drainage.

    Science.gov (United States)

    Hishikawa, S; Kobayashi, E; Sugimoto , K; Miyata, M; Fujimura, A

    2001-07-01

    To examine diurnal variation in biliary excretion of flomoxef. Flomoxef (1 g) was injected intravenously in eight patients with percutaneous transhepatic cholangiography with drainage at 09.00 h and 21.00 h by a cross-over design with a 36 h washout period. Drained biliary fluid was collected for 6 h after each dosing. These patients still had mild to moderate hepatic dysfunction. Bile flow and bile acid excretion for 6 h after dosing did not differ significantly between the 09.00 h and 21.00 h treatments. The maximum concentration of biliary flomoxef was significantly greater and its total excretion for 6 h tended to be greater after the 21.00 h dose [maximum concentration (microg ml(-1)): 34.2 +/- 29.9 (09.00 h dose) vs 43.5 +/- 28.3 (21.00 h dose) (95% confidence interval for difference: 2.6 approximately 15.9, P = 0.013); total excretion (mg 6 h(-1)): 1.4 +/- 1.3 (09.00 h dose) vs 1.6 +/- 1.2 (21.00 h dose) (95% confidence interval for difference: -26.8, 313.7, P = 0.087)]. The period that biliary flomoxef remained above the minimal inhibitory concentration did not differ significantly between the two treatment times. These results suggest that biliary excretion of flomoxef shows diurnal variation. However, as the difference was relatively small, flomoxef could be given at any time of day without any dosage adjustments.

  20. Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case ...

    African Journals Online (AJOL)

    Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case report and literature ... skin over the right leg from the knee joint up to the middle of the right foot. ... Examination indicated no signs of child abuse; the parents refused an ...

  1. A comparison between endoscopic ultrasound-guided rendezvous and percutaneous biliary drainage after failed ERCP for malignant distal biliary obstruction.

    Science.gov (United States)

    Bill, Jason G; Darcy, Michael; Fujii-Lau, Larissa L; Mullady, Daniel K; Gaddam, Srinivas; Murad, Faris M; Early, Dayna S; Edmundowicz, Steven A; Kushnir, Vladimir M

    2016-09-01

    Selective biliary cannulation is unsuccessful in 5 % to 10 % of patients undergoing endoscopic retrograde cholangiopancreatography (ERCP) for malignant distal biliary obstruction (MDBO). Percutaneous biliary drainage (PBD) has been the gold standard, but endoscopic ultrasound guided rendezvous (EUSr) have been increasingly used for biliary decompression in this patient population. Our aim was to compare the initial success rate, long-term efficacy, and safety of PBD and EUSr in relieving MDBO after failed ERC Patients and methods: A retrospective study involving 50 consecutive patients who had an initial failed ERCP for MDBO. Twenty-five patients undergoing EUSr between 2008 - 2014 were compared to 25 patients who underwent PBD immediately prior to the introduction of EUSr at our center (2002 - 2008). Comparisons were made between the two groups with regard to technical success, duration of hospital stay and adverse event rates after biliary decompression. The mean age at presentation was 66.5 (± 12.6 years), 28 patients (54.9 %) were female. The etiology of MDBO was pancreaticobiliary malignancy in 44 (88 %) and metastatic disease in 6 (12 %) cases. Biliary drainage was technically successful by EUSr in 19 (76 %) cases and by PBD in 25 (100 %) (P = 0.002). Median length of hospital stay after initial drainage was 1 day in the EUSr group vs 5 days in PBD group (P = 0.02). Repeat biliary intervention was required for 4 patients in the EUSr group and 15 in the PBD group (P = 0.001). Initial technical success with EUSr was significantly lower than with PBD, however when EUSr was successful, patients had a significantly shorter post-procedure hospital stay and required fewer follow-up biliary interventions. Meeting presentations: Annual Digestive Diseases Week 2015.

  2. Functional Self-Expandable Metal Stents in Biliary Obstruction

    Science.gov (United States)

    Kwon, Chang-Il; Ko, Kwang Hyun; Hahm, Ki Baik

    2013-01-01

    Biliary stents are widely used not only for palliative treatment of malignant biliary obstruction but also for benign biliary diseases. Each plastic stent or self-expandable metal stent (SEMS) has its own advantages, and a proper stent should be selected carefully for individual condition. To compensate and overcome several drawbacks of SEMS, functional self-expandable metal stent (FSEMS) has been developed with much progress so far. This article looks into the outcomes and defects of each stent type for benign biliary stricture and describes newly introduced FSEMSs according to their functional categories. PMID:24143314

  3. Anaesthesia for oesophageal atresia with or without tracheo ...

    African Journals Online (AJOL)

    Oesophageal atresia, with or without tracheo-oesophageal fistula, is one of the most challenging conditions with which the anaesthesiologist has to deal during the perioperative period. The patients are usually in their first few days of life, and might be premature with inherent airway problems. This is then compounded by ...

  4. Radiological evaluation of pulmonary atresia: An analysis of cineangiography in 32 cases

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Ho; Yeon, Kyung Mo; Park, Jae Hyung; Han, Man Chung; Yoon, Yong Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1983-06-15

    Total 32 cases of pulmonary atresia were diagnosed radiographically at Seoul National University Hospital from March, 1979 to August 1982. Some characteristic radiological findings were analyzed in chest PA and cineangiographies. The results were as follows: 1. In the evaluation of chest PA, cardiomegaly was noticed in 16 cases, dextrocardia in 8 cases, elevated cardiac apex in 7 cases and right-sided aortic arch in 6 cases. The pulmonary vascularties were mildly decreased in 20 cases, markedly decreased in 9 cases and decreased with reticular pattern in 3 cases. 2. As final diagnoses after cineangiography, pulmonary atresia was associated with Tetralogy of Fallot variant in 17 cases, transposition of great vessels in 7 cases, single ventricle in 5 cases, tricuspid atresia in 2 cases and intact ventricular septum in 1 case. 3. The classification according to the pattern of pulmonary artery is main pulmonary trunk with PDA(Type Ia) in 10 cases, pulmonary arterial confluence with PDA (Type Ib) in 10 cases, no pulmonary arterial confluence with PDA (Type Ic) in 5 cases, main pulmonary trunk without PDA (Type IIa) in 0 case, pulmonary arterial confluence without PDA (Type IIb) in 5 cases, and no pulmonary arterial confluence without PDA ( Type IIc) in 2 cases. 4. Pulmonary wedge venography was done and successful in 8 cases. Among them, confluence between right and left pulmonary arteries was noticed in 5 cases. 5. Biventricular cineangiograpy and/or pulmonary wedge venography, if necessary, is essential for the accurate diagnosis of pulmonary atresia to demonstrate detailed anatomy of pulmonary artery.

  5. Radiological evaluation of pulmonary atresia: An analysis of cineangiography in 32 cases

    International Nuclear Information System (INIS)

    Lee, Dong Ho; Yeon, Kyung Mo; Park, Jae Hyung; Han, Man Chung; Yoon, Yong Soo

    1983-01-01

    Total 32 cases of pulmonary atresia were diagnosed radiographically at Seoul National University Hospital from March, 1979 to August 1982. Some characteristic radiological findings were analyzed in chest PA and cineangiographies. The results were as follows: 1. In the evaluation of chest PA, cardiomegaly was noticed in 16 cases, dextrocardia in 8 cases, elevated cardiac apex in 7 cases and right-sided aortic arch in 6 cases. The pulmonary vascularties were mildly decreased in 20 cases, markedly decreased in 9 cases and decreased with reticular pattern in 3 cases. 2. As final diagnoses after cineangiography, pulmonary atresia was associated with Tetralogy of Fallot variant in 17 cases, transposition of great vessels in 7 cases, single ventricle in 5 cases, tricuspid atresia in 2 cases and intact ventricular septum in 1 case. 3. The classification according to the pattern of pulmonary artery is main pulmonary trunk with PDA(Type Ia) in 10 cases, pulmonary arterial confluence with PDA (Type Ib) in 10 cases, no pulmonary arterial confluence with PDA (Type Ic) in 5 cases, main pulmonary trunk without PDA (Type IIa) in 0 case, pulmonary arterial confluence without PDA (Type IIb) in 5 cases, and no pulmonary arterial confluence without PDA ( Type IIc) in 2 cases. 4. Pulmonary wedge venography was done and successful in 8 cases. Among them, confluence between right and left pulmonary arteries was noticed in 5 cases. 5. Biventricular cineangiograpy and/or pulmonary wedge venography, if necessary, is essential for the accurate diagnosis of pulmonary atresia to demonstrate detailed anatomy of pulmonary artery

  6. Formation of intestinal atresias in the Fgfr2IIIb-/- mice is not associated with defects in notochord development or alterations in Shh expression.

    Science.gov (United States)

    Reeder, Amy L; Botham, Robert A; Franco, Marta; Zaremba, Krzysztof M; Nichol, Peter F

    2012-09-01

    The etiology of intestinal atresia remains elusive but has been ascribed to a number of possible events including in utero vascular accidents, failure of recanalization of the intestinal lumen, and mechanical compression. Another such event that has been postulated to be a cause in atresia formation is disruption in notochord development. This hypothesis arose from clinical observations of notochord abnormalities in patients with intestinal atresias as well as abnormal notochord development observed in a pharmacologic animal model of intestinal atresia. Atresias in this model result from in utero exposure to Adriamycin, wherein notochord defects were noted in up to 80% of embryos that manifested intestinal atresias. Embryos with notochord abnormalities were observed to have ectopic expression of Sonic Hedgehog (Shh), which in turn was postulated to be causative in atresia formation. We were interested in determining whether disruptions in notochord development or Shh expression occurred in an established genetic model of intestinal atresia and used the fibroblast growth factor receptor 2IIIb homozygous mutant (Fgfr2IIIb-/-) mouse model. These embryos develop colonic atresias (100% penetrance) and duodenal atresias (42% penetrance). Wild-type and Fgfr2IIIb-/- mouse embryos were harvested at embryonic day (E) 10.5, E11.5, E12.5, and E13.5. Whole-mount in situ hybridization was performed on E10.5 embryos for Shh. Embryos at each time point were harvested and sectioned for hematoxylin-eosin staining. Sections were photographed specifically for the notochord and resulting images reconstructed in 3-D using Amira software. Colons were isolated from wild-type and Fgfr2IIIb-/- embryos at E10.5, then cultured for 48 hours in Matrigel with FGF10 in the presence or absence of exogenous Shh protein. Explants were harvested, fixed in formalin, and photographed. Fgfr2IIIb-/- mouse embryos exhibit no disruptions in Shh expression at E10.5, when the first events in atresia

  7. Resection of Concomitant Hepatic and Extrahepatic Metastases from Colorectal Cancer - A Worthwhile Operation?

    Science.gov (United States)

    Diaconescu, Andrei; Alexandrescu, Sorin; Ionel, Zenaida; Zlate, Cristian; Grigorie, Razvan; Brasoveanu, Vladislav; Hrehoret, Doina; Ciurea, Silviu; Botea, Florin; Tomescu, Dana; Droc, Gabriela; Croitoru, Adina; Herlea, Vlad; Boros, Mirela; Grasu, Mugur; Dumitru, Radu; Toma, Mihai; Ionescu, Mihnea; Vasilescu, Catalin; Popescu, Irinel

    2017-01-01

    Background: The benefit of hepatic resection in case of concomitant colorectal hepatic and extrahepatic metastases (CHEHMs) is still debatable. The purpose of this study is to assess the results of resection of hepatic and extrahepatic metastases in patients with CHEHMs in a high-volume center for both hepatobiliary and colorectal surgery and to identify prognostic factors that correlate with longer survival in these patients. It was performed a retrospective analysis of 678 consecutive patients with liver resection for colorectal cancer metastases operated in a single Centre between April 1996 and March 2016. Among these, 73 patients presented CHEHMs. Univariate analysis was performed to identify the risk factors for overall survival (OS) in these patients. Results: There were 20 CHMs located at the lymphatic node level, 20 at the peritoneal level, 12 at the ovary and lung level, 12 presenting as local relapses and 9 other sites. 53 curative resections (R0) were performed. The difference in overall survival between the CHEHMs group and the CHMs group is statistically significant for the entire groups (p 0.0001), as well as in patients who underwent R0 resection (p 0.0001). In CHEHMs group, the OS was statistically significant higher in patients who underwent R0 resection vs. those with R1/R2 resection (p=0.004). Three variables were identified as prognostic factors for poor OS following univariate analysis: 4 or more hepatic metastases, major hepatectomy and the performance of operation during first period of the study (1996 - 2004). There was a tendency toward better OS in patients with ovarian or pulmonary location of extrahepatic disease, although the difference was not statistically significant. In patients with concomitant hepatic and extrahepatic metastases, complete resection of metastatic burden significantly prolong survival. The patients with up to 4 liver metastases, resectable by minor hepatectomy benefit the most from this aggressive onco

  8. Feeding Difficulties in Children with Esophageal Atresia.

    Science.gov (United States)

    Mahoney, Lisa; Rosen, Rachel

    2016-06-01

    The current available literature evaluating feeding difficulties in children with esophageal atresia was reviewed. The published literature was searched through PubMed using a pre-defined search strategy. Feeding difficulties are commonly encountered in children and adults with repaired esophageal atresia [EA]. The mechanism for abnormal feeding includes both esophageal and oropharyngeal dysphagia. Esophageal dysphagia is commonly reported in patients with EA and causes include dysmotility, anatomic lesions, esophageal outlet obstruction and esophageal inflammation. Endoscopic evaluation, esophageal manometry and esophograms can be useful studies to evaluate for causes of esophageal dysphagia. Oropharyngeal dysfunction and aspiration are also important mechanisms for feeding difficulties in patients with EA. These patients often present with respiratory symptoms. Videofluoroscopic swallow study, salivagram, fiberoptic endoscopic evaluation of swallowing and high-resolution manometry can all be helpful tools to identify aspiration. Once diagnosed, management goals include reduction of aspiration during swallowing, reducing full column reflux into the oropharynx and continuation of oral feeding to maintain skills. We review specific strategies which can be used to reduce aspiration of gastric contents, including thickening feeds, changing feeding schedule, switching formula, trialing transpyloric feeds and fundoplication. Copyright © 2015 Elsevier Ltd. All rights reserved.

  9. Bilateral Choanal Atresia: Highlighting The Role Of Computerised ...

    African Journals Online (AJOL)

    A neonate delivered at UCH Ibadan was diagnosed to have bilateral choanal atresia being membranous on the right side and bony on the left side by computed tomography. She had surgical correction of the right sided obstruction but died on the seventh post operative day due to septicemia. The literature has been ...

  10. Repair of oesophageal atresia with tracheo- oesophageal fistula ...

    African Journals Online (AJOL)

    Right thoracotomy for oesophageal atresia (OA) with dextrocardia is technically challenging due to the heart being in the operative field, and also due to the possibility of right-sided aortic arch. We report a neonate with long- gap OA with tracheo-oesophageal fistula (TOF), dextrocardia, and left-sided aortic arch who was.

  11. [Biliary dysfunction in obese children].

    Science.gov (United States)

    Aleshina, E I; Gubonina, I V; Novikova, V P; Vigurskaia, M Iu

    2014-01-01

    To examine the state of the biliary system, a study of properties of bile "case-control") 100 children and adolescents aged 8 to 18 years, held checkup in consultative and diagnostic center for chronic gastroduodenitis. BMI children were divided into 2 groups: group 1-60 children with obesity (BMI of 30 to 40) and group 2-40 children with normal anthropometric indices. Survey methods included clinical examination pediatrician, endocrinologist, biochemical parameters (ALT, AST, alkaline phosphatase level, total protein, bilirubin, lipidogram, glucose, insulin, HOMA-index), ultrasound of the abdomen and retroperitoneum, EGD with aspiration of gallbladder bile. Crystallography bile produced by crystallization of biological substrates micromethods modification Prima AV, 1992. Obese children with chronic gastroduodenita more likely than children of normal weight, had complaints and objective laboratory and instrumental evidence of insulin resistance and motor disorders of the upper gastrointestinal and biliary tract, liver enlargement and biliary "sludge". Biochemical parameters of obese children indicate initial metabolic changes in carbohydrate and fat metabolism and cholestasis, as compared to control children. Colloidal properties of bile in obese children with chronic gastroduodenita reduced, as indicated by the nature of the crystallographic pattern. Conclusions: Obese children with chronic gastroduodenitis often identified enlarged liver, cholestasis and biliary dysfunction, including with the presence of sludge in the gallbladder; most often--hypertonic bile dysfunction. Biochemical features of carbohydrate and fat metabolism reflect the features of the metabolic profile of obese children. Crystallography bile in obese children reveals the instability of the colloidal structure of bile, predisposing children to biliary sludge, which is a risk factor for gallstones.

  12. [Endoscopic ultrasound guided rendezvous for biliary drainage].

    Science.gov (United States)

    Knudsen, Marie Høxbro; Vilmann, Peter; Hassan, Hazem; Karstensen, John Gésdal

    2015-04-27

    Endoscopic retrograde cholangiography (ERCP) is currently standard treatment for biliary drainage. Endoscopic ultrasound guided rendezvous (EUS-RV) is a novel method to overcome an unsuccessful biliary drainage procedure. Under endoscopic ultrasound guidance a guidewire is passed via a needle from the stomach or duodenum to the common bile duct and from there on to the duodenum enabling ERCP. With a relatively high rate of success EUS-RV should be considered as an alternative to biliary drainage and surgical intervention.

  13. Evolución a largo plazo de 55 pacientes operados de atresia esofágica

    Directory of Open Access Journals (Sweden)

    Ruperto Llanes Céspedes

    2003-06-01

    Full Text Available Los pacientes operados de atresia esofágica constituyen un reto para los médicos involucrados en su seguimiento. Se realizó un estudio retrospectivo de las historias clínicas de 55 pacientes vivos después de operarse de atresia esofágica, en un período de 15 años, donde se evaluaron las complicaciones encontradas a largo plazo, con el objetivo de identificarlas y conocer su tratamiento. La presencia de reflujo gastroesofágico en 30 niños (54,54 %, la dismotilidad esofágica en 28 (50,90 % y las manifestaciones respiratorias recurrentes en 26 pacientes (47,27 % fueron las más frecuentes, seguidas de las estenosis de la sutura en 11 (20 %, retardo del crecimiento y desarrollo en 10 (18,18 % y la recurrencia de la fístula en 2 niños (3,63 %. Se concluye que en el seguimiento a largo plazo de los pacientes operados de atresia esofágica se deben realizar el diagnóstico precoz y el tratamiento de múltiples complicaciones que definen su calidad de vida.The patients operated for esophageal atresia become a challenge for physicians involved in their follow-up. A retrospective study of the medical histories of 55 live patients after they had been operated for esophageal atresia in a period of 15 years was undertaken where long-term complications were evaluated to identify them and find out their treatment. Gastroesophageal reflux in 30 children (54,54 %, esophageal dismotility in 28 children (50,90 % and recurrent respiratory manifestations in 26 patients (47,27 % were the most frequent complications followed by suture stenosis in 11 (20 %, retarded growth and development in 10 (18,18 % and recurrent tracheoesophageal fistula in 2 children. It is concluded that early diagnosis and treatment of multiple complications, which define the quality of life of patients, should be made in the long-term follow-up of patients operated for esophageal atresia.

  14. The ability of anti-carbonic anhydrase II antibody to distinguish autoimmune cholangitis from primary biliary cirrhosis in Japanese patients.

    Science.gov (United States)

    Akisawa, N; Nishimori, I; Miyaji, E; Iwasaki, S; Maeda, T; Shimizu, H; Sato, N; Onishi, S

    1999-06-01

    Serum antibody against carbonic anhydrase (CA) II has been described as a serological marker for distinguishing autoimmune cholangitis (AIC) from primary biliary cirrhosis (PBC). To validate this finding in a Japanese population, we evaluated sera from patients with PBC and AIC for antibody to human CA II. An enzyme-linked immunosorbent assay was employed to quantify serum antibody against CA II in patients with PBC (n = 40), AIC (n = 23), autoimmune hepatitis (n = 10), and extrahepatic obstructive jaundice (n = 10). Compared with the finding of a 4% prevalence of anti-CAII antibody in healthy subjects (n = 24), a significantly higher prevalence of anti-CA II antibody was detected in patients with PBC (35%) and AIC (30%) (P jaundice. No significant difference was observed between PBC and AIC patients. These results showed that AIC and PBC would be indistinguishable by anti-CA II antibody testing in Japanese patients. However, the finding of serum anti-CA II antibody in patients with PBC and AIC supports the disease concept of autoimmune exocrinopathy.

  15. [Surgical treatment of intraoperative injuries and cicatricial strictures of extrahepatic bile ducts].

    Science.gov (United States)

    Tret'iakov, A A; Slepykh, N I; Kornilov, A K; Karimov, Z Kh

    1998-01-01

    The analysis of 70 cases of surgical treatment for intraoperative injuries and cicatricial strictures of extrahepatic bile ducts was carried out. In 25 patients surgical procedure was restorative and in 45--reconstructiver. Most common causes of corrective operations were: iatrogenic injuries of extrahepatic bile ducts (14) and cicatricial strictures of hepaticocholedochal duct due to intraoperative trauma (31). The problems of operative technique in performing biliobilio-, hepato-hepatico and hepatico-jejuno-anastomoses are considered. There were three deaths in the early postoperative period: 2 patients died of hepatic failure, pyogenic cholangiogenic intoxication caused by cholangioectasies and intrahepatic abscesses, and 1-due to generalyzed peritonitis caused by acute gastric ulcer perforation. Special attention is paid to the choice of the method of prolonged drainage used in reconstructive as well as in restorative operations.

  16. Equol inhibits growth, induces atresia, and inhibits steroidogenesis of mouse antral follicles in vitro

    International Nuclear Information System (INIS)

    Mahalingam, Sharada; Gao, Liying; Gonnering, Marni; Helferich, William; Flaws, Jodi A.

    2016-01-01

    Equol is a non-steroidal estrogen metabolite produced by microbial conversion of daidzein, a major soy isoflavone, in the gut of some humans and many animal species. Isoflavones and their metabolites can affect endogenous estradiol production, action, and metabolism, potentially influencing ovarian follicle function. However, no studies have examined the effects of equol on intact ovarian antral follicles, which are responsible for sex steroid synthesis and further development into ovulatory follicles. Thus, the present study tested the hypothesis that equol inhibits antral follicle growth, increases follicle atresia, and inhibits steroidogenesis in the adult mouse ovary. To test this hypothesis, antral follicles isolated from adult CD-1 mice were cultured with vehicle control (dimethyl sulfoxide; DMSO) or equol (600 nM, 6 μM, 36 μM, and 100 μM) for 48 and 96 h. Every 24 h, follicle diameters were measured to monitor growth. At 48 and 96 h, the culture medium was subjected to measurement of hormone levels, and the cultured follicles were subjected to gene expression analysis. Additionally, follicles were histologically evaluated for signs of atresia after 96 h of culture. The results indicate that equol (100 μM) inhibited follicle growth, altered the mRNA levels of bcl2-associated X protein and B cell leukemia/lymphoma 2, and induced follicle atresia. Further, equol decreased the levels of estradiol, testosterone, androstenedione, and progesterone, and it decreased mRNA levels of cholesterol side-chain cleavage, steroid 17-α-hydroxalase, and aromatase. Collectively, these data indicate that equol inhibits growth, increases atresia, and inhibits steroidogenesis of cultured mouse antral follicles. - Highlights: • Equol exposure inhibits antral follicle growth. • Equol exposure increases follicle atresia. • Equol exposure inhibits sex steroid hormone levels. • Equol exposure inhibits mRNA levels of certain steroidogenic enzymes.

  17. Equol inhibits growth, induces atresia, and inhibits steroidogenesis of mouse antral follicles in vitro

    Energy Technology Data Exchange (ETDEWEB)

    Mahalingam, Sharada, E-mail: mahalin2@illinois.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States); Gao, Liying, E-mail: lgao@uiuc.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States); Gonnering, Marni, E-mail: mgonne2@illinois.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States); Helferich, William, E-mail: helferic@illinois.edu [Department of Food Science and Human Nutrition, University of Illinois, 905 S. Goodwin, Urbana, IL 61801 (United States); Flaws, Jodi A., E-mail: jflaws@illinois.edu [Department of Comparative Biosciences, College of Veterinary Medicine, University of Illinois, 2001 S. Lincoln Ave, Urbana, IL 61802 (United States)

    2016-03-15

    Equol is a non-steroidal estrogen metabolite produced by microbial conversion of daidzein, a major soy isoflavone, in the gut of some humans and many animal species. Isoflavones and their metabolites can affect endogenous estradiol production, action, and metabolism, potentially influencing ovarian follicle function. However, no studies have examined the effects of equol on intact ovarian antral follicles, which are responsible for sex steroid synthesis and further development into ovulatory follicles. Thus, the present study tested the hypothesis that equol inhibits antral follicle growth, increases follicle atresia, and inhibits steroidogenesis in the adult mouse ovary. To test this hypothesis, antral follicles isolated from adult CD-1 mice were cultured with vehicle control (dimethyl sulfoxide; DMSO) or equol (600 nM, 6 μM, 36 μM, and 100 μM) for 48 and 96 h. Every 24 h, follicle diameters were measured to monitor growth. At 48 and 96 h, the culture medium was subjected to measurement of hormone levels, and the cultured follicles were subjected to gene expression analysis. Additionally, follicles were histologically evaluated for signs of atresia after 96 h of culture. The results indicate that equol (100 μM) inhibited follicle growth, altered the mRNA levels of bcl2-associated X protein and B cell leukemia/lymphoma 2, and induced follicle atresia. Further, equol decreased the levels of estradiol, testosterone, androstenedione, and progesterone, and it decreased mRNA levels of cholesterol side-chain cleavage, steroid 17-α-hydroxalase, and aromatase. Collectively, these data indicate that equol inhibits growth, increases atresia, and inhibits steroidogenesis of cultured mouse antral follicles. - Highlights: • Equol exposure inhibits antral follicle growth. • Equol exposure increases follicle atresia. • Equol exposure inhibits sex steroid hormone levels. • Equol exposure inhibits mRNA levels of certain steroidogenic enzymes.

  18. Copper Cu 64 Anti-CEA Monoclonal Antibody M5A PET in Diagnosing Patients With CEA Positive Cancer

    Science.gov (United States)

    2018-05-04

    Breast Cancer; Colon Cancer; Extrahepatic Bile Duct Cancer; Gallbladder Cancer; Gastrointestinal Cancer; Liver and Intrahepatic Biliary Tract Cancer; Lung Cancer; Metastatic Cancer; Pancreatic Cancer; Rectal Cancer; Thyroid Gland Medullary Carcinoma; Unspecified Adult Solid Tumor, Protocol Specific

  19. A Study of Technical Approach Methods to Transabdominal Ultrasonography of the Extrahepatic Bile Ducts and of Following Effects from the Scan Training

    International Nuclear Information System (INIS)

    Lee, In Ja; Kang, Dae Hyun; Kim, Bo Young

    2008-01-01

    The purposes of this study are to analyze abnormal dilatation of the extrahepatic bile ducts by using transabdominal ultrasound, to confirm the existence of bile ducts diseases and their interrelationship, and for it to give a new theoretical basis for the technical access to extrahepatic bile ducts, upon which to analyze the ripple effects of the scan training. After teaching technical access process based on the new theory about extrahepatic bile duct to the thirty students who are studying ultrasonography, we allocated three hours per one student (30 mins 6 times) to focus on the training of scanning skill. Training has been performed by one-to-one method. For evaluation, all the students have to perform the scans on (1) confluence of the right and left hepatic ducts (extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct, (3) the intrapancreatic bile duct, (4) intrapapilla Duct, based on the clearly divided concept. The existing training and methods have had low confidency about transabdominal ultrasonography of the extrahepatic bile duct and had limitation with which they could image only the suprapancreatic bile duct. The evaluation after finishing the train based on the new theory, however, all the students (30 students) can access to (1) confluence of the right and left hepatic ducts(extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct objectively. 24 students can access to (3) the intrapancreatic bile duct and only one student can even make an image for (4) the intrapapilla Duct Though the evaluation on extrahepatic bile duct has to be performed with multi-sided method considering intrahepatic cause, bile duct cause and pathophysiological cause, only if we can image the extrahepatic bile duct to ampular of Vater objectively and confidently, we can greatly reduce invasive procedure such as ERCP, which is for the purpose of simple differential diagnosis and painful to the patients. Therefore if we concentrate on

  20. An Unusual Cause of Biliary Obstruction

    Directory of Open Access Journals (Sweden)

    Sern Wei Yeoh

    2012-01-01

    Full Text Available Portal biliary ductopathy (PBD is a condition in which biliary and pancreatic ducts are extrinsically compressed by collateral branches of the portal venous system, which in turn have become dilated and varicosed due to portal hypertension. While the majority of patients with PBD are asymptomatic, a minority can present with symptoms of biliary obstruction and cholangitis with the potential of developing secondary chronic liver disease. This paper reports the case of a 29 year old male presenting with acute cholangitis, in whom PBD was diagnosed radiologically. A brief review of current literature regarding the diagnosis and management of this condition will also be presented.

  1. MRI-three dimensional reconstruction of biliary system in choledochal cyst

    International Nuclear Information System (INIS)

    Kaji, Tatsuru; Takamatsu, Hideo; Noguchi, Hiroyuki; Tahara, Hiroyuki; Fukushige, Takahiko; Kajiya, Hiroshi; Kajiya, Yoshiki

    1995-01-01

    We report a trial of MR cholangiography in children with choledochal cyst. Recently, three-dimensional reconstruction using magnetic resolution imaging of biliary system (MR cholangiography) has been reported as the less-invasive diagnostic method for obstructive lesions of biliary system. Forty-eight cases of choledochal cyst were treated at Kagoshima University Hospital in the past ten years. In 22 of them, intrahepatic duct dilatation was revealed by preoperative or operative cholangiogram. We tried MR cholangiography in nine cases of 22 cases pre- and/or post-operatively. Five cases had MR cholangiography preoperatively. Intrahepatic biliary dilatation was revealed in all of them and intrahepatic biliary stenosis was revealed in two cases. These findings were almost the same as those by preoperative or operative cholangiogram. MR cholangiography was applied on seven cases postoperatively: 3 cases had fine construction of biliary system, because they still had intrahepatic biliary dilatation, and no dilatation was seen in 4 cases, because of good operative results. This method has advantages of less-invasive in children, no need of contrast dye, and fair delineation of biliary system as samely as endoscopic retrograde cholangiography (ERCP) and percutaneous transhepatic cholangiography (PTC). In cases of huge biliary dilatation, MR cholangiography provides more information concerning intrahepatic biliary than ERCP. (author)

  2. Usefulness of endoscopic ultrasonography in the clinical suspicion of biliary disease Utilidad de la ultrasonografía endoscópica en la sospecha clínica de patología biliar

    Directory of Open Access Journals (Sweden)

    David del Pozo

    2011-07-01

    Full Text Available Background and aims: endoscopic ultrasound (EUS is a very sensitive and specific technique for the diagnosis of biliary diseases. This procedure has proven its usefulness in cases of high suspicion of biliary disease (history of gallstones and dilatation of the intrahepatic and/or extrahepatic bile ducts. We know less about its usefulness in cases of low suspicion of biliary pathology. The aim of this study was to assess the diagnostic accuracy of EUS in patients with low suspicion of biliary disease (patients with dilatation of the biliary tract were excluded. Methods: 33 patients with low suspicion of biliary disease were recruited in 12 months. All of them had no biliary findings in a previous abdominal ultrasound and computer tomography scan. All of them underwent EUS and were studied prospectively. The diagnosis was confirmed by surgery and/or by ERCP in patients with positive EUS or clinical follow-up in those with normal EUS. Time of follow-up was 9 months (range, 3-12 months. Results: seventeen patients (51.5% presented with abnormal biliary findings on EUS (7 choledocholithiasis, 3 cholelithiasis, 2 choledocholithiasis + cholelithiasis and 5 microlithiasis. Conclusion: EUS is a useful and safe procedure for diagnosing patients with low suspicion of biliary disease.Antecedentes y objetivos: la ultrasonografía endoscópica (USE es una técnica muy sensible y específica para el diagnóstico de enfermedades biliares. Este procedimiento ha demostrado su utilidad en casos de alta sospecha de enfermedad biliar (historia de colelitiasis y dilatación de la vía biliar intra- y/o extrahepática. Sabemos menos sobre su utilidad en casos de baja sospecha de patología biliar. El objetivo de este estudio fue evaluar la precisión diagnóstica de la USE en pacientes con baja sospecha de enfermedad biliar (los pacientes con dilatación de la vía biliar fueron excluidos. Métodos: 33 pacientes con baja sospecha de enfermedad biliar fueron

  3. An Exceptional Case of Spontaneous Fistulization of an Intrahepatic ...

    African Journals Online (AJOL)

    ADMIN

    Whenever they occur, usually, areassociated with diseases of extrahepatic biliary tree12-14. Spontaneousfistulization .... John G; Thai, Pham H. chapter 32. ... presented as an exophytic mass in the liver. PedSurgInternat 1998; 13:177-179. 10.

  4. Effect of ketamine, pentobarbital, and morphine on Tc-99m-DISIDA hepatobiliary kinetics

    International Nuclear Information System (INIS)

    Durakovic, A.; Dubois, A.

    1985-01-01

    The purpose of this study was to evaluate hapatobiliary kinetics of Tc-99m-DISIDA in dogs after administration of anesthetic sedative or narcotic agents. Four groups of six male Beagle dogs were studied as a non-treated control group and after parenteral administration of ketamine (30 mg/kg IM), pentobarbital (25 mg/kg IV) or morphine (1 mg/kg IV). Each animal was injected with 4 mCi Tc-99m-DISIDA and hepatobiliary scintigraphic studies were obtained using a gamma camera with parallel hole multipurpose collimator and an A/sup 3/ MDS computer. The authors determined; peak activity of Tc-99m-DISIDA in the liver, visualization and peak activity of gallbladder, and intestinal visualization of Tc-99m-DISIDA. Total bilirubin, LDH, SGOT and SGPT were not modified significantly after any drug compared to control. The results showed that two commonly used anesthetics and sedatives (ketamine and pentobarbital) have dramatic and opposite effects on extrahepatic biliary kinetics. Furthermore, ketamine, but not pentobarbital, significantly accelerates intrahepatic biliary kinetics. Finally, as expected, morphine delayed extrahepatic biliary kinetics. Thus, studies of biliary kinetics should be interpreted with caution when measurements are made after administration of anesthetic, sedative or narcotic agents

  5. Pathological aspects of so called "hilar cholangiocarcinoma".

    Science.gov (United States)

    Castellano-Megías, Víctor M; Ibarrola-de Andrés, Carolina; Colina-Ruizdelgado, Francisco

    2013-07-15

    Cholangiocarcinoma (CC) arising from the large intrahepatic bile ducts and extrahepatic hilar bile ducts share clinicopathological features and have been called hilar and perihilar CC as a group. However, "hilar and perihilar CC" are also used to refer exclusively to the intrahepatic hilar type CC or, more commonly, the extrahepatic hilar CC. Grossly, a major distinction can be made between papillary and non-papillary tumors. Histologically, most hilar CCs are well to moderately differentiated conventional type (biliary) carcinomas. Immunohistochemically, CK7, CK20, CEA and MUC1 are normally expressed, being MUC2 positive in less than 50% of cases. Two main premalignant lesions are known: biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the biliary tract (IPNB). IPNB includes the lesions previously named biliary papillomatosis and papillary carcinoma. A series of 29 resected hilar CC from our archives is reviewed. Most (82.8%) were conventional type adenocarcinomas, mostly well to moderately differentiated, although with a broad morphological spectrum; three cases exhibited a poorly differentiated cell component resembling signet ring cells. IPNB was observed in 5 (17.2%), four of them with an associated invasive carcinoma. A clear cell type carcinoma, an adenosquamous carcinoma and two gastric foveolar type carcinomas were observed.

  6. The mechanism of biliary lipid secretion and its defects

    NARCIS (Netherlands)

    Elferink, R. P.; Groen, A. K.

    1999-01-01

    Biliary lipid secretion is an important physiological event; not only for the disposal of cholesterol from the body, but also for the protection of cells lining the biliary tree against bile salts. Insight into the (patho)physiological role of biliary lipid secretion has been recently expanded

  7. Biliary Stricture Following Hepatic Resection

    Directory of Open Access Journals (Sweden)

    Jeffrey B. Matthews

    1991-01-01

    Full Text Available Anatomic distortion and displacement of hilar structures due to liver lobe atrophy and hypertrophy occasionally complicates the surgical approach for biliary stricture repair. Benign biliary stricture following hepatic resection deserves special consideration in this regard because the inevitable hypertrophy of the residual liver causes marked rotation and displacement of the hepatic hilum that if not anticipated may render exposure for repair difficult and dangerous. Three patients with biliary stricture after hepatectomy illustrate the influence of hepatic regeneration on attempts at subsequent stricture repair. Following left hepatectomy, hypertrophy of the right and caudate lobes causes an anteromedial rotation and displacement of the portal structures. After right hepatectomy, the rotation is posterolateral, and a thoracoabdominal approach may be necessary for adequate exposure. Radiographs obtained in the standard anteroposterior projection may be deceptive, and lateral views are recommended to aid in operative planning.

  8. Relationship between hepatic CTGF expression and routine blood tests at the time of liver transplantation for biliary atresia: hope or hype for a biomarker of hepatic fibrosis.

    Science.gov (United States)

    Haafiz, Allah; Farrington, Christian; Andres, Joel; Islam, Saleem

    2011-01-01

    Progressive hepatic fibrosis (HF) is a prominent feature of biliary atresia (BA), the most common indication for liver transplantation (LT) in children. Despite its importance in BA, HF is not evaluated in routine patient care because the invasiveness of liver biopsy makes histologic monitoring of fibrosis unfeasible. Therefore, the identification of noninvasive markers to assess HF is desirable especially in children. The main goal of this pilot project was to establish an investigational framework correlating hepatic expression of fibrogenic markers with routine blood tests in BA. Using liver explants from patients with BA (n = 26), immune-expression of connective tissue growth factor (CTGF), a key fibrogenic cytokine was determined using horseradish-labeled antibodies. Expression intensities of lobular (L-CTGF) and portal (P-CTGF) CTGF were determined by using ImageJ software. These CTGF intensities were correlated with blood tests performed at the time of LT. Correlation coefficients were determined for each blood test variable versus mean L-CTGF and P-CTGF expression intensities. A P-value of less than 0.05 was considered statistically significant. All patients had end-stage liver disease and persistent cholestasis at the time of LT. Kendall tau (τ) rank correlation coefficient for L-CTGF and white blood cell (WBC) was inversed (-0.52; P ≤ 0.02). Similar but statistically nonsignificant inverse relationships were noted between L-CTGF and prothrombin time (PT) (-0.15; P ≤ 0.4), international normalized ratio (INR) (-0.14; P ≤ 0.5), and platelet count (-0.36; P ≤ 0.09). Inversed (τ) rank correlation coefficients were also evident between P-CTGF expression and gamma-glutamyl transpeptidase (GGT), PT, INR, and platelet count. Pearson correlation coefficients for combinational analysis of standardized total bilirubin (TB), alkaline phosphatase, GGT, and platelet count with L-CTGF (0.33; P = 0.3) and P-CTGF (0.06; P = 0.8), were not significant. Similar

  9. [Association of biliary calculosis and portal cavernomatosis].

    Science.gov (United States)

    Crespi, C; De Giorgio, A M

    1992-08-01

    This paper reports the case of a woman, who underwent surgery because of cholelithiasis, with intraoperative finding of prehepatic portal hypertension from portal vein thrombosis ("portal cavernoma") with healthy liver, later confirmed by angiographic studies. This rare pathologic association carries a higher risk of major operative complications; therefore the Authors agree with the general belief that, for these cases, biliary tract surgery should be as simple and safe as possible. In the case of preoperative diagnosis of biliary disease associated with portal cavernoma, should a surgical approach on the biliary tract be required, we agree on the advisability of performing a shunting procedure before any kind of biliary surgery. In case of variceal bleeding endoscopic sclerotherapy will be the first choice; surgical procedures (shunting) should be seen as a second choice in case of rebleeding after sclerotherapy.

  10. Adult bile duct strictures: differentiating benign biliary stenosis from cholangiocarcinoma.

    Science.gov (United States)

    Nguyen Canh, Hiep; Harada, Kenichi

    2016-12-01

    Biliary epithelial cells preferentially respond to various insults under chronic pathological conditions leading to reactively atypical changes, hyperplasia, or the development of biliary neoplasms (such as biliary intraepithelial neoplasia, intraductal papillary neoplasm of the bile duct, and cholangiocarcinoma). Moreover, benign biliary strictures can be caused by a variety of disorders (such as IgG4-related sclerosing cholangitis, eosinophilic cholangitis, and follicular cholangitis) and often mimic malignancies, despite their benign nature. In addition, primary sclerosing cholangitis is a well-characterized precursor lesion of cholangiocarcinoma and many other chronic inflammatory disorders increase the risk of malignancies. Because of these factors and the changes in biliary epithelial cells, biliary strictures frequently pose a diagnostic challenge. Although the ability to differentiate neoplastic from non-neoplastic biliary strictures has markedly progressed with the advance in radiological modalities, brush cytology and bile duct biopsy examination remains effective. However, no single modality is adequate to diagnose benign biliary strictures because of the low sensitivity. Therefore, understanding the underlying causes by compiling the entire clinical, laboratory, and imaging data; considering the under-recognized causes; and collaborating between experts in various fields including cytopathologists with multiple approaches is necessary to achieve an accurate diagnosis.

  11. Biliary parasites: diagnostic and therapeutic strategies.

    Science.gov (United States)

    Khandelwal, Niraj; Shaw, Joanna; Jain, Mamta K

    2008-04-01

    Parasitic infections of the biliary tract are a common cause of biliary obstruction in endemic areas. This article focuses on primary biliary parasites: Ascaris lumbricoides, Clonorchis sinensis, Opisthorchis viverrini, Opisthorchis felineus, Dicrocoelium dendriticum, Fasciola hepatica, and Fasciola gigantica. Tropical and subtropical countries have the highest incidence and prevalence of these infections. Diagnosis is made primarily through direct microscopic examination of eggs in the stool, duodenal, or bile contents. Radiologic imaging may show intrahepatic ductal dilatation, whereas endoscopic retrograde cholangiopancreatography can be used diagnostically and therapeutically. However, oral treatment is inexpensive and effective for most of these parasites and can prevent untoward consequences. Primary and alternative treatments are available and are reviewed in this article.

  12. Congenital biliary tract malformation resembling biliary cystadenoma in a captive juvenile African lion (Panthera leo).

    Science.gov (United States)

    Caliendo, Valentina; Bull, Andrew C J; Stidworthy, Mark F

    2012-12-01

    A captive 3-mo-old white African lion (Panthera leo) presented with clinical signs of acute pain and a distended abdomen. Despite emergency treatment, the lion died a few hours after presentation. Postmortem examination revealed gross changes in the liver, spleen, and lungs and an anomalous cystic structure in the bile duct. Histologic examination identified severe generalized multifocal to coalescent necrotizing and neutrophilic hepatitis, neutrophilic splenitis, and mild interstitial pneumonia, consistent with bacterial septicemia. The abnormal biliary structures resembled biliary cystadenoma. However, due to the age of the animal, they were presumed to be congenital in origin. Biliary tract anomalies and cystadenomas have been reported previously in adult lions, and this case suggests that at least some of these examples may have a congenital basis. It is unclear whether the lesion was an underlying factor in the development of hepatitis.

  13. Ultrasound-guided percutaneous transhepatic biliary drainage: Experiences in 146 patients

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jai Keun [Sohwa Children' s Hospital, Seoul(Korea, Republic of); Yu, Jeong Sik; Kim, Ki Whang; Chung, Soo Yoon; Jeong, Mi Gyoung [Yonsei University College of Medicine, Seoul (Korea, Republic of); Choi, Deuk Lin; Kwon, Gui Hyang; Lee, Hae Kyung [Soonchunhyang University College of Medicine, Seoul (Korea, Republic of)

    1999-03-15

    Percutaneous biliary drainage is an important technique for palliative therapy of obstructive biliary disease and diagnostic information. The purpose of this study is to review and evaluate the experiences of ultrasound-guided percutaneous transhepatic biliary drainage. Ultrasound-guided percutaneous transhepatic biliary drainage was performed on 146 occasions in 134 patients. The causes of biliary obstruction were: benign diseases (19 cases, 14.2%) such as bile duct stones or stricture, cholangiocarcinoma (37 cases, 27.6%), pancreatic carcinoma (35 cases, 26.1%), metastasis (22 cases, 16.5%), gall bladder cancer (14 cases, 10.4%), ampulla of Vater cancer (4 cases, 3.0%), hepatocellular carcinoma (3 cases, 2.2%). Retrospectively reviewing medical records, we found out frequency of external or external/internal biliary drainages, puncture of left or right hepatic duct, and presence of bileinfection. Ultrasound-guided percutaneous transhepatic biliary drainage was compared with conventional biliary drainage of previous reports on the basis of frequency of complications. External (124 procedures, 84.9%) and external/internal biliary drainage (22 procedures, 15.1%) were carried out by puncture of dilated right (59.6%) or left (40.4%) intrahepatic duct. Sixty-nine complications occurred in 47 patients. Catheter related complications (33/69, 47.8%) were most common: catheter dislodgement (17/69, 24.6%), malfunction (9/69, 13.1%), leakage (7/69, 10.1%). Other minor complications such as simple fever (16/69, 23.2%), cholangitis (7/69, 10.1%), hemobilia (4/69, 5.8%), biloma (2/69, 2.9%) and wound infection (1/69, 1.5%) occurred. Major complications including sepsis (4/69, 5.8%) and bile peritonitis (2/69, 2.9%) were also noted. Puncture-related complications such as hemobilia, biloma and bile peritonitis occurred in 8 cases (5.5%). Comparing with conventional X-ray guided drainage, ultrasound-guided percutaneous transhepatic biliary drainage is a safe procedure for

  14. CASE REPORT CASE Unusual case of pulmonary valve atresia

    African Journals Online (AJOL)

    atresia with a ventricular septal defect (VSD) and anomalous origin of the left pulmonary artery arising from ... Anomalous origin of either pulmonary artery branch from the aorta has been reported. The association of ... Department of Radiology, Pretoria Academic Hospital and University of. Pretoria. F Takawira, MB ChB, FC ...

  15. Hepatic Encephalopathy

    Medline Plus

    Full Text Available ... Related Liver Disease Alpha-1 Antitrypsin Deficiency Autoimmune Hepatitis Benign Liver Tumors Biliary Atresia Cirrhosis of the ... Disease Type 1 (von Gierke) Hemochromatosis Hepatic Encephalopathy Hepatitis A Hepatitis B Hepatitis C Intrahepatic Cholestasis of ...

  16. Spontaneous cholangiohepatitis in broiler chickens: immunohistochemical study of Ito cells

    Directory of Open Access Journals (Sweden)

    E Handharyani

    2001-12-01

    Full Text Available The function of Ito cells is expanding from a fat-storing site to a center of extracellular matrix metabolism and mediator production in the liver. Immunohistochemical reactivities of Ito cells were examined in eight livers of broiler chickens affected with spontaneous cholangiohepatitis and six chicken livers with malformation of extrahepatic biliary tracts. The livers in both groups revealed severe diffuse fibrosis. Ito cells expressing HHF35 muscle actin and desmin actively proliferated in the fibrotic foci of the all livers. The immunoreactivities of Ito cells to antibodies were enhanced compared with those in normal livers. There were no immunohistochemical differences between the Ito cells of two groups. From these findings, it was suggested that Ito cells actively proliferate and show enhanced immunoreactivities in the livers affected with cholangiohepatitis andmalformation of extrahepatic biliary tracts.

  17. Immunohistochemical study of Ito cells of spontaneous cholangiohepatitis in broiler chickens

    Directory of Open Access Journals (Sweden)

    E Handharyani

    2001-12-01

    Full Text Available The function of Ito cells is expanding from a fat-storing site to a center of extracellular matrix metabolism and mediator production in the liver. Immunohistochemical reactivities of Ito cells were examined in eight livers of broiler chickens affected with spontaneous cholangiohepatitis and six chicken livers with malformation of extrahepatic biliary tracts. The livers in both groups revealed severe diffuse fibrosis. Ito cells expressing HHF35 muscle actin and desmin actively proliferated in the fibrotic foci of the all livers. The immunoreactivities of Ito cells to antibodies were enhanced compared with those in normal livers. There were no immunohistochemical differences between the Ito cells of two groups. From these findings, it was suggested that Ito cells actively proliferate and show enhanced immunoreactivities in the livers affected with cholangiohepatitis and malformation of extrahepatic biliary tracts.

  18. Recurrent pyogenic cholangitis in Asian immigrants: use of ultrasonography, computed tomography, and cholangiography

    International Nuclear Information System (INIS)

    Federle, M.P.; Cello, J.P.; Laing, F.C.; Jeffery, R.B. Jr.

    1982-01-01

    Five cases of recurrent pyogenic cholangitis (RPC) were studied by ultrasonography, computed tomography (CT), and cholangiography. All patients were recent immigrants from the Orient or Indonesia and had had recurrent attacks of cholangitis for many years. The bile was infected by E. coli and the biliary ducts were dilated; in addition, extrahepatic bile-pigment calculi we represent in all 5 and intrahepatic calculi in 4. Abdominal ultrasound usually failed to demonstrate duct calculi and extrahepatic dilatation due to the soft, mud-like consistency of the stones. CT was successful in showing the calculi and the full extent of dilatation. The authors conclude that preoperative diagnosis of RPC is best achieved by awareness of the characteristic clinical presentation and the findings on abdominal CT. Preoperative cholangiography provides excellent detail, but poses the danger of biliary sepsis requiring antibiotics

  19. EUS-guided biliary rendezvous using a short hydrophilic guidewire.

    Science.gov (United States)

    Dhir, Vinay; Kwek, Boon Eu Andrew; Bhandari, Suryaprakash; Bapat, Mukta; Maydeo, Amit

    2011-10-01

    BACKGROUND AND STUDY AIMS: EUS-guided rendezvous technique for biliary access requires expert manipulation of the guidewire across the downstream stricture or papilla. Published literature reports usage of the long-wire system to prevent loss of wire during scope exchange. We studied the efficacy of using a short hydrophilic guidewire in EUS-guided rendezvous. PATIENTS AND METHODS: This is a retrospective study conducted in a tertiary care referral centre. 15 patients underwent EUS-guided biliary rendezvous with short wire. EUS-guided transduodenal/transgastric puncture of the biliary system was performed, followed by anterograde placement of a hydrophilic short-wire (260 cm) across the downstream stricture and/or papilla. Retrograde access was then achieved by retrieving the trans-papillary wire, followed by standard ERCP intervention. Main outcome measurements were rates of procedural success and complications. RESULTS: EUS-guided biliary rendezvous was successful in 14 patients (93.3%). Failure was seen in one patient due to a tight malignant biliary stricture. One patient had peri-choledochal bile tracking which did not require any specific treatment. CONCLUSIONS: Short-wire system in EUS-guided biliary rendezvous is highly effective and safe. It is a useful salvage procedure for biliary cannulation in patients with accessible papilla.

  20. Treatment of biliary stricture by percutaneous transhepatic insertion of metallic stent

    International Nuclear Information System (INIS)

    He Xiaofeng; Shan Hong; Chen Yong; Li Yanhao

    1997-01-01

    Purpose: Percutaneous transhepatic insertion of biliary stent (PTIBS) was adopted for treating malignant and benign biliary stricture. The therapeutic effect, technique and complications were investigated. Materials and methods: Twenty-five patients with biliary stricture were treated by PTIBS, including hilar biliary cancer 16 cases, pancreatic cancer 4 cases, hilar metastatic cancer 3 cases, and post-operative biliary stricture of bile duct 2 cases. Results: The technical success rate was 96.0% (24/25). Two stents were placed in the right and left hepatic ducts respectively in 2 cases. The survival rate of 6 and 12 months were 73.3% and 46.6%. Restenosis rate was 40.0% in 6 months. Mild hemobilia (44.0%) and retrograde infection (8.0%) were the main complications which could be managed by conservative treatment. Conclusion: It was suggested that PTIBS was a safe and effective method in the treatment of biliary stricture. There was higher success rate of PTIBS as compared to endoscopic retrograde biliary, approach especially in patients with hilar biliary stricture. If combined with chemotherapy, the survival rate of malignant biliary stricture could be further improved

  1. Split thickness skin graft for cervicovaginal reconstruction in congenital atresia of cervix.

    Science.gov (United States)

    Zhang, Xuyin; Han, Tiantian; Ding, Jingxin; Hua, Keqin

    2015-10-01

    To introduce a new technique that combines laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in patients with congenital atresia of the cervix. Video article introducing a new surgical technique. University hospital. A 16-year-old patient with congenital cervical atresia, vaginal dysgenesis, and ovarian endometrial cyst. An original technique of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft for cervicovaginal reconstruction. A midline incision at the vaginal introitus was made, and a 9-cm canal was made between the bladder and the rectum using sharp and blunt dissection along the anatomic vaginal route, with the aid of laparoscopy to ensure correct orientation. A 14 × 12 cm split thickness skin graft was harvested from the right lateral thigh. By laparoscopy, the level of the lowest pole of the uterine cavity was exposed and the cervix was incised by shape dissection. The proximal segment of the harvested skin to the lower uterine segment was secured, and the distal segment was sutured with the upper margin of vulva vaginally. Surgical technique reports in anonymous patients are exempted from ethical approval by the Institutional Review Board. The patient gave consent to use the video in the article. The procedure was successfully completed. Since February 2013, our experiences of combined laparoscopic and vaginal cervicovaginal reconstruction using split thickness skin graft in 10 patients with congenital atresia of cervix were positive, with successful results and without complications or cervical, or vaginal stenosis. Our technique is feasible and safe for congenital atresia of cervix, with successful results and without complications or cervical or vaginal stenosis. Copyright © 2015 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  2. Effects of albumin administration in serum liver enzymes of rats in the presence of extrahepatic biliary obstruction Efeitos da administração de albumina nos níveis séricos de enzimas hepáticas em ratos com obstrução biliar extra-hepática

    Directory of Open Access Journals (Sweden)

    Jorge Fernando Rebouças Lessa

    2011-01-01

    Full Text Available PURPOSE: To study the influence of albumin on changes of liver function in the extrahepatic biliary obstruction through an experimental model in rats. METHODS: Sixty rats were divided into four groups: Group C (Control: 6 animals. Group M (Fictitious Operation: 18 rats underwent laparotomy and handling of the bile ducts; Groups O (extrahepatic biliary obstruction and A (Treated with 2% albumin: 18 animals in each group underwent ligation of the ductus liver; The animals in groups M, O and A were divided into three subgroups of 6 animals each to be killed in the 7, 14 and 21 days postoperative (POD. Blood was drawn for determination of total bilirubin (TB, indirect bilirubin (IB, direct bilirubin (DB, alkaline phosphatase (ALP, aspartate aminotransferase (AST and alanine aminotransferase (ALT. RESULTS: On POD 7, BI levels were 4.5 mg / dl in group O and 2.1 mg / dl in group A (p = 0.025. On the 14th POD, the levels of PA were 1185.2 U / l in the group and O 458.3 U / l in group A (p = 0.004. ALT levels were 101.7 U / l in the group O and 75.7 U / l in group A (= 0.037. On POD 21, the levels of ALP were 1069.5 U / l in the group O and 468.3 U / l in group A (p = 0, 004. CONCLUSION: The administration of albumin reduced the serum levels of bilirubin in the 7th day of supplementationOBJETIVO: Estudar a influência da albumina em alterações funcionais do fígado na obstrução biliar extra-hepática por meio de um modelo experimental desenvolvido em ratos. MÉTODOS: 60 ratos distribuídos em quatro grupos: Grupo C (Controle: 6 animais. Grupo M (Operação Fictícia: 18 ratos submetidos à laparotomia e manuseio das vias biliares; Grupos O (Obstrução Biliar Extra-hepática e A (Tratados com albumina a 2%: 18 animais, em cada grupo, submetidos à ligadura do ducto hepático; Os animais dos grupos M, O e A foram distribuídos em três subgrupos de 6 animais cada, para serem mortos nos 7°, 14° e 21° dias pós- operatórios (DPO. Foi colhido sangue

  3. Biliary enhanced MR imaging by Gd-DTPA

    International Nuclear Information System (INIS)

    Ohkawa, Shinichi; Fujikura, Yuji; Kanai, Toshio; Hiramatsu, Kyoichi.

    1992-01-01

    Biliary enhanced MRI (BEMRI) by Gd-DTPA via PTCD and/or PTGBD tube for obstructive jaundice was performed in 8 patients. In all cases, biliary tract was clearly visualised as high signal intensity on T1 weighted images. On same images, primary lesion such as common bile duct cancer was also visualised as well as portal system. In addition, MR angiography (MRA) by 2D-time of flight method was performed. MRA with BEMRI shows portal encasement on the same image as biliary tract obstruction. This suggests MRA with BEMRI may replace the other modality for obstructive jaundice. (author)

  4. Biliary and pancreatic secretions in abdominal irradiation

    International Nuclear Information System (INIS)

    Becciolini, A.; Cionini, L.; Cappellini, M.; Atzeni, G.

    1979-01-01

    The biliary and pancreatic secretions have been determined in patients given pelvic or para-aortic irradiation, with a dose of 50 Gy in the former group and between 36 and 40 Gy in the latter. A test meal containing polyethylene glycol (PEG) as reference substance was used. Each sample of the duodenal content was assayed for volume, PEG content, amylase and trypsin activity, pH and biliary secretion. No significant modifications of biliary and pancreatic secretions were demonstrated after irradiation, suggesting that these functions are not involved in the pathogenesis of the malabsorption radiation syndrome. (Auth.)

  5. The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

    OpenAIRE

    Rommel, Nathalie; Rayyan, Maissa; Scheerens, Charlotte; Omari, Taher

    2017-01-01

    Infants and children with esophageal atresia commonly present with swallowing dysfunction or dysphagia. Dysphagia can lead to a range of significant consequences such as aspiration pneumonia, malnutrition, dehydration, and food impaction. To improve oral intake, the clinical diagnosis of dysphagia in patients with esophageal atresia should focus on both the pharynx and the esophagus. To characterize the complex interactions of bolus flow and motor function between mouth, pharynx, and esophagu...

  6. Brain Oxygenation During Thoracoscopic Repair of Long Gap Esophageal Atresia

    NARCIS (Netherlands)

    Stolwijk, Lisanne J; van der Zee, David C; Tytgat, Stefaan; van der Werff, Desiree; Benders, Manon J N L; van Herwaarden, Maud Y A; Lemmers, Petra M A

    2017-01-01

    Background: Elongation and repair of long gap esophageal atresia (LGEA) can be performed thoracoscopically, even directly after birth. The effect of thoracoscopic CO2-insufflation on cerebral oxygenation (rScO2) during the consecutive thoracoscopic procedures in repair of LGEA was evaluated.

  7. Surgical treatment of choanal atresia with transnasal endoscopic approach with stentless single side-hinged flap technique: 5 year retrospective analysis

    Directory of Open Access Journals (Sweden)

    Carmelo Saraniti

    Full Text Available Abstract Introduction: Choanal atresia is a rare congenital malformation of the nasal cavity characterized by the complete obliteration of the posterior choanae. In 67% of cases choanal atresia is unilateral, affecting mainly (71% the right nasal cavity. In contrast to the unilateral form, bilateral choanal atresia is a life-threatening condition often associated with respiratory distress with feeding and intermittent cyanosis exacerbated by crying. Surgical treatment remains the only therapeutic option. Objective: To report our experience in the use of a transnasal endoscopic approach with stentless single side-hinged flap technique for the surgical management of choanal atresia. Methods: A 5 year retrospective analysis of surgical outcomes of 18 patients treated for choanal atresia with a transnasal technique employing a single side-hinged flap without stent placement. All subjects were assessed preoperatively with a nasal endoscopy and a Maxillofacial computed tomography scan. Results: Ten males and eight females with a mean age at the time of surgery of 20.05 ± 11.32 years, underwent surgery for choanal atresia. Fifteen subjects (83.33% had a bony while 3 (26.77% a mixed bony-membranous atretic plate. Two and sixteen cases suffered from bilateral and unilateral choanal atresia respectively. No intra- and/or early postoperative complications were observed. Between 2 and 3 months after surgery two cases (11.11% of partial restenosis were found. Only one of these presented a relapse of the nasal obstruction and was subsequently successfully repaired with a second endoscopic procedure. Conclusion: The surgical technique described follows the basic requirements of corrective surgery and allows good visualization, evaluation and treatment of the atretic plate and the posterior third of the septum, in order to create the new choanal opening. We believe that the use of a stent is not necessary, as recommended in case of other surgical techniques

  8. Interventional radiology in benign diseases of the biliary tract

    International Nuclear Information System (INIS)

    Juliani, G.; Gandini, G.

    1986-01-01

    Most references in the literature on interventional radiology of the biliary tract refer to the treatment of cancer; only occasionally are benign conditions mentioned. An updated list of radiosurgical instruments on the market in Italy is presented. The operating technique from the preparation of the patient to the performance of percutaneous transhepatic cholangiography (PTC), biliary drainage, transhepatic bilioplasty, percutaneous extraction and chemical cholelitholisis of biliary calculi and drainage of biliary collections is then described. A personal series is then presented. It consist of 93 patients in whom one or more of the following conditions were diagnosed: exclusively intrahepatic calculosis (3 cases), calculosis of the common bile duct (23 percutaneous treatments), empyema of the gallbladder (6 cases), suppurating cholangitis (46 cases), sclerotic or inflammatory stenosis (16 cases), biliary collections (14 cases). Results are reported and commented on

  9. Biliary duodenostomy: a safe and easier biliary drainage procedure ...

    African Journals Online (AJOL)

    another simple alternative for biliary drainage after CC excision. ... group II suffered from ascending cholangitis 6 months postoperatively. ... including all cases below 18 years of age with a diagnosis of CC, from ... All patients remained in the high-dependency unit for ... between two groups with quantitative data was carried.

  10. Endoscopic Stenting and Clipping for Anastomotic Stricture and Persistent Tracheoesophageal Fistula after Surgical Repair of Esophageal Atresia in an Infant

    Directory of Open Access Journals (Sweden)

    Mohammed Amine Benatta

    2014-01-01

    Full Text Available Anastomotic stricture (AS and recurrent tracheoesophageal fistula (TEF are two complications of surgical repair of esophageal atresia (EA. Therapeutic endoscopic modalities include stenting, tissue glue, and clipping for TEF and endoscopic balloon dilation bougienage and stenting for esophageal strictures. We report herein a two-month infant with both EA and TEF who benefited from a surgical repair for EA, at the third day of life. Two months later he experienced deglutition disorders and recurrent chest infections. The esophagogram showed an AS and a TEF confirmed with blue methylene test at bronchoscopy. A partially covered self-expanding metal type biliary was endoscopically placed. Ten weeks later the stent was removed. This allows for easy passage of the endoscope in the gastric cavity but a persistent recurrent fistula was noted. Instillation of contrast demonstrated a fully dilated stricture but with a persistent TEF. Then we proceeded to placement of several endoclips at the fistula site. The esophagogram confirmed the TEF was obliterated. At 12 months of follow-up, he was asymptomatic. Stenting was effective to alleviate the stricture but failed to treat the TEF. At our knowledge this is the second case of successful use of endoclips placement to obliterate recurrent TEF after surgical repair of EA in children.

  11. Epidemiology of small intestinal atresia in Europe: a register-based study.

    LENUS (Irish Health Repository)

    Best, Kate E

    2012-09-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe.

  12. Signet Ring Cell Carcinoma of the Extrahepatic Bile Duct Diagnosed by Preoperative Biopsy: A Case Report

    Directory of Open Access Journals (Sweden)

    Emiri Kita

    2014-11-01

    Full Text Available A 73-year-old woman was admitted because of obstructive jaundice. Computed tomography revealed a stricture in the lower bile duct with enhanced bile duct wall. Endoscopic retrograde cholangiopancreatography (ERCP revealed a tapering stenosis at the lower bile duct. Transpapillary histological biopsy using biopsy forceps through ERCP was performed; the diagnosis of signet ring cell carcinoma (SRCC of the bile duct was established. Regional lymph node enlargement and distant metastases were not detected on diagnostic imaging. Pancreaticoduodenectomy with pylorus preservation was performed. Histological examination of the resected specimen confirmed SRCC of the extrahepatic bile duct coexisting with adenocarcinoma (ADC of the extrahepatic bile duct with negative resection margins. However, tumor cells directly invaded the pancreatic parenchyma and the muscle layer of the duodenum, prompting us to administer adjuvant chemotherapy to the patient, with no sign of tumor recurrence at 1-year follow-up. Almost all tumors originating from the extrahepatic bile duct are ADC and other histological variants are rare. Of these, SRCC is extremely rare and only four cases have been reported. Furthermore, to the best of our knowledge, this is the first case report regarding the preoperative diagnosis of SRCC of the bile duct. Current reports indicate that younger age and Asian ethnicity are the clinical features of SRCC of the extrahepatic bile duct. Immunohistochemical staining of CK7, CK20 and MUC2 may be useful for predicting prognosis. Chemotherapy has not resulted in increased survival rates and only surgical resection currently serves as a curative treatment.

  13. Sonographic assessment of endoscopically-placed biliary endoprostheses. A prospective study

    Energy Technology Data Exchange (ETDEWEB)

    Mirk, P; Belli, P; Filemi, A; Costamagna, G; Coppola, R; Nuzzo, G; Colagrande, C

    1988-01-01

    Endoscopic retrograde biliary drainage by means of transtumoral endoprostheses is an effective technique for pallliative decompression of malignant biliary obstruction. However, serial follow-up is required for an early detection of eventual long-term complications. In the present study 37 patients with malignant biliary obstruction, trated by endoscopic insertion of one or more biliary stents, were prospectively evaluated by sonography, with serial clinical and US examinations up to 10 months. In this experience, sonography could correctly identify both the endoprostheses and their location in the biliary tract. Most important, sonography has proved to be a sensitive method to detect possible stent dysfunctions, besides providing with information about the prograssion of the underlying malignancy. 19 refs.

  14. Aortic atresia with normal sized left ventricle

    OpenAIRE

    Priya Jagia; Arun Sharma; Saurabh K Gupta; Munish Guleria

    2016-01-01

    Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best ...

  15. Dysphagia in children with repaired oesophageal atresia

    OpenAIRE

    Coppens, C.H.; Engel-Hoek, L. van den; Scharbatke, H.E.; Groot, S.A. de; Draaisma, J.M.T.

    2016-01-01

    Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral and pharyngeal dysphagia based on videofluoroscopic swallow study (VFSS) findings in a limited number of children in this cohort. Medical records of 111 patients, born between January 1996 and July ...

  16. [SURGICAL TACTICS IN CHRONIC PANCREATITIS WITH SIGNS OF BILIARY HYPERTENSION].

    Science.gov (United States)

    Usenko, O Yu; Kopchak, V M; Pylypchuk, V I; Kopchak, K V; Andronik, S V

    2015-08-01

    The results of treatment of 84 patients for chronic pancreatitis with the biliary hypertension signs were depicted. In 83 patients operative interventions were performed, and in 1--positive results were achieved after pancreatic cyst puncture under ultrasonographic control. In 51 patients the conduction of Frey operation have permitted to achieve a lower pressure inside biliary system, in 25--the additional procedures were applied for a biliary hypertension elimination. In 20 patients a method of pressure measurement in biliary system was used.

  17. Extrahepatic Arteries Originating from Hepatic Arteries: Analysis Using CT During Hepatic Arteriography and Visualization on Digital Subtraction Angiography

    International Nuclear Information System (INIS)

    Ozaki, Kumi; Kobayashi, Satoshi; Matsui, Osamu; Minami, Tetsuya; Koda, Wataru; Gabata, Toshifumi

    2017-01-01

    PurposeTo investigate the prevalence and site of origin of extrahepatic arteries originating from hepatic arteries on early phase CT during hepatic arteriography (CTHA) was accessed. Visualization of these elements on digital subtraction hepatic angiography (DSHA) was assessed using CTHA images as a gold standard.Materials and MethodsA total of 943 patients (mean age 66.9 ± 10.3 years; male/female, 619/324) underwent CTHA and DSHA. The prevalence and site of origin of extrahepatic arteries were accessed using CTHA and visualized using DSHA.ResultsIn 924 (98.0%) patients, a total of 1555 extrahepatic branches, representing eight types, were found to originate from hepatic arteries on CTHA. CTHA indicated the following extrahepatic branch prevalence rates: right gastric artery, 890 (94.4%); falciform artery, 386 (40.9%); accessory left gastric artery, 161 (17.1%); left inferior phrenic artery (IPA), 43 (4.6%); posterior superior pancreaticoduodenal artery, 33 (3.5%); dorsal pancreatic artery, 26 (2.8%); duodenal artery, 12 (1.3%); and right IPA, 4 (0.4%). In addition, 383 patients (40.6%) had at least one undetectable branch on DSHA. The sensitivity, specificity, and accuracy of visualization on DSHA were as follows: RGA, 80.0, 86.8, and 80.4%; falciform artery, 53.9, 97.7, and 80.0%; accessory LGA, 64.6, 98.6, and 92.3%; left IPA, 76.7, 99.8, and 98.7%; PSPDA, 100, 99.7, and 99.9%; dorsal pancreatic artery, 57.7, 100, and 98.8%; duodenal artery, 8.3, 99.9, and 98.7%; and right IPA, 0, 100, and 99.6%, respectively.ConclusionExtrahepatic arteries originating from hepatic arteries were frequently identified on CTHA images. These arteries were frequently overlooked on DSHA.

  18. Extrahepatic Arteries Originating from Hepatic Arteries: Analysis Using CT During Hepatic Arteriography and Visualization on Digital Subtraction Angiography

    Energy Technology Data Exchange (ETDEWEB)

    Ozaki, Kumi, E-mail: ozakik-rad@umin.org [Kanazawa University Graduate School of Medical Science, Department of Radiology (Japan); Kobayashi, Satoshi [Kanazawa University Graduate School of Medical Science, Department of Quantum Medicine Technology (Japan); Matsui, Osamu; Minami, Tetsuya; Koda, Wataru; Gabata, Toshifumi [Kanazawa University Graduate School of Medical Science, Department of Radiology (Japan)

    2017-06-15

    PurposeTo investigate the prevalence and site of origin of extrahepatic arteries originating from hepatic arteries on early phase CT during hepatic arteriography (CTHA) was accessed. Visualization of these elements on digital subtraction hepatic angiography (DSHA) was assessed using CTHA images as a gold standard.Materials and MethodsA total of 943 patients (mean age 66.9 ± 10.3 years; male/female, 619/324) underwent CTHA and DSHA. The prevalence and site of origin of extrahepatic arteries were accessed using CTHA and visualized using DSHA.ResultsIn 924 (98.0%) patients, a total of 1555 extrahepatic branches, representing eight types, were found to originate from hepatic arteries on CTHA. CTHA indicated the following extrahepatic branch prevalence rates: right gastric artery, 890 (94.4%); falciform artery, 386 (40.9%); accessory left gastric artery, 161 (17.1%); left inferior phrenic artery (IPA), 43 (4.6%); posterior superior pancreaticoduodenal artery, 33 (3.5%); dorsal pancreatic artery, 26 (2.8%); duodenal artery, 12 (1.3%); and right IPA, 4 (0.4%). In addition, 383 patients (40.6%) had at least one undetectable branch on DSHA. The sensitivity, specificity, and accuracy of visualization on DSHA were as follows: RGA, 80.0, 86.8, and 80.4%; falciform artery, 53.9, 97.7, and 80.0%; accessory LGA, 64.6, 98.6, and 92.3%; left IPA, 76.7, 99.8, and 98.7%; PSPDA, 100, 99.7, and 99.9%; dorsal pancreatic artery, 57.7, 100, and 98.8%; duodenal artery, 8.3, 99.9, and 98.7%; and right IPA, 0, 100, and 99.6%, respectively.ConclusionExtrahepatic arteries originating from hepatic arteries were frequently identified on CTHA images. These arteries were frequently overlooked on DSHA.

  19. Usefulness of ultrasonography and biochemical features in the ...

    African Journals Online (AJOL)

    [2] Identification of a normal gall bladder on sonogram is highly predictive of ... and is also influenced by the patient feeding or fasting, even though biliary atresia can ..... failure to detect jaundice clinically in dark-skinned babies. There was no ...

  20. When Your Child Needs a Liver Transplant

    Science.gov (United States)

    ... failure is biliary atresia . This happens when the liver's bile ducts (tubes that carry bile out of the liver) ... the sick liver and put in the donated liver. Blood vessels and bile ducts from the new liver will be attached to ...

  1. Variability of human hepatic UDP-glucuronosyltransferase activity

    NARCIS (Netherlands)

    Little, JM; Lester, R; Kuipers, F; Vonk, R; Mackenzie, PI; Drake, RR; Frame, L; Radominska-Pandya, A

    1999-01-01

    The availability of a unique series of liver samples from human subjects, both control patients (9) and those with liver disease (6; biliary atresia (2), retransplant, chronic tyrosinemia type I, tyrosinemia, Wilson's disease) allowed us to characterize human hepatic UDP-glucuronosyltransferases

  2. [Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis].

    Science.gov (United States)

    Marí-Alfonso, Begoña; Amengual-Guedan, María José; Vergara-Gómez, Mercè; Simeón-Aznar, Carmen Pilar; Fonollosa-Plà, Vicente; Jove-Buxeda, Esther; Oliva-Morera, Juan; Tolosa-Vilella, Carles

    2016-01-01

    Primary biliary cirrhosis (PBC) is associated to any systemic autoimmune disease (SAD), in particular systemic sclerosis (SSc). To investigate the prevalence of SAD in a cohort of patients with PBC, specifically the prevalence of SSc and its clinical subtypes, and determining the clinical and biological profile of patients with associated PBC and SSc. Observational study of 62 patients with PBC following a protocol that included an anamnesis and physical examination to detect the presence of SAD as well as a nailfold capillaroscopy and an immunological study with specific SSc autoantibodies. A comparative analysis was conducted between patients with isolated PBC and patients with PBC and an associated SAD. SAD was associated to PBC in 22 patients (35,4%), and SSc was the most frequent illness, identified in 13 cases (21%). Five patients (8%) without previous diagnosis of SAD fulfilled pre-scleroderma criteria, according to LeRoy and Medsger criteria. The presence of anticentromere antibodies (54,5% vs. 5%, P<.001) was the unique immunological determination identified more frequently in patients with PBC-SAD. The SSc suggestive capillary pattern was visualized in 11 patients (20,4%), mainly the slow pattern. No factors associated with greater morbi-mortality were identified in the PBC-SAD group. It does exist a subgroup of patients with PBC and clinical-biological features suggestive of an SAD, which advise a protocolized study to detect early the association to an SAD. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

  3. Frey procedure combined with biliary diversion in chronic pancreatitis.

    Science.gov (United States)

    Merdrignac, Aude; Bergeat, Damien; Rayar, Michel; Harnoy, Yann; Turner, Kathleen; Courtin-Tanguy, Laetitia; Boudjema, Karim; Meunier, Bernard; Sulpice, Laurent

    2016-11-01

    The Frey procedure has become the standard operative treatment in chronic painful pancreatitis. Biliary diversion could be combined when associated with common bile duct obstruction. The aim of the present study was to evaluate the impact of the type of biliary diversion combined with the Frey procedure on late morbidity. The data from consecutive patients undergoing the Frey procedure and having a minimum follow-up of 2 years were extracted from a maintained prospective database. The mean endpoint was the rate of secondary biliary stricture after the Frey procedure combined with biliary diversion (bilioenteric anastomosis or common bile duct reinsertion in the resection cavity). Between 2006 and 2013, 55 consecutive patients underwent the Frey procedure. Twenty-nine patients had common bile duct obstruction (52.7%). The technique of biliary diversion resulted in bilioenteric anastomosis in 19 patients (65.5%) and common bile duct reinsertion in 10 patients (34.5%). Preoperative characteristics and early surgical outcomes were comparable. Pain control was similar. There was significantly more secondary biliary stricture after common bile duct reinsertion than after bilioenteric anastomosis (60% vs 11%, P = .008). Combined bilioenteric anastomosis during the Frey procedure is an efficient technique for treating common bile duct obstruction that complicates chronic painful pancreatitis. Bilioenteric anastomosis was associated with less secondary biliary stricture than common bile duct reinsertion in the pancreatic resection cavity. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Biliary cholesterol secretion : More than a simple ABC

    NARCIS (Netherlands)

    Dikkers, Arne; Tietge, Uwe J. F.

    2010-01-01

    Biliary cholesterol secretion is a process important for 2 major disease complexes, atherosclerotic cardiovascular disease and cholesterol gallstone disease With respect to cardiovascular disease, biliary cholesterol secretion is regarded as the final step for the elimination of cholesterol

  5. Targeted Therapy for Biliary Tract Cancer

    International Nuclear Information System (INIS)

    Furuse, Junji; Okusaka, Takuji

    2011-01-01

    It is necessary to establish effective chemotherapy to improve the survival of patients with biliary tract cancer, because most of these patients are unsuitable candidates for surgery, and even patients undergoing curative surgery often have recurrence. Recently, the combination of cisplatin plus gemcitabine was reported to show survival benefits over gemcitabine alone in randomized clinical trials conducted in the United Kingdom and Japan. Thus, the combination of cisplatin plus gemcitabine is now recognized as the standard therapy for unresectable biliary tract cancer. One of the next issues that need to be addressed is whether molecular targeted agents might also be effective against biliary tract cancer. Although some targeted agents have been investigated as monotherapy for first-line chemotherapy, none were found to exert satisfactory efficacy. On the other hand, monoclonal antibodies such as bevacizumab and cetuximab have also been investigated in combination with a gemcitabine-based regimen and have been demonstrated to show promising activity. Furthermore, clinical trials using new targeted agents for biliary tract cancer are also proposed. This cancer is a relatively rare and heterogeneous tumor consisting of cholangiocarcinoma and gallbladder carcinoma. Therefore, a large randomized clinical trial is necessary to confirm the efficacy of chemotherapy, and international collaboration is important

  6. The role of B/D ratio and A/D ratio to defferentiate malignancy from benignancy in distal extrahepatic bile duct obstruction

    International Nuclear Information System (INIS)

    Rhim, Hyun Chul; Baek, JUng Hwan; Jeon, Eui Yong; Koh, Byung Hee; Cho, On Koo; Kim, Young Hwan

    1994-01-01

    To determine wheter bilirubin/extrahepatic bile duct diameter ratio(B/D ratio) or alkalinephosphatase/extrahepatic bile duct diameter ratio (A/D ratio) can be used to differentiate malignant from benigndisease in distal extrahepatic bile duct obstruction during ultrasonographic examination. We retrospectively reviewed the sonograms and laboratory data of 100 patients with obstructive jaundice (benign : n=50, malignant: : n=50). The diagnosis was confirmed either surgically (n=66) or clinically (n=34). The B/D ratio and A/D radio were calculated by means of dividing total bilirubin (mg/dl) and alkaline phosphatase (I.U.)respectively by maximum extrahepatic bile duct diameter(mm) on ultrasonogram. Significant difference in B/D ratio was found between the benignancy (0.28+0.25) and malignancy (0.98+0.84) groups (P<0.001). Significant difference in A/D ratio was also found between the benignancy (15.00+10.22) and malignancy (32.44+30.28) groups(P<0.001) Accuracies to predict malignancy according to criteria of B/D and A/D ratios were less than 75% and 65%respectively. On the other hand, the positive predictive value and specificity were relatively high. The B/D ratio and A/D ratio calculated from ultrasonograms can be used as a valuable screening index todifferentiate malignancy from benignacy in patients with distal extrahepatic bile duct obstruction, especially when the sonography is difficult because of the interposition of bowel gas or obesity

  7. Carcinoid tumors of the extrahepatic bile ducts: a study of seven cases

    NARCIS (Netherlands)

    Maitra, A.; Krueger, J. E.; Tascilar, M.; Offerhaus, G. J.; Angeles-Angeles, A.; Klimstra, D. S.; Hruban, R. H.; Albores-Saavedra, J.

    2000-01-01

    The authors report seven patients with carcinoid tumors of the extrahepatic bile ducts (EHBDs). All patients were women, with an average age at diagnosis of 49.8 years (range, 37-67 yrs). The most common presenting symptom was painless jaundice with or without pruritus. Although one patient had

  8. On the mechanical behavior of the human biliary system

    Institute of Scientific and Technical Information of China (English)

    Xiaoyu Luo; Wenguang Li; Nigel Bird; Swee Boon Chin; NA Hill; Alan G Johnson

    2007-01-01

    This paper reviews the progress made in understanding the mechanical behaviour of the biliary system.Gallstones and diseases of the biliary tract affect more than 10% of the adult population. The complications of gallstones, i.e. acute pancreatitis and obstructive jandice, can be lethal, and patients with acalculous gallbladder pain often pose diagnostic difficulties and undergo repeated ultrasound scans and oral cholecystograms. Moreover, surgery to remove the gallbladder in these patients, in an attempt to relieve the symptoms, gives variable results. Extensive research has been carried out to understand the physiological and pathological functions of the biliary system, but the mechanism of the pathogenesis of gallstones and pain production still remain poorly understood. It is believed that the mechanical factors play an essential role in the mechanisms of the gallstone formation and biliary diseases. However, despite the extensive literature in clinical studies, only limited work has been carried out to study the biliary system from the mechanical point of view. In this paper, we discuss the state of art knowledge of the fluid dynamics of bile flow in the biliary tract, the solid mechanics of the gallbladder and bile ducts, recent mathematical and numerical modelling of the system,and finally the future challenges in the area.

  9. Diagnosis and treatment of gastroesophageal reflux in patients with esophageal atresia

    NARCIS (Netherlands)

    J.H.L.J. Bergmeijer (Jan Hein)

    2002-01-01

    textabstractIn the last two decades, surgical treatment of children born with esophageal atresia has become a standard procedure. Postoperative mortality- now negligible in those born at term without other severe anomalies- mainly relates to patients with associated severe congenital cardiac

  10. Missed diagnosis of atresia of the right pulmonary artery in woman with left-sided pneumothorax

    DEFF Research Database (Denmark)

    Dagnegård, Hanna; Ryom, Philip

    2016-01-01

    Isolated pulmonary atresia is an uncommon condition, which can go undiagnosed for a long time in asymptomatic patients. Sometimes, diagnosis can be made at pregnancy due to respiratory symptoms. There is no known increased risk of pneumothorax. We here present a case where a second-time pregnant...... woman with an unknown atresia of the right pulmonary artery received a left-sided pneumothorax. The diagnosis was initially missed in spite of adequate imaging and the condition progressed to respiratory stop. We describe the course of diagnostics and the chosen strategy of treatment....

  11. Radiotherapy Is Associated With Improved Survival in Adjuvant and Palliative Treatment of Extrahepatic Cholangiocarcinomas

    International Nuclear Information System (INIS)

    Shinohara, Eric T.; Mitra, Nandita; Guo Mengye; Metz, James M.

    2009-01-01

    Purpose: Extrahepatic cholangiocarcinomas (EHC) are rare tumors of the biliary tree because of their low incidence, large randomized studies examining radiotherapy (RT) for EHC have not been performed. The purpose of this study was to examine the role of adjuvant and palliative RT in the treatment of EHC in a large patient population. Methods and Materials: This was a retrospective analysis of 4,758 patients with EHC collected from the Surveillance, Epidemiology, and End Results database. The primary endpoint was overall survival. Results: Patients underwent surgery (28.8%), RT (10.0%), surgery and RT (14.7%), or no RT or surgery (46.4%). The median age of the patient population was 73 years (range, 23-104), 52.5% were men, and 80.7% were white. The median overall survival time was 16 months (95% confidence interval [CI] 15-17), 9 months (95% CI 9-11), 9 months (95% CI 9-10), and 4 months (95% CI 3-4) for surgery and RT, surgery, RT, and no RT or surgery, respectively. The overall survival was significantly different between the surgery and surgery and RT groups (p < .0001) and RT and no RT or surgery groups (p < .0001) on the log-rank test. The propensity score-adjusted analyses of surgery and RT vs. surgery (hazard ratio, 0.94; 95% CI, 0.84-1.05) were not significantly different, but that for RT vs. no RT or surgery (hazard ratio, 0.61; 95% CI, 0.54-0.70) was significantly different. Conclusion: These results suggest that palliative RT prolongs survival in patients with EHC. The benefit associated with surgery and RT was significant on univariate analysis but not after controlling for potential confounders using the propensity score. Future studies should evaluate the addition of chemotherapy and biologic agents for the treatment of EHC.

  12. Inflammation-based prognostic score is a useful predictor of postoperative outcome in patients with extrahepatic cholangiocarcinoma.

    Science.gov (United States)

    Oshiro, Yukio; Sasaki, Ryoko; Fukunaga, Kiyoshi; Kondo, Tadashi; Oda, Tatsuya; Takahashi, Hideto; Ohkohchi, Nobuhiro

    2013-03-01

    Recent studies have revealed that the Glasgow prognostic score (GPS), an inflammation-based prognostic score, is useful for predicting outcome in a variety of cancers. This study sought to investigate the significance of GPS for prognostication of patients who underwent surgery with extrahepatic cholangiocarcinoma. We retrospectively analyzed a total of 62 patients who underwent resection for extrahepatic cholangiocarcinoma. We calculated the GPS as follows: patients with both an elevated C-reactive protein (>10 mg/L) and hypoalbuminemia (L) were allocated a score of 2; patients with one or none of these abnormalities were allocated a s ore of 1 or 0, respectively. Prognostic significance was analyzed by the log-rank test and a Cox proportional hazards model. Overall survival rate was 25.5 % at 5 years for all 62 patients. Venous invasion (p = 0.01), pathological primary tumor category (p = 0.013), lymph node metastasis category (p GPS (p = 0.008) were significantly associated with survival by univariate analysis. A Cox model demonstrated that increased GPS was an independent predictive factor with poor prognosis. The preoperative GPS is a useful predictor of postoperative outcome in patients with extrahepatic cholangiocarcinoma.

  13. Autoimmunity and Extrahepatic Manifestations in Treatment-Naïve Children with Chronic Hepatitis C Virus Infection

    Directory of Open Access Journals (Sweden)

    Giuseppe Indolfi

    2012-01-01

    Full Text Available Hepatitis C virus (HCV infection has been associated with autoimmunity and extrahepatic manifestations in adults. Few data are available on these topics in children. Nonorgan specific auto-antibodies development is part of the natural course of chronic hepatitis C in children. Smooth muscle autoantibody is the most common autoantibody found, while liver-kidney microsomal type-1 antibody positivity is the most peculiar autoimmune feature of children with HCV infection. The clinical significance of non-organ specific autoantibodies in the course of paediatric chronic hepatitis C is still debated. Autoantibody positivity can be considered neutral for most patients, while it can be associated with negative connotations for others, especially those positive for liver-kidney microsomal type-1 autoantibody. Subclinical hypothyroidism but not autoimmune thyroiditis has been demonstrated in HCV infection in children, while only few cases of HCV-associated membranoproliferative glomerulonephritis have been described. Single reports are available in the literature reporting the anecdotal association between chronic hepatitis C and other extrahepatic manifestations such as myopathy and opsoclonus-myoclonus syndrome. Despite the low incidence of extrahepatic manifestations of chronic hepatitis C in children, overall, available data suggest a careful monitoring.

  14. Intrahepatic biliary tract adenocarcinoma. Review of literature

    International Nuclear Information System (INIS)

    Encalada, Edmundo; Engracia, Ruth; Calle, Carlos; Rivera, Tania; Marengo, Carlos

    2002-01-01

    A seven years old patient, with a biliary tract tumoration, diagnosed by computerized tomography and eco, which had practice an exploratory laparotomy, finding an intrahepatic tumor at the left hepatic tract level, with a pathological diagnosis of papillary adenocarcinoma moderately differentiated the biliary tract. The surgery is the main treatment, auxiliary treatments with chemotherapy and radiotherapy. (The author)

  15. Aneurysmal malformation of the extrahepatic portal vein: a case report

    International Nuclear Information System (INIS)

    Lee, Jeong Min; Kang, Sung Soo; Kim, Chong Soo; Han, Hyeun Young

    1999-01-01

    Aneurysmal malformation of the portal vein is a rare entity. To our knowledge, only scattered reports of portal vein aneurysms appear in the literature in English, and there is no previously published report in Korean. We describe a case exhibiting aneurysmal malformation of the extrahepatic portal vein at the hepatic hilum ; the findings demonstrated by ultrasound, CT and angiography are discussed, a review of previously described cases is included

  16. 3 T MR cholangiopancreatography appearances of biliary ascariasis

    Energy Technology Data Exchange (ETDEWEB)

    Ding, Z.X., E-mail: hangzhoudzx73@126.co [Department of Radiology, Zhejiang Provincial People' s Hospital, Hangzhou (China); Yuan, J.H. [Department of Radiology, Zhejiang Provincial People' s Hospital, Hangzhou (China); Chong, V. [Department of Diagnostic Imaging, National University Health System, Yong Loo Lin School of Medicine, National University of Singapore (Singapore); Zhao, D.J. [Department of Hepatobiliary Surgery, Zhejiang Provincial People' s Hospital, Hangzhou (China); Chen, F.H.; Li, Y.M. [Department of Radiology, Zhejiang Provincial People' s Hospital, Hangzhou (China)

    2011-03-15

    Aim: To evaluate the 3 T MR cholangiopancreatography (MRCP) appearances of biliary ascariasis. Materials and methods: Nine patients with a MRCP diagnosis of biliary ascariasis were reviewed. All patients had endoscopic retrograde cholangiopancreatography (ERCP) or surgical confirmation of the disease. Results: On thin-slab MRCP imaging, Ascaris worms are clearly demarcated within the biliary tree. All the identified worms demonstrated a characteristic three-parallel-lines appearance. The middle high-signal intensity line is sandwiched between two low-signal intensity lines and they are in turn surrounded by high signal bile. On thick-slab MRCP the worms also show the three-line sign but with less clarity. However, thick-slab MRCP has the advantage of providing three-dimensional ERCP-like images of the pancreaticobiliary system. Conclusion: The 'three-line' sign appears to be a characteristic sign of biliary ascariasis on 3 T MRCP.

  17. Sequential algorithm analysis to facilitate selective biliary access for difficult biliary cannulation in ERCP: a prospective clinical study.

    Science.gov (United States)

    Lee, Tae Hoon; Hwang, Soon Oh; Choi, Hyun Jong; Jung, Yunho; Cha, Sang Woo; Chung, Il-Kwun; Moon, Jong Ho; Cho, Young Deok; Park, Sang-Heum; Kim, Sun-Joo

    2014-02-17

    Numerous clinical trials to improve the success rate of biliary access in difficult biliary cannulation (DBC) during ERCP have been reported. However, standard guidelines or sequential protocol analysis according to different methods are limited in place. We planned to investigate a sequential protocol to facilitate selective biliary access for DBC during ERCP. This prospective clinical study enrolled 711 patients with naïve papillae at a tertiary referral center. If wire-guided cannulation was deemed to have failed due to the DBC criteria, then according to the cannulation algorithm early precut fistulotomy (EPF; cannulation time > 5 min, papillary contacts > 5 times, or hook-nose-shaped papilla), double-guidewire cannulation (DGC; unintentional pancreatic duct cannulation ≥ 3 times), and precut after placement of a pancreatic stent (PPS; if DGC was difficult or failed) were performed sequentially. The main outcome measurements were the technical success, procedure outcomes, and complications. Initially, a total of 140 (19.7%) patients with DBC underwent EPF (n = 71) and DGC (n = 69). Then, in DGC group 36 patients switched to PPS due to difficulty criteria. The successful biliary cannulation rate was 97.1% (136/140; 94.4% [67/71] with EPF, 47.8% [33/69] with DGC, and 100% [36/36] with PPS; P EPF, 314.8 (65.2) seconds in DGC, and 706.0 (469.4) seconds in PPS (P EPF, DGC, and PPS may be safe and feasible for DBC. The use of EPF in selected DBC criteria, DGC in unintentional pancreatic duct cannulations, and PPS in failed or difficult DGC may facilitate successful biliary cannulation.

  18. Recent progress in ERCP for biliary and pancreatic diseases