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

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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

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 ...

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.

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.

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

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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

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

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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.

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...

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...

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

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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.

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

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...

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.

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.

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

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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.

Biliary atresia: lessons learned from the voluntary German registry.

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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

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...

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 ...

[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.

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.

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

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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

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.

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

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).

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

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...

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...

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).

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

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

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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.

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

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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.

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.

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

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.

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.

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

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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.

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.

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.

“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

Biliary Innate Immunity: Function and Modulation

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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.

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.

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 ...

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.

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

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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.

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.

Bone Health in a Nonjaundiced Population of Children with Biliary Atresia

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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.

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...

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.)

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.)

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

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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.

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

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

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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.

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

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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.

[Bile duct atresia: outline for a solution].

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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

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

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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.

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

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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.

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

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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.

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

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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.

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

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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.

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.

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.

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)

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

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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.

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

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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.

Endotoxin and CD14 in the progression of biliary atresia

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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.

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

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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

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

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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

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...

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

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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.

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

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.

Anomalous pancreatic ductal system allowing distal bowel gas with duodenal atresia

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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.

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.)

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

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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.)

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...

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

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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.

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

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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.

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

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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.

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

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

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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.

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

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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.

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

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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.

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...

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

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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.

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

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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.

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

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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.

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.

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

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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.

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

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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.

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

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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.)

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.)

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

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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.

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 ...

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

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.

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

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)

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

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 ...

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 ...

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.

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 ...

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)

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...

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

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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.

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.)

Prenatal diagnosis of horseshoe lung and esophageal atresia

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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.)

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.)

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.)

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

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

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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.

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

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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.)

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.

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 ...

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

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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

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

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

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

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

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.

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

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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

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

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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.

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

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.

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.

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

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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.

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.

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

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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.

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

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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.)

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.)

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 ...

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...

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

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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

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

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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.

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

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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

Apple-peel atresia presenting as foetal intestinal obstruction

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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.

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

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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.

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

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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.

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.

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

Aortic atresia with normal sized left ventricle

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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.

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...

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.

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.

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.

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.)

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.

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.

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.

Future developments in biliary stenting

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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

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

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.

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

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.

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

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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.

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

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.

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.

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.)

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.

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.

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

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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.)

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.)

MicroRNAs: New Insight in Modulating Follicular Atresia: A Review

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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.

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...

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....

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.

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.

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.

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.

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

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

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

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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.

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

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

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.

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

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 ...

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.

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.

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 ...

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.

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.

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.)

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)

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.

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

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

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.

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.

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

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...

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

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.

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.

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.

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....

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...

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

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

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 ...

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.

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

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.

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)

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.

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....

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.)

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.)

Broncho-biliary fistula secondary to biliary obstruction and lung abscess in a patient with pancreatic neuro-endocrine tumor

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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.

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

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

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

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.

False Computed Tomography Findings in Bilateral Choanal Atresia

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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.

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.

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.

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

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.

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.

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

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.

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...

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.

[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.

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.

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.

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

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

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

Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators

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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.

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.

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....

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

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

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

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.

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.

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

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.

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

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.

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

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.

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.)

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.

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.

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.

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...

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

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.

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 ...

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.

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

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 ...

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.

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

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

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.

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 ...

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.

[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.

[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.

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.

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.

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.

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.

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)

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

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.

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

[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.

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.

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.

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.

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

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 ...

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 ...

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.

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

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.

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)

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.)

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...

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.

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

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

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.

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.

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.

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.

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 ...

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 ...

[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.

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

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 ...

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 ...

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

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.

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.

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

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

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.

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

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....

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...

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)

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.

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.

Recent progress in ERCP for biliary and pancreatic diseases

Directory of Open Access Journals (Sweden)

MIAO Lin

2014-12-01

Full Text Available In recent years, with the continuous development of endoscopic and interventional techniques, many new devices and methods have been used in clinical practice, and the application of endoscopic retrograde cholangiopancreatography (ERCP in biliary and pancreatic diseases has developed rapidly. This paper reviews and summarizes the recent progress in ERCP among patients with biliary and pancreatic diseases, including those with altered gastrointestinal anatomy, pregnant patients, patients with benign and malignant biliary strictures, and patients with pancreatic pseudocysts, as well as the application of SpyGlass, photodynamic therapy, and radiofrequency ablation, the management of ERCP-related duodenal perforation, and the prevention of post-ERCP pancreatitis. All the progress has made a great contribution to the diagnosis and treatment of biliary and pancreatic diseases.

Role of stents and laser therapy in biliary strictures

Science.gov (United States)

Chennupati, Raja S.; Trowers, Eugene A.

2001-05-01

The most frequent primary cancers causing malignant obstructive jaundice were pancreatic cancer (57%), hilar biliary cancer (19% including metastatic disease), nonhilar biliary cancer (14%) and papillary cancer (10%). Endoscopic stenting has widely replaced palliative surgery for malignant biliary obstruction because of its lower risk and cost. Self-expandable metal stents are the preferred mode of palliation for hilar malignancies. Plastic stents have a major role in benign biliary strictures. Major complications and disadvantages associated with metallic stents include high cost, cholangitis. malposition, migration, unextractability, and breakage of the stents, pancreatitis and stent dysfunction. Dysfunction due to tumor ingrowth can be relieved by thermal methods (argon plasma coagulator therapy). We present a concise review of the efficacy of metallic stents for palliation of malignant strictures.

Primary biliary cirrhosis and scleroderma complicated by Barrett's ...

African Journals Online (AJOL)

1991-04-06

Apr 6, 1991 ... primary biliary cirrhosis, CREST syndrome, and chronic pancreatitis. Thorax. 1983; 38: 316-317. 9. Okano Y, Nisbikai M, Sato A. Scleroderma, primary biliary cirrhosis, and. Sjogren's syndrome after cosmetic breast augmentation with silicone injec- tion: a case reporfof possible human adjuvant disease.

Benign Biliary Strictures and Leaks.

Science.gov (United States)

Devière, Jacques

2015-10-01

The major causes of benign biliary strictures include surgery, chronic pancreatitis, primary sclerosing cholangitis, and autoimmune cholangitis. Biliary leaks mainly occur after surgery and, rarely, abdominal trauma. These conditions may benefit from a nonsurgical approach in which endoscopic retrograde cholangiopancreatography (ERCP) plays a pivotal role in association with other minimally invasive approaches. This approach should be evaluated for any injury before deciding about the method for repair. ERCP, associated with peroral cholangioscopy, plays a growing role in characterizing undeterminate strictures, avoiding both unuseful major surgeries and palliative options that might compromise any further management. Copyright © 2015 Elsevier Inc. All rights reserved.

Metallic stents for management of malignant biliary obstruction

International Nuclear Information System (INIS)

Lee, Byung Hee; Do, Young Soo; Byun, Hong Sik; Kim, Kie Hwan; Chin, Soo Yil

1992-01-01

In patients with inoperable malignant biliary obstruction, percutaneous transhepatic biliary drainage (PTBD) has been the method of choice for palliative treatment. All patients except three had undergone PTBD, and the stents were placed 5-7 days after the initial drainage procedure. Three patients underwent stent placement on the same day of PTBD. External drainage catheter is converted to various types of tube endoprostheses with associated physiologic and psychologic benefits. Tube stents, however, have some problems such as migration, occlusion, and traumatic implantation procedure. We report our experiences and clinical results of percutaneous placement of metallic stents in 40 patients with malignant biliary obstruction

Metallic stents for management of malignant biliary obstruction

Energy Technology Data Exchange (ETDEWEB)

Lee, Byung Hee; Do, Young Soo; Byun, Hong Sik; Kim, Kie Hwan; Chin, Soo Yil [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

1992-11-15

In patients with inoperable malignant biliary obstruction, percutaneous transhepatic biliary drainage (PTBD) has been the method of choice for palliative treatment. All patients except three had undergone PTBD, and the stents were placed 5-7 days after the initial drainage procedure. Three patients underwent stent placement on the same day of PTBD. External drainage catheter is converted to various types of tube endoprostheses with associated physiologic and psychologic benefits. Tube stents, however, have some problems such as migration, occlusion, and traumatic implantation procedure. We report our experiences and clinical results of percutaneous placement of metallic stents in 40 patients with malignant biliary obstruction.

Palliation of Malignant Biliary and Duodenal Obstruction with Combined Metallic Stenting

International Nuclear Information System (INIS)

Akinci, Devrim; Akhan, Okan; Ozkan, Fuat; Ciftci, Turkmen; Ozkan, Orhan S.; Karcaaltincaba, Musturay; Ozmen, Mustafa N.

2007-01-01

Purpose. The purpose of this study is to evaluate the efficacy of palliation of malignant biliary and duodenal obstruction with combined metallic stenting under fluoroscopy guidance. Materials and Methods. A retrospective analysis of 9 patients (6 men and 3 women) who underwent biliary and duodenal stenting was performed. The mean age of patients was 61 years (range: 42-80 years). The causes of obstruction were pancreatic carcinoma in 7 patients, cholangiocellular carcinoma in one, and duodenal carcinoma in the other. Biliary and duodenal stents were placed simultaneously in 4 patients. In other 5 patients dudodenal stents were placed after biliary stenting when the duodenal obstruction symptoms have developed. In two patients duodenal stents were advanced via transgastric approach. Results. Technical success rate was 100 %. After percutaneous biliary drainage and stenting bilirubin levels decreased to normal levels in 6 patients and in remaining 3 patients mean reduction of 71% in bilirubin levels was achieved. Tumoral ingrowth occurred in one patient and percutaneous biliary restenting was performed 90 days after the initial procedure. Of the 9 patients, 6 patients were able to tolerate solid diet, whereas 2 patients could tolerate liquid diet and one patient did not show any improvement. Mean survival periods were 111 and 73 days after biliary and duodenal stenting, respectively. Conclusion. Combined biliary and duodenal stent placement which can be performed under fluoroscopic guidance without assistance of endoscopy is feasible and an effective method of palliation of malignant biliary and duodenal obstructions. If transoral and endoscopic approaches fail, percutaneous gastrostomy route allows duodenal stenting

The rendezvous technique involving insertion of a guidewire in a percutaneous transhepatic gallbladder drainage tube for biliary access in a case of difficult biliary cannulation.

Science.gov (United States)

Sunada, Fumiko; Morimoto, Naoki; Tsukui, Mamiko; Kurata, Hidekazu

2017-05-01

Endoscopic retrograde cholangiopancreatography (ERCP) is a diagnostic method and treatment approach for biliary diseases. However, biliary cannulation can be difficult in some cases. We performed ERCP in a 97-year-old woman with abdominal pain resulting from acute cholangitis caused by choledocholithiasis and observed difficult biliary cannulation. Eventually, the patient was successfully treated with the rendezvous technique. We could not cannulate the biliary duct during ERCP twice. Therefore, we placed a percutaneous transhepatic gallbladder drainage (PTGBD) tube without intrahepatic dilation. The rendezvous technique was performed using the PTGBD tube. The patient did not experience pancreatitis or perforation.

An Update on Endoscopic Management of Post-Liver Transplant Biliary Complications

Directory of Open Access Journals (Sweden)

Hyun Woo Lee

2017-09-01

Full Text Available Biliary complications are the most common post-liver transplant (LT complications with an incidence of 15%–45%. Furthermore, such complications are reported more frequently in patients who undergo a living-donor LT compared to a deceased-donor LT. Most post-LT biliary complications involve biliary strictures, bile leakage, and biliary stones, although many rarer events, such as hemobilia and foreign bodies, contribute to a long list of related conditions. Endoscopic treatment of post-LT biliary complications has evolved rapidly, with new and effective tools improving both outcomes and success rates; in fact, the latter now consistently reach up to 80%. In this regard, conventional endoscopic retrograde cholangiopancreatography remains the preferred initial treatment. However, percutaneous transhepatic cholangioscopy is now central to the management of endoscopy-resistant cases involving complex hilar or multiple strictures with associated stones. Many additional endoscopic tools and techniques—such as the rendezvous method, magnetic compression anastomosis , and peroral cholangioscopy—combined with modified biliary stents have significantly improved the success rate of endoscopic management. Here, we review the current status of endoscopic treatment of post-LT biliary complications and discuss conventional as well as the aforementioned new tools and techniques.

Preoperative biliary drainage for pancreatic cancer.

Science.gov (United States)

Van Heek, N T; Busch, O R; Van Gulik, T M; Gouma, D J

2014-04-01

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 salts, impaired liver function and altered nutritional status due to obstructive jaundice have been characterized as factors for development of complications after surgery. Whereas PBD was to yield beneficial effects in the experimental setting, conflicting results have been observed in clinical studies. The meta-analysis from relative older studies as well as more importantly a recent clinical trial showed that PBD should not be performed routinely. PBD for patients with a distal biliary obstruction is leading to more serious complications compared with early surgery. Arguments for PBD have shifted from a potential therapeutic benefit towards a logistic problem such as patients suffering from cholangitis and severe jaundice at admission or patients who need extra diagnostic tests, or delay in surgery due to a referral pattern or waiting list for surgery as well as candidates for neoadjuvant chemo(radio)therapy. If drainage is indicated in these patients it should be performed with a metal stent to reduce complications after the drainage procedure such as stent occlusion and cholangitis. Considering a change towards more neoadjuvant therapy regimes improvement of the quality of the biliary drainage concept is still important.

Gastric serosal tear due to congenital pyloric atresia: A rare anomaly ...

African Journals Online (AJOL)

Congenital pyloric atresia (CPA) is a very rare malformation with unknown aetiology. It has has numerous complications including gastric perforation, aspiration pneumonia. Gastric perforations in newborns occur by three mechanisms: trauma, ischaemia, or spontaneous. Here, we report a newborn with CPA presenting with ...

Biliary ascariasis: MR cholangiography findings in two cases

International Nuclear Information System (INIS)

Hwang, Cheol Mok; Kim, Tae Kyoung; Ha, Hyun Kwon; Kim, Pyo Nyun; Lee, Moon Gyu

2001-01-01

We describe the imaging features of two cases of biliary ascariasis. Ultrasonography and CT showed no specific abnormal findings, but MR cholangiography clearly demonstrated an intraductal linear filling defect that led to the correct diagnosis. MR cholangiography is thus a useful technique for the diagnosis of biliary ascariasis

Meconial peritonitis in a rare association of partial ileal apple-peel atresia with small abdominal wall defect

Directory of Open Access Journals (Sweden)

V. Insinga

2014-06-01

Full Text Available Intestinal atresia type III B (apple peel and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation. Paralytic ileus and infections are the main causes of morbidity and mortality at neonatal age. In our case, in spite of the mild phenotype, prognosis has been complicated by the onset of functional bowel obstruction, caused by chemical peritonitis resulting from contact with either amniotic fluid and meconium.

Contemporary Management of Acute Biliary Pancreatitis

Directory of Open Access Journals (Sweden)

Orhan Ozkan

2014-03-01

Full Text Available Acute biliary pancreatitis is one of the major causes of acute pancreatitis.Gallstones, biliary sludge and microlithiasis, especially in pancreatitis without detectable reason, can be the cause of acute pancreatitis. Acute biliary pancreatitis has many controversions in the literature, and its classification and guidelines are being updated very frequently. Atlanta classifications which determine the definitions and guidelines about acute pancreatitis were renewed and published in 2013. It has various clinical aspects, ranging from a mild form which is easily treated, to a severe form that causes complications leading to mortality. The pathogenesis of this disease has not been fully elucidated and several theories have been suggested. New scoring systems and laboratory methods such as proteomics have been suggested for both diagnosis and to predict disease severity, and research on these topics is still in progress. Novel therapeutic approaches with technological developments such as ERCP, ES, MRCP, and EUS are also suggested.

Balloon dilatation biopsy of the biliary stricture through the percutaneous transhepatic biliary drainage tract: Feasibility and diagnostic accuracy

Energy Technology Data Exchange (ETDEWEB)

Hong, Ji Hoon; Ryeom, Hun Kyu; Jang, Yun Jin; Kim, Gab Chul; Cho, Seung Hyun; Song, Jung Hup [Kyungpook National University Hospital, Daegu (Korea, Republic of)

2016-01-15

To evaluate the feasibility and diagnostic accuracy of the balloon dilatation biopsy for the biliary stricture through the percutaneous transhepatic biliary drainage (PTBD) tract. The study included 35 patients who underwent balloon dilatation biopsy for the biliary stricture through the PTBD tract. Balloon dilatation was done with a balloon catheter of 10-mm or 12-mm diameter. Soft tissue adherent to the retrieved balloon catheter and soft tissue components separated by gauze filtration of evacuated bile were sampled for histopathologic examination. The results were compared with the final diagnosis which was made by clinical and imaging follow-up for mean 989 days (n = 34) and surgery with histopathologic examination (n = 1). Procedure-related complications and diagnostic accuracy were assessed. Tissues suitable for histopathologic examination were obtained in 31 out of 35 patients (88.6%). In 3 patients, self-limiting hemobilia was noted. No major complication was noted. The sensitivity, specificity, diagnostic accuracy, positive and negative predictive values for diagnosis of malignant stricture were 70.0%, 100%, 90.3%, 100%, and 87.5%, respectively. Balloon dilatation biopsy of the biliary stricture through the PTBD tract is a feasible and accurate diagnostic method. It can be a safe alternative to the endoscopic retrograde cholangiography biopsy or forceps biopsy through the PTBD tract.

Interval biliary stent placement via percutaneous ultrasound guided cholecystostomy: another approach to palliative treatment in malignant biliary tract obstruction.

Science.gov (United States)

Harding, James; Mortimer, Alex; Kelly, Michael; Loveday, Eric

2010-12-01

Percutaneous cholecystostomy is a minimally invasive procedure for providing gallbladder decompression, often in critically ill patients. It can be used in malignant biliary obstruction following failed endoscopic retrograde cholangiopancreatography when the intrahepatic ducts are not dilated or when stent insertion is not possible via the bile ducts. In properly selected patients, percutaneous cholecystostomy in obstructive jaundice is a simple, safe, and rapid option for biliary decompression, thus avoiding the morbidity and mortality involved with percutaneous transhepatic biliary stenting. Subsequent use of a percutaneous cholecystostomy for definitive biliary stent placement is an attractive concept and leaves patients with no external drain. To the best of our knowledge, it has only been described on three previous occasions in the published literature, on each occasion forced by surgical or technical considerations. Traditionally, anatomic/technical considerations and the risk of bile leak have precluded such an approach, but improvements in catheter design and manufacture may now make it more feasible. We report a case of successful interval metal stent placement via percutaneous cholecystostomy which was preplanned and achieved excellent palliation for the patient. The pros and cons of the procedure and approach are discussed.

Interval Biliary Stent Placement Via Percutaneous Ultrasound Guided Cholecystostomy: Another Approach to Palliative Treatment in Malignant Biliary Tract Obstruction

International Nuclear Information System (INIS)

Harding, James; Mortimer, Alex; Kelly, Michael; Loveday, Eric

2010-01-01

Percutaneous cholecystostomy is a minimally invasive procedure for providing gallbladder decompression, often in critically ill patients. It can be used in malignant biliary obstruction following failed endoscopic retrograde cholangiopancreatography when the intrahepatic ducts are not dilated or when stent insertion is not possible via the bile ducts. In properly selected patients, percutaneous cholecystostomy in obstructive jaundice is a simple, safe, and rapid option for biliary decompression, thus avoiding the morbidity and mortality involved with percutaneous transhepatic biliary stenting. Subsequent use of a percutaneous cholecystostomy for definitive biliary stent placement is an attractive concept and leaves patients with no external drain. To the best of our knowledge, it has only been described on three previous occasions in the published literature, on each occasion forced by surgical or technical considerations. Traditionally, anatomic/technical considerations and the risk of bile leak have precluded such an approach, but improvements in catheter design and manufacture may now make it more feasible. We report a case of successful interval metal stent placement via percutaneous cholecystostomy which was preplanned and achieved excellent palliation for the patient. The pros and cons of the procedure and approach are discussed.

Rescue EUS-guided intrahepatic biliary drainage for malignant hilar biliary stricture after failed transpapillary re-intervention.

Science.gov (United States)

Minaga, Kosuke; Takenaka, Mamoru; Kitano, Masayuki; Chiba, Yasutaka; Imai, Hajime; Yamao, Kentaro; Kamata, Ken; Miyata, Takeshi; Omoto, Shunsuke; Sakurai, Toshiharu; Watanabe, Tomohiro; Nishida, Naoshi; Kudo, Masatoshi

2017-11-01

Treatment of unresectable malignant hilar biliary stricture (UMHBS) is challenging, especially after failure of repeated transpapillary endoscopic stenting. Endoscopic ultrasonography-guided intrahepatic biliary drainage (EUS-IBD) is a recent technique for intrahepatic biliary decompression, but indications for its use for complex hilar strictures have not been well studied. The aim of this study was to assess the feasibility and safety of EUS-IBD for UMHBS after failed transpapillary re-intervention. Retrospective analysis of all consecutive patients with UMHBS of Bismuth II grade or higher who, between December 2008 and May 2016, underwent EUS-IBD after failed repeated transpapillary interventions. The technical success, clinical success, and complication rates were evaluated. Factors associated with clinical ineffectiveness of EUS-IBD were explored. A total of 30 patients (19 women, median age 66 years [range 52-87]) underwent EUS-IBD for UMHBS during the study period. Hilar biliary stricture morphology was classified as Bismuth II, III, or IV in 5, 13, and 12 patients, respectively. The median number of preceding endoscopic interventions was 4 (range 2-14). EUS-IBD was required because the following procedures failed: duodenal scope insertion (n = 4), accessing the papilla after duodenal stent insertion (n = 5), or achieving desired intrahepatic biliary drainage (n = 21). Technical success with EUS-IBD was achieved in 29 of 30 patients (96.7%) and clinical success was attained in 22 of these 29 (75.9%). Mild peritonitis occurred in three of 30 (10%) and was managed conservatively. Stent dysfunction occurred in 23.3% (7/30). There was no procedure-related mortality. On multivariable analysis, Bismuth IV stricture predicted clinical ineffectiveness (odds ratio = 12.7, 95% CI 1.18-135.4, P = 0.035). EUS-IBD may be a feasible and effective rescue alternative with few major complications after failed transpapillary endoscopic re-intervention in patients

Biliary excretion of ciprofloxacin and piperacillin in the obstructed biliary tract

NARCIS (Netherlands)

van den Hazel, S. J.; de Vries, X. H.; Speelman, P.; Dankert, J.; Tytgat, G. N.; Huibregtse, K.; van Leeuwen, D. J.

1996-01-01

Biliary excretion of ciprofloxacin and piperacillin was determined in cholestatic patients who had undergone endoscopic cholangiography. The median concentration of ciprofloxacin (n = 9) was 2.36 micrograms/ml (range, 0.29 to 19.8 micrograms/ml) in bile compared with 1.66 micrograms/ml (range, 0.73

Urgent endoscopic retrograde cholangiopancreatography is not superior to early ERCP in acute biliary pancreatitis with biliary obstruction without cholangitis.

Science.gov (United States)

Lee, Hee Seung; Chung, Moon Jae; Park, Jeong Youp; Bang, Seungmin; Park, Seung Woo; Song, Si Young; Chung, Jae Bock

2018-01-01

Acute pancreatitis is a common diagnosis worldwide, with gallstone disease being the most prevalent cause (50%). The American College of Gastroenterology recommends urgent endoscopic retrograde cholangiopancreatography (ERCP) (within 24 h) for patients with biliary pancreatitis accompanied by cholangitis. Most international guidelines recommend that ERCP be performed within 72 h in patients with biliary pancreatitis and a bile duct obstruction without cholangitis, but the optimal timing for endoscopy is controversial. We investigated the optimal timing for ERCP in patients with biliary pancreatitis and a bile duct obstruction without cholangitis, and whether performing endoscopy within 24 h is superior to performing it after 24 h. We analyzed the clinical data of 505 patients with newly diagnosed acute pancreatitis, from January 1, 2005 to December 31, 2014. We divided the patients into two groups according to the timing of ERCP: pancreatitis and a bile duct obstruction without cholangitis. The mean age of the patients was 55 years (range: 26-90 years). Bile duct stones and biliary sludge were identified on endoscopy in 45 (61.6%) and 11 (15.0%) patients, respectively. The timing of ERCP within 72 h was not associated with ERCP-related complications (P = 0.113), and the total length of hospital stay was not different between urgent and early ERCP (5.9 vs. 5.7 days, P = 0.174). No significant differences were found in total length of hospitalization or procedural-related complications, in patients with biliary pancreatitis and a bile duct obstruction without cholangitis, according to the timing of ERCP (< 24 h vs. 24-72 h).

Percutaneous transhepatic biliary stenting in patients with intradiverticular papillae and biliary strictures caused by ampullary carcinoma: A case report

OpenAIRE

NIU, HONG-TAO; HUANG, QIANG; ZHAI, REN-YOU

2014-01-01

Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy is a well-established procedure for the treatment of bile duct strictures. However, the procedure is difficult to perform in patients with intradiverticular papillae or tumor infiltration of the major papilla. Percutaneous transhepatic biliary stenting (PTBS) is commonly used in the management of malignant biliary stricture. The current study reports two cases of PTBS performed to treat malignant obstructive jaundic...

Unusual case of pulmonary valve atresia | Steyn | SA Journal of ...

African Journals Online (AJOL)

We report on a 12-year-old boy with a rare form of pulmonary valve atresia with a ventricular septal defect (VSD) and anomalous origin of the left pulmonary artery arising from the aortic arch. He also has an absent right pulmonary artery, ... severe PHT of the left lung. South African Journal of Radiology Vol. 12 (1) 2008: pp.

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...

Biliary strictures and liver transplantation : clinical and biomedical aspects

NARCIS (Netherlands)

Sebib Korkmaz, Kerem

2014-01-01

The current thesis describes short and long term results of orthotopic liver transplantation (OLT) performed with livers from donation after brain death (DBD) and livers from donation after cardiac death (DCD) with an emphasis on biliary complications, especially nonanastomotic biliary strictures

Isolated unilateral cytomegalovirus retinitis: a rare long-term complication after pediatric liver transplantation.

Science.gov (United States)

Squires, James E; Sisk, Robert A; Balistreri, William F; Kohli, Rohit

2013-02-01

To highlight the rare yet devastating complication of CMV retinitis in a minimally immunosuppressed patient eight yr after liver transplantation for biliary atresia. A 22-yr-old female status-post deceased donor liver transplant at age 13 secondary to biliary atresia receiving single agent immunosuppression presented with acute, unilateral, profound decrease in visual acuity. The patient was diagnosed to have acute onset unilateral CMV retinitis. Retinal examination uncovered classical appearance of retinal whitening and retinal hemorrhages with extensive macular involvement. CMV retinitis can occur as a late complication following liver transplantation. Additionally, CMV retinal disease can occur in the absence of laboratory evidence of CMV infection and independent of additional clinical features suggesting CMV disease. Currently, there is no standard of care regarding screening for CMV retinitis, and thus, further research is needed to define the need for potential changes in current clinical practices and post-transplant screening protocols. © 2012 John Wiley & Sons A/S.

Three-dimensional assessment of the temporal bone and mandible deformations in patients with congenital aural atresia.

Science.gov (United States)

Fu, Yaoyao; Li, Chenlong; Dai, Peidong; Zhang, Tianyu

2017-10-01

To investigate the deformations of temporal bone and mandible combined with congenital aural atresia. A total of 158 patients with congenital aural atresia were included in the study. The raw CT data of the temporal bone was imported into MIMICS v 12 and threshold dissection, region growing and three-dimensional (3D) calculation were used to calculate 3D models. The 3D characteristics of the temporal bone and upper part of mandible were assessed. The tympanic part of the temporal bone was all undeveloped. Of all the patients included, 14 patients were found to have severe maxillofacial malformations. Among them, 2 cases have floating arch, 4 cases have interrupted arch, 5 cases have mandibular processes hypoplasia and 3 cases have interrupted arch combined with severe maxillary malformation. Ten of the 14 patients were suffered from dysplasia of the mastoid part of the temporal bone as well. Maxillofacial malformations may sometimes coexist with congenital aural atresia. Otolaryngologists should not neglect the coexisted maxillofacial malformations and give timely referral to maxillofacial surgeons. Copyright © 2017. Published by Elsevier B.V.

Dysphagia among adult patients who underwent surgery for esophageal atresia at birth.

Science.gov (United States)

Huynh Trudeau, Valérie; Maynard, Stéphanie; Terzic, Tatjana; Soucy, Geneviève; Bouin, Mickeal

2015-03-01

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. To determine the motor and anatomical causes of dysphagia. A total of 41 adults, followed at the Esophageal Atresia Clinic at Hôpital Saint-Luc (Montreal, Quebec), were approached to particpate in the present prospective study. Evaluation was completed using upper endoscopy, manometry and barium swallow for the participants who consented. The medical charts of respondents were systematically reviewed from the neonatal period to 18 years of age to assess medical and surgical history. All 41 patients followed at the clinic consented and were included in the study. Dysphagia was present in 73% of patients. Esophagogastroduodenoscopy was performed in 32 patients: hiatal hernia was present in 62% (n=20); esophageal diverticulum in 13% (n=4); macroscopic Barrett esophagus in 31% (n=10); and esophagitis in 19% (n=6). Histological esophagitis was present in 20% and intestinal metaplasia in 10%. There were no cases of dysplagia or adenocarcinoma. Esophageal manometry was performed on 56% of the patients (n=23). Manometry revealed hypomotility in 100% of patients and included an insufficient number of peristaltic waves in 96%, nonpropagating peristalsis in 78% and low-wave amplitude in 95%. Complete aperistalsis was present in 78%. The lower esophageal sphincter was abnormal in 12 (52%) patients, with incomplete relaxation the most common anomaly. Of the 41 patients, 29 (71%) consented to a barium swallow, which was abnormal in 13 (45%). The anomalies found were short esophageal dilation in 28%, delay in esophageal emptying in 14%, diverticula in 14% and stenosis in 7

Reverse gastric tube oesophageal substitution for staged repair of oesophageal atresia and tracheo-oesophageal fistula

Directory of Open Access Journals (Sweden)

Christopher Olusanjo Bode

2014-01-01

Full Text Available The management of oesophageal atresia and tracheo-oesophageal atresia (OATOF is very challenging. While in developed countries survival of patients with this condition has improved, the outcome in many developing countries has been poor. Primary repair through a thoracotomy (or video-assisted thoracoscopic surgery where available is the gold standard treatment of OATOF. However, in our setting where patients typically present late and with minimum support resources such as Neonatal Intensive Care Unit and total parenteral nutrition; staged repair may be the only hope of survival of these patients and this communication highlights the essential steps of this mode of treatment.

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...

Biliary intervention for malignant obstructive jaundice

International Nuclear Information System (INIS)

Naoi, Yutaka; Suzuki, Fuminao.

1990-01-01

Currently, diagnosis of obstructive jaundice has become easier with CT and USEG, and percutaneous transhepatic cholangialdrainage (PTCD) for obstructive jaundice has also become much safer using USEG control. We have performed PTCD in 277 cases, from December 1976 to May 1989 at Saitama Cancer Center with specially designed thin needle. And using a PTCD fistula, we have been attempted radiotherapy for 7 cases of the bile duct cancer using Remoto After Loading System (RALS), and hyperthermia for 5 cases of bile duct cancer using antenna of microwaves. From autopsy cases, we evaluated treated lesion pathologically, and we obtained the following results. Dosage of biliary RALS need up to 50 Gy at the point of 1 cm from 60 Co sourse. Biliary hyperthermia using microwave seem to be unsuccessful, and further improvement to the antenna of microwave were necessary. Furthermore, we have attempted biliary endoprosthesis 27 cases, for better quality of life to the patients with malignant obstructive jaundice. These methods seem to be effective in prolonging patient's lives, comparing of cases in which PTCD of an external fistula has been performed. (author)

Spontaneous perforation of a choledochal cyst, clues for diagnosis

African Journals Online (AJOL)

The diagnosis is often delayed because of its nonspecific presentation; hence, it is very rarely made preoperatively. The presenta- tion of CM perforation may be .... biliary atresia and. Table 1 Patient profile and clinical features at the time of presentation. Patients. Age/sex. Abdominal distension. Clinical jaundice. Acholic.

Obstructive Jaundice in Early Infancy | Pretorius | South African ...

African Journals Online (AJOL)

The aetiology of obstructive jaundice, as encountered in 113 Black and 17 White infants, as well as the clinical manifestations and prognosis, are discussed, together with a review of the literature. The commonest causes in Black infants were syphilitic hepatitis (28 patients), neonatal hepatitis (27), elltlahepiltic biliary atresia ...

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

Directory of Open Access Journals (Sweden)

Haafiz A

2011-04-01

Full Text Available Allah Haafiz1, Christian Farrington1, Joel Andres1, Saleem Islam21Hepatology and Liver Transplantation, Division of Pediatric Gastroenterology, Hepatology and Nutrition, 2Division of Pediatric Surgery, University of Florida College of Medicine, Gainesville, FL, USABackground: 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.Purpose: 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.Methods: 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.Results: All patients had end-stage liver disease and persistent cholestasis at the time of LT. Kendall tau (t 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 (t rank correlation coefficients were also evident between P

Pyloric atresia: A report of ten patients | Ksia | African Journal of ...

African Journals Online (AJOL)

Pyloric atresia (PA) is uncommon. It occurs in 1:100000 live births. Neonates usually present soon after birth with copious non-bilious vomiting. The treatment is surgical and its prognosis is poor, especially, when it is associated with epidermolysis bullosa (EB). The aim of this study was to evaluate the clinical presentation, ...

A newborn with duodenal atresia and a gastric perforation | Akçora ...

African Journals Online (AJOL)

... 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. Key words: Duodenal atresia, gastric perforation, newborn ...

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.

Acute iliofemoral venous thrombosis in patients with atresia of the inferior vena cava can be treated successfully with catheter-directed thrombolysis

DEFF Research Database (Denmark)

Broholm, Rikke; Jørgensen, Maja; Just, Sven

2011-01-01

To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT).......To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT)....

Gianturco metallic biliary stent in malignant biliary obstruction: results of follow-up in dead patients

Energy Technology Data Exchange (ETDEWEB)

Roh, Byung Suk; Kim, Chan Soo; Lee, Kyung Soo; Choi, See Sung; Won, Jong Jin; Kim, Haak Cheul; Chae, Kwon Mook [Wonkwang University School of Medicine, Iri (Korea, Republic of)

1994-04-15

In order to study the patency, restenosis, efficacy, and complication of the metallic stent in the course of treatment of malignant biliary obstruction, the results of follow up of the dead patients after stent insertion were reviewed. Self-expandable Gianturco metallic stent with 10-mm diameter was successfully inserted in 33 patients: 10 with Klatskin tumor, 7 with common bile duct cancer, 7 with gallbladder cancer, 5 with pancreatic cancer, 2 with recurred stomach cancer, one with periampullary cancer, one with hepatocellular carcinoma. The overall duration of survival and patency of the stents in 33 patients were 5.2 months(1-12 months) and 4.9 months(1-14 months), respectively. Restenosis of metallic stents was found in 9 cases(27%), after 6.1 months in average. Causes of stent occlusion were overgrowing of tumor in 5, overgrowing and ingrowing of tumor in 3, extraductal dislodgement in one case. Two cases of symptomatic cholangitis after stent placement were successfully treated with percutaneous cholecystostomy. Three cases of destruction and migration of metallic stents were found after 6 months. On the basis of our experience, insertion of Gianturco metallic biliary stent is an acceptable treatment method in the malignant biliary obstruction, especially for whom short term survival is expected.

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

Spontaneous perforation of bile duct, clinical presentation, laboratory work up, treatment and outcome

International Nuclear Information System (INIS)

Malik, H.S.; Cheema, H.A.; Fayyaz, Z.; Hashmi, M.A.

2016-01-01

Spontaneous perforation of bile duct (SPBD) is a rare and often misdiagnosed entity. Though rare, it is the second most common surgical cause of jaundice in infants, after biliary atresia. This study was planned to determine the clinical presentation, study different diagnostic modalities, treatment and outcome of patients with spontaneous perforation of bile duct. Methods: This descriptive case series, comprising 22 patients with spontaneous perforation of bile duct over a period of 24 months. Clinical presentation, biochemical abnormalities, imaging details, treatment options and outcome were studied. Results: Total 22 patients (12 Males and 10 Females) between ages of 1.5-36 months were studied. Associated anatomical defects included choledochal cyst in 7 (31.8%) while acquired biliary atresia in 1 (4.5%). Elevated liver enzymes (ALT and AST) were present in 16 patients (72.7%) and 5 (22.7%) had bilirubin above 3 mg/dl. Coagulopathy was seen in 8 (36.6%) patients. Abdominal USG showed presence of ascites in all 22 (100%), hydrocele in 2 (9.0%), inguinal hernia in 1 (4.5%), choledochal cyst in 7 (31.8%) and atretic gall bladder suggestive of acquired biliary atresia in one (4.5%) patient. HIDA scan was diagnostic in all 17 (77.27%) in which it was performed. MRCP was done in 3 (13.6%) patients. Mortality frequency was 3/22 (13.6%); one died of post-surgical sepsis second one was cirrhotic at time of presentation and didnot make It. Two were lost to follow up one which died at home while we lost contact with fourth patient. Conclusion: Spontaneous perforation of bile duct can present and should be suspected as an important cause of neonatal biliary ascites or peritonitis. Most patients can be managed with intravenous antibiotics, percutaneous drainage and t-tube insertion while patients with choledochal cysts required cholecystectomy with roux en y choledochjejunostomy. Timely recognition and intervention is associated with favourable outcome. (author)

2q24 deletion in a 9-month old girl with anal atresia, hearing impairment, and hypotonia.

Science.gov (United States)

Zhao, Peiwei; Mao, Bing; Cai, Xiaonan; Jiang, Jun; Liu, Zhisheng; Lin, Jun; He, Xuelian

2018-06-01

Deletion of 2q24.2 is a rare cytogenetic aberration in patients, exhibiting heterogeneous clinical features, and common phenotypes included developmental delay, intellectual disability, hypotonia, and mild dysmorphic features. Hearing impairment and anal atresia are rarely described. Here we described a 9-month-old female patient with hypotonia in all four limbs, developmental delay, and intellectual disability. In addition, congenital anal atresia was diagnosed and treated after birth, and hearing impairment was found in right ear. Single nucleotide polymorphisms (SNP) array detected a 5.2 Mb deletion on 2q24.2q24.3, including 19 genes (ITGB6; TBR1; SLC4A10; KCNH7 SCN3A; SCN2A et al.). Among these genes, it is affirmative that TBR1 is a causative gene for intellectual disability; however, the pathogenic genes of other phenotypes remain unclear. We briefly review the knowledge of genes likely involved in these clinical features, including hearing impairment, anal atresia, and developmental delay. Copyright © 2018 Elsevier B.V. All rights reserved.

Annals of Pediatric Surgery - Vol 12, No 3 (2016)

African Journals Online (AJOL)

Biliary atresia with hyaline cartilage at the porta hepatis: a novel finding of undetermined significance: a case report · EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. Rakesh Kumar Gupta, Vineeta V. Batra, Mamta Sengar, Puja Sakhuja, Sheetal N. Rooge, 122-125 ...

Spontaneous bile duct perforation in an infant, managed with simple ...

African Journals Online (AJOL)

Spontaneous bile duct perforation is a very rare but important cause of surgical jaundice in pediatric patients and one of the most common causes of surgical jaundice during infancy after biliary atresia. Preoperative diagnosis may not be possible in most of the cases. The exact cause of the perforation remains unclear.

Author Details

African Journals Online (AJOL)

Left hepatic lobectomy in a long-term biliary atresia survivor. Abstract · Vol 10, No 1 (2013) - Articles Giant omental lipoblastoma and CD56 expression. Abstract · Vol 10, No 3 (2013) - Articles Video-assisted thoracoscopic double lobectomy for bronchiectasis: A case report and literature review. Abstract. ISSN: 0189-6725.

Definitive management of isolated esophageal atresia: Experience at NICH Karachi

Directory of Open Access Journals (Sweden)

Jan Iftikhar

2006-01-01

Full Text Available Background: Definitive treatment of isolated esophageal atresia (IEA is still controversial. A study was conducted to review cases of IEA in our department with a view to evaluate the long term results of definitive surgery in these patients. Materials and Methods: Nine consecutive patients with IEA were included in the study. All babies initially underwent cervical esophagostomy and feeding gastrostomy. One baby also had anorectal atresia and needed a colostomy. In 6 babies, end esophagostomy was performed while in 3 babies, lateral esophagostomy was performed. Of the 6 babies with end esophagostomy, two underwent jejunal Interpositioning, two had serial extra-thoracic lengthening and two reverse gastric tube interpositiong. Three babies with lateral esophagostomy were planned for delayed primary repair. Results: Both babies with jejunal interpositiong initially did well; serious gastro-esophageal reflux occurred in one baby who needed antireflux surgery. At 6 years follow-up both children had redundant Jejunum. Recurrent Respiratory tract infection remained a problem in one child while other is doing well. Of the two babies with extrathoracic lengthening it was possible to perform end to end anastomosis in one baby but in the other extrathoracic lengthening did not work and gastric transpositioning was performed. Of the two babies with reverse gastric tube interpositioning one developed esophageal stenosis at the site of anastomosis and is on serial dilatations. Other died due to anastomotic leak and mediastinitis. In all the three babies with lateral esophagostomy spontaneous lengthening of esophagus was noted. End to end anastomosis was possible in two of these babies who are doing well. One is waiting definitive surgery. Number of complications, hospital stay and cost of surgery was least in patients with lateral esophagostomy. Conclusion: Various methods of definitive treatment have been proposed for isolated esophageal atresia but each

Decisional Conflict in Parents Considering Bone-Anchored Hearing Devices in Children With Unilateral Aural Atresia.

Science.gov (United States)

Graham, M Elise; Haworth, Rebecca; Chorney, Jill; Bance, Manohar; Hong, Paul

2015-12-01

The benefits of bone-anchored hearing devices (BAHD) in children with unilateral aural atresia are controversial. We sought to determine whether there is parental decisional conflict surrounding elective placement of BAHD for this indication. Caregivers of pediatric patients with unilateral aural atresia and normal contralateral ear undergoing percutaneous BAHD consultation were enrolled. All consultations were carried out by one pediatric otolaryngologist in a consistent manner. After consultation, the participants completed a demographics form and the Decisional Conflict Scale (DCS) questionnaire. Twenty-three caregivers of 15 male (65.2%) and 8 female (34.8%) children (mean age 5.65 years) participated. The overall median DCS score was 15.63 (standard error = 4.21). Significant decisional conflict (DCS score ≥ 25) was found in 10 participants (43.5%). The median DCS score in the group choosing surgery was 5.47, and it was 23.44 in those who did not choose surgery (Mann-Whitney U = 39, Z = -1.391, P = .164). The median DCS score for mothers and fathers was 25 and 3.91, respectively. Many parents experienced significant decisional conflict when considering percutaneous BAHD surgery in children with unilateral aural atresia in our study population. Future research should explore the impact of decisional conflict on health outcomes. © The Author(s) 2015.

High-dose-rate afterloading intracavitary irradiation and expandable metallic biliary endoprosthesis for malignant biliary obstruction

Energy Technology Data Exchange (ETDEWEB)

Yoshimura, Hitoshi; Ohishi, Hajime; Yoshioka, Tetsuya [Nara Medical Univ., Kashihara (Japan); and others

1989-04-01

A double lumen catheter was developed as an applicator for the remote afterloading system (RALS) of {sup 60}Co for the intracavitary irradiation of an obstructed common bile duct due to gallbladder cancer in 1 case and by cholangiocarcinoma in 7 cases. This was followed by the biliary endoprosthesis with expandable metallic stents to maintain patency. The mean survival period after treatment was not long (14 weeks). However, removal of the external drainage tube was possible in 7 of the 8 cases, and none of the 8 cases showed dislodgement or deformity of the stent, or obstruction of the bile duct in the stent-inserted area. This combination effectively provided palliation, and has considerable potential for malignant biliary obstruction. (author).

Surgical Management of Benign Biliary Stricture in Chronic Pancreatitis: A Single-Center Experience.

Science.gov (United States)

Ray, Sukanta; Ghatak, Supriyo; Das, Khaunish; Dasgupta, Jayanta; Ray, Sujay; Khamrui, Sujan; Sonar, Pankaj Kumar; Das, Somak

2015-12-01

Biliary stricture in chronic pancreatitis (CP) is not uncommon. Previously, all cases were managed by surgery. Nowadays, three important modes of treatment in these patients are observation, endoscopic therapy, and surgery. In the modern era, surgery is recommended only in a subset of patients who develop biliary symptoms or those who have asymptomatic biliary stricture and require surgery for intractable abdominal pain. We want to report on our experience regarding surgical management of CP-induced benign biliary stricture. Over a period of 5 years, we have managed 340 cases of CP at our institution. Bile duct stricture was found in 62 patients. But, surgical intervention was required in 44 patients, and the remaining 18 patients were managed conservatively. Demographic data, operative procedures, postoperative complications, and follow-up parameters of these patients were collected from our prospective database. A total 44 patients were operated for biliary obstruction in the background of CP. Three patients were excluded, so the final analysis was based on 41 patients. The indication for surgery was symptomatic biliary stricture in 27 patients and asymptomatic biliary stricture with intractable abdominal pain in 14 patients. The most commonly performed operation was Frey's procedure. There was no inhospital mortality. Thirty-five patients were well at a mean follow-up of 24.4 months (range 3 to 54 months). Surgery is still the best option for CP-induced benign biliary stricture, and Frey's procedure is a versatile operation unless you suspect malignancy as the cause of biliary obstruction.

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.

Diagnosis of liver, biliary tract and gastrointestine

International Nuclear Information System (INIS)

Aburano, Tamio

1981-01-01

The role of RI imaging in the diagnosis of lesions of the liver, biliary tracts and gastrointestinal tracts are reviewed, and representative cases are shown. Liver scintigraphy was of value for the diagnosis of lesions limitted to the liver such as primary and metastatic liver cancer and inflammatory liver diseases. However, RI methods were less useful in the diagnosis of lesions of the biliary tracts and stomach. RI scintigraphy was more sensitive than angiography in the detection of Meckel's deverticulum, Ballet's esophagus, and gastrointestinal hemorrhage. (Tsunoda, M.)

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.

Prospective observational multicenter study to define a diagnostic algorithm for biliary candidiasis.

Science.gov (United States)

Lenz, Philipp; Eckelskemper, Franziska; Erichsen, Thomas; Lankisch, Tim; Dechêne, Alexander; Lubritz, Gabriele; Lenze, Frank; Beyna, Torsten; Ullerich, Hansjörg; Schmedt, Andre; Domagk, Dirk

2014-09-14

To develop an algorithm to improve the diagnosis and treatment of patients with biliary candidiasis. We performed a prospective study of 127 patients who underwent endoscopic retrograde cholangiopancreatography, for various biliary disorders, at 3 tertiary referral centers in Germany from July 2011 through July 2012 (ClinicalTrials.gov: NCT01109550). Bile, buccal, and stool samples were collected. When indicated, endoscopic transpapillary bile duct biopsies were performed to clarify the etiology of bile duct strictures and to prove invasive fungal infections. Candida species were detected in 38 of the 127 bile samples (29.9%). By multivariate analysis patients' age and previous endoscopic sphincterotomy were independent risk factors for biliary candidiasis (P 7 d) (P = 0.089) tend to be at risk for biliary candidiasis. One patient was negative in mycological culture of bile fluid but invasive biliary candidiasis was diagnosed histologically. Of Candida subspecies detected, 36.7% were azole-resistant, such as C glabrata. Eight patients received anti-mycotic therapy, based on our algorithm. Of these, 3 had cancer with biliary tract involvement, 2 had secondary sclerosing cholangitis, 1 had retroperitoneal fibrosis, and 5 had septicemia. In all patients contamination was ruled out by smears of the endoscope channel. Gastroenterologists should be aware of frequent candida colonization in patients with cholangitis and biliary disorders. Our suggested algorithm facilitates the further clinical management.

Prospective observational multicenter study to define a diagnostic algorithm for biliary candidiasis

Science.gov (United States)

Lenz, Philipp; Eckelskemper, Franziska; Erichsen, Thomas; Lankisch, Tim; Dechêne, Alexander; Lubritz, Gabriele; Lenze, Frank; Beyna, Torsten; Ullerich, Hansjörg; Schmedt, Andre; Domagk, Dirk

2014-01-01

AIM: To develop an algorithm to improve the diagnosis and treatment of patients with biliary candidiasis. METHODS: We performed a prospective study of 127 patients who underwent endoscopic retrograde cholangiopancreatography, for various biliary disorders, at 3 tertiary referral centers in Germany from July 2011 through July 2012 (ClinicalTrials.gov: NCT01109550). Bile, buccal, and stool samples were collected. When indicated, endoscopic transpapillary bile duct biopsies were performed to clarify the etiology of bile duct strictures and to prove invasive fungal infections. RESULTS: Candida species were detected in 38 of the 127 bile samples (29.9%). By multivariate analysis patients’ age and previous endoscopic sphincterotomy were independent risk factors for biliary candidiasis (P 7 d) (P = 0.089) tend to be at risk for biliary candidiasis. One patient was negative in mycological culture of bile fluid but invasive biliary candidiasis was diagnosed histologically. Of Candida subspecies detected, 36.7% were azole-resistant, such as C glabrata. Eight patients received anti-mycotic therapy, based on our algorithm. Of these, 3 had cancer with biliary tract involvement, 2 had secondary sclerosing cholangitis, 1 had retroperitoneal fibrosis, and 5 had septicemia. In all patients contamination was ruled out by smears of the endoscope channel. CONCLUSION: Gastroenterologists should be aware of frequent candida colonization in patients with cholangitis and biliary disorders. Our suggested algorithm facilitates the further clinical management. PMID:25232260

[Surgical treatment of chronic pancreatitis complicated by biliary hypertension].

Science.gov (United States)

Pylypchuk, V I

2015-01-01

The results of 29 patients treatment, suffering chronic pancreatitis, complicated by biliary hypertension, in whom operative interventions in Department of Surgery of Regional Ivano-Frankivsk clinical hospital in 2009 - 2014 yrs, were analyzed. The drainage, resectional and combined interventions were performed. Direct intervention on pancreatic gland was not applied in 5 (17.2%) patients. Operation to Frey was performed in 7 (24.1%) patients, in 4--it was added by choledochojejunoanastomosis formation, longitudinal pancreatojejunostomy--in 13 (44.8%). In 4 (14.8%) patients while functional disorders of adjacent organs present a pancreaticoduodenal resection to Whipple was done. For biliary hypertension diagnosis (including the occult one) the method of intraoperative pressure measurement in common biliary duct (CBD) was proposed. The operation was added by biliodigestive anastomosis formation, using choledochoenterostomy to Roux method if while operations to Frey or Beger after intervention on pancreatic head with the intrapancreatic CBD freeing the intraductal pressure witnessed the biliary hypertension presence. In all the patients good and satisfactory results of operative treatment were noted.

Safety and Efficacy of Percutaneous Biliary Covered Stent Placement in Patients with Malignant Biliary Hilar Obstruction; Correlation with Liver Function

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Hyun, Hyeran; Choi, Sun Young, E-mail: medmath@hanmail.net [School of Medicine Ewha Womans University, Department of Radiology and Medical Research Institute (Korea, Republic of); Kim, Kyung Ah [St. Vincent’s Hospital, The Catholic University of Korea, Department of Radiology (Korea, Republic of); Ko, Soo Bin [College of Arts and Science Case Western Reserve University, Department of Biology (United States)

2016-09-15

PurposeTo estimate the safety and efficacy of percutaneous ePTFE-covered biliary stent placement and the relationship between underlying liver function and stent patency in patients with malignant hilar obstruction.Materials and MethodsFrom March 2012 to June 2015, 41 patients [22 females, 19 males; mean age 69.8 (range 34–94) years] with malignant biliary obstruction underwent percutaneous biliary stent placement (31 patients with unilateral, 10 patients with bilateral side-by-side). Cumulative patient survival and stent patency rate curves were derived using the Kaplan–Meier method. A Cox model was used to explore the relationship between liver function and patient survival, and also biliary stent patency. Pearson correlation coefficient was used to analyze the relationship between patient survival and stent patency.ResultsTechnical success rate was 100 % and clinical success rate was 95 %. During follow-up, four complications occurred (two bilomas and two cases of acute cholecystitis) and were treated successfully with percutaneous drainage. No other complication occurred. Mean serum bilirubin level was 11.34 ± 7.35 mg/dL before drainage and 5.00 ± 4.83 mg/dL 2 weeks after stent placement. The median patent survival duration was 147 days (95 % CI, 69.6–224.4 days). The median stent patency duration was 101 days (95 % CI, 70.0–132.0 days). The cumulative stent patency rates at 1, 3, 6, and 12 months were 97, 57.6, 30.3, and 17.0 %, respectively. Child–Pugh score was correlated significantly with patient survival (P = 0.011) and stent patency (P = 0.007). MELD score was correlated significantly with stent patency (P = 0.044). There was a correlation between patient survival and stent patency (r = 0.778, P < 0.001).ConclusionPercutaneous placement of ePTFE-covered biliary stent was a safe and an effective method for malignant biliary obstruction. Underlying liver function seemed to be one of the important factors affecting

Safety and Efficacy of Percutaneous Biliary Covered Stent Placement in Patients with Malignant Biliary Hilar Obstruction; Correlation with Liver Function

International Nuclear Information System (INIS)

Hyun, Hyeran; Choi, Sun Young; Kim, Kyung Ah; Ko, Soo Bin

2016-01-01

PurposeTo estimate the safety and efficacy of percutaneous ePTFE-covered biliary stent placement and the relationship between underlying liver function and stent patency in patients with malignant hilar obstruction.Materials and MethodsFrom March 2012 to June 2015, 41 patients [22 females, 19 males; mean age 69.8 (range 34–94) years] with malignant biliary obstruction underwent percutaneous biliary stent placement (31 patients with unilateral, 10 patients with bilateral side-by-side). Cumulative patient survival and stent patency rate curves were derived using the Kaplan–Meier method. A Cox model was used to explore the relationship between liver function and patient survival, and also biliary stent patency. Pearson correlation coefficient was used to analyze the relationship between patient survival and stent patency.ResultsTechnical success rate was 100 % and clinical success rate was 95 %. During follow-up, four complications occurred (two bilomas and two cases of acute cholecystitis) and were treated successfully with percutaneous drainage. No other complication occurred. Mean serum bilirubin level was 11.34 ± 7.35 mg/dL before drainage and 5.00 ± 4.83 mg/dL 2 weeks after stent placement. The median patent survival duration was 147 days (95 % CI, 69.6–224.4 days). The median stent patency duration was 101 days (95 % CI, 70.0–132.0 days). The cumulative stent patency rates at 1, 3, 6, and 12 months were 97, 57.6, 30.3, and 17.0 %, respectively. Child–Pugh score was correlated significantly with patient survival (P = 0.011) and stent patency (P = 0.007). MELD score was correlated significantly with stent patency (P = 0.044). There was a correlation between patient survival and stent patency (r = 0.778, P < 0.001).ConclusionPercutaneous placement of ePTFE-covered biliary stent was a safe and an effective method for malignant biliary obstruction. Underlying liver function seemed to be one of the important factors affecting

Risk factors and outcome in patients with primary sclerosing cholangitis with persistent biliary candidiasis.

Science.gov (United States)

Rupp, Christian; Bode, Konrad Alexander; Chahoud, Fadi; Wannhoff, Andreas; Friedrich, Kilian; Weiss, Karl-Heinz; Sauer, Peter; Stremmel, Wolfgang; Gotthardt, Daniel Nils

2014-10-23

Candidiasis is commonly observed in patients with primary sclerosing cholangitis (PSC), but the clinical risk factors associated with its presence have not been fully investigated. In this study, we aimed to analyse the incidence, risk factors, and transplantation-free survival in primary sclerosing cholangitis (PSC) patients with persistent biliary candidiasis. We retrospectively analysed patients diagnosed with PSC who were admitted to our department during 2002 to 2012. One-hundred fifty patients whose bile cultures were tested for fungal species were selected, and their clinical and laboratory parameters were investigated. The results of endoscopic retrograde cholangiography (ERC) and bile cultures were analysed using chart reviews. The cases of biliary candidiasis were sub-classified as transient or persistent. Thirty out of 150 (20.0%) patients had biliary candidiasis. Although all patients demonstrated comparable baseline characteristics, those with biliary candidiasis showed significantly reduced transplantation-free survival (p candidiasis. A subgroup analysis showed reduced survival with a greater necessity for orthotopic liver transplantation (OLT) only in patients with persistence of Candida (p = 0.007). The survival in the patients with transient biliary candidiasis was comparable to that in candidiasis-free patients. In a multivariate regression analysis that included Mayo risk score (MRS), sex, age, dominant stenosis, inflammatory bowel disease, autoimmune hepatitis overlap syndrome, and number of times ERC was performed, biliary candidiasis was an independent risk factor for reduced survival (p = 0.008). Risk factors associated with acquisition of biliary candidiasis were age at PSC diagnosis and number of ERCs. The persistence of biliary candidiasis is associated with markedly reduced transplantation-free survival in PSC patients. By contrast, actuarial survival in patients with transient biliary candidiasis approaches that for patients without any

Management of Acquired Atresia of the External Auditory Canal.

Science.gov (United States)

Bajin, Münir Demir; Yılmaz, Taner; Günaydın, Rıza Önder; Kuşçu, Oğuz; Sözen, Tevfik; Jafarov, Shamkal

2015-08-01

The aim was to evaluate surgical techniques and their relationship to postoperative success rate and hearing outcomes in acquired atresia of the external auditory canal. In this article, 24 patients with acquired atresia of the external auditory canal were retrospectively evaluated regarding their canal status, hearing, and postoperative success. Acquired stenosis occurs more commonly in males with a male: female ratio of 2-3:1; it seems to be a disorder affecting young adults. Previous ear surgery (13 patients, 54.2%) and external ear trauma (11 patients, 45.8%) were the main etiological factors of acquired ear canal stenosis. Mastoidectomy (12/13) and traffic accidents (8/11) comprise the majority of these etiological factors. Endaural incision is performed in 79.2% and postauricular incision for 20.8% of cases during the operation. As types of surgical approach, transcanal (70.8%), transmastoid (20.8%), and combined (8.4%) approaches are chosen. The atretic plate is generally located at the bony-cartilaginous junction (37.5%) and in the cartilaginous canal (33.3%); the bony canal is involved in a few cases only. Preserved healthy canal skin, split- or full-thickness skin grafts, or pre- or postauricular skin flaps are used to line the ear canal, but preserved healthy canal skin is preferred. The results of surgery are generally satisfactory, and complications are few if surgical principles are followed.

Percutaneous transhepatic biliary stenting in patients with intradiverticular papillae and biliary strictures caused by ampullary carcinoma: A case report.

Science.gov (United States)

Niu, Hong-Tao; Huang, Qiang; Zhai, Ren-You

2014-04-01

Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy is a well-established procedure for the treatment of bile duct strictures. However, the procedure is difficult to perform in patients with intradiverticular papillae or tumor infiltration of the major papilla. Percutaneous transhepatic biliary stenting (PTBS) is commonly used in the management of malignant biliary stricture. The current study reports two cases of PTBS performed to treat malignant obstructive jaundice caused by ampullary carcinoma complicated with intradiverticular papillae. PTBS is potentially a safe technique for this relatively rare condition.

Risk of primary biliary cirrhosis in patients with coeliac disease

DEFF Research Database (Denmark)

Sørensen, Henrik Toft; Thulstrup, Ane Marie; Blomqvist, P

1999-01-01

BACKGROUND: Several case reports, but only a few studies, have examined the coexistence of coeliac disease and primary biliary cirrhosis. AIM: To estimate the risk of primary biliary cirrhosis in two national cohorts of patients with coeliac disease in Denmark and Sweden. METHODS: Through record...... linkage all Danish patients hospitalised with coeliac disease were followed for possible occurrence of primary biliary cirrhosis from 1 January 1977 until 31 December 1992. All patients hospitalised with coeliac disease in Sweden from 1987 to 1996 were also followed in a separate analysis. RESULTS......: A total of 896 patients with coeliac disease were identified in Denmark with a median follow up period of 9.1 years for a total of 8040 person-years at risk. Two cases of primary biliary cirrhosis were observed where 0.07 were expected, giving a standardised incidence ratio of 27.6 (95% confidence...

MRI in children following surgery for anal and rectal atresia

International Nuclear Information System (INIS)

Krahe, T.; Herold, A.; Doelken, W.; Hoecht, B.; Wuerzburg Univ.

1989-01-01

MRI of the pelvis was performed in 17 children following surgical correction of anal and rectal atresias and in five children without ano-rectal malformations. A muscle score was used to characterize the muscles of the pelvic floor and their relationship to the rectum. There was close agreement between the MRI muscle score and clinical continence. MRI provided additional information that should improve continence following conservative and surgical treatment. (orig.) [de

Percutaneous transhepatic biliary drainage: analysis of 175 cases

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Suh, Kyung Jin; Lee, Sang Kwon; Kim, Tae Hun; Kim, Yong Joo; Kang, Duk Sik [College of Medicine, Kyungpook National Univ., Daegu (Korea, Republic of)

1990-10-15

Percutaneous transhepatic biliary drainage is a safe, effective and palliative means of treatment in biliary obstruction, especially in cases with malignant obstruction which are inoperable. 175 cases of transhepatic biliary drainage were performed on 119 patients with biliary obstruction from January 1985 to June 1989 at Kyung-pook National University Hospital. The causes of obstructive jaundice were 110 malignant diseases and 9 benign diseases. The most common indication for drainage was palliative intervention of obstruction secondary to malignant tumor in 89 cases. 86 cases of external drainage were performed including 3 cases of left duct approach, 29 cases of external-internal drainage and 60 cases of endoprosthesis. In external and external-internal drainages, immediate major complications (11.9%) occurred, including not restricted to, but sepsis, bile peritonitis and hemobilia. Delayed major complications (42.9%) were mainly catheter related. The delayed major complication of endoprosthesis resulted from obstruction of the internal stent. The mean time period to reobstruction of the internal stent was about 12 weeks. To improve management status, regular follow-up is required, as is education of both patients and their families as to when immediate clinical attention is mandated. Close communication amongst the varying medical specialities involved will be necessary to provide optional treatment for each patient.

Percutaneous transhepatic biliary drainage: analysis of 175 cases

International Nuclear Information System (INIS)

Suh, Kyung Jin; Lee, Sang Kwon; Kim, Tae Hun; Kim, Yong Joo; Kang, Duk Sik

1990-01-01

Percutaneous transhepatic biliary drainage is a safe, effective and palliative means of treatment in biliary obstruction, especially in cases with malignant obstruction which are inoperable. 175 cases of transhepatic biliary drainage were performed on 119 patients with biliary obstruction from January 1985 to June 1989 at Kyung-pook National University Hospital. The causes of obstructive jaundice were 110 malignant diseases and 9 benign diseases. The most common indication for drainage was palliative intervention of obstruction secondary to malignant tumor in 89 cases. 86 cases of external drainage were performed including 3 cases of left duct approach, 29 cases of external-internal drainage and 60 cases of endoprosthesis. In external and external-internal drainages, immediate major complications (11.9%) occurred, including not restricted to, but sepsis, bile peritonitis and hemobilia. Delayed major complications (42.9%) were mainly catheter related. The delayed major complication of endoprosthesis resulted from obstruction of the internal stent. The mean time period to reobstruction of the internal stent was about 12 weeks. To improve management status, regular follow-up is required, as is education of both patients and their families as to when immediate clinical attention is mandated. Close communication amongst the varying medical specialities involved will be necessary to provide optional treatment for each patient

Benign biliary strictures refractory to standard bilioplasty treated using polydoxanone biodegradable biliary stents: retrospective multicentric data analysis on 107 patients.

Science.gov (United States)

Mauri, Giovanni; Michelozzi, Caterina; Melchiorre, Fabio; Poretti, Dario; Pedicini, Vittorio; Salvetti, Monica; Criado, Eva; Falcò Fages, Joan; De Gregorio, Miguel Ángel; Laborda, Alicia; Sonfienza, Luca Maria; Cornalba, Gianpaolo; Monfardini, Lorenzo; Panek, Jiri; Andrasina, Tomas; Gimenez, Mariano

2016-11-01

To assess mid-term outcome of biodegradable biliary stents (BBSs) to treat benign biliary strictures refractory to standard bilioplasty. Institutional review board approval was obtained and patient consent was waived. 107 patients (61 males, 46 females, mean age 59 ± 16 years), were treated. Technical success and complications were recorded. Ninety-seven patients (55 males, 42 females, aged 57 ± 17 years) were considered for follow-up analysis (mean follow-up 23 ± 12 months). Fisher's exact test and Mann-Whitney U tests were used and a Kaplan-Meier curve was calculated. The procedure was always feasible. In 2/107 cases (2 %), stent migration occurred (technical success 98 %). 4/107 patients (4 %) experienced mild haemobilia. No major complications occurred. In 19/97 patients (18 %), stricture recurrence occurred. In this group, higher rate of subsequent cholangitis (84.2 % vs. 12.8 %, p = 0.001) and biliary stones (26.3 % vs. 2.5 %, p = 0.003) was noted. Estimated mean time to stricture recurrence was 38 months (95 % C.I 34-42 months). Estimated stricture recurrence rate at 1, 2, and 3 years was respectively 7.2 %, 26.4 %, and 29.4 %. Percutaneous placement of a BBS is a feasible and safe strategy to treat benign biliary strictures refractory to standard bilioplasty, with promising results in the mid-term period. • Percutaneous placement of a BBS is 100 % feasible. • The procedure appears free from major complications, with few minor complications. • BBSs offer promising results in the mid-term period. • With a BBS, external catheter/drainage can be removed early. • BBSs represent a new option in treating benign biliary stenosis.

Author Details

African Journals Online (AJOL)

Sengar, Mamta. Vol 12, No 3 (2016) - Articles Biliary atresia with hyaline cartilage at the porta hepatis: a novel finding of undetermined significance: a case report. Abstract PDF. ISSN: 1687-4137. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL ...

A comparative evaluation of early stent occlusion among biliary conventional versus wing stents.

Science.gov (United States)

Khashab, Mouen A; Hutfless, Susan; Kim, Katherine; Lennon, Anne Marie; Canto, Marcia I; Jagannath, Sanjay B; Okolo, Patrick I; Shin, Eun Ji; Singh, Vikesh K

2012-06-01

Conventional plastic stents with a lumen typically have limited patency. The lumenless wing stent was engineered to overcome this problem. The objective of this study was to compare the incidence of early stent occlusion (symptomatic occlusion/cholangitis necessitating re-insertion within 90 days) for wing stents and conventional plastic stents. Patients with biliary pathology treated with plastic biliary stenting during the period 2003-2009 comprised the study cohort. Patients who had at least one biliary wing stent placed comprised the wing stent group, whereas patients who underwent only conventional stent plastic placement comprised the conventional stent group. Patients were stratified by indication: benign biliary strictures (group 1), malignant biliary strictures (group 2), or benign biliary non-stricture pathology (group 3). The association of stent type with the occurrence of primary outcome by indication was analyzed by use of multivariable logistic regression. Three-hundred and forty-six patients underwent 612 ERCP procedures with placement of plastic biliary stent(s). On multivariate analysis, early stent occlusion did not differ between the wing and conventional groups in groups 1, 2, and 3. Among patients who achieved primary outcome in group 2, significantly fewer patients in the wing group had cholangitis (6.7% vs. 39.1%, P = 0.03). Among patients who achieved primary outcome in group 3, significantly fewer patients in the wing group had cholangitis (10% vs. 50%, P = 0.03). Early stent occlusion was similar for wing stents and conventional plastic stents. Wing stents, however, were associated with a lower incidence of cholangitis in patients with malignant biliary obstruction and benign non-stricturing biliary pathology.

Depression of biliary glutathione excretion by chronic ethanol feeding in the rat

International Nuclear Information System (INIS)

Vendemiale, G.; Jayatilleke, E.; Shaw, S.; Lieber, C.S.

1984-01-01

The effects of chronic alcohol feeding on biliary glutathione excretion were studied in rats pair fed diets containing either ethanol (36% of total energy) or isocaloric carbohydrate for 4-6 weeks. An exteriorized biliary-duodenal fistula was established and total glutathione (GSH) and oxidized glutathione (GSSG) were measured. A significant decrease was observed in rats fed alcohol chronically compared to their pair fed controls in the biliary excretion of GSH (55.7 +/- 37.0 vs 243.1 +/- 29.0 μg/ml bile, p 35 -L-methionine incorporation into hepatic and biliary GSH was unchanged or even increased after chronic ethanol feeding. 22 references, 4 figures

Hepcidin is an antibacterial, stress-inducible peptide of the biliary system.

Directory of Open Access Journals (Sweden)

Pavel Strnad

Full Text Available BACKGROUND/AIMS: Hepcidin (gene name HAMP, an IL-6-inducible acute phase peptide with antimicrobial properties, is the key negative regulator of iron metabolism. Liver is the primary source of HAMP synthesis, but it is also produced by other tissues such as kidney or heart and is found in body fluids such as urine or cerebrospinal fluid. While the role of hepcidin in biliary system is unknown, a recent study demonstrated that conditional gp130-knockout mice display diminished hepcidin levels and increased rate of biliary infections. METHODS: Expression and localization of HAMP in biliary system was analyzed by real time RT-PCR, in-situ hybridization, immunostaining and -blotting, while prohepcidin levels in human bile were determined by ELISA. RESULTS: Hepcidin was detected in mouse/human gallbladder and bile duct epithelia. Biliary HAMP is stress-inducible, in that it is increased in biliary cell lines upon IL-6 stimulation and in gallbladder mucosa of patients with acute cholecystitis. Hepcidin is also present in the bile and elevated prohepcidin levels were observed in bile of primary sclerosing cholangitis (PSC patients with concurrent bacterial cholangitis compared to PSC subjects without bacterial infection (median values 22.3 vs. 8.9; p = 0.03. In PSC-cholangitis subjects, bile prohepcidin levels positively correlated with C-reactive protein and bilirubin levels (r = 0.48 and r = 0.71, respectively. In vitro, hepcidin enhanced the antimicrobial capacity of human bile (p<0.05. CONCLUSION: Hepcidin is a stress-inducible peptide of the biliary epithelia and a potential marker of biliary stress. In the bile, hepcidin may serve local functions such as protection from bacterial infections.

The T2-Shortening Effect of Gadolinium and the Optimal Conditions for Maximizing the CNR for Evaluating the Biliary System: a Phantom Study

International Nuclear Information System (INIS)

Lee, Mi Jung; Kim, Myung Joon; Yoon, Choon Sik; Song, Si Young; Park, Kyung Soo; Kim, Woo Sun

2011-01-01

Clear depiction of the common bile duct is important when evaluating neonatal cholestasis in order to differentiate biliary atresia from other diseases. During MR cholangiopancreatography, the T2-shortening effect of gadolinium can increase the contrast-to-noise ratio (CNR) of the bile duct and enhance its depiction. The purpose of this study was to confirm, by performing a phantom study, the T2-shortening effect of gadolinium, to evaluate the effect of different gadolinium chelates with different gadolinium concentrations and different magnetic field strengths for investigating the optimal combination of these conditions, and for identifying the maximum CNR for the evaluation of the biliary system. MR imaging using a T2-weighted single-shot fast spin echo sequence and T2 relaxometry was performed with a sponge phantom in a syringe tube. Two kinds of contrast agents (Gd-DTPA and Gd-EOB-DTPA) with different gadolinium concentrations were evaluated with 1.5T and 3T scanners. The signal intensities, the CNRs and the T2 relaxation time were analyzed. The signal intensities significantly decreased as the gadolinium concentrations increased (p < 0.001) with both contrast agents. These signal intensities were higher on a 3T (p < 0.001) scanner. The CNRs were higher on a 1.5T (p < 0.001) scanner and they showed no significant change with different gadolinium concentrations. The T2 relaxation time also showed a negative correlation with the gadolinium concentrations (p < 0.001) and the CNRs showed decrease more with Gd-EOB-DTPA (versus Gd-DTPA; p < 0.001) on a 3T scanner (versus 1.5T; p < 0.001). A T2-shortening effect of gadolinium exhibits a negative correlation with the gadolinium concentration for both the signal intensities and the T2 relaxation time. A higher CNR can be obtained with Gd-DTPA on a 1.5T MRI scanner.

Retrograde cholangiopancreatography in the diagnosis of biliary and pancreatic duct diseases

International Nuclear Information System (INIS)

Vasil'ev, Yu.D.; Sedletskaya, T.N.

1980-01-01

Results of retrograde cannulation with the aid of flexible fibroduodenoscopes with subsequent introduction of a contrast substance into biliary and pancreatic ducts are presented. The investigation is carried out on 120 patients with different diseases of hepatopancreatoduodenal zone. The standard technique of X-ray examination has been applied permitting to obtain the most exhaustive information. Using retrograde cholangiopancreatography revealed have been choledocholithiasis, deformation of biliary ducts after surgical intervention, pancreatic cyst, tumor of the main pancreatic duct etc. Results of investigation of biliary and pancreatic ducts using retrograde cannulation are reaffirmed with the data of operations on biliary tract in 72 patients. Intraoperational cholangiography has been carried out on 36 of them during operation. An attempt to cannulate big duodenal papilla in 12 patients proved to be ineffective. No complications have been observed during examination

Atresia biliar: a experiência Brasileira

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Elisa de Carvalho

2010-12-01

Full Text Available OBJETIVO: Avaliar as características epidemiológicas, clínicas e prognósticas de crianças com atresia biliar. MÉTODOS: Dados sobre portoenterostomia, transplante hepático (TxH, idade no último seguimento e sobrevida foram coletados dos prontuários de pacientes acompanhados em seis centros no Brasil (1982-2008 e comparados em relação às décadas do procedimento cirúrgico. RESULTADOS: Dos 513 pacientes, 76,4% foram submetidos a portoenterostomia [idade: 60,0-94,7 (82,6±32,8 dias] e 46,6% foram submetidos a TxH. Em 69% dos casos, o TxH foi realizado após a portoenterostomia, enquanto em 31% dos casos o TxH foi realizado como cirurgia primária. Os pacientes da região Nordeste foram submetidos a portoenterostomia mais tardiamente do que as crianças das regiões Sul (p = 0,008 e Sudeste (p = 0,0012, embora, mesmo nas duas últimas regiões, a idade no momento da portoenterostomia tenha sido superior ao desejável. Ao longo das décadas, houve aumento progressivo do número de TxH realizados. A sobrevida global foi de 67,6%. A sobrevida aumentou nas últimas décadas (anos 1980 versus 1990, p = 0,002; anos 1980 versus 2000, p 90 dias, respectivamente. Os pacientes transplantados apresentaram taxas de sobrevida mais elevadas (88,3%. A sobrevida de 4 anos com fígado nativo foi de 36,8%, inversamente correlacionada à idade no momento da portoenterostomia (54, 33,3, 26,6% para 90 dias, respectivamente. CONCLUSÕES: Este estudo multicêntrico demonstrou que o encaminhamento tardio das crianças portadoras de atresia biliar ainda é um problema no Brasil, influenciando a sobrevida destes pacientes. Estratégias que proporcionam o encaminhamento precoce estão sendo desenvolvidas com o objetivo de reduzir a necessidade de transplante hepático nos primeiros anos de vida.

Importance of early diagnosis of pancreaticobiliary maljunction without biliary dilatation

Science.gov (United States)

Takuma, Kensuke; Kamisawa, Terumi; Tabata, Taku; Hara, Seiichi; Kuruma, Sawako; Inaba, Yoshihiko; Kurata, Masanao; Honda, Goro; Tsuruta, Koji; Horiguchi, Shin-ichiro; Igarashi, Yoshinori

2012-01-01

AIM: To clarify the strategy for early diagnosis of pancreaticobiliary maljunction (PBM) without biliary dilatation and to pathologically examine gallbladder before cancer develops. METHODS: The anatomy of the union of the pancreatic and bile ducts was assessed by using endoscopic retrograde cholangiopancreatography (ERCP). Patients with a long common channel in which communication between the pancreatic and bile ducts was maintained even during sphincter contraction were diagnosed as having PBM. Of these, patients in which the maximal diameter of the bile duct was less than 10 mm were diagnosed with PBM without biliary dilatation. The process of diagnosing 54 patients with PBM without biliary dilatation was retrospectively investigated. Histopathological analysis of resected gallbladder specimens from 8 patients with PBM without biliary dilatation or cancer was conducted. RESULTS: Thirty-six PBM patients without biliary dilatation were diagnosed with gallbladder cancer after showing clinical symptoms such as abdominal or back pain (n = 16) or jaundice (n = 12). Radical surgery for gallbladder cancer was only possible in 11 patients (31%) and only 4 patients (11%) survived for 5 years. Eight patients were suspected as having PBM without biliary dilatation from the finding of gallbladder wall thickening on ultrasound and the diagnosis was confirmed by ERCP and/or magnetic resonance cholangiopancreatography (MRCP). The median age of these 8 patients was younger by a decade than PBM patients with gallbladder cancer. All 8 patients underwent prophylactic cholecystectomy and bile duct cancer has not occurred. Wall thickness and mucosal height of the 8 resected gallbladders were significantly greater than controls, and hyperplastic changes, hypertrophic muscular layer, subserosal fibrosis, and adenomyomatosis were detected in 7 (88%), 5 (63%), 7 (88%) and 5 (63%) patients, respectively. Ki-67 labeling index was high and K-ras mutation was detected in 3 of 6 patients

Atresia Hymenalis with Haematometrocolpos: A Benefit of Teaching a Case Series and Review of the Literature

LENUS (Irish Health Repository)

Whitla, L

2017-09-01

Imperforate hymen or atresia hymenalis is a rare clinical presentation, the condition is estimated to be present in 0.5\\/1000 females1. It is the most common obstructive anomaly of the female genital tract, and results from failure of canalization of the vaginal plate in utero. Atresia hymenalis can present in neonates as bulging hymen due to accumulation of secretions by the uterovaginal mucosa2,3. Usually the imperforate hymen is asymptomatic until menarche, when haematometrocolpos results in symptoms such as abdominal pain, abdominal mass, urinary retention and constipation. Many cases are diagnosed in the evaluation of primary amenorrhoea or recurrent abdominal pain2,3. We present 4 cases diagnosed over a 7 month period in our ED.

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.

Malignant Biliary Obstruction: Evidence for Best Practice

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Leonardo Zorrón Cheng Tao Pu

2016-01-01

Full Text Available What should be done next? Is the stricture benign? Is it resectable? Should I place a stent? Which one? These are some of the questions one ponders when dealing with biliary strictures. In resectable cases, ongoing questions remain as to whether the biliary tree should be drained prior to surgery. In palliative cases, the relief of obstruction remains the main goal. Options for palliative therapy include surgical bypass, percutaneous drainage, and stenting or endoscopic stenting (transpapillary or via an endoscopic ultrasound approach. This review gathers scientific foundations behind these interventions. For operable cases, preoperative biliary drainage should not be performed unless there is evidence of cholangitis, there is delay in surgical intervention, or intense jaundice is present. For inoperable cases, transpapillary stenting after sphincterotomy is preferable over percutaneous drainage. The use of plastic stents (PS has no benefit over Self-Expandable Metallic Stents (SEMS. In case transpapillary drainage is not possible, Endoscopic Ultrasonography- (EUS- guided drainage is still an option over percutaneous means. There is no significant difference between the types of SEMS and its indication should be individualized.

Adaptive remodeling of the biliary tree: the essence of liver progenitor cell expansion.

Science.gov (United States)

Kok, Cindy Yuet-Yin; Miyajima, Atsushi; Itoh, Tohru

2015-07-01

The liver progenitor cell population has long been thought to exist within the liver. However, there are no standardized criteria for defining the liver progenitor cells, and there has been intense debate about the origin of these cells in the adult liver. The characteristics of such cells vary depending on the disease model used and also on the method of analysis. Visualization of three-dimensional biliary structures has revealed that the emergence of liver progenitor cells essentially reflects the adaptive remodeling of the hepatic biliary network in response to liver injury. We propose that the progenitor cell exists as a subpopulation in the biliary tree and show that the appearance of liver progenitor cells in injured parenchyma is reflective of extensive remodeling of the biliary structure. © 2015 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

The Emerging Role of Soluble Adenylyl Cyclase in Primary Biliary Cholangitis

NARCIS (Netherlands)

Chang, Jung-Chin; Beuers, Ulrich; Oude Elferink, Ronald P. J.

2017-01-01

Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is a chronic fibrosing cholangiopathy with the signature of an autoimmune disease and features of intrahepatic cholestasis. Immunosuppressing treatments are largely unsuccessful. Responsiveness to ursodeoxycholic

Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

International Nuclear Information System (INIS)

Holmqvist, C.; Hochbergs, P.; Bjoerkhem, G.; Brockstedt, S.; Laurin, S.

2000-01-01

To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5±4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD

Pre-operative evaluation with MR in tetralogy of Fallot and pulmonary atresia with ventricular septal defect

Energy Technology Data Exchange (ETDEWEB)

Holmqvist, C.; Hochbergs, P. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology; Bjoerkhem, G. [Univ. Hospital, Lund (Sweden). Dept of Paediatrics; Brockstedt, S.; Laurin, S. [Univ. Hospital, Lund (Sweden). Dept of Diagnostic Radiology

2000-01-01

To assess whether MR imaging could replace angiography in pre-operative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. Fourteen patients with tetralogy of Fallot (n=10) or pulmonary atresia with VSD (n=4), mean age 7.5{+-}4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supra valvular stenosis, but the agreement was somewhat lower for the sub valvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the sub valvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.

Effect of cisapride on symptoms and biliary drainage in patients with postcholecystectomy syndrome

International Nuclear Information System (INIS)

Farup, P.G.; Tjora, S.; Tholfsen, K.

1991-01-01

The study evaluates the effect of 20 mg cisapride twice daily on symptoms and biliary drainage in patients with the postcholecystectomy syndrome. 19 patients, all female, went through a randomized, double-blind, placebo-controlled, crossover trial with two 4-week treatment periods separated by a 2-week washout period. Symptoms were registered on diary cards. Biliary drainage was studied with dynamic cholescinitigraphy. The down slope of the time-activity curve was used as a measure of the biliary drainage. More symptoms were registered during cisapride therapy than with placebo. This unfavourable effect of cisapride was statistically significant in a subgroup of patients with postcholecystectomy complaints identical to the biliary pain they experienced during injection of contrast at the endoscopic retrograde cholangiopancreatographic examination. Cisapride statistically significantly hastened biliary drainage. The median T 1/2 values were 24 and 28 min after cisapride and placebo, respectively. In conclusion, cisapride promoted biliary drainage in patients with the postcholecystectomy syndrome, but had an unfavourable symptomatic effect in those with bile duct triggered postcholecystectomy complaints. 22 refs., 3 figs

Transcriptome profiling of the theca interna from bovine ovarian follicles during atresia.

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Nicholas Hatzirodos

Full Text Available The theca interna is a specialized stromal layer that envelops each growing ovarian follicle. It contains capillaries, fibroblasts, immune cells and the steroidogenic cells that synthesize androgens for conversion to estradiol by the neighboring granulosa cells. During reproductive life only a small number of follicles will grow to a sufficient size to ovulate, whereas the majority of follicles will undergo regression/atresia and phagocytosis by macrophages. To identify genes which are differentially regulated in the theca interna during follicular atresia, we undertook transcriptome profiling of the theca interna from healthy (n = 10 and antral atretic (n = 5 bovine follicles at early antral stages (<5 mm. Principal Component Analyses and hierarchical classification of the signal intensity plots for the arrays showed primary clustering into two groups, healthy and atretic. A total of 543 probe sets were differentially expressed between the atretic and healthy theca interna. Further analyses of these genes by Ingenuity Pathway Analysis and Gene Ontology Enrichment Analysis Toolkit software found most of the genes being expressed were related to cytokines, hormones and receptors as well as the cell cycle and DNA replication. Cell cycle genes which encode components of the replicating chromosome complex and mitotic spindle were down-regulated in atretic theca interna, whereas stress response and inflammation-related genes such as TP53, IKBKB and TGFB1 were up-regulated. In addition to cell cycle regulators, upstream regulators that were predicted to be inhibited included Retinoblastoma 1, E2 transcription factor 1, and hepatocyte growth factor. Our study suggests that during antral atresia of small follicles in the theca interna, arrest of cell cycle and DNA replication occurs rather than up- regulation of apoptosis-associated genes as occurs in granulosa cells.

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)

Percutaneous transluminal angioplasty of an occluded surgical splenorenal shunt in a 4-year-old child after liver transplantation

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Yussim, Ethan; Belenky, Alexander; Atar, Eli [Department of Diagnostic Radiology, Rabin Medical Center, Vascular and Interventional Radiology Unit, Petah Tikva (Israel); Shapiro, Rivka [Schneider Children' s Medical Center, Institute of Pediatric Gastroenterology and Nutrition, Petah Tikva (Israel); Mor, Eytan [Rabin Medical Center, Department of Organ Transplantation, Petah Tikva (Israel)

2005-07-01

Percutaneous transluminal angioplasty is increasingly used in children. We present a case of successful balloon angioplasty of an occluded surgical splenorenal shunt in a 4-year-old child who underwent liver transplantation because of biliary atresia. Percutaneous reopening of the shunt is a relatively safe procedure that may spare patients of surgical intervention. (orig.)

Percutaneous transluminal angioplasty of an occluded surgical splenorenal shunt in a 4-year-old child after liver transplantation

International Nuclear Information System (INIS)

Yussim, Ethan; Belenky, Alexander; Atar, Eli; Shapiro, Rivka; Mor, Eytan

2005-01-01

Percutaneous transluminal angioplasty is increasingly used in children. We present a case of successful balloon angioplasty of an occluded surgical splenorenal shunt in a 4-year-old child who underwent liver transplantation because of biliary atresia. Percutaneous reopening of the shunt is a relatively safe procedure that may spare patients of surgical intervention. (orig.)

Bile Culture and Susceptibility Testing of Malignant Biliary Obstruction via PTBD

Energy Technology Data Exchange (ETDEWEB)

Yu Haipeng; Guo Zhi, E-mail: jieruke@yahoo.com.cn; Xing Wenge; Guo Xiuying; Liu Fang; Li Baoguo [Tinajin Medical University Cancer Institute and Hospital, Department of Interventional Therapy, Tianjin Key Cancer Prevention and Treatment Laboratory (China)

2012-10-15

Purpose: To assess the information obtained by bile culture and susceptibility testing for malignant biliary obstruction by a retrospective one-center study. Methods: A total of 694 patients with malignant biliary obstruction received percutaneous transhepatic biliary drainage during the period July 2003 to September 2010, and subsequently, bile specimens were collected during the procedure. Among the 694 patients, 485 were men and 209 were women, ranging in age from 38 to 78 years (mean age 62 years). Results: A total of 42.9% patients had a positive bile culture (298 of 694). Further, 57 species of microorganisms and 342 strains were identified; gram-positive bacteria accounted for 50.9% (174 of 342) and gram-negative bacteria accounted for 41.5% (142 of 342) of these strains. No anaerobes were obtained by culture during this study. The most common microorganisms were Enterococcus faecalis (41 of 342, 11.9%), Escherichia coli (34 of 342, 9.9%), Klebsiella pneumoniae (28 of 342, 8.2%), Staphylococcus epidermidis (19 of 342, 5.5%), Enterococcus (18 of 342, 5.3%), and Enterobacter cloacae (16 of 342, 4.7%). The percentage of {beta}-lactamase-producing gram-positive bacteria was 27.6% (48 of 174), and the percentage of gram-negative bacteria was 19.7% (28 of 142). The percentage of enzyme-producing Escherichia coli was 61.7% (21 of 34). Conclusion: The bile cultures in malignant biliary obstruction are different from those in the Tokyo Guidelines and other benign biliary obstruction researches, which indicates that a different antibacterial therapy should be applied. Thus, knowledge of the antimicrobial susceptibility data could aid in the better use of antibiotics for the empirical therapy of biliary infection combined with malignant biliary obstruction.

Bile Culture and Susceptibility Testing of Malignant Biliary Obstruction via PTBD

International Nuclear Information System (INIS)

Yu Haipeng; Guo Zhi; Xing Wenge; Guo Xiuying; Liu Fang; Li Baoguo

2012-01-01

Purpose: To assess the information obtained by bile culture and susceptibility testing for malignant biliary obstruction by a retrospective one-center study. Methods: A total of 694 patients with malignant biliary obstruction received percutaneous transhepatic biliary drainage during the period July 2003 to September 2010, and subsequently, bile specimens were collected during the procedure. Among the 694 patients, 485 were men and 209 were women, ranging in age from 38 to 78 years (mean age 62 years). Results: A total of 42.9% patients had a positive bile culture (298 of 694). Further, 57 species of microorganisms and 342 strains were identified; gram-positive bacteria accounted for 50.9% (174 of 342) and gram-negative bacteria accounted for 41.5% (142 of 342) of these strains. No anaerobes were obtained by culture during this study. The most common microorganisms were Enterococcus faecalis (41 of 342, 11.9%), Escherichia coli (34 of 342, 9.9%), Klebsiella pneumoniae (28 of 342, 8.2%), Staphylococcus epidermidis (19 of 342, 5.5%), Enterococcus (18 of 342, 5.3%), and Enterobacter cloacae (16 of 342, 4.7%). The percentage of β-lactamase-producing gram-positive bacteria was 27.6% (48 of 174), and the percentage of gram-negative bacteria was 19.7% (28 of 142). The percentage of enzyme-producing Escherichia coli was 61.7% (21 of 34). Conclusion: The bile cultures in malignant biliary obstruction are different from those in the Tokyo Guidelines and other benign biliary obstruction researches, which indicates that a different antibacterial therapy should be applied. Thus, knowledge of the antimicrobial susceptibility data could aid in the better use of antibiotics for the empirical therapy of biliary infection combined with malignant biliary obstruction.

Percutaneous transluminal biopsy using 7F forceps for diagnosing malignant biliary obstruction

Energy Technology Data Exchange (ETDEWEB)

Bahn, Young Eun; Kim, Young Hwan; An, Eun Jung; Kim, See Hyung [Keimyung Univ. College of Medicine, Daegu (Korea, Republic of)

2012-07-15

To evaluate the usefulness of the percutaneous transluminal biopsy using 7-F forceps for diagnosing malignant biliary obstruction. One hundred and seven consecutive patients with obstructive jaundice underwent transluminal forceps biopsy. The lesions involved the common bile duct (n = 33), common hepatic duct (n = 13), hilum (n = 17), right or left intrahepatic bile duct (n = 32), multiple sites (extra and intrahepatic ducts, n = 7), or anastomotic sites (n = 5). In each patient, an average of three specimens was taken with 7F biopsy forceps through a transhepatic biliary drainage tract. The final diagnosis was confirmed with pathologic findings, or a clinical and radiologic follow up. The final diagnoses showed malignancies in 75 patients and benign biliary obstructions in 32 patients. Pathologic classifications of malignancies established by forceps biopsy included 67 adenocarcinomas, 1 adenosquamous cell carcinoma, and 1 hepatocelluar carcinoma. There were 6 false-negative diagnoses. The diagnostic performance of transluminal forceps biopsy in malignant biliary obstruction had a sensitivity of 92%, specificity of 100%, positive predictive value of 100%, a negative predictive value of 84.2%, and an accuracy of 94.2%. Percutaneous transluminal forceps biopsy is a safe procedure that is easy to perform through a transhepatic biliary drainage tract. It is a highly accurate technique for diagnosing malignant biliary obstructions.

The diagnostic significance of endoscopic cytology in evaluating pancreatic and biliary lesions

OpenAIRE

Nilüfer ONAK KANDEMİR; Banu DOĞAN GÜN; Sibel BEKTAŞ; Figen BARUT1; Burak BAHADIR; Gamze YURDAKAN; Şükrü Oğuz ÖZDAMAR; Gamze MOCAN KUZEY

2007-01-01

Cytology is a widely performed technique in evaluating biliary and pancreatic ductal lesions. The contribution of cytological methods to the diagnosis of the disorders causing biliary stricture, and biopsy diagnoses of the cases were evaluated together with clinical follow-up outcomes and the results presented in this study.From January 2005 to December 2006, in Zonguldak Karaelmas University, Faculty of Medicine, Department of Gastroenterology, 20 biliary endoscopic brush cytology and 2 exfo...

Biliary Cast Syndrome: Hepatic Artery Resistance Index, Pathological Changes, Morphology and Endoscopic Therapy

Directory of Open Access Journals (Sweden)

Hu Tian

2015-01-01

Full Text Available Background: Biliary cast syndrome (BCS was a postoperative complication of orthotopic liver transplantation (OLT, and the reason for BSC was considered to relate with ischemic type biliary lesions. This study aimed to evaluate the relationship between BCS following OLT and the hepatic artery resistance index (HARI, and to observe pathological changes and morphology of biliary casts. Methods: Totally, 18 patients were diagnosed with BCS by cholangiography following OLT using choledochoscope or endoscopic retrograde cholangiopancreatography. In addition, 36 patients who did not present with BCS in the corresponding period had detectable postoperative HARI on weeks 1, 2, 3 shown by color Doppler flow imaging. The compositions of biliary casts were analyzed by pathological examination and scanning electron microscopy. Results: HARI values of the BCS group were significantly decreased as compared with the non-BCS group on postoperative weeks 2 and 3 (P 1 (OR = 1.300; 1.223; and 1.889, respectively. The OR of HARI 3 was statistically significant (OR = 1.889; 95% confidence interval = 1.166-7.490; P = 0.024. The compositions of biliary casts were different when bile duct stones were present. Furthermore, vascular epithelial cells were found by pathological examination in biliary casts. Conclusions: HARI may possibly serve as an independent risk factor and early predictive factor of BCS. Components and formation of biliary casts and bile duct stones are different.

Endoscopic Ultrasonography-Guided Techniques for Accessing and Draining the Biliary System and the Pancreatic Duct.

Science.gov (United States)

Rimbaş, Mihai; Larghi, Alberto

2017-10-01

When endoscopic retrograde cholangiopancreatography (ERCP) fails to decompress the biliary system or the pancreatic duct, endoscopic ultrasonography (EUS)-guided biliary or pancreatic access and drainage can be used. Data show a high success rate and acceptable adverse event rate for EUS-guided biliary drainage. The outcomes of EUS-guided biliary drainage seem equivalent to percutaneous drainage and ERCP, whereas only retrospective studies are available for pancreatic duct drainage. In this article, revision of the technical and clinical status and the current evidence of interventional EUS-guided biliary and pancreatic duct access and drainage are presented. Copyright © 2017 Elsevier Inc. All rights reserved.

Preoperative Biliary Drainage for Cancer of the Head of the Pancreas

NARCIS (Netherlands)

van der Gaag, Niels A.; Rauws, Erik A. J.; van Eijck, Casper H. J.; Bruno, Marco J.; van der Harst, Erwin; Kubben, Frank J. G. M.; Gerritsen, Josephus J. G. M.; Greve, Jan Willem; Gerhards, Michael F.; de Hingh, Ignace H. J. T.; Klinkenbijl, Jean H.; Nio, Chung Y.; de Castro, Steve M. M.; Busch, Olivier R. C.; van Gulik, Thomas M.; Bossuyt, Patrick M. M.; Gouma, Dirk J.

2010-01-01

BACKGROUND The benefits of preoperative biliary drainage, which was introduced to improve the postoperative outcome in patients with obstructive jaundice caused by a tumor of the pancreatic head, are unclear. METHODS In this multicenter, randomized trial, we compared preoperative biliary drainage

A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis

NARCIS (Netherlands)

Nevens, Frederik; Andreone, Pietro; Mazzella, Giuseppe; Strasser, Simone I.; Bowlus, Christopher; Invernizzi, Pietro; Drenth, Joost P. H.; Pockros, Paul J.; Regula, Jaroslaw; Beuers, Ulrich; Trauner, Michael; Jones, David E.; Floreani, Annarosa; Hohenester, Simon; Luketic, Velimir; Shiffman, Mitchell; van Erpecum, Karel J.; Vargas, Victor; Vincent, Catherine; Hirschfield, Gideon M.; Shah, Hemant; Hansen, Bettina; Lindor, Keith D.; Marschall, Hanns-Ulrich; Kowdley, Kris V.; Hooshmand-Rad, Roya; Marmon, Tonya; Sheeron, Shawn; Pencek, Richard; MacConell, Leigh; Pruzanski, Mark; Shapiro, David; Angus, Peter; Roberts, Stuart; Vogel, Wolfgang; Graziadei, Ivo; de Lédinghen, Victor; Berg, Thomas; Gotthardt, Daniel; Hartmann, Heinz; Kremer, Andreas E.; Lammert, Frank; Manns, Michael P.; Rust, Christian; Schramm, Christoph; Trautwein, Christian; Zeuzem, Stefan; Carbone, Marco; van Nieuwkerk, Carin C. M. J.; Celinski, Krzysztof; Gonciarz, Maciej; Hartleb, Marek; Milkiewicz, Piotr; Parés, Albert; Bramley, Peter; Thorburn, Douglas; Mookerjee, Rajeshwar P.; Burroughs, Andrew; Chapman, Roger; Dillon, John F.; Greer, John A.; Tripathi, Dhiraj; McCune, Anne; Ryder, Stephen; Bacon, Bruce R.; Naik, Jahnavi; Wang, Lan Sun; Bodenheimer, Henry C.; Bowlus, Christopher L.; Chalasani, Naga; Forman, Lisa M.; Gordon, Stuart C.; Luketic, Velimir A.; Mayo, Marlyn; Muir, Andrew J.; Reddy, K. Gautham; Talwalker, Jayant T.; Vierling, John M.

2016-01-01

BACKGROUND Primary biliary cholangitis ( formerly called primary biliary cirrhosis) can progress to cirrhosis and death despite ursodiol therapy. Alkaline phosphatase and bilirubin levels correlate with the risk of liver transplantation or death. Obeticholic acid, a farnesoid X receptor agonist, has

Diabetes in relation to biliary tract cancer and stones: a population-based study in Shanghai, China

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Shebl, F M; Andreotti, G; Rashid, A; Gao, Y-T; Yu, K; Shen, M-C; Wang, B-S; Li, Q; Han, T-Q; Zhang, B-H; Fraumeni, J F; Hsing, A W

2010-01-01

Background: Biliary tract cancers are rare but fatal malignancies. Diabetes has been related to biliary stones, but its association with biliary tract cancers is less conclusive. Methods: In a population-based case–control study of 627 cancers, 1037 stones, and 959 controls in Shanghai, China, we examined the association between diabetes and the risks of biliary tract cancer and stones, as well as the effect of potential mediating factors, including serum lipids and biliary stones (for cancer), contributing to the causal pathway from diabetes to biliary diseases. Results: Independent of body mass index (BMI), diabetes was significantly associated with gallbladder cancer and biliary stones ((odds ratio (OR) (95% confidence interval)=2.6 (1.5–4.7) and 2.0 (1.2–3.3), respectively). Biliary stones and low serum levels of high-density lipoprotein (HDL) were significant mediators of the diabetes effect on gallbladder cancer risk, accounting for 60 and 17% of the diabetes effect, respectively. High-density lipoprotein was also a significant mediator of the diabetes effect on biliary stones, accounting for 18% of the diabetes effect. Conclusions: Independent of BMI, diabetes is a risk factor for gallbladder cancer, but its effect is mediated in part by biliary stones and serum HDL levels, suggesting that gallbladder cancer risk may be reduced by controlling diabetes, stones, and HDL levels. PMID:20517308

Duodenal atresia in an infant with triple-X syndrome: a new associated malformation in 47,XXX.

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Rolle, Udo; Linse, Barbara; Glasow, Simone; Sandig, Klaus Rainer; Richter, Thomas; Till, Holger

2007-08-01

An association between the triple-X syndrome (47,XXX) and gastrointestinal malformations is extremely rare. Most 47,XXX patients present with a normal phenotype, but genitourinary malformations have been described. We report a case of a child with 47,XXX and duodenal atresia. Antenatal ultrasound scan showed a dilated fetal stomach and upper part of the duodenum (double bubble phenomenon) at 31 weeks of gestation in a 31-year-old woman with polyhydramnion. The amniotic fluid karyotype showed 47,XXX. After a scheduled delivery, duodenal atresia was confirmed and treated with duodeno-duodenostomy. The possible association of gastrointestinal and genitourinary tract anomalies requires a detailed postnatal clinical investigation and ultrasonographic examination of the abdomen, retroperitoneum, and pelvis on all triple-X syndrome patients. 2007 Wiley-Liss, Inc.

Short term efficacy of interventional therapy for hilar biliary obstruction

International Nuclear Information System (INIS)

Zhai Renyou; Dai Dingke; Wang Jianfeng; Yu Ping; Wei Baojie

2006-01-01

Objective: To analyze the method and short term efficacy of interventional therapy for hilar biliary obstructive jaundice. Methods: 100 consecutive patients with perihilar biliary obstruction admitted before May 2004 were treated with percutaneous transhepatic biliary drainage (PTBD) or placement of metallic stents. Among them, 39 patients were found with bile duct cancer, 6 with adenocarcinoma of gallbladder, 22 with metastatic carcinoma, 15 with primary liver carcinoma and 18 with bile duct strait after liver transplantation. Serum total bilirubin before operation and 3-7 days, 8-14 days after procedure were analysed by t test. Results: 79 patients with PTBD (including simple external drainage and combined internal and external drainage), and 21 patients with stents placement (including 31 stents of 4 different kinds) were all carried out successfully. There were significant differences in serum total bilirubin before and 3-7 days, 8-14 days after the procedure, P<0.05 vs P<0.01. Conclusion: Interventional therapy is simple, safe, and effective for hilar biliary obstruction, the latter showed more significance than the former with short term satisfaction. (authors)

Percutaneous Treatment of Intrahepatic Biliary Leak: A Modified Occlusion Balloon Technique

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Nasser, Felipe; Rocha, Rafael Dahmer, E-mail: rafaeldrocha@gmail.com; Falsarella, Priscila Mina; Motta-Leal-Filho, Joaquim Maurício da; Azevedo, André Arantes; Valle, Leonardo Guedes Moreira; Cavalcante, Rafael Noronha; Garcia, Rodrigo Gobbo; Affonso, Breno Boueri; Galastri, Francisco Leonardo [Hospital Israelita Albert Einstein, Department of Interventional Radiology (Brazil)

2016-05-15

PurposeTo report a novel modified occlusion balloon technique to treat biliary leaks.MethodsA 22-year-old female patient underwent liver transplantation with biliary-enteric anastomosis. She developed thrombosis of the common hepatic artery and extensive ischemia in the left hepatic lobe. Resection of segments II and III was performed and a biliary-cutaneous leak originating at the resection plane was identified in the early postoperative period. Initial treatment with percutaneous transhepatic drainage was unsuccessful. Therefore, an angioplasty balloon was coaxially inserted within the biliary drain and positioned close to the leak.ResultsThe fistula output abruptly decreased after the procedure and stopped on the 7th day. At the 3-week follow-up, cholangiography revealed complete resolution of the leakage.ConclusionThis novel modified occlusion balloon technique was effective and safe. However, greater experience and more cases are necessary to validate the technique.

A self-retaining looped catheder for percutaneous biliary drainage

International Nuclear Information System (INIS)

Guenther, R.; Klose, K.; Daehnert, W.

1983-01-01

A percutaneous catheter which can be looped by means of a nylon thread was used in 32 patients for percutaneous biliary drainage. The catheter can be fixed in this way and is thereby prevented from migrating from the biliary system. The catheter is not suitable for proximal obstructions. Problems may arise during the removal of the nylon thread and loss of looping of the point of catheter. (orig.) [de

Usefulness of a Biliary Manipulation Catheter in Percutaneous Transhepatic Biliary Drainage

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Paek, Auh Whan [Dept. of Radiology, Virginia University Heath Center, Charlottesville (United States); Won, Je Hwan; Lee, Jei Hee; Sun, Joo Sung; Kwak, Kyu Sung; Bae, Jae Ik [Dept. of Radiology, Ajou University School of Medicine, Suwon (Korea, Republic of)

2011-04-15

To evaluate usefulness of a manipulation catheter in percutaneous transhepatic biliary drainage (PTBD). A biliary manipulation catheter was used for the aspiration of retained bile and lesion crossing during an initial PTBD in 91 consecutive patients over a 6 month period. This catheter allowed for a 0.035 inch guide wire made of 5F short steel braided polyurethane. The terminal 1 cm segment was tapered and 45 degree angulated. Two side holes were made in the terminal segment to facilitate the aspiration of bile. The safety of this procedure was evaluated based on whether the catheters caused complications during insertion and manipulation, and whether cholangitis was aggravated after the procedure. Effectiveness of the procedure was evaluated based on the ability to aspirate retained bile and to cross the lesion. Both the insertion of a 0.035 inch hydrophilic guide wire and aspiration of sufficient retained bile were successful with the catheter. Crossing the common bile duct (CBD) lesion had a 98.1% success rate during the initial PTBD. Crossing the hilar obstruction lesion was had a 94.7% success rate to the CBD and 92.1% to the contralateral lobe. Cholangitis improved in 97% of cases, and aggravated transiently in only 3% of cases after PTBD.

Usefulness of a Biliary Manipulation Catheter in Percutaneous Transhepatic Biliary Drainage

International Nuclear Information System (INIS)

Paek, Auh Whan; Won, Je Hwan; Lee, Jei Hee; Sun, Joo Sung; Kwak, Kyu Sung; Bae, Jae Ik

2011-01-01

To evaluate usefulness of a manipulation catheter in percutaneous transhepatic biliary drainage (PTBD). A biliary manipulation catheter was used for the aspiration of retained bile and lesion crossing during an initial PTBD in 91 consecutive patients over a 6 month period. This catheter allowed for a 0.035 inch guide wire made of 5F short steel braided polyurethane. The terminal 1 cm segment was tapered and 45 degree angulated. Two side holes were made in the terminal segment to facilitate the aspiration of bile. The safety of this procedure was evaluated based on whether the catheters caused complications during insertion and manipulation, and whether cholangitis was aggravated after the procedure. Effectiveness of the procedure was evaluated based on the ability to aspirate retained bile and to cross the lesion. Both the insertion of a 0.035 inch hydrophilic guide wire and aspiration of sufficient retained bile were successful with the catheter. Crossing the common bile duct (CBD) lesion had a 98.1% success rate during the initial PTBD. Crossing the hilar obstruction lesion was had a 94.7% success rate to the CBD and 92.1% to the contralateral lobe. Cholangitis improved in 97% of cases, and aggravated transiently in only 3% of cases after PTBD.

Peritoneal seeding of cholangiocarcinoma in patients with percutaneous biliary drainage

International Nuclear Information System (INIS)

Miller, G.A. Jr.; Heaston, D.K.; Moore, A.V. Jr.; Mills, S.R.; Dunnick, N.R.

1983-01-01

Percutaneous transhepatic catheter decompression is performed increasingly as an adjunct or alternative to surgery in patients with benign or malignant biliary obstruction. The authors recently saw three patients with cholangiocarcinoma in whom metastatic seeding of the peritoneal serosa was identified some months after initial percutaneous transhepatic biliary drainage. Although no tumor was found along the hepatic tract of the biliary drainage catheters to implicate the drainage tubes as the direct source of peritoneal spread, the occurrence of this rare type of metastasis of cholangiocarcinoma in patients with potential access of tumor cells to the peritoneal cavity via the catheter tracts does suggest such a relation. The clinical history of one patient is presented

Clinical analysis and management of infections relative to percutaneous biliary drainage or stenting dilation

International Nuclear Information System (INIS)

Yu Ping; Dai Dingke; Qian Xiaojun; Zhai Renyou

2007-01-01

Objective: To analyze the occurrence of infectious relative to percutaneous biliary drainage (PTBD)or stenting for malignant obstructive jaundice and explore the therapy and prevention. Methods: 181 patients (130 male and 51 female; median age 64.5 years old) with malignant biliary obstructive jaundice were investigated including 81 hepatobiliary cancers, 42 pancreatico-ampullae tumors, 58 gestro-intestinal portal lymphatic metastasis. All cases accepted PTBD or placement of metallic stents and the perioperative complications were recorded and analysed including the occurance and treatment. Results: All cases accepted PTBD or stenting successfully. The perioperative biliary infection was the major complication including 50 out of 62 preoperative infected cases (34.25%). 18 cases (15.13%)suffered from biliary infection after operation with 13 under control, 5 without control, 4 complicated with pulmonary infection and 17 (9.39%)died of serious biliary infections. Gram-negative bacilli and endotoxin were the main cause of the severe biliary infection. Postoperative mild pancreatitis occurred in 65 cases (35.91%)without severe necrotic changes and were cured alter anti-inflammatory treatment. Hepatic abscess due to biliary leak occurred in 1 case (0.55%), and was cured by CT-guided drainage. Conclusion: Biliary infection is the most common complication after interventional therapy and should be promptly under control for preventing mortality and prolonging survival. Simultaneously, acute pancreatitis should also be on alert but good prognosis would be obtained with apt therapy. (authors)

[Diagnosis and treatment of congenital biliary duct cyst: twenty-year experience].

Science.gov (United States)

Peng, S; Shi, L; Peng, C; Yang, D; Ji, Z; Wu, Y; Liu, Y; Gao, N; Chen, H

2001-12-01

To summarize the experience in diagnosis and treatment of congenital biliary duct cyst. Clinical data from 108 patients treated from 1980 to 2000 were analyzed retrospectively. Abdominal pain, jaundice and abdominal mass were presented in most pediatric patients. Clinical symptoms in adult patients were non-specific, resulting in delayed diagnosis frequently. Fifty-seven patients (52.7%) had coexistent pancreatic biliary disease. Carcinoma of the biliary duct occurred in 18 patients (16.6%). Ultrasonic examination was performed in 94 patients, ERCP in 46, and CT in 71. All of the patients were correctly diagnosed before operation. Abnormal pancreatobiliary duct junction was found in 39 patients. Before 1985, the diagnosis and classification of congenital biliary duct cyst were established by ultrasonography preoperatively and confirmed during operation. The main procedure was internal drainage by cyst-enterostomy. After 1985, the diagnosis was decided with ERCP and CT, the procedure was cyst excision with Roux-en-Y hepaticojejunostomy. In 1994, we used a new and simplified operative procedure to reduce the risk of malignancy of choledochal cyst. Retrograde infection of the biliary tract the major postoperative complication, could be controlled by the administration of antibiotics. The concept in diagnosis and treatment of congenital choledochal cyst has been changed greatly. CT and ERCP are of great help in the classification of the disease. Currently, cyst excision with Roux-en-Y hepaticojejunostomy is strongly recommended as the treatment of choice for patients with type I and IV cysts. Piggyback orthotopic liver transplantation is indicated for type V cysts (Caroli's disease) with frequently recurrent cholangitis, resulting in biliary cirrhosis.

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

Atresia of large ovarian follicles of the rat

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Maria Słomczyn´ ska

2011-08-01

Full Text Available In the rat, at the beginning of pregnancy a cohort of antral follicles develops until the preovulatory stage. However, these follicles, differentiating in the hyperprolactinemic milieu, produce only small amount of estradiol, do not ovulate and undergo rapid degeneration. They constitute an interesting physiological model of atresia. In the present study, we analysed the development and subsequent degeneration of such follicles. The study was performed on Wistar female rats killed in succession between days 1-9 of pregnancy. Excised ovaries were submitted to a routine histological procedure. Paraffin sections were subjected to hematoxylin and eosin staining or in situ DNA labelling. Histological and TUNEL staining revealed that the investigated group of follicles grew slower than that on the corresponding days of the estrous cycle and reached a preovulatory size and morphological appearance on day 5 of pregnancy. They did not ovulate and between days 6 and 9 of pregnancy an increasing number of apoptotic cells appeared within these follicles. They were localized predominantly in the antral granulosa layer, especially near the cumulus oophorus complex (COC and in the region linking the COC with the follicular wall. The COC and the theca layer were much less affected. In late stages of atresia, also cumulus cells became apoptotic but degenerating oocytes did not exhibit positive TUNEL staining. Only limited number of the theca cells have undergone apoptosis and generally they were not hypertrophied. Our findings indicate that much smaller than normal amount of intrafollicular estradiol was sufficient to support a normal, according to the morphological criteria, although slower development of antral follicles to the late preovulatory stage.

Histopathologic observations of anorectal abnormalities in anal atresia.

Science.gov (United States)

Meier-Ruge, W A; Holschneider, A M

2000-01-01

Over the years from 1992 to 1997, 41 anorectal malformations (ARM) with histopathologic alterations were investigated to determine which morphologic abnormalities of the distal rectum accompany ARMs. Three other cases showed normal neuromuscular morphology; 9 further cases could not be evaluated owing to scanty biopsies. All resected specimens were caudocranially coiled and cryostat cut at -20 degrees C into serial sections, which were stained with a lactic dehydrogenase, succinic dehydrogenase, nitroxide synthase, and acetylcholinesterase reaction as well as hemalum and sirius red. Ten low, 15 intermediate, and 10 high forms of anal atresia (AA) were studied. In addition, six cloacal abnormalities were investigated. In 7 cases (17%) (5 intermediate, 2 low AAs), the characteristics of Hirschsprung's disease were observed. Oligoneuronal hypoganglionosis of the myenteric plexus proximal to the anal floor was diagnosed in 7 AAs (12%). In 10 children with high-type AA and resection of 1-5 cm distal rectum and in all cloacal anomalies (n = 6) defects of the muscularis propria were seen in the rectal-atresia sac. These defects were characterized by hypoplasia of the circular-muscle layer and/or the internal anal sphincter (IAS). Intestinal neuronal dysplasia of the submucous plexus was most frequently observed (12%) in high-type AA. A correlation between innervation anomalies or anomalies of the muscularis propria and the type of fistula could not be seen. In conclusion, all cases with high-type AA and cloacal anomalies were characterized by anomalies of the muscularis propria and/or IAS but this was not the case in intermediate and low-type AAs. Anomalies of the enteric nervous system were diagnosed in 60% of AAs.

Biliary cystadenoma with bile duct communication depicted on liver-specific contrast agent-enhanced MRI in a child

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Marrone, Gianluca; Carollo, Vincenzo; Luca, Angelo [Mediterranean Institute of Transplantation and High Specialization Therapy (ISMETT), Diagnostic and Interventional Radiology, Palermo (Italy); Maggiore, Giuseppe [University Hospital S. Chiara, Gastroenterology and Hepatology, Department of Paediatrics, Pisa (Italy); Sonzogni, Aurelio [Riuniti Hospital, Pathology Department, Bergamo (Italy)

2011-01-15

Biliary cystadenoma is a benign, but potentially malignant, cystic neoplasm of the biliary ducts occurring most commonly in middle-aged females and very rarely in children. We present a 9-year-old boy with biliary cystadenoma, diagnosed by MRI using a new liver-specific contrast agent (gadoxetic acid) that is eliminated by the biliary system. The images clearly demonstrate the communication between the multiloculated cystic mass and the biliary tree, suggesting the possibility of biliary cystadenoma. Due to the malignant potential of a cystadenoma, the lesion was resected. The resection was complete and the postoperative course was uneventful. (orig.)

Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

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Abdel-Aziz Mosaad

2012-06-01

Full Text Available Abstract Background Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis. Case presentation A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma. Conclusions Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology.

Sirenomelia and ndash; Mermaid Syndrome with Oesophageal Atresia: A Rare Case Report

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Veena Raja

2015-09-01

Full Text Available Sirenomelia or Mermaid syndrome is a very rare congenital deformity in which legs are fused together and commonly associated with abnormal kidney development, genital, and rectal abnormalities. In this present case, sirenomelia was associated with oesophageal atresia, which is a rare association and occurs in about 20-35% of cases. [J Interdiscipl Histopathol 2015; 3(3.000: 113-116

The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

Science.gov (United States)

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 esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA) method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus. PMID:28680874

The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

Directory of Open Access Journals (Sweden)

Nathalie Rommel

2017-06-01

Full Text Available 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 esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus.

The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia.

Science.gov (United States)

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 esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA) method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus.

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.

Atresia coli in a Japanese black calf diagnosed by a barium sulphate enema contrast radiograph in the standing position: a case report

International Nuclear Information System (INIS)

Abouelnasr, K.; Ishii, M.; Inokuma, H.; Kobayashi, Y.; Lee, K.; Yamada, K.

2012-01-01

A three day-old Japanese black calf was admitted with a history of abdominal distension and absence of defecation. Dilated loops of the small intestine and hypoplasia of the colon and rectum was observed upon a contrast radiographic examination in the standing position. At necropsy atresia coli with undeveloped rectum and patent anal opening was found. We conclude that a contrast radiograph in the standing position is useful for diagnosing atresia coli in such cases

Y-shaped endoprosthesis stent. A new device for biliary drainage in malignant obstruction

Energy Technology Data Exchange (ETDEWEB)

Hauenstein, K H; Beck, A; Sontheimer, J; Krueger, H J; Salm, R

1988-05-01

Biliary decompression in cases of central tumorous biliary obstruction requires surgical or internal/external catheter bypass techniques. The development of a 14-F Y-shaped-polyurethane endoprosthesis stent provides the possibility to drain the left and right biliary system simultaneously. The endoprosthesis is placed by a combination of external transhepatic and endoscopic approach. The tip of the singular choledochal stent segment is placed within the choledochus or duodenum.

The observation and nursing of patients receiving interventional management for biliary complications occurred after liver transplantation

International Nuclear Information System (INIS)

Li Xiaohui; Zhu Kangshun; Lian Xianhui; Qiu Xuanying

2009-01-01

Objective: To discuss the perioperative nursing norm for patients who are suffering from biliary complications occurred after liver transplantation and who will receive interventional management to treat the complications. Methods: Interventional therapies were performed in 20 patients with biliary complications due to liver transplantation. The interventional procedures performed in 20 cases included percutaneous biliary drainage (n = 13), percutaneous biliary balloon dilatation (n = 5) and biliary stent implantation (n = 7). The clinical results were observed and analyzed. Results: Biliary tract complications occurred after liver transplantation were seen frequently. Proper interventional management could markedly improve the successful rate of liver transplantation and increase the survival rate of the patients. In accordance with the individual condition, proper nursing measures should be taken promptly and effectively. Conclusion: Conscientious and effective nursing can contribute to the early detection of biliary complications and, therefore, to improve the survival rate of both the transplanted liver and the patients. (authors)

[Cost-effectiveness trial of self-expandable metal stents and plastic biliary stents in malignant biliary obstruction].

Science.gov (United States)

Daróczi, Tímea; Bor, Renáta; Fábián, Anna; Szabó, Ella; Farkas, Klaudia; Bálint, Anita; Czakó, László; Rutka, Mariann; Szűcs, Mónika; Milassin, Ágnes; Molnár, Tamás; Szepes, Zoltán

2016-02-14

Self-expandable metal and plastic stents can be applied in the palliative endoscopic treatment of patients with unresectable malignant biliary obstruction. The use of metal stentsis recommended if the patient's life expectancy is more than four months. To compare the therapeutic efficacy and cost-effectiveness of metal and plastic stents in the treatment of malignant biliary obstruction. The authors retrospectively enrolled patients who received metal (37 patients) or plastic stent (37 patients). The complication rate, stent patency and cumulative cost of treatment were assessed in the two groups. The complication rate of metal stents was lower (37.84% vs. 56.76%), but the stent patency was higher compared with plastic stents (19.11 vs. 8.29 weeks; p = 0.0041). In the plastic stent group the frequency of hospitalization of patients in context with stent complications (1.18 vs. 2.32; p = 0.05) and the necessity of reintervention for stent dysfunction (17 vs. 27; p = 0.033) were substantially higher. In this group multiple stent implantation raised the stent patency from 7.68 to 10.75 weeks. There was no difference in the total cost of treatment of malignant biliary obstruction between the two groups (p = 0.848). Considering the cost of treatment and the burden of patients the authors recommend self-expandable metal sten timplantation if the life expectancy of patients is more than two months. In short survival cases multiple plastic stent implantation is recommended.

Hepatobiliary scintigraphy for early diagnosis of biliary atresia

DEFF Research Database (Denmark)

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

2016-01-01

-glutamyl transpeptidase (GGTP) than non-BA patients with non-draining HS (p = 0.019) or draining HS (p = 0.0001). CONCLUSIONS: HS plays an important role in the diagnostic strategy of infantile jaundice due to conjugated hyperbilirubinaemia. It is a non-invasive method that only seldomly calls for sedation. A high...

Acceptable Toxicity After Stereotactic Body Radiation Therapy for Liver Tumors Adjacent to the Central Biliary System

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Eriguchi, Takahisa; Takeda, Atsuya; Sanuki, Naoko; Oku, Yohei; Aoki, Yousuke [Radiation Oncology Center, Ofuna Chuo Hospital, Kanagawa (Japan); Shigematsu, Naoyuki [Department of Radiology, Keio University School of Medicine, Tokyo (Japan); Kunieda, Etsuo, E-mail: kunieda-mi@umin.ac.jp [Department of Radiation Oncology, Tokai University, Kanagawa (Japan)

2013-03-15

Purpose: To evaluate biliary toxicity after stereotactic body radiation therapy (SBRT) for liver tumors. Methods and Materials: Among 297 consecutive patients with liver tumors treated with SBRT of 35 to 50 Gy in 5 fractions, patients who were irradiated with >20 Gy to the central biliary system (CBS), including the gallbladder, and had follow-up times >6 months were retrospectively analyzed. Toxicity profiles, such as clinical symptoms and laboratory and radiologic data especially for obstructive jaundice and biliary infection, were investigated in relation to the dose volume and length relationship for each biliary organ. Results: Fifty patients with 55 tumors were irradiated with >20 Gy to the CBS. The median follow-up period was 18.2 months (range, 6.0-80.5 months). In the dose length analysis, 39, 34, 14, and 2 patients were irradiated with >20 Gy, >30 Gy, >40 Gy, and >50 Gy, respectively, to >1 cm of the biliary tract. Seven patients were irradiated with >20 Gy to >20% of the gallbladder. Only 2 patients experienced asymptomatic bile duct stenosis. One patient, metachronously treated twice with SBRT for tumors adjacent to each other, had a transient increase in hepatic and biliary enzymes 12 months after the second treatment. The high-dose area >80 Gy corresponded to the biliary stenosis region. The other patient experienced biliary stenosis 5 months after SBRT and had no laboratory changes. The biliary tract irradiated with >20 Gy was 7 mm and did not correspond to the bile duct stenosis region. No obstructive jaundice or biliary infection was found in any patient. Conclusions: SBRT for liver tumors adjacent to the CBS was feasible with minimal biliary toxicity. Only 1 patient had exceptional radiation-induced bile duct stenosis. For liver tumors adjacent to the CBS without other effective treatment options, SBRT at a dose of 40 Gy in 5 fractions is a safe treatment with regard to biliary toxicity.

Congenital bronchopulmonary foregut malformation initially diagnosed as esophageal atresia type C: challenging diagnosis and treatment

NARCIS (Netherlands)

Boersma, Doeke; Koot, Bart G.; van der Griendt, Erik Jonas; van Rijn, Rick R.; van der Steeg, Alida F.

2012-01-01

Communicating bronchopulmonary foregut malformations are extremely rare congenital malformations, characterized by a communicating fistula between an isolated part of the respiratory system and the esophagus or the stomach. In this article, we present a case of esophageal atresia type C, later

Manometric assessment of esophageal motor function in patients with primary biliary cirrhosis.

Science.gov (United States)

Bektas, Mehmet; Seven, Gulseren; Idilman, Ramazan; Yakut, Mustafa; Doğanay, Beyza; Kabacam, Gökhan; Ustun, Yusuf; Korkut, Esin; Kalkan, Çağdaş; Sahin, Günay; Cetinkaya, Hulya; Bozkaya, Hakan; Yurdaydin, Cihan; Bahar, Kadir; Cinar, Kubilay; Soykan, Irfan

2014-03-01

Primary biliary cirrhosis is associated with other autoimmune diseases including Sjögren's syndrome, and scleroderma. Esophageal dysmotility is well known in scleroderma, and Sjögren's syndrome. The aim of this study is to investigate whether any esophageal motor dysfunction exists in patients with primary biliary cirrhosis. The study was performed in 37 patients (36 women, mean age: 56.29 ± 10.01 years) who met diagnostic criteria for primary biliary cirrhosis. Thirty-seven functional dyspepsia patients, were also included as a control group. Patients entering the study were asked to complete a symptom questionnaire. Distal esophageal contraction amplitude, and lower esophageal sphincter resting pressure were assessed. Manometric findings in primary biliary cirrhosis patients vs. controls were as follows: Median lower esophageal sphincter resting pressure (mmHg): (24 vs 20, p=0.033); median esophageal contraction amplitude (mmHg): (71 vs 56, p=0.050); mean lower esophageal sphincter relaxation duration (sc, x ± SD): (6.10 ± 1.18 vs 8.29 ± 1.92, pesophageal sphincter relaxation (%) (96 vs 98, p=0.019); respectively. No significant differences were evident in median peak velocity (sc) (3.20 vs 3.02, p=0.778) between patients with primary biliary cirrhosis and the functional dyspepsia patients. Esophageal dysmotility was found in 17 (45.9%) primary biliary cirrhosis patients (non-specific esophageal motor disorder in ten patients, hypomotility of esophagus in five patients, nutcracker esophagus in one patient and hypertensive lower esophageal sphincter in one patient). Esophageal dysmotility was detected in 45.9% of patients. The study suggests that subclinic esophageal dysmotility is frequent in patients with primary biliary cirrhosis. Crown Copyright © 2014. Published by Elsevier B.V. All rights reserved.

Endoscopic treatment of acute biliary pancreatitis: A national survey among Dutch gastroenterologists

NARCIS (Netherlands)

van Geenen, Erwin-Jan M.; Mulder, Chris J. J.; van der, Donald L.; Fockens, Paul; Bruno, Marco J.

2010-01-01

Objective. Based on the ampullary obstruction and reflux theory, six endoscopic retrograde cholangiopancreatography (ERCP) studies have investigated the effect of (early) biliary decompression versus conservative management on the course and outcome of patients with acute biliary pancreatitis (ABP)

Ursodeoxycholic acid for primary biliary cirrhosis.

Science.gov (United States)

Rudic, Jelena S; Poropat, Goran; Krstic, Miodrag N; Bjelakovic, Goran; Gluud, Christian

2012-12-12

Ursodeoxycholic acid is administered to patients with primary biliary cirrhosis, a chronic progressive inflammatory autoimmune-mediated liver disease with unknown aetiology. Despite its controversial effects, the U.S. Food and Drug Administration has approved its usage for primary biliary cirrhosis. To assess the beneficial and harmful effects of ursodeoxycholic acid in patients with primary biliary cirrhosis. We searched for eligible randomised trials in The Cochrane Hepato-Biliary Group Controlled Trials Register, The Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library, MEDLINE, EMBASE, Science Citation Index Expanded, LILACS, Clinicaltrials.gov, and the WHO International Clinical Trials Registry Platform. The literature search was performed until January 2012. Randomised clinical trials assessing the beneficial and harmful effects of ursodeoxycholic acid versus placebo or 'no intervention' in patients with primary biliary cirrhosis. Two authors independently extracted data. Continuous data were analysed using mean difference (MD) and standardised mean difference (SMD). Dichotomous data were analysed using risk ratio (RR). Meta-analyses were conducted using both a random-effects model and a fixed-effect model, with 95% confidence intervals (CI). Random-effects model meta-regression was used to assess the effects of covariates across the trials. Trial sequential analysis was used to assess risk of random errors (play of chance). Risks of bias (systematic error) in the included trials were assessed according to Cochrane methodology bias domains. Sixteen randomised clinical trials with 1447 patients with primary biliary cirrhosis were included. One trial had low risk of bias, and the remaining fifteen had high risk of bias. Fourteen trials compared ursodeoxycholic acid with placebo and two trials compared ursodeoxycholic acid with 'no intervention'. The percentage of patients with advanced primary biliary cirrhosis at baseline varied

Karyotype analysis of a male exhibiting Meckel's diverticulum and aural atresia

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Frizzell, B.; Hicks, M.F. (David Lipscomb Univ., Nashville, TN (United States))

Patau's Syndrome is caused by inheritance of an extra chromosome 13. It is characterized primarily by severe mental retardation, cleft palate, and retarded growth. Most fetuses expressing Patau's Syndrome spontaneously abort, and those that are born usually die before one year. Both Meckel's diverticulum and aural atresia are defects found in patients with Patau's at levels higher than those in the general population. An otherwise asymptomatic male expressing only Meckel's diverticulum and aural atresia has a female sibling whose son expressed Patau's syndrome. Twenty percent of patients with Patau's show a translocation of part of chromosome 13 to another D chromosome. If a translocation were the cause of the expression of Patau's in this family, it is possible that the normal male inherited a balanced translocation and the Patau's male received an unbalanced translocation. A karyotype analysis of the non-Patau's male was done to determine if such a translocation were present.

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.

Hilar biliary neurofibroma without neurofibromatosis: case report with contrast-enhanced ultrasound findings.

Science.gov (United States)

Guo, Huan-Ling; Chen, Li-da; Wang, Zhu; Huang, Yang; Liu, Jin-Ya; Shan, Quan-Yuan; Xie, Xiao-Yan; Lu, Ming-de; Wang, Wei

2016-10-01

Solitary neurofibroma located in the hilum of the liver is extremely rare, particularly without neurofibromatosis. We herein report a case of hilar biliary neurofibroma without signs of von Recklinghausen's disease. A 36-year-old man was admitted to our department with progressive jaundice. The case was diagnosed as hilar cholangiocarcinoma based on preoperative imaging. The patient consequently received a Roux-en-Y hepaticojejunostomy and was confirmed with neurofibroma pathologically. This is the first reported imaging finding of hilar biliary neurofibroma using contrast-enhanced ultrasound, emphasizing the differential diagnosis of biliary tumors.

Specific echocardiographic findings useful for the diagnosis of common pulmonary vein atresia

Directory of Open Access Journals (Sweden)

Hiroyuki Nagasawa

2015-12-01

Full Text Available In this paper, we report a case of common pulmonary vein atresia, which is a very rare disease characterized by cyanosis, heart failure and pulmonary hypertension. Reverse flow in the pulmonary artery at end-diastole as well as in the isthmus of the aorta from early systole to end-diastole detected by echocardiography were found to be specific features useful in diagnosing the disease.

The role of interventional radiology in biliary complications after orthotopic liver transplantation: a single-center experience

International Nuclear Information System (INIS)

Civelli, Enrico Maria; Cozzi, Guido; Milella, Marco; Suman, Laura; Severini, Aldo; Meroni, Roberta; Vercelli, Ruggero

2004-01-01

This study evaluated interventional radiological experience in the management of biliary complications of OLT at the National Cancer Institute of Milan. Seventeen patients who had undergone orthotopic liver transplantation in various hospital were referred to our unit with biliary complications. Group I consisted of 8 patients with anastomotic biliary fistula who came to our attention a short time after transplantation. Group II consisted of 9 patients with anastomotic strictures who came to our attention in a longer period. Two different interventional radiological approaches were used: (a) percutaneous transhepatic biliary drainage (PTBD) in the presence of fistulas in patients of group I; and (b) percutaneous transhepatic biliary drainage combined with dilatation of the strictures with a balloon catheter in patients of group II. On the whole resolution of the biliary complications was achieved in 13 of the 17 cases treated (76.5%), 5 of 8 in group I and 8 of 9 in group II. No secondary stenosis after PTBD were observed in group I, whereas two patients of group II needed a second dilatation. Percutaneous biliary drainage is indicated as a valid treatment in the management of biliary complications, either to allow closure of the fistula either to perform balloon dilatation of stenosis. (orig.)

ATRESIA CONGÉNITA DEL OÍDO Y SU MANEJO

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Dr. Daniel Orfila

2016-11-01

Los implantes cocleares siguen siendo los más usados y en las que se tiene mayor experiencia. Las prótesis de conducción ósea implantables o semi implantables cambiaron el manejo de las atresias y malformaciones de oído externo y medio. Pese a lo prometedor que se visualiza el presente y futuro con el uso de estos dispositivos, siempre se debe tener presente que requieren de un acto quirúrgico para su implantación y que no están exentas de complicaciones, por lo cual se debe elegir juiciosamente la prótesis a usar.

Risk factors for biliary complications after liver transplantation from donation after cardiac death

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LYU Guoyue

2015-12-01

Full Text Available Liver transplantation has become the effective therapeutic method for end-stage liver disease, but the incidence of biliary complications after liver transplantation remains high. With an increasing number of liver transplantation procedures from donation after cardiac death (DCD, it is necessary to investigate the risk factors for biliary complications after liver transplantation from DCD and enhance our understanding of such risk factors in order to reduce biliary complications after liver transplantation from DCD.

Percutaneous balloon dilatation and long-term drainage as treatment of anastomotic and nonanastomotic benign biliary strictures

NARCIS (Netherlands)

Janssen, Jan Jaap; van Delden, Otto M.; van Lienden, Krijn P.; Rauws, Erik A. J.; Busch, Olivier R. C.; van Gulik, Thomas M.; Gouma, Dirk J.; Laméris, Johan S.

2014-01-01

This study was designed to determine the effectiveness of percutaneous balloon dilation and long-term drainage of postoperative benign biliary strictures. Medical records of patients with postoperative benign biliary strictures, in whom percutaneous transhepatic biliary drainage (PTBD) and balloon

Percutaneous Transhepatic Biliary Stenting with Uncovered Self-Expandable Metallic Stents in Patients with Malignant Biliary Obstruction - Efficacy and Survival Analysis.

Science.gov (United States)

Pranculis, Andrius; Kievišas, Mantas; Kievišienė, Lina; Vaičius, Artūras; Vanagas, Tomas; Kaupas, Rytis Stasys; Dambrauskas, Žilvinas

2017-01-01

The aim of this study was to assess short- and long-term outcomes of malignant biliary obstruction (MBO) treatment by percutaneous transhepatic biliary stenting (PTBS) with uncovered selfexpandable metallic stents (SEMS), and to identify predictors of survival. A nine-year, single-centre study from a prospectively collected database included 222 patients with inoperable MBO treated by PTBS with uncovered nitinol SEMS. Technical and clinical success rates were 95.9% and 82.4%, respectively. The total rate of postprocedural complications was 14.4%. The mean durations of the primary and secondary stent patency were 114.7±15.1 and 146.4±21.2 days, respectively. The 30-day mortality rate was 15.3% with no procedure-related deaths. The mean estimated length of survival was 143.3±20.6 days. Independent predictors increasing the risk of death included higher than 115 μmol/L serum bilirubin 2-5 days after biliary stenting (HR 3.274, P =0.019), distal (non-hilar) obstruction of the bile ducts (HR 3.711, P =0.008), Bismuth-Corlette type IV stricture (HR 2.082, P =0.008), obstruction due to gallbladder cancer (HR 31.029, P =0.012) and only partial drainage of liver parenchyma (HR 4.158, P =0.040). PTBS with uncovered SEMS is an effective and safe method for palliative treatment of MBO. Serum bilirubin higher than 115 μmol/L 2-5 days after the procedure has a significant negative impact on patients' survival. Lower survival is also determined by distal bile duct obstruction, Bismuth- Corlette type IV stricture, biliary obstruction caused by gallbladder cancer and when only partial liver drainage is applied.

Should patients with esophageal atresia be submitted to esophageal substitution before they start walking?

Science.gov (United States)

Tannuri, U; Tannuri, A C A

2011-01-01

Esophagocoloplasty and gastric transposition are two major methods for esophageal substitution in children with esophageal atresia, and there is broad agreement that these operations should not be performed before the children start walking. However, there are some reported advantages of performing such operations in the first months of life or in the neonatal period. In this study, we compared our experience with esophageal substitution procedures performed in walking children with esophageal atresia, with the outcomes of children who had the operation before the third month of life reported in the literature. The purpose of this study was to establish if we have to wait until the children start walking before indicating the esophageal replacement procedure. From February 1978 to October 2009, 129 children with esophageal atresia underwent esophageal replacement in our hospital (99 colonic interpositions and 30 gastric transpositions). The records of these patients were reviewed for data regarding demographics, complications (leaks, graft failures, strictures, and graft torsion), and mortality and compared with those reported in the two main articles on esophageal replacement in the neonatal period or in patients less than 3 months of age. The main complication of our casuistic was cervical anastomosis leakage, which sealed spontaneously in all except in four patients. One patient of the esophagocoloplasty group developed graft necrosis and three patients in the gastric transposition group had gastric outlet obstruction, secondary to axial torsion of the stomach placed in the retrosternal space. The long-term outcome of the patients in both groups was considered good to excellent in terms of normal weight gain, absence of dysphagia, and other gastrointestinal symptoms. The comparisons of the main complications and mortality rates in walking children with esophageal substitutions performed in the first months of life showed that the incidences of cervical

Spontaneous Expulsion of a Biliary Stent via the abdominal wall: A ...

African Journals Online (AJOL)

We report a rare complication of a biliary stent inserted via endoscopic retrograde cholangio-pancreatography (ERCP) for benign biliary disease in a 60 years-old female presented with abdominal pain and a tender left iliac fossa (LIF) mass. She had change of the colour of the skin over the hypogastric region. Crepitus was ...

Dosing-time-dependent variation in biliary excretion of flomoxef in rats.

Science.gov (United States)

Hishikawa, Shuji; Sugimoto, Koh-ichi; Kobayashi, Eiji; Kumagai, Yuji; Fujimura, Akio

2003-05-01

We previously reported that the biliary excretion of flomoxef, an oxacephem antibiotic, was greater after dosing at 21:00 than at 09:00 h in diurnally active human subjects. The present study was undertaken to examine whether the biliary excretion of flomoxef is also dependent on its dosing time in rats. Adult male Wistar rats were housed under light on at 07:00 h and off at 19:00 h. Bile fluid was completely drained through a polyethylene catheter from conscious animals. Flomoxef (20 mg/kg) was injected into the tail vein at 09:00 or 21:00 h by a cross-over design, and drained bile fluid was collected for 8 h after each dosing. The maximum concentration of biliary flomoxef was significantly greater and its total excretion tended to be greater after dosing at 09:00 than 21:00 h. These results suggest the biliary excretion of flomoxef is enhanced after dosing at the beginning of the rest period in rats, as it is in humans.

Coexistence of primary biliary cirrhosis and myasthenia gravis: a case study.

Science.gov (United States)

Horigome, H; Nomura, T; Saso, K; Joh, T; Ohara, H; Akita, S; Sobue, S; Mizuno, Y; Kato, Y; Itoh, M

2000-01-01

We present a case that suggests a relationship between primary biliary cirrhosis and myasthenia gravis. A 43-year-old Japanese woman was admitted to the Nagoya City University Medical School, First Department of Internal Medicine with abnormal liver function in August 1991. She had had ptosis of the right eye since 1990. She had not been treated for liver disease. Ptosis of the right eye and hepatomegaly were present. Serum laboratory examinations revealed elevated biliary enzymes and IgM levels; tests were positive for antimitochondrial antibody and antiacetylcholine antibody. Liver histology revealed chronic non-suppurative destructive cholangitis and led to a diagnosis of primary biliary cirrhosis. The tensilon test was positive. Electromyography with repetitive motor nerve stimulation revealed a neuromuscular junction defect; i.e., the primary characteristic of myasthenia gravis. The patient was diagnosed with myasthenia gravis. Although the development of myasthenia gravis has previously been reported in patients with primary biliary cirrhosis during D-penicillamine administration, this is a very rare case of the coexistence of both diseases before such treatment.

Atresia pulmonar con comunicación interventricular

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Tomasa Centella Hernández

2014-04-01

Full Text Available La atresia pulmonar con comunicación interventricular es una cardiopatía congénita cianótica, severa y rara, de alta complejidad, que se caracteriza por la ausencia de conexión entre el ventrículo derecho y las arterias pulmonares. Coexiste con una comunicación interventricular. El flujo hacia el territorio pulmonar puede realizarse a través del ductus arterioso o de colaterales sistémico-pulmonares. La dificultad de esta cardiopatía viene determinada por los diferentes niveles de interrupción desde el ventrículo derecho hasta el territorio pulmonar, y por la diferencia anatómica de las fuentes del flujo hacia dicho territorio, lo que determina diferentes tipos de abordaje quirúrgico.

Lymphocytes contribute to biliary injury and fibrosis in experimental xenobiotic-induced cholestasis

International Nuclear Information System (INIS)

Joshi, Nikita; Kopec, Anna K.; Cline-Fedewa, Holly; Luyendyk, James P.

2017-01-01

The etiology of chronic bile duct injury and fibrosis in patients with autoimmune cholestatic liver diseases is complex, and likely involves immune cells such as lymphocytes. However, most models of biliary fibrosis are not autoimmune in nature. Biliary fibrosis can be induced experimentally by prolonged exposure of mice to the bile duct toxicant alpha-naphthylisothiocyanate (ANIT). We determined whether lymphocytes contributed to ANIT-mediated biliary hyperplasia and fibrosis in mice. Hepatic accumulation of T-lymphocytes and increased serum levels of anti-nuclear-autoantibodies were evident in wild-type mice exposed to ANIT (0.05% ANIT in chow). This occurred alongside bile duct hyperplasia and biliary fibrosis. To assess the role of lymphocytes in ANIT-induced biliary fibrosis, we utilized RAG1 −/− mice, which lack T- and B-lymphocytes. ANIT-induced bile duct injury, indicated by increased serum alkaline phosphatase activity, was reduced in ANIT-exposed RAG1 −/− mice compared to ANIT-exposed wild-type mice. Despite this reduction in biliary injury, ANIT-induced bile duct hyperplasia was similar in wild-type and RAG1 −/− mice. However, hepatic induction of profibrogenic genes including COL1A1, ITGβ6 and TGFβ2 was markedly attenuated in ANIT-exposed RAG1 −/− mice compared to ANIT-exposed wild-type mice. Peribiliary collagen deposition was also reduced in ANIT-exposed RAG1 −/− mice. The results indicate that lymphocytes exacerbate bile duct injury and fibrosis in ANIT-exposed mice without impacting bile duct hyperplasia.

Dysmotility in Esophageal Atresia: Pathophysiology, Characterization, and Treatment

Science.gov (United States)

Faure, Christophe; Righini Grunder, Franziska

2017-01-01

Esophageal dysmotility is almost universal after esophageal atresia (EA) repair and is mainly related to the developmental anomaly of the esophagus. Esophageal dysmotility is involved in the pathophysiology of numerous symptoms and comorbidities associated with EA such as gastroesophageal reflux disease, aspiration and respiratory complications, and symptoms of dysphagia and feeding disorders. High-resolution esophageal manometry (HREM) has facilitated the characterization of the dysmotility, but there is an incomplete correlation between symptoms and manometrical patterns. Impedance coupled to HREM should help to predict the clinical outcome and therefore personalize patient management. Nowadays, the management of esophageal dysmotility in patients with EA is essentially based on treatment of associated inflammation related to peptic or eosinophilic esophagitis. PMID:28620599

Pulmonary ventilation and perfusion abnormalities and ventilation perfusion imbalance in children with pulmonary atresia or extreme tetralogy of Fallot

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Dowdle, S.C.; Human, D.G.; Mann, M.D. (Univ. of Cape Town (South Africa))

1990-08-01

Xenon-133 lung ventilation and perfusion scans were done preoperatively after cardiac catheterization and cineangiocardiography in 19 children; 6 had pulmonary atresia with an intact ventricular septum and hypoplastic right ventricle, 4 pulmonary atresia with associated complex univentricular heart, and 9 extreme Tetralogy of Fallot. The four patients with discrepancies in the sizes of the left and right pulmonary arteries on angiography had marked asymmetry of pulmonary perfusion and ventilation-perfusion imbalance on scintigraphy. Similar degrees of asymmetry and imbalance were present in 6 of the 15 children with equal-size pulmonary vessels. Asymmetry of pulmonary perfusion and ventilation-perfusion imbalance were associated with a poor prognosis.

Pulmonary ventilation and perfusion abnormalities and ventilation perfusion imbalance in children with pulmonary atresia or extreme tetralogy of Fallot

International Nuclear Information System (INIS)

Dowdle, S.C.; Human, D.G.; Mann, M.D.

1990-01-01

Xenon-133 lung ventilation and perfusion scans were done preoperatively after cardiac catheterization and cineangiocardiography in 19 children; 6 had pulmonary atresia with an intact ventricular septum and hypoplastic right ventricle, 4 pulmonary atresia with associated complex univentricular heart, and 9 extreme Tetralogy of Fallot. The four patients with discrepancies in the sizes of the left and right pulmonary arteries on angiography had marked asymmetry of pulmonary perfusion and ventilation-perfusion imbalance on scintigraphy. Similar degrees of asymmetry and imbalance were present in 6 of the 15 children with equal-size pulmonary vessels. Asymmetry of pulmonary perfusion and ventilation-perfusion imbalance were associated with a poor prognosis

A forgotten biliary stent for 17 years: Presented with perforated gallbladder and stentolith

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Tejas J Patel

2014-01-01

Full Text Available Endoscopic biliary stent placement is a well-established, safe, and minimally invasive modality for the treatment of choledocholithiasis and other biliary diseases. Over the past decade, there has been an increase in its prevalence and use. We present an unusual case of forgotten biliary stent for 17 years who presented now with gangrenous cholecystitis and sealed the perforation. A large stentolith had also developed which was cleared with endoscopic retrograde cholengiography and re-stenting followed by laparoscopic cholecystectomy.

Long-term pulmonary function in esophageal atresia-A case-control study

DEFF Research Database (Denmark)

Pedersen, Rikke N; Markøv, Simone; Kruse-Andersen, Søren

2017-01-01

BACKGROUND: Esophageal atresia (EA) is a congenital anomaly associated with substantial pulmonary morbidity throughout childhood. AIM: The aim of this study was to evaluate pulmonary complications among 59 five to 15-year-old children and adolescents with surgically corrected congenital EA. METHO...... ventilatory impairment occurring in EA is probably due to poor lung growth after thoracotomy. No single factor predicted ventilatory impairment in children and adolescents with EA. Pediatr Pulmonol. 2016; 9999:XX-XX. © 2016 Wiley Periodicals, Inc....

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.

Pancreatic Calculus Causing Biliary Obstruction: Endoscopic Therapy for a Rare Initial Presentation of Chronic Pancreatitis.

Science.gov (United States)

Shetty, Anurag J; Pai, C Ganesh; Shetty, Shiran; Balaraju, Girisha

2015-09-01

Biliary obstruction in chronic calcific pancreatitis (CCP) is often caused by inflammatory or fibrotic strictures of the bile duct, carcinoma of head of pancreas or less commonly by compression from pseudocysts. Pancreatic calculi causing ampullary obstruction and leading to obstructive jaundice is extremely rare. The medical records of all patients with CCP or biliary obstruction who underwent endoscopic retrograde cholangiopancreatography (ERCP) over 4 years between 2010-2014 at Kasturba Medical College, Manipal were analyzed. Five patients of CCP with impacted pancreatic calculi at the ampulla demonstrated during ERCP were identified. All 5 presented with biliary obstruction and were incidentally detected to have CCP when evaluated for the same; 3 patients had features of cholangitis. All the patients were managed successfully by endoscopic papillotomy and extraction of pancreatic calculi from the ampulla with resolution of biliary obstruction. Pancreatic calculus causing ampullary obstruction, though very rare, should be considered as a possibility in patients with CCP complicated by biliary obstruction. Endoscopic therapy is affective in the resolution of biliary obstruction in such patients.

Pancreatitis of biliary origin, optimal timing of cholecystectomy (PONCHO trial: study protocol for a randomized controlled trial

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Bouwense Stefan A

2012-11-01

Full Text Available Abstract Background After an initial attack of biliary pancreatitis, cholecystectomy minimizes the risk of recurrent biliary pancreatitis and other gallstone-related complications. Guidelines advocate performing cholecystectomy within 2 to 4 weeks after discharge for mild biliary pancreatitis. During this waiting period, the patient is at risk of recurrent biliary events. In current clinical practice, surgeons usually postpone cholecystectomy for 6 weeks due to a perceived risk of a more difficult dissection in the early days following pancreatitis and for logistical reasons. We hypothesize that early laparoscopic cholecystectomy minimizes the risk of recurrent biliary pancreatitis or other complications of gallstone disease in patients with mild biliary pancreatitis without increasing the difficulty of dissection and the surgical complication rate compared with interval laparoscopic cholecystectomy. Methods/Design PONCHO is a randomized controlled, parallel-group, assessor-blinded, superiority multicenter trial. Patients are randomly allocated to undergo early laparoscopic cholecystectomy, within 72 hours after randomization, or interval laparoscopic cholecystectomy, 25 to 30 days after randomization. During a 30-month period, 266 patients will be enrolled from 18 hospitals of the Dutch Pancreatitis Study Group. The primary endpoint is a composite endpoint of mortality and acute re-admissions for biliary events (that is, recurrent biliary pancreatitis, acute cholecystitis, symptomatic/obstructive choledocholithiasis requiring endoscopic retrograde cholangiopancreaticography including cholangitis (with/without endoscopic sphincterotomy, and uncomplicated biliary colics occurring within 6 months following randomization. Secondary endpoints include the individual endpoints of the composite endpoint, surgical and other complications, technical difficulty of cholecystectomy and costs. Discussion The PONCHO trial is designed to show that early

Tracheal Atresia with Segmental Esophageal Duplication: An Unusual Anatomic Arrangement.

Science.gov (United States)

Gaerty, Kirsten; Thomas, Joseph T; Petersen, Scott; Tan, Edwin; Kumar, Sailesh; Gardener, Glenn; Armes, Jane

2016-01-01

An unusual anatomic configuration of segmental tracheal agenesis/atresia with esophageal duplication on autopsy in a fetus that demised in utero at 29 weeks is reported. The mother was scanned initially for a cardiac anomaly at 20 weeks and on follow-up scan at 27 weeks had polyhydramnios and underwent amnioreduction. The final autopsy diagnosis was vertebral, ano-rectal, cardiac, tracheoesophageal, renal, and limb malformations (VACTERL). We discuss the autopsy findings along with the embryological mechanisms and compare the configuration with Floyd's classification for tracheal agenesis. The difficulties in prenatal diagnosis are discussed.

Manganese accumulation in the brain: MR imaging

Energy Technology Data Exchange (ETDEWEB)

Uchino, A.; Nomiyama, K.; Takase, Y.; Nakazono, T.; Nojiri, J.; Kudo, S. [Saga Medical School, Department of Radiology, Saga (Japan); Noguchi, T. [Kyushu University, Department of Clinical Radiology, Graduate School of Medicine, Fukuoka (Japan)

2007-09-15

Manganese (Mn) accumulation in the brain is detected as symmetrical high signal intensity in the globus pallidi on T1-weighted MR images without an abnormal signal on T2-weighted images. In this review, we present several cases of Mn accumulation in the brain due to acquired or congenital diseases of the abdomen including hepatic cirrhosis with a portosystemic shunt, congenital biliary atresia, primary biliary cirrhosis, congenital intrahepatic portosystemic shunt without liver dysfunction, Rendu-Osler-Weber syndrome with a diffuse intrahepatic portosystemic shunt, and patent ductus venosus. Other causes of Mn accumulation in the brain are Mn overload from total parenteral nutrition and welding-related Mn intoxication. (orig.)

Questionnaire Based Assessment of Risk Factors for Primary Biliary Cirrhosis

Science.gov (United States)

Lammert, Craig; Nguyen, Douglas L.; Juran, Brian D.; Schlicht, Erik; Larson, Joseph J.; Atkinson, Elizabeth J.; Lazaridis, Konstantinos N.

2013-01-01

Background Primary Biliary Cirrhosis is a cholestatic liver disease characterized by immune-mediated destruction of bile ducts. Its pathogenesis is largely unknown, although complex interactions between environment and genetic predisposition are proposed. Aims Identify disease risk factors using a detailed patient questionnaire and compare study findings to 3 published reports. Methods Questionnaire data were prospectively collected from 522 cases and 616 controls of the Mayo Clinic Primary Biliary Cirrhosis Genetic Epidemiology Registry. Case and control responses were compared using logistic regression, adjusting for recruitment age, sex, and education level. Results Cases reported ever regularly smoking cigarettes more frequently than controls (P < 0.001). History of urinary tract infection (UTI) was similar between groups; however, cases reported multiple UTIs more commonly than controls (P < 0.001). Frequency of other autoimmune disease was higher in cases than controls (P < 0.001). As well, prevalence of primary biliary cirrhosis among first-degree relatives was higher in case families than control families (P < 0.001). Conclusions Our study confirms prior reported risk factors associated with disease risk. Given the potential importance of gene and environment interactions, further examination of environmental risk factors considering genetic background may provide new insight into primary biliary cirrhosis pathogenesis. PMID:23490343

Interventional drainage technique for patients with multiple biliary tracts obstruction

International Nuclear Information System (INIS)

Xie Zonggui; Yi Yuhai; Zhang Xuping; Zhang Lijun

2000-01-01

Objective: To evaluate the methodology and effectiveness of interventional biliary drainage for patients with multiple biliary tract obstruction (MBO). Methods: Twenty-one patients with MBO caused by cholangiocarcinoma in 13 cases, primary hepatocellular carcinoma in 5 cases and porta hepatic metastases in 3 cases were included. According to types of biliary tract occlusion, the authors performed different combined interventional draining procedures. That is, thirteen cases were performed with right and left bile duct stent implantation respectively; three cases with stent insertion between left and right bile ducts and catheter for external draining in right bile duct; three cases with right bile duct stent placement and catheter for external draining in left bile duct; two cases with anterior right bile tract stent placement and posterior right bile tract for external draining while left bile duct for internal (one case) or external (one case) draining. Results: All together 36 stents were implanted in 21 patients. 35 stents have obtained satisfactory internal draining function and one stent has not shown function due to malposition. Jaundice disappeared completed in 19 of 21 cases, and disappeared incompletely in 2 cases. Conclusions: Multiform biliary internal and/or external drainage is effective for most patients with MBO

Lymph node metastasis in the biliary tract carcinoma: CT evaluation

International Nuclear Information System (INIS)

Lee, Byung Hee; Lee, Ah Ra; Kim, Kie Hwan; Do, Young Soo; Chin, Soo Yil

1994-01-01

The primary biliary carcinoma is usually unresectable at presentation, because of early lymphatic spread. To determine the incidence and the spread pattern of lymph node metastases according to the location of the primary tumor, we analyzed the CT scans of the patients with primary biliary adenocarcinoma. We reviewed the CT scans of 92 patients with pathologically proven primary biliary adenocacinima, including 45 peripheral cholangiocarcinomas, 22 hilar cholangiocarcinomas, 18 gallbladder carcinomas, and 7 common bile duct carcinomas. Positive adenopathy was diagnosed when the node exceeded 10 mm in short axis. The overall incidence of nodal metastases was 59.8% (55/92); 66.7% in peripheral cholangiocarcinoma, 54.5% in hilar cholangiocarcinoma, 55.6% in gallbladder carcinoma, and 42.9% in common bile duct carcinoma. The most commonly involved nodal group was the lesser omentum, followed by the celiac, periaortic, and peripancreatic group. The phrenic node group was only involved in the cases with the peripheral or hilar cholangiocarcinoma. The primary biliary carcinoma has a high incidence of lymph node metastases at the time of diagnosis, and shows different nodal spread pattern according to the location of the primary tumor. Involvement of the phrenic node was limited to the peripheral and hilar cholangiocarcinoma

Lymph node metastasis in the biliary tract carcinoma: CT evaluation

Energy Technology Data Exchange (ETDEWEB)

Lee, Byung Hee; Lee, Ah Ra; Kim, Kie Hwan; Do, Young Soo; Chin, Soo Yil [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

1994-07-15

The primary biliary carcinoma is usually unresectable at presentation, because of early lymphatic spread. To determine the incidence and the spread pattern of lymph node metastases according to the location of the primary tumor, we analyzed the CT scans of the patients with primary biliary adenocarcinoma. We reviewed the CT scans of 92 patients with pathologically proven primary biliary adenocacinima, including 45 peripheral cholangiocarcinomas, 22 hilar cholangiocarcinomas, 18 gallbladder carcinomas, and 7 common bile duct carcinomas. Positive adenopathy was diagnosed when the node exceeded 10 mm in short axis. The overall incidence of nodal metastases was 59.8% (55/92); 66.7% in peripheral cholangiocarcinoma, 54.5% in hilar cholangiocarcinoma, 55.6% in gallbladder carcinoma, and 42.9% in common bile duct carcinoma. The most commonly involved nodal group was the lesser omentum, followed by the celiac, periaortic, and peripancreatic group. The phrenic node group was only involved in the cases with the peripheral or hilar cholangiocarcinoma. The primary biliary carcinoma has a high incidence of lymph node metastases at the time of diagnosis, and shows different nodal spread pattern according to the location of the primary tumor. Involvement of the phrenic node was limited to the peripheral and hilar cholangiocarcinoma.

Combined atresia of one left-sided and one right-sided cardiac valve in a premature newborn.

Science.gov (United States)

Roberts, William C; Sing, Alan C; Guileyardo, Joseph M

2017-10-01

Described herein is the heart of a 2-day-old newborn, the product of a 25-week gestation, with atresia of two cardiac valves, one on the right side and one on the left side, apparently a previously undescribed entity.

Early laparoendoscopic rendezvous for acute biliary pancreatitis: preliminary results.

Science.gov (United States)

Borzellino, G; Lombardo, F; Minicozzi, A M; Donataccio, M; Cordiano, C

2010-02-01

Early restored patency of the papilla has been hypothesized to reduce complications and mortality of acute biliary pancreatitis. The aim of this study was to evaluate the role of urgent laparoscopic cholecystectomy with intraoperative cholangiography and rendezvous when necessary in acute biliary pancreatitis natural history. Patients observed in the early stage of an acute biliary pancreatitis were included in the study. Operative risk assessment based on American Society of Anesthesiologists (ASA) score allowed the performance of urgent laparoscopic cholecystectomy within 72 h from onset of symptoms in 55 patients and a delayed intervention during the same admission in 21 patients. Intraoperative cholangiography was performed in all cases, and clearance of common bile duct was performed by flushing when possible, or rendezvous when necessary. Evolution of pancreatitis was evaluated with clinical and radiological monitoring. Urgent laparoscopic cholecystectomy was performed in all cases without conversion. At intraoperative cholangiography common bile duct was free in 25 patients, a papillary spasm was observed in 9, and common bile duct stones in 21 patients. Patency of the papilla was restored by flushing in 13 patients, while a rendezvous was necessary in 17 patients. The rate of organ failure and pancreatic necrosis was 1.8%, overall mortality was 1.8%, and overall morbidity 21.8%. No infectious complications of peripancreatic collections were observed. Urgent laparoscopic cholecystectomy with selective intraoperative rendezvous may be considered as a treatment option in the early stage of acute biliary pancreatitis.

Endoscopic management of hilar biliary strictures

Science.gov (United States)

Singh, Rajiv Ranjan; Singh, Virendra

2015-01-01

Hilar biliary strictures are caused by various benign and malignant conditions. It is difficult to differentiate benign and malignant strictures. Postcholecystectomy benign biliary strictures are frequently encountered. Endoscopic management of these strictures is challenging. An endoscopic method has been advocated that involves placement of increasing number of stents at regular intervals to resolve the stricture. Malignant hilar strictures are mostly unresectable at the time of diagnosis and only palliation is possible.Endoscopic palliation is preferred over surgery or radiological intervention. Magnetic resonance cholangiopancreaticography is quite important in the management of these strictures. Metal stents are superior to plastic stents. The opinion is divided over the issue of unilateral or bilateral stenting.Minimal contrast or no contrast technique has been advocated during endoscopic retrograde cholangiopancreatography of these patients. The role of intraluminal brachytherapy, intraductal ablation devices, photodynamic therapy, and endoscopic ultrasound still remains to be defined. PMID:26191345

Outcomes and risk factors for cancer patients undergoing endoscopic intervention of malignant biliary obstruction

OpenAIRE

Haag, Georg-Martin; Herrmann, Thomas; Jäger, Dirk; Stremmel, Wolfgang; Schemmer, Peter; Sauer, Peter; Gotthardt, Daniel Nils

2015-01-01

Background: Malignant bile duct obstruction is a common problem among cancer patients with hepatic or lymphatic metastases. Endoscopic retrograde cholangiography (ERC) with the placement of a stent is the method of choice to improve biliary flow. Only little data exist concerning the outcome of patients with malignant biliary obstruction in relationship to microbial isolates from bile. Methods: Bile samples were taken during the ERC procedure in tumor patients with biliary obstruction. Clin...

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

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.