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Sample records for congenital cystic biliary

  1. 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.

  2. Congenital cystic eyeball

    Directory of Open Access Journals (Sweden)

    Gupta V

    1990-01-01

    Full Text Available A rare case of histopathologically proved case of congenital cystic eye in a one day old girl is described. It was an unusually large cystic mass bulging forwards stretching the upperlid. There was no rudimentary eyeball in the orbit. The cystic eye′s predilection for the left eye has been pointed out for the first time in this article.

  3. Congenital cystic lung malformations

    International Nuclear Information System (INIS)

    Stoever, B.; Scheer, I.; Bassir, C.; Chaoui, R.; Henrich, W.; Schwabe, M.; Wauer, R.

    2006-01-01

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  4. 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.

  5. Congenital cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Chaudhry, A.K.; Azam, M.; Maqsood, R.; Naz, B.; Salam, A.

    2003-01-01

    This case report presents the clinical picture, diagnostic methodology and surgical treatment of a female child who presented with chronic cough and dyspnoea due to congenital malformation of lung. A discussion of diagnosis and management is presented at the end. (author)

  6. Congenital cystic adenomatoid lung malformation of newborn

    International Nuclear Information System (INIS)

    Reither, M.; Peltner, H.U.; Weigel, W.; Braune, M.; Heiming, E.

    1980-01-01

    The congenital cystic adenomatoid malformation (CCAM) of the newborn is a particular form among the cystic disorders of the lung. The clinical findings, illustrated by four cases, and especially the roentgenographic symptoms are typical. Different radiologic examinations, including the computertomography, are discussed. The differential diagnosis of the disease is various, and therefore a correct and on time diagnosis is necessary, because the prognosis of the patient depends on an adequate therapy. (orig.) [de

  7. Congenital Cystic Adenomatoid Malformation of Lung-Rare Case Report

    Directory of Open Access Journals (Sweden)

    N. S. Kamakeri

    2016-10-01

    Full Text Available Congenital cystic adenomatoid malformation of lung associated with Cystic dysplasia of kidney, cystic disease of liver with mixed gonadal dysgenesis is rare and is not reported in literature so far. Hence an attempt is made to present this rarest entity.

  8. Congenital cystic lung malformations; Konnatale zystische Lungenfehlbildungen

    Energy Technology Data Exchange (ETDEWEB)

    Stoever, B.; Scheer, I.; Bassir, C. [Klinik fuer Strahlenheilkunde, Berlin (Germany). Abt. Paediatrische Radiologie, Charite; Mau, H. [Campus Virchow-Klinikum, Klinik fuer Kinderchirurgie, Berlin (Germany); Chaoui, R. [Campus Mitte, Klinik fuer Geburtsmedizin, Berlin (Germany); Henrich, W. [Campus Virchow-Klinikum, Klinik fuer Geburtsmedizin, Berlin (Germany); Schwabe, M. [Campus Mitte, Inst. fuer Pathologie, Berlin (Germany); Wauer, R. [Campus Mitte, Klinik fuer Neonatologie, Berlin (Germany)

    2006-04-15

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  9. Radiological findings in congenital cystic disease of the lung in infancy

    International Nuclear Information System (INIS)

    Heymer, R.; Benz-Bohm, G.; Arnold, G.; Koeln Univ.

    1982-01-01

    Three infants with congenital cystic disease of the lung are described. They consisted of a solitary bronchial cyst, an adenomatous cystic malformation and congenital lobar emphysema. The radiological appearance and differential diagnosis of these three conditions are discussed. (orig.) [de

  10. Congenital cystic masses of the face and neck: CT evaluation

    International Nuclear Information System (INIS)

    Chung, Hae Gyeong; Kim, Hyung Jin; Kim, Jae Hyoung; Hwang, Eui Gee; Jeon, Sea Young; Kim, Sun Young; Chung, Sung Hoon

    1991-01-01

    Recognition of the congenital cystic masses of the face and neck is important because they are usually benign, and can be completely cured by surgical excision. We retrospective analyzed CT scan of 18 surgically proven congenital cystic masses of the face and neck. The cases included 5 thyroglossal duct cysts, 4 cystic hygromas, 5 dermoid cysts, 1 branchial cleft cyst, and 3 fissural cysts of the face. Of five cases of thyroglossal duct cysts, CT showed either a well-marginated, rim enhancing unilocular cystic mass (n=3), or a diffuse but heterogeneous highly enhancing soft tissue mass (n=2). The latter two cases were confirmed later as infected thyroglossal duct cysts. Four cases of cystic hygromas were seen as either an irregularly-marginated (n=3) or a well-marginated (n=1) rim enhancing multiseptated cystic mass with a fluid-fluid level. Five cases of dermoid cysts appeared as well-marginated rim enhancing unilocular ovoid masses. The content of each mass was predominantly fluid in four cases, of which additional solid components were found in two, and interspersed fat globules in one. One case was composed of a homogeneous fatty density. One case of branchial cleft cyst was in the anterior triangle near the left mandibular angle, and appeared as a well-marginated enhancing cystic mass with a thick rim. In this case displacement of the adjacent structures was noted also. All three cases of fissural cysts of the face were seen as a well-marginated, rim enhancing cystic mass, causing a smooth pressure erosion of the adjacent bones. We conclude that CT is useful for the evaluation of the congenital cystic masses of the face and neck, because it can differentiate various forms of the congenital lesions and is able to clearly reveal the relation of the mass to the adjacent structures

  11. Congenital cystic masses of the face and neck: CT evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Hae Gyeong; Kim, Hyung Jin; Kim, Jae Hyoung; Hwang, Eui Gee; Jeon, Sea Young; Kim, Sun Young; Chung, Sung Hoon [Gyeongsang National University, Jinju (Korea, Republic of)

    1991-09-15

    Recognition of the congenital cystic masses of the face and neck is important because they are usually benign, and can be completely cured by surgical excision. We retrospective analyzed CT scan of 18 surgically proven congenital cystic masses of the face and neck. The cases included 5 thyroglossal duct cysts, 4 cystic hygromas, 5 dermoid cysts, 1 branchial cleft cyst, and 3 fissural cysts of the face. Of five cases of thyroglossal duct cysts, CT showed either a well-marginated, rim enhancing unilocular cystic mass (n=3), or a diffuse but heterogeneous highly enhancing soft tissue mass (n=2). The latter two cases were confirmed later as infected thyroglossal duct cysts. Four cases of cystic hygromas were seen as either an irregularly-marginated (n=3) or a well-marginated (n=1) rim enhancing multiseptated cystic mass with a fluid-fluid level. Five cases of dermoid cysts appeared as well-marginated rim enhancing unilocular ovoid masses. The content of each mass was predominantly fluid in four cases, of which additional solid components were found in two, and interspersed fat globules in one. One case was composed of a homogeneous fatty density. One case of branchial cleft cyst was in the anterior triangle near the left mandibular angle, and appeared as a well-marginated enhancing cystic mass with a thick rim. In this case displacement of the adjacent structures was noted also. All three cases of fissural cysts of the face were seen as a well-marginated, rim enhancing cystic mass, causing a smooth pressure erosion of the adjacent bones. We conclude that CT is useful for the evaluation of the congenital cystic masses of the face and neck, because it can differentiate various forms of the congenital lesions and is able to clearly reveal the relation of the mass to the adjacent structures.

  12. [Symptoms, diagnosis and treatment of pancreaticobiliary maljunction associated with congenital cystic dilatation of bile duct].

    Science.gov (United States)

    Funabiki, T; Matsubara, T; Ochiai, M

    1996-08-01

    The concept of congenital biliary dilatation (CBD) was established by Alonso-Lej whose classification has been widely accepted and become a standard. Todani, Komi and several investigators then added additional subclassifications and/or made a proposal of new classification. The trias, abdominal pain, abdominal mass and jaundice, had been characteristically found in these cases, but recently asymptomatic cases are often found. Many reports have shown that the cases with cystic dilation of the biliary duct frequently associated with biliary carcinoma and pancreaticobiliary maljunction (PBM), an anomalous union of pancreatic duct with biliary tree since the Rabbit's first report. Whereas extracorporeal ultrasound and CT scan are minimally invasive diagnostic procedures for these cases, a direct cholangiography, ERCP and PTC are the diagnostic procedures that make a definite diagnosis for the appropriate treatment. Recently, endoscopic ultrasound (EUS), helical CT and magnetic resonance cholangiopan-creatography (MRCP) have drawn an attention since these methods are less invasive than those ever being used. In particular, MRCP will be suitable for the screening of preoperative examination. Whereas cystoduodenostomy used to be performed in the past for these cases, the resection of dilatted bule duct along with the diversion of bile from pancreatic juice should be performed to prevent biliary carcinoma. Cholecystectomy along with the resection of bile duct and the biliary diversion from pancreatic juice should also be performed for cases of PBM without CBD because mutagenicity of bile mixed with pancreatic juice and K-ras point mutation in noncancerous bile duct epithelium in cases of PBM without CBD were demonstrated.

  13. Prenatal diagnosis of congenital cystic adenomatoid malformation of the lung: A case report

    International Nuclear Information System (INIS)

    Shin, Hyun Ja; Shin, M. J.; Yoo, Y. J.; Park, J. M.; Kim, J. R.

    1990-01-01

    Congenital cystic adenomatoid malformation is one of a rare congenital malformation usually unilateal in volving a part of lobe or a whole lobe of the fetal lung, characterized by excessive growing of terminal respiratory element. We made a prenatal diagnosis in a case of congenital cystic adenomdtoid malformation with diffuse bilateral involvement, Stocker Type III which is associated with fetal hydrops

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

    Science.gov (United States)

    Cohen, J; Schanen, N C

    2000-01-01

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

  15. Congenital cystic adenomatoid malformation: case presentation in a two months old infant

    International Nuclear Information System (INIS)

    Aqrabawi, H.E.; Shabatat, M.; Abbadi, B.M.

    2015-01-01

    Congenital cystic adenomatoid malformation (CCAM) is a rare abnormality of lung development; it is increasingly detected by the routine ultrasound scan during pregnancy. The severity of the abnormality is very variable. Herein, we present a case of congenital cystic adenomatoid malformation that presented in a two months old infant who had normal initial chest X rays. (author)

  16. Inadvertent chest tube insertion in congenital cystic adenomatoid malformation and congenital lobar emphysema-highlighting an important problem

    International Nuclear Information System (INIS)

    Prabhu, Shailesh M; Choudhury, Subhasis Roy; Solanki, Ravi S; Shetty, Gurucharan S; Agarwala, Surenderkumar

    2013-01-01

    Chest tube insertion in congenital cystic lung lesions is an important problem in children with acute respiratory distress having a cystic lucent lesion on chest radiograph. To evaluate the imaging findings and complications in cases of congenital cystic lung lesions with chest tube insertion and suggest the role of appropriate imaging for management of these patients. Chest radiographs and CT scans of children with congenital cystic lung lesions who had inadvertent chest tube insertion preoperatively were retrospectively reviewed for imaging appearances and complications. Fifteen patients comprising 10 cases of congenital cystic adenomatoid malformation (CCAM) and 5 cases of congenital lobar emphysema (CLE) were included. Majority of the cases were infants. CCAM was misdiagnosed as complicated pneumatocele (n = 5) and pneumothorax (n = 5), while CLE was misdiagnosed as tension pneumothorax (n = 5) on the chest radiograph findings. Final diagnosis was made on CT and operative findings with histopathology. Complications noted were pneumothorax, hydropneumothorax, and infection in cases of CCAM, and change in imaging appearance and pneumothorax in cases of CLE. Chest tube insertion in congenital cystic lesions increases the rate of associated complications. Chest CT has a definite role in early diagnosis and deciding appropriate management in these cases

  17. [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.

  18. Pleural pneumatocoeles mimicking congenital cystic adenomatoid malformation of the lung. A case report

    International Nuclear Information System (INIS)

    Aurora, P.; McHugh, K.

    1998-01-01

    We present the plain radiographic and CT appearances of large intrapleural pneumatocoeles in a 13-week-old infant, resulting in compression atelectasis of the left upper and lower lobes, and mimicking congenital cystic adenomatoid malformation. (orig.)

  19. Association of two respiratory congenital anomalies: tracheal diverticulum and cystic adenomatoid malformation of the lung

    International Nuclear Information System (INIS)

    Restrepo, S.; Villamil, M.A.; Rojas, I.C.; Lemos, D.F.; Echeverri, S.; Angarita, M.; Triana, G.

    2004-01-01

    Many associations of congenital anomalies of the respiratory system have been reported, but the combination of tracheal diverticulum and cystic adenomatoid malformation (CCAM) is unique. We present a patient with these two anomalies and analyze their embryological correlation. (orig.)

  20. [Autosomal-recessive renal cystic disease and congenital hepatic fibrosis: clinico-anatomic case].

    Science.gov (United States)

    Rostol'tsev, K V; Burenkov, R A; Kuz'micheva, I A

    2012-01-01

    Clinico-anatomic observation of autosomal-recessive renal cystic disease and congenital hepatic fibrosis at two fetuses from the same family was done. Mutation of His3124Tyr in 58 exon of PKHD1 gene in heterozygous state was found out. The same pathomorphological changes in the epithelium of cystic renal tubules and bile ducts of the liver were noted. We suggest that the autopsy research of fetuses with congenital abnormalities, detected after prenatal ultrasonic screening, has high diagnostic importance.

  1. 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...

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

    International Nuclear Information System (INIS)

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

    2013-01-01

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

  3. Rapidly Evoluting Congenital Cystic Neuroblastoma in a Neonate

    Energy Technology Data Exchange (ETDEWEB)

    Yun, Tae Jun; Kim, Myung Jun; Han, Seok Joo; Lee, Mi Jung [Severance Children' s Hospital, Yonsei University, College of Medicine, Seoul(Korea, Republic of)

    2012-08-15

    Perinatal detection of neonatal suprarenal masses has increased. Here, we report an unusual case of an adrenal cystic neuroblastoma that presented as a purely cystic lesion upon initial postnatal ultrasonography (US) and showed rapid evolution to a mixed cystic and solid mass during follow-up US and MRI. We suggest a short-term (two weeks) follow-up US for neonatal adrenal cystic lesions, even if they appear as purely cystic.

  4. Diagnosis of congenital cystic adenomatoid malformation of the lung in newborn infants and children

    NARCIS (Netherlands)

    Heij, H. A.; Ekkelkamp, S.; Vos, A.

    1990-01-01

    Seventeen patients were diagnosed as having congenital cystic adenomatoid malformation of the lung during 1970-88. One case was associated with congenital diaphragmatic hernia and the child died before operation. The other 16 children underwent successful surgery. The patients presented in one of

  5. Dynamic MR cholangiography after fatty meal loading. Cystic contractility and dynamic evaluation of biliary stasis

    International Nuclear Information System (INIS)

    Omata, Takayuki; Saito, Kazuhiro; Kotake, Fumio; Mizokami, Yuji; Matsuoka, Takeshi; Abe, Kimihiko

    2002-01-01

    Dynamic MR cholangiography was conducted on patients with cholelithiasis or choledocholithiasis who had consumed a fatty test meal (Molyork) and the cystic contractility and dynamics of biliary stasis was evaluated. The subjects were 25 with intracystic cholelithiasis, 10 with choledocholithiasis and 10 normal controls. For an imaging sequence, the rapid acquisition with relaxation enhancement (RARE) method was employed and imaging was conducted for 40 min (every 30 s following Molyork administration) without breath-holding. The gallbladder contraction ratio was computed and the contractile ratio for the common bile duct was calculated. To determine the bile flow to the duodenum, the high-intensity signal, indicating the flow from the lower common bile duct, and perfusion of the duodenum were observed in dynamic mode on the monitor with the naked eye and interpreted as positive bile flow. The frequency of this flow was visually monitored. The gallbladder contractile ratio was significantly reduced in patients with cholelithiasis or choledocholithiasis compared with the controls. In a comparison with the normal controls, no sequential changes were noted in the mean contractile ratio of the common bile duct of the patients with cholelithiasis or choledocholithiasis. The mean frequency of bile flow observed for each 40 min period was 13±2.4, 6±2.2, and 4±1.3 times for the controls, those with intracystic cholelithiasis, and those with choledocholithiasis, respectively. Compared with the controls, the latter two patient groups showed evident reductions in the frequency of bile flow to the duodenum (p<0.001). Dynamic MRC combined with Molyork loading makes it possible to compute cystic contractile ratios and perform a dynamic examination of bile flow under non-invasive, near-physiological conditions. (author)

  6. Large congenital cystic asdenomatous malformation of the lung in a newborn

    Directory of Open Access Journals (Sweden)

    İlyas Yolbaş

    2013-12-01

    Full Text Available Congenital cystic adenomatous malformation (CCAM oflung is a rare form of congenital hamartomatous lesionsof the lung consisting of cysts filled with air. The generalclinic presentation of CCAM is dyspnea in newborns.CCAM may mimic congenital pneumonia or respiratorydistress syndrome. After the delivery, the newborn malewho had low Apgar score and severe respiratory distresswas intubated and admitted to neonatal intensive careunit. Patient was ventilated for 50 days and weaned fromthe mechanical ventilator at 50th day. Type II CCAM of thelung was diagnosed according to the chest radiographsand computed tomography scan signs. Although the surgeonssuggested lobectomy considering the patient’s notcompletely asymptomatic, family did not accept this operationdue to the risk of death. The patient was dischargedfrom the hospital until the next control.Key word: Congenital cystic adenomatous malformation of lunch, newborn, conservative treatment

  7. Comparison of Clinico-Radiological Features between Congenital Cystic Neuroblastoma and Neonatal Adrenal Hemorrhagic Pseudocyst

    Energy Technology Data Exchange (ETDEWEB)

    Eo, Hong; Kim, Ji Hye; Jang, Kyung Mi; Yoo, So Young [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of); Lim, Gye Yeon [St. Mary' s Hospital Catholic University, Seoul (Korea, Republic of); Kim, Myung Joon [Severance Hospital Yonsei University, Seoul (Korea, Republic of); Kim, Ok Hwa [Ajou University Hospital, Suwon (Korea, Republic of)

    2011-02-15

    To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas

  8. Comparison of Clinico-Radiological Features between Congenital Cystic Neuroblastoma and Neonatal Adrenal Hemorrhagic Pseudocyst

    International Nuclear Information System (INIS)

    Eo, Hong; Kim, Ji Hye; Jang, Kyung Mi; Yoo, So Young; Lim, Gye Yeon; Kim, Myung Joon; Kim, Ok Hwa

    2011-01-01

    To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas

  9. Congenital cystic adenomatoid malformation of the lung: hazards of delayed diagnosis.

    LENUS (Irish Health Repository)

    Collins, Anne M

    2012-02-01

    Congenital cystic adenomatoid malformation is a rare pulmonary developmental anomaly, which typically manifests in neonates and infants. Presentation in adulthood is uncommon, with <60 cases reported in the literature. The majority of cases involve one lobe only. We report a case of type 1 congenital cystic adenomatoid malformation in an adult presenting with a respiratory tract infection and haemoptysis. At thoracotomy, complex cystic masses were noted in the right upper and lower lobes. Lung-sparing surgery, in the form of two segmentectomies and a non-anatomical resection, was performed in order to avoid pneumonectomy. Such presentations may be problematic as potentially incomplete resections may increase the risk of complications and malignant transformation. This suggests the importance of appropriate clinical and radiological follow up.

  10. [Rare case of congenital cystic adenomatoid malformation associated with polycystic kidney disease].

    Science.gov (United States)

    Jabłoński, Janusz; Jankowski, Zbigniew; Sitkiewicz, Anna; Lewandowska, Małgorzata; Andrzejewska, Ewa

    2011-01-01

    Congenital cystic adenomatoid malformation (CCAM) is a rare pulmonary abnormality that results from aberrant fetal lung development. It about 4-26% of cases it can be associated with other congenital abnormalities. We describe a case of congenital cystic adenomatoid malformation 2 associated with polycystic kidney disease. The association of these two congenital malformations is exceptional. Only four similar cases have been reported in the literature. A 2-year-old girl was referred to the Department of Paediatric Surgery and Oncology Medical University of Lodz with pneumonia and left pneumothorax. For three weeks prior to referral the patient was treated with antibiotics. Chest x-ray revealed hyperinflation of left upper lobe with mediastinal shift to right. Computer tomographic scan of the lung revealed multiple cyst in the left upper lobe, left-site pneumothorax and mediastinal shift to the right. The patient underwent thoracotomy. Intraoperatively, multiple cysts in the left upper lobe were found and left upper lobectomy was performed. Histologic study was compatible with type 2 congenital cystic adenomatoid malformation. Ultrasound examination showed multilocular cysts in both kidneys. The dimensions of the cysts were: MWR4. 54x45x45 mm and 25x21x24 mm on the left and right sides, respectively. Significant increase in cyst size on the left side was observed. Ten months after first hospitalization resection of the cystic lower pole of the left kidney was performed. The presence of even a single renal cyst in a child with CCAM is an indication for further follow up examinations.

  11. Macrophage recruitment by fibrocystin-defective biliary epithelial cells promotes portal fibrosis in congenital hepatic fibrosis.

    Science.gov (United States)

    Locatelli, Luigi; Cadamuro, Massimiliano; Spirlì, Carlo; Fiorotto, Romina; Lecchi, Silvia; Morell, Carola Maria; Popov, Yury; Scirpo, Roberto; De Matteis, Maria; Amenduni, Mariangela; Pietrobattista, Andrea; Torre, Giuliano; Schuppan, Detlef; Fabris, Luca; Strazzabosco, Mario

    2016-03-01

    Congenital hepatic fibrosis (CHF) is a disease of the biliary epithelium characterized by bile duct changes resembling ductal plate malformations and by progressive peribiliary fibrosis, in the absence of overt necroinflammation. Progressive liver fibrosis leads to portal hypertension and liver failure; however, the mechanisms leading to fibrosis in CHF remain elusive. CHF is caused by mutations in PKHD1, a gene encoding for fibrocystin, a ciliary protein expressed in cholangiocytes. Using a fibrocystin-defective (Pkhd1(del4/del4)) mouse, which is orthologous of CHF, we show that Pkhd1(del4/del4) cholangiocytes are characterized by a β-catenin-dependent secretion of a range of chemokines, including chemokine (C-X-C motif) ligands 1, 10, and 12, which stimulate bone marrow-derived macrophage recruitment. We also show that Pkhd1(del4/del4) cholangiocytes, in turn, respond to proinflammatory cytokines released by macrophages by up-regulating αvβ6 integrin, an activator of latent local transforming growth factor-β1. While the macrophage infiltrate is initially dominated by the M1 phenotype, the profibrogenic M2 phenotype increases with disease progression, along with the number of portal myofibroblasts. Consistent with these findings, clodronate-induced macrophage depletion results in a significant reduction of portal fibrosis and portal hypertension as well as of liver cysts. Fibrosis can be initiated by an epithelial cell dysfunction, leading to low-grade inflammation, macrophage recruitment, and collagen deposition; these findings establish a new paradigm for biliary fibrosis and represent a model to understand the relationship between cell dysfunction, parainflammation, liver fibrosis, and macrophage polarization over time. © 2015 by the American Association for the Study of Liver Diseases.

  12. TWO-STAGE SURGICAL TREATMENT OF A CHILD OF ONE YEAR FROM CONGENITAL HEART DISEASE AND BILIARY CIRRHOSIS

    Directory of Open Access Journals (Sweden)

    S. V. Gautier

    2014-01-01

    Full Text Available Aim: Clinical case of successful two-stage surgical treatment of a 1-year-old child with congenital heart disease and biliary cirrhosis is represented in this article. At the first day of life laparotomy was performed because of high intestinal obstruction. Kasai procedure and Roux-en-Y choledochojejunostomy were per- formed on 12th day and at the end of second month of life, respectively. Liver biopsy showed the signs of biliary cirrhosis. At the same time ventricular septal defect and atrial septal defect with pulmonary hyper- tension were diagnosed. The first step of treatment was the surgical septal defects closure. No complications during procedure, cardiopulmonary bypass and post-operative period were registered. There were no nega- tive effects on liver function after cardiac surgery. 11 months later living-donor liver transplantation was performed without any complications. Patient was discharged at 35th post-transplant day with stable graft function. 

  13. [Congenital cystic lung lesions--review of the literature with three clinical cases].

    Science.gov (United States)

    Slancheva, B; Hitrova, S; Markov, D; Vakrilova, L; Pramatarova, T; Yarukova, N; Brankov, O

    2013-01-01

    Congenital cystic lung lesions are rare. Mainly affects the lower respiratory patishta.i are congenital cystic malformation and adematozna bronchopulmonary sequestration (BPS). The pathogenesis of the occurrence of these malformations is not clear but they have a common clinical course. In most cases, the anomaly is asymptomatic and occurs with infections of the lung during the first year of life. Currently congenital lung lesions were classified into five types and is considered by most authors. The anomaly is due to the abnormal proliferation of terminal bronchioles accompanied by inhibition of alveolar development between 7-17 weeks, obstructed airway dysplasia and metaplasia of normal lung tissue. Early diagnosis is vital in making a medical decision on how to treat CCAM. Associated with abnormalities of the urinary tract, cardiovascular system, gastrointestinal atresia, diaphragmatic hernia skeletal abnormalities. In pregnancies in which prenatal lung lesions weighs registered necessary series of ultrasound examinations to track finding and using the Doppler to assess how the blood supply of the fault. The clinical presentation of malformations is respiratory distress, respiratory infection, and dyspnea. The use of CT and MRA allows better visualization of the pulmonary lesions. With its combination with arteriography and bronchoscopy are used to differentiate CCAM and pulmonary sequestration. We present three cases with lung lesions were born in Neonatologia clinic at the University Hospital of Obstetrics and Gynecology "Maternity" Sofia for the period 2010-2012 three cases CCAMs type 1, operated by 5 meters after birth with a good final outcome without complications in the postoperative period and lack of pulmonary symptoms up to 1 year after birth.

  14. Congenital Lobar Emphysema Associated with Cystic Adenomatoid Malformation Type I: Case Report

    International Nuclear Information System (INIS)

    Morales Riveros, Myriam; Henao, Liliana; Jaramillo B, Lina

    2011-01-01

    Congenital lung abnormalities are a heterogeneous group of alterations in lung development, and many of them are asymptomatic, but is important to know, they must enter the differential diagnosis of patients respiratory distress syndrome. Some of these lesions can coexist, the point where the pulmonary sequestration may present with adenomatoid malformation cystic type II in over 50% of cases. Although the final diagnosis for this histological type of injury is, images play an important role for initial approach. Natural history of these malformations depends lung the size of the lung mass and physiological problem that this causes. The CT multidetector computed expanded knowledge of lung anatomy and improves visualization of the tracheobronchial tree in the pediatric population.

  15. Congenital cystic adenomatoid malformations (CCAM) - prenatal MRI diagnosis: a case report

    International Nuclear Information System (INIS)

    Gagov, E.; Iieva, E.; Gvanska, G.

    2012-01-01

    Full text: Congenital Cystic Adenomatoid Malformation/CCAM (recently termed Congenital Pulmonary Airway Malformation/CPAM) is a rare lung lesion that is believed to result from a cessation of bronchiolar maturation with overgrowth of mesenchymal elements. The differential diagnosis of a mass in the fetal thorax includes CCAM/CPAM, congenital diaphragmatic hernia, and pulmonary sequestration. We present a case of CCAM/CPAM detected on prenatal ultrasound examination with prenatal magnetic resonance imaging (MRI) correlation. A multicystic lesion in the right hemithorax of the fetus was identified on ultrasound at 26th weeks of gestation which was slightly increasing in size on follow-up examinations. MRI was performed for further evaluation and selecting the appropriate management. On MRI the lesion was confined to the right middle lobe, consisting of a single large 3 cm cyst surrounded by multiple smaller cysts and compressed normal parenchyma of the upper and lower lobes of the right lung (type I, Stocker and al. classification). No mediastinal shift or other abnormalities to the contralateral lung were detected. No complications, such as hydrothorax or polyhydramnios were identified. Based on the MR findings postnatal surgical removal of the lung lesion was planned. Continuous weekly ultrasound follow-up examination was recommended. Improvements in magnetic resonance imaging (MRI) now permit diagnostic images of the fetus to be obtained. Ultrasound (US) remains vital in all aspects of fetal imaging but MR serves as a useful second line imaging test. MR imaging can provide excellent tissue contrast with more accurate analysis of the fetal anatomy and superior differentiation between the abnormalities and adjacent structures, thereby allowing early planning of pre- and postnatal management

  16. Fetal lung interstitial tumor: the first Japanese case report and a comparison with fetal lung tissue and congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3.

    Science.gov (United States)

    Yoshida, Mariko; Tanaka, Mio; Gomi, Kiyoshi; Iwanaka, Tadashi; Dehner, Louis P; Tanaka, Yukichi

    2013-10-01

    Fetal lung interstitial tumor, a newly recognized lung lesion in infants, was first reported in 2010. Here, we report the first Japanese case of fetal lung interstitial tumor which was originally diagnosed as atypical congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. A 7-day-old girl was referred to our hospital with respiratory distress and a left lung mass and she subsequently underwent left lower lobectomy. The specimen showed a 5 cm solid mass with a fibrous capsule. Histological examination revealed immature airspaces and interstitium, containing bronchioles and cartilage. The epithelial and interstitial cells contained abundant glycogen granules. Immunohistochemistry showed nuclear/cytoplasmic expression of β-catenin in the epithelial and interstitial cells. β-catenin gene mutations and trisomy 8 were not detected, so a neoplastic origin could not be confirmed. The histological findings were partly consistent with normal fetal lung at the canalicular stage, pulmonary interstitial glycogenosis, and congenital cystic adenomatoid malformation/congenital pulmonary airway malformation type 3. In this report, we compare the above conditions and discuss the pathogenesis of fetal lung interstitial tumor. © 2013 The Authors. Pathology International © 2013 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

  17. A "blind" vascular ring in association with congenital cystic adenomatoid malformation: A case report.

    Science.gov (United States)

    Xia, Bo; Hong, Chun; Tang, Jing; Liu, Cuifen; Yu, Gang

    2017-12-01

    The occurrence of congenital cystic adenomatoid malformation (CCAM) and vascular ring (VR) is extremely rare. We present a case of left CCAM with VR consisting of a left aortic arch and right descending aorta with left tracheal compression causing atelectasis. A high-risk male neonate with the diagnosis of left CCAM was diagnosed at 20 weeks gestational age by antenatal ultrasound. Chest CT revealed multiple cysts in the left inferior lung. Cardiac CT showed VR consisting of a left aortic arch and right descending aorta with left tracheal compression causing atelectasis. left inferior lobectomy was performed. Cardiac CT showed VR consisting of a left aortic arch and right descending aorta with left tracheal compression causing atelectasis. Descending aorta transposition was performed. The patient recovered smoothly and remained asymptomatic during the 12-months of postoperative follow-up period. We report this rare case of CCAM with VR consisting of left aortic arch and right descending aorta with left tracheal compression causing atelectasis. From the findings of this report, early surgical treatment is recommended. Although the prognosis after surgery remained good, second surgery can be avoided if VR was detected early. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  18. Altered intestinal bile salt biotransformation in a cystic fibrosis (Cftr-/-) mouse model with hepato-biliary pathology.

    Science.gov (United States)

    Bodewes, Frank A J A; van der Wulp, Mariëtte Y M; Beharry, Satti; Doktorova, Marcela; Havinga, Rick; Boverhof, Renze; James Phillips, M; Durie, Peter R; Verkade, Henkjan J

    2015-07-01

    Cftr(-/-tm1Unc) mice develop progressive hepato-biliary pathology. We hypothesize that this liver pathology is related to alterations in biliary bile hydrophobicity and bile salt metabolism in Cftr(-/-tm1Unc) mice. We determined bile production, biliary and fecal bile salt- and lipid compositions and fecal bacterial composition of C57BL/6J Cftr(-/-tm1Unc) and control mice. We found no differences between the total biliary bile salt or lipid concentrations of Cftr(-/-) and controls. Compared to controls, Cftr(-/-) mice had a ~30% higher bile production and a low bile hydrophobicity, related to a ~7 fold higher concentration of the choleretic and hydrophilic bile salt ursocholate. These findings coexisted with a significantly smaller quantity of fecal Bacteroides bacteria. Liver pathology in Cftr(-/-tm1Unc) is not related to increased bile hydrophobicity. Cftr(-/-) mice do however display a biliary phenotype characterized by increased bile production and decreased biliary hydrophobicity. Our findings suggest Cftr dependent, alterations in intestinal bacterial biotransformation of bile salts. Copyright © 2014. Published by Elsevier B.V.

  19. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner′s syndrome

    Directory of Open Access Journals (Sweden)

    Jane Jackie David

    2016-01-01

    Full Text Available We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner′s syndrome.

  20. 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.

  1. 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.)

  2. Clinicopathological features and prognosis of mucin-producing bile duct tumor and mucinous cystic tumor of the liver: a multi-institutional study by the Japan Biliary Association.

    Science.gov (United States)

    Kubota, Keiichi; Nakanuma, Yasuni; Kondo, Fukuo; Hachiya, Hiroyuki; Miyazaki, Masaru; Nagino, Masato; Yamamoto, Masakazu; Isayama, Hiroyuki; Tabata, Masami; Kinoshita, Hisafumi; Kamisawa, Terumi; Inui, Kazuo

    2014-03-01

    The aim of this study was to determine the clinicopathological features and surgical outcomes of mucinous cystic neoplasm of the liver (MCN) and mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB). We performed a multi-institutional, retrospective study of patients with MCN or M-IPNB pathologically defined by the presence or absence of an ovarian-like stroma. The M-IPNB and MCN were diagnosed in 119 and nine patients, respectively. MCN was observed in female patients, while M-IPNB produced symptoms of cholangitis. M-IPNBs were classed as low or intermediate grade in 53 cases, high grade in 23 and invasive carcinoma in 43. Fifty-one of the M-IPNBs were the pancreatobiliary type (PT), 33 were the intestinal type (IT), 23 were the oncocytic type (OT), and 12 were the gastric type (GT). The 1-, 5- and 10-year survival rates for the 105 patients with M-IPNB were 96%, 84% and 81%, respectively, while the 5-year survival rate for patients with MCN was 100%. OT and GT M-IPNB had better 10-year survival rates than PT and IT M-IPNB. Although MCN has different features from M-IPNB, both diseases have a good prognosis after resection. The cellular type of M-IPNB appears to predict outcome. © 2013 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

  3. Mesenteric cystic lymphangioma: a congenital and an acquired anomaly? Two cases and a review of the literature

    NARCIS (Netherlands)

    Weeda, Viola B.; Booij, Klaske A. C.; Aronson, Daniel C.

    2008-01-01

    Mesenteric cystic lymphangioma is an uncommon benign abdominal mass. Two cases of mesenteric cystic lymphangioma are presented, both in combination with malrotation and intermittent volvulus. Both mesenteric cystic lymphangiomas were located near the duodenojejunal junction, the usual area of

  4. Mesenteric cystic lymphangioma: a congenital and an acquired anomaly? Two cases and a review of the literature.

    NARCIS (Netherlands)

    Weeda, V.B.; Booij, K.A.; Aronson, D.C.

    2008-01-01

    Mesenteric cystic lymphangioma is an uncommon benign abdominal mass. Two cases of mesenteric cystic lymphangioma are presented, both in combination with malrotation and intermittent volvulus. Both mesenteric cystic lymphangiomas were located near the duodenojejunal junction, the usual area of

  5. Clinical features of congenital cystic disease of the mediastinum. Diagnostic usefulness of computed tomography and magnetic resonance imaging

    International Nuclear Information System (INIS)

    Bando, Masashi; Ishii, Yoshiki; Sugiyama, Yukihiko; Furuse, Makoto; Murayama, Fumio; Sohara, Yasunori

    2000-01-01

    We describe 18 cases of congenital mediastinal cysts, including 4 bronchogenic cysts, 5 pericardial cysts, and 9 thymic cysts. Fifteen of the 18 patients (62.5%) were asymptomatic, and the cysts were found incidentally on a chest radiograph conducted as a part of a physical screening. In 2 of the 4 cases of bronchogenic cysts, the cystic lesions were located in the posterior mediastinum and in the cephalad recess of the azgos vein (CRAzV). In 3 of the 5 cases of pericardial cysts, the lesions were located in the right cardio-phrenic region. In the remaining 2 cases, the cysts were located in the CRAzV and in the posterior mediastinum. Seventeen patients who had histologically proven mediastinal cysts were investigated by magnetic resonance (MR) imaging. In all 3 cases of bronchogenic cysts, MR signals of the cysts were of higher intensity than those of muscle on T1-weighted images. Neither the pericardial cysts nor the thymic cysts showed high signal intensity on T1-weighted images. Macroscopically, the intracystic fluids in all of the bronchogenic cysts contained viscid mucus that probably caused high signal intensities on the T1-weighted images. MR imaging is not only useful in revealing the location of mediastinal cysts, but also in the differential diagnosis of congenital mediastinal cysts. (author)

  6. 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 ...

  7. 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

  8. 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.

  9. Congenital dilatation of the large and segmental intrahepatic bile ducts (Caroli's disease in two Golden retriever littermates : clinical communication

    Directory of Open Access Journals (Sweden)

    R.D. Last

    2006-06-01

    Full Text Available Two, sibling, male Golden retriever puppies, 13 weeks of age, were presented with congenital biliary cysts of the liver involving both hepatic and segmental bile ducts, as well as bilateral polycystic kidney disease. Ultrasonography of the livers of both pups demonstrated segmental cystic lesions that were contiguous with the bile ducts. Histopathology revealed cystic ectatic bile duct hyperplasia and dysplasia with variable portal fibrosis in the liver, while in the kidneys there were radially arranged, cylindrically dilated cysts of the collecting ducts, which extended through the medulla and cortex. This pathology was compatible with that of congenital dilatation of the large and segmental bile ducts (Caroli's disease described in humans, dogs and rats. In humans Caroli's disease has an autosomal recessive inheritance pattern, while in rats activation of the MEK5/ERK cascade initiates the biliary dysgenesis of Caroli's disease in this species. However, the exact mode of inheritance and pathogenesis of Caroli's disease in dogs is as yet unknown. Previous reports on congenital hepatic cystic diseases of the dog have described Caroli's disease like lesions in various breeds, but these are believed to be the 1st reported cases in the Golden retriever breed.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  11. 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.

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

    LENUS (Irish Health Repository)

    Hennessey, Derek B

    2012-02-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1989-07-01

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

  14. Is congenital bilateral absence of vas deferens a primary form of cystic fibrosis? Analyses of the CFTR gene in 67 patients

    Energy Technology Data Exchange (ETDEWEB)

    Mercier, B.; Verlingue, C.; Audrezet, M.P.; Ferec, C. [Centre de Biogenetique C.D.T.S., Brest (France); Lissens, W.; Bonduelle, M. [University Hospital VUB, Brussels (United Kingdom); Silber, S.J. [St. Luke`s Hospital, St. Louis, MO (United States); Novelli, G. [Catholic Univ. of Rome (Italy)

    1995-01-01

    Congenital bilateral absence of the vas deferens (CBAVD) is an important cause of sterility in men. Although the genetic basis of this condition is still unclear, it has been shown recently that some of these patients carry mutations in their cystic fibrosis transmembrane conductance regulator (CFTR) genes. To extend this observation, we have analyzed the entire coding sequence of the CFTR gene in a cohort of 67 men with CBAVD, who are otherwise healthy. We have identified four novel missense mutations (A800G, G149R, R258G, and E193K). We have shown that 42% of subjects were carriers of one CFTR allele and that 24% are compound heterozygous for CFTR alleles. Thus, we have been unable to identify 76% of these patients as carrying two CFTR mutations. Furthermore, we have described the segregation of CFTR haplotypes in the family of one CBAVD male; in this family are two male siblings, with identical CFTR loci but displaying different phenotypes, one of them being fertile and the other sterile. The data presented in this family, indicating a discordance between the CBAVD phenotype and a marked carrier ({delta}F508) chromosome, support the involvement of another gene(s), in the etiology of CBAVD. 35 refs., 2 figs., 1 tab.

  15. Cystic thymic diseases: CT manifestations

    International Nuclear Information System (INIS)

    Song, Soon Young; Choi, Yo Won; Jeon, Eui Yong; Jeon, Seok Chol; Seo, Heung Suk; Hahm, Chang Kok

    1995-01-01

    To describe CT findings and differential points of cystic thymic lesions. We evaluated retrospectively total 19 masses with well marginated cystic lesions at thymic area on CT scans. They were 10 teratomas, 3 congenital thymic cysts, 2 multilocular thymic cysts(associated with thymoma and myasthenia gravis in each), 2 cysts Assciated with thymic Hodgkin's lymphomas an ectopic parathyroid cyst, and an infected thymic cyst. The radiological abnormalities evaluated were thickness of the wall, presence or abscene of septa, mural nodule, solid component, calcification and fat component. All three cases of congenital thymic cysts and an ectopic parathyroid cyst appeared as thin-walled unilocular cyst with homogeneous internal density and without identifiable solid component. In multilocular thymic cyst, there were thick wall and solid components(n =2), thick internal septa and calcifications(n = 1). The cysts of teratomas manifested thick walls(n = 9), internal septa(n = 4), calcifications(n = 6), fat components(n = 4), and solid components(n = 4). Cysts in Hodgkin's diseases appeared as multilocular or unilocular and had thick wall and septa without calcification. Infected thymic cyst presented with multilocular cystic mass with identifiable wall and septa, calcification, and solid components. The thymic diseases with cystic lesion include teratomas, congenital thymic cysts, multilocular thymic cysts, parathyroid cyst, and Hodgkin's disease. Congenital thymic cyst and ectopic parathyroid cyst are thin-walled unilocular cystic lesions. Cystic lesions associated with teratoma, Hodgkin's disease, and multilocular thymic cyst are thick-walled cystic lesions with or without solid component

  16. Cystic duct remnant mucocele in a liver transplant recipient

    Energy Technology Data Exchange (ETDEWEB)

    Ahlawat, Sushil K. [Georgetown University Hospital, Department of Medicine, Division of Gastroenterology, Washington, DC (United States); University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, NJ (United States); Fishbien, Thomas M. [Georgetown University Hospital, Department of Medicine, Division of Gastroenterology, Washington, DC (United States); Haddad, Nadim G. [Georgetown University Hospital, Department of Surgery, Division of Transplant Surgery, Washington, DC (United States)

    2008-08-15

    Cystic duct remnant mucocele is an extremely rare complication of liver transplantation in children. Surgical correction is usually required for cystic duct remnant mucocele when it causes biliary obstruction. We describe a 14-month-old liver transplant recipient who presented with biliary obstruction 1 month after orthotopic liver transplantation with an end-to-end choledochocholedocal biliary anastomosis for hepatoblastoma. US, CT and cholangiography findings were consistent with mucocele of the allograft cystic duct remnant. Surgery was not needed in our patient because the mucocele and biliary obstruction had resolved on repeat imaging most likely due to guidewire manipulation during cholangiography, resulting in opening of the cystic duct remnant orifice and drainage into the common duct. (orig.)

  17. Cystic duct remnant mucocele in a liver transplant recipient

    International Nuclear Information System (INIS)

    Ahlawat, Sushil K.; Fishbien, Thomas M.; Haddad, Nadim G.

    2008-01-01

    Cystic duct remnant mucocele is an extremely rare complication of liver transplantation in children. Surgical correction is usually required for cystic duct remnant mucocele when it causes biliary obstruction. We describe a 14-month-old liver transplant recipient who presented with biliary obstruction 1 month after orthotopic liver transplantation with an end-to-end choledochocholedocal biliary anastomosis for hepatoblastoma. US, CT and cholangiography findings were consistent with mucocele of the allograft cystic duct remnant. Surgery was not needed in our patient because the mucocele and biliary obstruction had resolved on repeat imaging most likely due to guidewire manipulation during cholangiography, resulting in opening of the cystic duct remnant orifice and drainage into the common duct. (orig.)

  18. Congenital orbital teratoma

    OpenAIRE

    Aiyub, Shereen; Chan, Weng Onn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-01-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The p...

  19. Mediastinal Cystic Lymphangioma in a Patient with Situs Inversus Totalis

    Directory of Open Access Journals (Sweden)

    Teruya Komatsu

    2014-01-01

    Full Text Available We present a case of cystic lymphangioma of the mediastinum complicated with situs inversus totalis. The 70-year-old man underwent thoracoscopic resection of a mediastinal cystic tumor, which was diagnosed as cystic lymphangioma. Cystic lymphangiomas are congenital cystic abnormalities of the lymphatic system. The head and neck area is often involved while the mediastinum is rarely affected. The rarity of this case is further attributed to the coexistence of situs inversus totalis.

  20. Fatal Airway Obstruction in a Man With a Cystic Hygroma.

    Science.gov (United States)

    Wygant, Cassandra Maria; Cohle, Stephen D

    2018-05-03

    We describe a 24-year-old man with a cystic hygroma of the left side of the lower neck that led to sudden death. Cystic hygroma (cystic lymphangioma) is a congenital malformation of the lymphatic system. The patient, who had a tracheostomy because of airway obstruction from the cystic hygroma, was found dead with his tracheostomy tube on the floor next to him. Complications of cystic hygroma include infiltration of the neck causing airway obstruction, dysphagia, pain, and obstructive sleep apnea.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2004-12-15

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

  2. 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.

  3. Cystic thymic diseases: CT manifestations

    Energy Technology Data Exchange (ETDEWEB)

    Song, Soon Young; Choi, Yo Won; Jeon, Eui Yong; Jeon, Seok Chol; Seo, Heung Suk; Hahm, Chang Kok [School of Medicine, Hanyang University, Seoul (Korea, Republic of)

    1995-09-15

    To describe CT findings and differential points of cystic thymic lesions. We evaluated retrospectively total 19 masses with well marginated cystic lesions at thymic area on CT scans. They were 10 teratomas, 3 congenital thymic cysts, 2 multilocular thymic cysts(associated with thymoma and myasthenia gravis in each), 2 cysts Assciated with thymic Hodgkin's lymphomas an ectopic parathyroid cyst, and an infected thymic cyst. The radiological abnormalities evaluated were thickness of the wall, presence or abscene of septa, mural nodule, solid component, calcification and fat component. All three cases of congenital thymic cysts and an ectopic parathyroid cyst appeared as thin-walled unilocular cyst with homogeneous internal density and without identifiable solid component. In multilocular thymic cyst, there were thick wall and solid components(n =2), thick internal septa and calcifications(n = 1). The cysts of teratomas manifested thick walls(n = 9), internal septa(n = 4), calcifications(n = 6), fat components(n = 4), and solid components(n = 4). Cysts in Hodgkin's diseases appeared as multilocular or unilocular and had thick wall and septa without calcification. Infected thymic cyst presented with multilocular cystic mass with identifiable wall and septa, calcification, and solid components. The thymic diseases with cystic lesion include teratomas, congenital thymic cysts, multilocular thymic cysts, parathyroid cyst, and Hodgkin's disease. Congenital thymic cyst and ectopic parathyroid cyst are thin-walled unilocular cystic lesions. Cystic lesions associated with teratoma, Hodgkin's disease, and multilocular thymic cyst are thick-walled cystic lesions with or without solid component.

  4. Cystic medulloblastoma

    International Nuclear Information System (INIS)

    Mahapatra, A.K.; Paul, H.K.; Sarkar, C.

    1989-01-01

    In children medulloblastoma is a commonly encountered posterior fossa midline tumour in which cystic degeneration is not uncommon. A cystic medulloblastoma without solid component has, however, not been described. We report a 12-year-old boy with a posterior fossa midline cystic lesion on CT with surgical and histological confirmation of the diagnosis. (orig.)

  5. Liver manifestations of cystic fibrosis

    International Nuclear Information System (INIS)

    Akata, Deniz; Akhan, Okan

    2007-01-01

    Chronic liver disease is one of the major complications of cystic fibrosis (CF). Significant liver disease is seen in 13-25% of children with CF. Improved life expectancy and prolonged follow-up have favored better characterization of the hepatic manifestations of CF and allowed direct observation of an increasing number of liver-related events. Liver disease typically develops in the first decade of life, with the incidence dropping rapidly after the age of 10 years. The wide spectrum of liver disease ranging from asymptomatic gallbladder abnormalities to biliary cirrhosis will be reviewed in this article

  6. Mucocele of the cystic duct remnant after orthotopic liver transplant: a problem revisited.

    Science.gov (United States)

    Chatterjee, Suvadip; Das, Debasish; Hudson, Mark; Bassendine, Margaret Fiona; Scott, John; Oppong, Kofi Ernest; Sen, Gourab; French, Jeremy J

    2011-06-01

    Mucocele of the cystic duct remnant is an uncommon hepatobiliary complication of a liver transplant. Current practice usually involves either excising the cystic duct, or incorporating the distal end of the transected cystic duct into the suture line of the biliary anastomosis to ensure drainage. We report a patient who developed cystic duct remnant mucocele after the latter approach was adopted. We believe that this is likely related to delayed anastomotic stricturing, which prevented draining from the remnant cystic duct. We also discuss the incidence, pathology, investigations, and treatment of this condition.

  7. Congenital orbital teratoma.

    Science.gov (United States)

    Aiyub, Shereen; Chan, Wengonn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-12-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  8. Congenital orbital teratoma

    Directory of Open Access Journals (Sweden)

    Shereen Aiyub

    2013-01-01

    Full Text Available We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  9. A case report of corgenotal cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Jun, Soon Ae; Cha, Kyung Sub; Chi, Je Geun

    1987-01-01

    Congenital cystic adnomatoid malformation (CCAM) is rare pulmonary cystic disease. CCAM has been detected on prematurity, stillborn and respiratory distress infant or child by chest X-ray film and CT scan. One case of CCAM diagnosed in utero at gestational age 22 weeks is reported with sonographic findings and autopsy findings. Ultrasonographic findings are large cystic lesion in fetal thorax and fetal hydrops without hydramnios. The survival of these infants is very poor despite accurate prenatal diagnosis and maximal postnatal care

  10. Giant cystic abdominal masses in children

    International Nuclear Information System (INIS)

    Wootton-Gorges, Sandra L.; Thomas, Kristen B.; Harned, Roger K.; Wu, Sarah R.; Stein-Wexler, Rebecca; Strain, John D.

    2005-01-01

    In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis. (orig.)

  11. Giant cystic abdominal masses in children

    Energy Technology Data Exchange (ETDEWEB)

    Wootton-Gorges, Sandra L.; Thomas, Kristen B.; Harned, Roger K.; Wu, Sarah R.; Stein-Wexler, Rebecca; Strain, John D. [University of California, Davis Health Center, Sacramento, CA (United States); Davis Children' s Hospital, Department of Radiology, Sacramento, CA (United States)

    2005-12-01

    In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis. (orig.)

  12. Plastic biliary stents for malignant biliary diseases

    NARCIS (Netherlands)

    Huibregtse, Inge; Fockens, Paul

    2011-01-01

    Plastic biliary endoprostheses have not changed much since their introduction more than 3 decades ago. Although their use has been challenged by the introduction of metal stents, plastic stents still remain commonly used. Much work has been done to improve the problem of stent obstruction but

  13. 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

  14. Congenital urethrovaginal fistula associated with imperforate hymen causing fetal urinary ascites and abdominal cystic lesions: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Koichi Ohno

    2015-02-01

    Full Text Available A primipara presented to our hospital due to fetal ascites, bilateral hydronephrosis and hydrometrocolpos. The female baby had two giant abdominal cysts, a single atrium and foot polydactyly. Absence of the vaginal orifice was noticed, and the hymen was broken through; the left cyst collapsed after fluid drainage. The right cyst was punctured and drained, which improved the hydronephrosis. Contrast imaging showed medium pouring into the abdominal cavity via the uterine tube; the right cyst was a 6 × 5 cm closed cavity. Vaginal uroplania persisted even after hospital discharge. At the age of 4 years, cystoscopy showed a fistulous opening on the urethra 5 mm distal from the internal urethral orifice. A catheter inserted via the opening was found in the vagina. In our case, urine poured into the abdominal cavity through the fistula and uterine tube; following obstruction of the uterine tube, giant hydrometrocolpos and hydrosalpinx appeared. Three cases of congenital urethrovaginal fistula (CUVF with imperforate hymen have been reported. A remnant of communication between the uterovaginal primordium and urogenital sinus may be responsible for CUVF.

  15. Pulmonary sequestration with histologic changes of cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Morin, C.; Filiatrault, D.; Russo, P.

    1989-01-01

    Pulmonary sequestration and congenital cystic adenomatoid malformation (CCAM) are two infrequent congenital pulmonary diseases. The combination of these two entities is rare. We report a case where the antenatal ultrasonography showed a left pulmonary mass suggesting CCAM. The US done after birth revealed an aberrant vascularisation. Pathologic examination confirmed the association of both lesions. (orig.)

  16. Extrahepatic biliary atresia in a border collie.

    Science.gov (United States)

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

    2000-01-01

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

  17. 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....

  18. Hepatic echinococcosis ruptured into the biliary tract

    International Nuclear Information System (INIS)

    Van Steenbergen, W.; Fevery, J.; Broeckaert, L.; Ponette, E.; Marchal, G.; Baert, A.; Penninckx, F.; Kerremans, R.; De Groote, J.

    1987-01-01

    Three patients are described with hepatic involvement by Echinococcus granulosus, complicated by spontaneous rupture into the biliary tract. Abdominal computed tomography, showing the cystic wall, the presence of wall calcifications, daughter cysts and wall enhancement, provided a correct diagnosis of hepatic hydatidosis in all patients. Dilatation of the bile ducts with the presence of intraluminal material was clearly shown by sonography and endoscopic retrograde cholangiography. On sonography, the intraluminal material appeared as amorphous, sludge-like hydatid sand, and as daughter cysts. On ERCP, the intrabiliary parasitic material appeared as non-homogeneous, irregularly shaped and mobile filling defects. Other findings at ERCP were displacement and distortion of intrahepatic bile ducts by the hepatic cysts and a mild dilatation of the pancreatic duct. (Auth.)

  19. Childhood abdominal cystic lymphangioma

    Energy Technology Data Exchange (ETDEWEB)

    Konen, Osnat; Rathaus, Valeria; Shapiro, Myra [Department of Diagnostic Imaging, Meir General Hospital, Sapir Medical Centre, Kfar Saba (Israel); Dlugy, Elena [Department of Paediatric Surgery, Schneider Medical Centre, Sackler School of Medicine, Tel-Aviv University (Israel); Freud, Enrique [Department of Paediatric Surgery, Sapir Medical Centre, Sackler School of Medicine, Tel-Aviv University (Israel); Kessler, Ada [Department of Diagnostic Imaging, Sourasky Medical Centre, Tel-Aviv (Israel); Horev, Gadi [Department of Diagnostic Imaging, Schneider Medical Centre, Tel-Aviv (Israel)

    2002-02-01

    Background: Abdominal lymphangioma is a rare benign congenital malformation of the mesenteric and/or retroperitoneal lymphatics. Clinical presentation is variable and may be misleading; therefore, complex imaging studies are necessary in the evaluation of this condition. US and CT have a major role in the correct preoperative diagnosis and provide important information regarding location, size, adjacent organ involvement, and expected complications. Objective: To evaluate the clinical and imaging findings of seven children with proven abdominal cystic lymphangioma. Materials and methods: Clinical and imaging files of seven children with pathologically proven abdominal lymphangioma, from three university hospitals, were retrospectively evaluated. Patient's ages ranged from 1 day to 6 years (mean, 2.2 years). Symptoms and signs included evidence of inflammation, abnormal prenatal US findings, chronic abdominal pain, haemorrhage following trauma, clinical signs of intestinal obstruction, and abdominal distension with lower extremities lymphoedema. Plain films of five patients, US of six patients and CT of five patients were reviewed. Sequential imaging examinations were available in two cases. Results: Abdominal plain films showed displacement of bowel loops by a soft tissue mass in five of six patients, two of them with dilatation of small bowel loops. US revealed an abdominal multiloculated septated cystic mass in five of six cases and a single pelvic cyst in one which changed in appearance over 2 months. Ascites was present in three cases. CT demonstrated a septated cystic mass of variable sizes in all available five cases. Sequential US and CT examinations in two patients showed progressive enlargement of the masses, increase of fluid echogenicity, and thickening of walls or septa in both cases, with multiplication of septa in one case. At surgery, mesenteric lymphangioma was found in five patients and retroperitoneal lymphangioma in the other two

  20. Childhood abdominal cystic lymphangioma

    International Nuclear Information System (INIS)

    Konen, Osnat; Rathaus, Valeria; Shapiro, Myra; Dlugy, Elena; Freud, Enrique; Kessler, Ada; Horev, Gadi

    2002-01-01

    Background: Abdominal lymphangioma is a rare benign congenital malformation of the mesenteric and/or retroperitoneal lymphatics. Clinical presentation is variable and may be misleading; therefore, complex imaging studies are necessary in the evaluation of this condition. US and CT have a major role in the correct preoperative diagnosis and provide important information regarding location, size, adjacent organ involvement, and expected complications. Objective: To evaluate the clinical and imaging findings of seven children with proven abdominal cystic lymphangioma. Materials and methods: Clinical and imaging files of seven children with pathologically proven abdominal lymphangioma, from three university hospitals, were retrospectively evaluated. Patient's ages ranged from 1 day to 6 years (mean, 2.2 years). Symptoms and signs included evidence of inflammation, abnormal prenatal US findings, chronic abdominal pain, haemorrhage following trauma, clinical signs of intestinal obstruction, and abdominal distension with lower extremities lymphoedema. Plain films of five patients, US of six patients and CT of five patients were reviewed. Sequential imaging examinations were available in two cases. Results: Abdominal plain films showed displacement of bowel loops by a soft tissue mass in five of six patients, two of them with dilatation of small bowel loops. US revealed an abdominal multiloculated septated cystic mass in five of six cases and a single pelvic cyst in one which changed in appearance over 2 months. Ascites was present in three cases. CT demonstrated a septated cystic mass of variable sizes in all available five cases. Sequential US and CT examinations in two patients showed progressive enlargement of the masses, increase of fluid echogenicity, and thickening of walls or septa in both cases, with multiplication of septa in one case. At surgery, mesenteric lymphangioma was found in five patients and retroperitoneal lymphangioma in the other two. Conclusions: US

  1. Biliary cystadenoma associated with Opisthorchis viverrini infection in a domestic cat (Felis catus).

    Science.gov (United States)

    Tiwananthagorn, Saruda; Srivorakul, Saralee; Khochakul, Varangpicha; Pringproa, Kidsadagon

    2018-05-05

    A 12-year-old, female, domestic cat (Felis catus) presented with dehydration, emaciation, anorexia, and lethargy. The cat was unresponsive to medical treatment and euthanized; the carcass was submitted for pathological diagnosis. Necropsy revealed icteric mucous membranes. The liver was enlarged, with multinodular, cystic, white masses, 0.5-4.0 cm in diameter, scattered throughout. Microscopically, the biliary epithelium presented with a proliferation of multifocal cystic masses, occasionally with periodic acid-Schiff-positive fluid within the cysts. Simple cuboidal epithelial cells showed small, round to oval, vesicular nuclei and rare mitotic figures. There were also multifocal trematode-like parasites situated within the biliary tracts. Immunohistochemistry of the cystic masses was positive for pan-cytokeratin and proliferating cell nuclear antigen, while negative for vimentin. Molecular analysis and gene sequencing of the parasite indicated that it was Opisthorchis viverrini. Based on the pathological findings and molecular analysis, the cat was diagnosed with biliary cystadenoma related to O. viverrini infection. This report described an unusual case of O. viverrini infection associated with biliary tumor in a cat, and raises the possibility of domestic cats as a reservoir host of the human liver fluke. Copyright © 2018 Elsevier B.V. All rights reserved.

  2. 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

  3. 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

  4. Conservative Treatment for Cystic Duct Stenosis in a Child

    Directory of Open Access Journals (Sweden)

    Marco Gasparetto

    2013-01-01

    Full Text Available Introduction. Few cases of common bile duct stenosis have been reported in the literature, and observations of strictures in the cystic duct are even more rare. Surgical cholecystectomy is the treatment needed in most cases of gallbladder hydrops. This paper describes the diagnosis and successful medical treatment of a rare pediatric case of cystic duct stenosis and gallbladder hydrops. Case Report. A formerly healthy one-year-old girl was admitted with colicky abdominal pain. Blood tests were normal, except for an increase in transaminases. Abdominal ultrasound excluded intestinal intussusception and identified a distended gallbladder with biliary sludge. MR cholangiography revealed a dilated gallbladder containing bile sediment and no detectable cystic duct, while the rest of the intra- and extrahepatic biliary tree and hepatic parenchyma were normal. This evidence was consistent with gallbladder hydrops associated with cystic duct stenosis. The baby was treated with i.v. hydration, corticosteroids, antibiotics, and ursodeoxycholic acid. Her general condition rapidly improved, with no further episodes of abdominal pain and normalization of liver enzymes. This allowed to avoid cholecystectomy, and the child is well 1.5 years after diagnosis. Conclusions. Although cholecystectomy is usually necessary in case of gallbladder hydrops, our experience suggests that surgical procedures can be avoided when the distension is caused by a cystic duct stenosis.

  5. Spiral computed tomography-intravenous cholangiography with three-dimensional reconstructions for imaging the biliary tree

    International Nuclear Information System (INIS)

    Alibrahim, E; Gibson, R.N.; Vincent, J; Speer, T.; Collier, N.; Jardine, C.

    2006-01-01

    This study aimed to assess the ability of CT-i.v. cholangiography to show the perihilar biliary and cystic duct anatomy and to assess the relative performance of axial, maximum intensity projection and surface rendered displays. We also assessed the correlation between serum bilirubin levels and adequacy of biliary opacification. Spiral CT was carried out following infusion of 100 mL of Biliscopin in 181 patients with suspected biliary disease. The display of biliary anatomy was of high quality, with 91% of patients having good opacification of at least first-order bile ducts and 84% having good opacification of at least third-order right and left hepatic ducts. The quality of biliary opacification correlated inversely to serum bilirubin levels, with levels above two to three times the normal value being associated with lower rates of good opacification. Maximum intensity projection and surface rendered reformats aided anatomical interpretation to a similar degree. The relative frequency of types of perihilar branching patterns and cystic duct junctional anatomy correlated closely to those reported from previous anatomical studies. Copyright (2006) Blackwell Science Pty Ltd

  6. Congenital cystic adenomatoid malformation of the lung

    International Nuclear Information System (INIS)

    Beluffi, G.; Brokensha, C.; Kozlowski, K.; Lucaya, J.; Morris, L.; Rosso, R.

    1989-01-01

    Sixteen cases of CCAM are presented, one with bilateral disease, diagnosed at different times, and one with an associated prune belly syndrome, to be added to the 405 already reported in the literature, and their clinical, radiological and pathological features are described. (orig./MG) [de

  7. Non-neoplastic cystic and cystic-like lesions of the pancreas: may mimic pancreatic cystic neoplasms.

    Science.gov (United States)

    Goh, Brian K P; Tan, Yu-Meng; Chung, Yaw-Fui A; Chow, Pierce K H; Ong, Hock-Soo; Lim, Dennis T H; Wong, Wai-Keong; Ooi, London L P J

    2006-05-01

    epithelial (congenital) cyst (n = 3), retention cyst (n = 1) and mucinous non-neoplastic cyst (n = 1). At a median follow up of 20 months (range, 3-34 months), none of the patients had any evidence of recurrent disease. Non-neoplastic cystic and cystic-like lesions of the pancreas are rare causes of pancreatic cystic lesions that are generally benign and do not require surgery when asymptomatic. However, despite advances in diagnostic investigations such as endoscopic ultrasound with fluid aspirate and magnetic resonance imaging, the preoperative diagnosis remains unreliable. Hence, the challenge for all clinicians is to recognize these lesions preoperatively and to avoid 'unnecessary' surgery.

  8. Annular pancreas causing extrahepatic biliary obstruction

    International Nuclear Information System (INIS)

    Ogulin, M.; Jamar, B.

    2004-01-01

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

  9. Malformação congénita das vias aéreas pulmonares: Experiência de cinco centros Congenital cystic adenomatoid malformation of the lung: The experience of five medical centres

    Directory of Open Access Journals (Sweden)

    Gustavo Rocha

    2007-07-01

    lesão assintomática é controverso; a cirurgia está indicada devido à baixa morbilidade e possibilidade de prevenção de complicações tardias, como a degenerescência maligna.Background: The clinical spectrum of congenital cystic adenomatoid malformation of the lung (CCAML ranges from asymptomatic lesions to neonatal respiratory distress and hydrops fetalis. Aim: To review our experience with CCAML, emphasising natural history, management and outcome. Material and methods: A retrospective chart review of all CCAML-diagnosed neonates admitted to the neonatal intensive care units of five tertiary medical centres in the north of Portugal between 1996 and 2005. Results: Fifteen neonates with CCAML were identified, 9F/6M, birth weight 3100 g (645-3975, gestational age 38 weeks (24-40. The incidence of CCAML was 1: 9300 births. There were 11 (73% cases of cystic lung lesion diagnosed during pregnancy, median age 22 weeks (19-30. The lesion was right sided in six (40% and left sided in nine (60% cases. In utero spontaneous regression of the lesion was observed in two cases. Antenatal intervention (pleurocentesis and thoracoamniotic shunting was performed in one foetus with impending hydrops. Normal lung radiographic findings at birth were present in five cases, with an abnormal CT scan. Three (20% neonates became symptomatic during the neonatal period (respiratory distress and one (70% after the neonatal period (spontaneous pneumothorax. Two neonates (13% died. Six (40% patients underwent thoracotomy and appropriate excisional surgery. Histological examination showed definitive features of CCAML (Stocker classification: type I = 4; type II = 1; type III = 2. Eight (53% patients remain asymptomatic and did not undergo surgery. Conclusions: Antenatally diagnosed CCAML has a good prognosis in the absence of severe foetal distress; normal radiographic findings at birth do not rule out CCAML; treatment of asymptomatic CCAML is controversial; surgery may be advocated

  10. Future developments in biliary stenting

    Directory of Open Access Journals (Sweden)

    Hair CD

    2013-06-01

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

  11. 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....

  12. Dual Pathology Causing Congenital Bladder Outlet Obstruction.

    Science.gov (United States)

    Kwong, Ruth; Johal, Navroop S; Upasani, Anand; Paul, Anu; Cuckow, Peter

    2017-12-07

    Anterior urethral syringocele is an uncommon congenital deformity characterised by cystic dilatation of bulbo-urethral gland ducts and is usually asymptomatic. We present a case on 4-day-old male neonate who presented with bilateral antenatal hydroureteronephrosis and renal impairment and found to have urethral syringocele and posterior urethral valves (PUV). Copyright © 2017. Published by Elsevier Inc.

  13. Congenital Hypothyroidism

    Science.gov (United States)

    ... Disease Featured Resource Find an Endocrinologist Search Congenital Hypothyroidism March 2012 Download PDFs English Espanol Editors Rosalind S. ... Resources MedlinePlus (NIH) Mayo Clinic What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

  14. Cystic Fibrosis (CF): Chloride Sweat Test

    Science.gov (United States)

    ... on this topic for: Parents Kids Teens Cystic Fibrosis Cystic Fibrosis and Nutrition Cystic Fibrosis (CF) Respiratory Screen: Sputum Cystic Fibrosis: Diet and Nutrition Cystic Fibrosis Cystic Fibrosis: Diet and Nutrition View more Partner Message ...

  15. Cystic fibrosis: case report

    International Nuclear Information System (INIS)

    Park, Si Hyun; Lee, Hyun Ju; Kim, Ji Hye; Park, Chol Heui

    2002-01-01

    Cystic fibrosis is an autosomal recessive genetic disease. Among Caucasians, it is the most common cause of pulmonary insufficiency during the first three decades of life. The prevalence of cystic fibrosis varies according to ethnic origin: it is common among Caucasians but rare among Asians. We report a case in which cystic fibrosis with bronchiectasis and hyperaeration was revealed by high-resolution CT, and mutation of the cystic fibrosis conductance transmembrane regulator gene (CFTR) by DNA analysis

  16. Cystic fibrosis: case report

    International Nuclear Information System (INIS)

    Park, Si Hyun; Lee, Hyun Ju; Kim, Ji Hye; Park, Chol Heui

    2002-01-01

    Cystic fibrosis is a autosomal recessive genetic disease. Among caucasians, it is the most common cause of pulmonary insufficiency during the first three decades of life. The prevalence of cystic fibrosis varies according to ethnic origin: it is common among caucasians but rare among Asians. We report a case in which cystic fibrosis with bronchiectasis and hyperaeration was revealed by high-resolution CT, and mutation of the cystic fibrosis conductance transmembrane regulator gene (CFTR) by DNA analysis

  17. Outcome following Resection of Biliary Cystadenoma: A Single Centre Experience and Literature Review

    Directory of Open Access Journals (Sweden)

    M. Pitchaimuthu

    2015-01-01

    Full Text Available Background. Biliary cystadenomas (BCAs are rare, benign, potentially malignant cystic lesions of the liver, accounting for less than 5% of cystic liver tumours. We report the outcome following resection of biliary cystadenoma from a single tertiary centre. Methods. Data of patients who had resection of BCA between January 1993 and July 2014 were obtained from liver surgical database. Patient demographics, clinicopathological characteristics, operative data, and postoperative outcome were analysed. Results. 29 patients had surgery for BCA. Male : female ratio was 1 : 28. Clinical presentation was abdominal pain (74%, jaundice (20%, abdominal mass (14%, and deranged liver function tests (3%. Cyst characteristics included septations (48%, wall thickening (31%, wall irregularity (38%, papillary projections (10%, and mural nodule (3%. Surgical procedures included atypical liver resection (52%, left hemihepatectomy (34%, right hemihepatectomy (10%, and left lateral segmentectomy (3%. Median length of stay was 7 (IQ 6.5–8.5 days. Two patients developed postoperative bile leak. No patients had malignancy on final histology. Median follow-up was 13 (IQ 6.5–15.7 years. One patient developed delayed biliary stricture and one died of cholangiocarcinoma 11 years later. Conclusion. Biliary cystadenomas can be resected safely with significantly low morbidity. Malignant transformation and recurrence are rare. Complete surgical resection provides a cure.

  18. Percutaneous transhepatic biliary endoprostheses

    International Nuclear Information System (INIS)

    Lammer, J.

    1985-01-01

    Eighty biliary endoprostheses were introduced by the transhepatic route in sixty patients with obstructive jaundice. Complication rate was 21% (no mortality). Average survival time is sixteen weeks (maximum 53 weeks). In most patients, a 12 F teflon endoprosthesis was introduced four to five days after the initial catheter drainage. Patients in poor condition had a primary implant of a 9 F endoprosthesis. A combined transhepatic/transoral implantation was carried out five times. Results have shown that implantation of a prosthesis is as effective as a surgical bypass operation for palliation of obstructive jaundice and that it is better than catheter drainage. In-vitro experiments have indicated that failure of a 12 F prosthesis due to encrustation may be expected in about 23 weeks. This is in line with the survival time of patients with carcinomas. (orig.) [de

  19. Heterotopic pancreatic tissue presenting as a solid and cystic lung lesion : A very unusual bronchopulmonary foregut malformation

    NARCIS (Netherlands)

    De Kruger, RR; Albers, MJIJ; Mooi, WJ; Bogers, J.J.C.

    2004-01-01

    We describe the history and lung pathology of a premature female infant, who presented with respiratory distress immediately after birth. A thoracic computerized tomography scan showed abnormalities suggestive of congenital cystic adenomatoid malformation of the left lung. In addition,

  20. Congenital extrahepatic portosystemic shunts

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-09-01

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

  1. Congenital extrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

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

    2003-01-01

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

  2. Congenital tuberculosis

    African Journals Online (AJOL)

    Prof Ezechukwu

    2012-06-20

    Jun 20, 2012 ... Key words: Congenital tuberculo- sis, case report, miliary tuberculosis. Introduction. Congenital tuberculosis defines tuberculosis in infants of .... tary TB and otitis media, resulting in seizures, deafness, and death. It is therefore not surprising that the index case who presented at twelve weeks of age, had ...

  3. Congenital Abnormalities

    Science.gov (United States)

    ... tube defects. However, there is also a genetic influence to this type of congenital anomaly. Unknown Causes The vast majority of congenital abnormalities have no known cause. This is particularly troubling for parents who plan to have more children, because there is no way to predict if ...

  4. Neonatal cystic fibrosis screening test

    Science.gov (United States)

    Cystic fibrosis screening - neonatal; Immunoreactive trypsinogen; IRT test; CF - screening ... Cystic fibrosis is a disease passed down through families. CF causes thick, sticky mucus to build up in ...

  5. 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.

  6. 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.

  7. 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.

  8. Antenatal diagnosis of cystic adenomatoid malformation: Effect on patient management

    International Nuclear Information System (INIS)

    Claiborne, A.K.; McAlister, W.H.; Martin, C.M.; Gast, M.J.

    1985-01-01

    Congenital adenomatoid malformation (CAM) of the lung was diagnosed at 30 weeks gestation. The mother presented with preterm labor and polyhydramnios. A complex cystic mass was seen in the right lung of the fetus. Additional radiographic and sonographic investigations prior to delivery allowed differentiation of this rare lesion from other cystic thoracic pathology of the fetal period. Careful hospital obstetric management of the mother and fetus for over 3 weeks allowed the delivery of an infant with adequate pulmonary maturity to permit stabilization and surgery on the baby in the first days of life. (orig.)

  9. 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

  10. Congenital intestinal lymphangiectasia. A case report

    Directory of Open Access Journals (Sweden)

    Georgios Mitsiakos

    2017-12-01

    Full Text Available Congenital intestinal lymphangiectasia, first described by Waldmann et al. in 1961, is a rare congenital malformation of the lymphatics, presented with generalized edema, hypoproteinemia and lymphopenia. Diagnosis is based on endoscopy findings and pathology.We present here a case of a male neonate, second child of an indigenous woman, delivered by caesarean section. Prenatally, multiple cystic abdominal masses were identified by ultrasound. The patient was treated successfully with enterectomy and anastomosis. Histopathology revealed primary intestinal lymphangiectasia with no features of malignancy.Intestinal lymphangiectasia is a rare pathology, which should be differentiated while exploring abdominal masses, hypoproteinemia and edema especially in neonates.

  11. Congenital generalized lipodystrophia: a case report

    International Nuclear Information System (INIS)

    Malheiros, N.R.; Marchiori, E.; Praxedes, M.C.; Machado, D.M.; Carvalho, A.A.V.

    1995-01-01

    Congenital generalized lipodystrophia is a rare genetic disorder, transmitted as an autosomal recessive trait and is prevalent on female. This paper presents a case of a man, 36-year-old, suffering from congenital generalized lipodystrophia with clinical features of diabetes mellitus and dyspnea. Radiographic findings have shown cystic areas in the skeleton, interstitial pulmonary fibrosis and paucity of abdominal fat. Radiological and anatomo-pathological aspects are presented as well as a review of the medical literature about the case. (author). 8 refs, 4 figs

  12. Diagnosis of cystic fibrosis

    NARCIS (Netherlands)

    H.J. Veeze

    1995-01-01

    textabstractApplying the sweat-test as the first choice of test when a diagnosis of cystic fibrosis is suspected is still common practice and advisable. Since the cloning of the CFTR gene more than 400 different cystic fibrosis (CF) mutations have already been identified. The use of CF mutation

  13. Congenital pulmonary airway malformation in a 36 year-old female

    OpenAIRE

    Barreiro, Timothy J.; Henn, Lucas; Ingnam, Sisham; Sypert, Michael

    2015-01-01

    Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation (CCAM), is an inborn abnormality of the lower respiratory system. Most often diagnosed in the perinatal period, these anomalies usually present with tachypnea, cyanosis, and respiratory distress. However, rare cases are asymptomatic and undiagnosed until adulthood.

  14. Pure Laparoscopic Left Hemihepatectomy for Hepatic Peribiliary Cysts with Biliary Intraepithelial Neoplasia

    Directory of Open Access Journals (Sweden)

    Akira Umemura

    2016-01-01

    Full Text Available Introduction. Hepatic peribiliary cysts (HPCs usually originate due to the cystic dilatation of the intrahepatic extramural peribiliary glands. We describe our rare experience of pure laparoscopic left hemihepatectomy (PLLH in a patient with HPCs accompanied by a component of biliary intraepithelial neoplasia (BilIN. Case Presentation. A 65-year-old man was referred for further investigation of mild hepatic dysfunction. Contrast-enhanced computed tomography showed dilatation of the left-sided intrahepatic bile duct, and biliary cytology showed class III cells. The patient was highly suspected of having left side-dominated cholangiocarcinoma and underwent PLLH. Microscopic findings revealed multiple cystic dilatations of the extramural peribiliary glands; hence, this lesion was diagnosed as HPCs. The resected intrahepatic bile duct showed that the normal ductal lumen comprised low columnar epithelia; however, front formation on the BilIN was observed in some parts of the intrahepatic bile duct, indicating that the BilIN coexisted with HPCs. Conclusion. We chose surgical therapy for this patient owing to the presence of some features of biliary malignancy. We employed noble PLLH as a minimally invasive procedure for this patient.

  15. Biliary scanning with /sup 99m/Tc pyridoxylideneglutamate. The effect of food in normal subjects: concise communication

    International Nuclear Information System (INIS)

    Baker, R.J.; Marion, M.A.

    1977-01-01

    Technetium-99m pyridoxylideneglutamate biliary scans were performed in 19 normal subjects in both the fasted and nonfasted state. The effect of eating was to reduce visualization of the gallbladder from 100% (fasted) to 47% (nonfasted). The common bile duct was seen in 84% on both occasions but intrahepatic and cystic ducts were seen less frequently in the nonfasted group. Preparation of patients by fasting is essential if information concerning gallbladder function is sought

  16. Congenital rubella

    Science.gov (United States)

    ... that usually closes shortly after birth remains open ( patent ductus arteriosus ) Narrowing of the large artery that ... prior to pregnancy can prevent congenital rubella. Pregnant women who have not had the vaccine should avoid ...

  17. Biliary interventionism in benign disease

    International Nuclear Information System (INIS)

    Marin, Jorge H

    2003-01-01

    The interventionism non-surgical biliary begins with the description of the drainage biliary percutaneous that has presented in the last 20 years a special development with the improvement of the techniques and materials for percutaneous and endoscopic use. At the present time the echographic technique allows a quick diagnosis of the possible causes of the obstructive jaundice and in most of the cases to approach the level of the obstruction; however, for a complete morphologic definition of the biliary tree; the cholangiography retrogrades endoscopic is used like first line of diagnosis and therapeutic leaving the transhepatic percutaneous cholangiography for some patients with bankrupt endoscopy or previous surgery with alteration of the anatomy and impossibility for the endoscopic canulation. Additionally, with the continuous improvement of the resonance images in the biliary duct and the new techniques of multiplanar reconstruction of the TAC have taken to that these techniques are of first diagnostic line and that the percutaneous boarding, is carried out with therapeutic and non diagnostic intention

  18. 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

  19. Imaging findings in congenital hepatic fibrosis

    International Nuclear Information System (INIS)

    Akhan, Okan; Karaosmanoglu, Ali Devrim; Ergen, Bilge

    2007-01-01

    Congenital hepatic fibrosis (CHF) is a rare congenital multisystemic disorder, mostly inherited in autosomal recessive fashion, primarily affecting renal and hepatobiliary systems. Main underlying process of the disease is the malformation of the ductal plate, the embryological precursor of the biliary system, and secondary biliary strictures and periportal fibrosis ultimately leading to portal hypertension. The natural course of the disease is highly variable ranging from minimally symptomatic disease to true cirrhosis of the liver. However, in most patients the most common manifestations of the diseases that are related to portal hypertension, particularly splenomegaly and bleeding varices. Many other disease processes may co-exist with the disease including Caroli's disease, choledochal cysts and autosomal recessive polycystic kidney disease (ARPKD) reflecting the mulstisystemic nature of the disease. The associating biliary ductal disease led the authors to think that all these entities are a continuum and different reflections of the same underlying pathophysiological process. Although, conventional method of diagnosis of CHF is the liver biopsy the advent of imaging technologies and modalities, today, may permit the correct diagnosis in a non-invasive manner. Characteristic imaging features are generally present and recognition of these findings may obviate liver biopsy while preserving the diagnostic accuracy. In this article, it is aimed to increase the awareness of the practising radiologists to the imaging findings of this uncommon clinical disorder and trail the blaze for future articles relating to this issue

  20. Imaging findings in congenital hepatic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Akhan, Okan [Department of Radiology, Hacettepe University, School of Medicine, 06100 Ankara (Turkey)]. E-mail: akhano@tr.net; Karaosmanoglu, Ali Devrim [Department of Radiology, Hacettepe University, School of Medicine, 06100 Ankara (Turkey); Ergen, Bilge [Department of Radiology, Hacettepe University, School of Medicine, 06100 Ankara (Turkey)

    2007-01-15

    Congenital hepatic fibrosis (CHF) is a rare congenital multisystemic disorder, mostly inherited in autosomal recessive fashion, primarily affecting renal and hepatobiliary systems. Main underlying process of the disease is the malformation of the ductal plate, the embryological precursor of the biliary system, and secondary biliary strictures and periportal fibrosis ultimately leading to portal hypertension. The natural course of the disease is highly variable ranging from minimally symptomatic disease to true cirrhosis of the liver. However, in most patients the most common manifestations of the diseases that are related to portal hypertension, particularly splenomegaly and bleeding varices. Many other disease processes may co-exist with the disease including Caroli's disease, choledochal cysts and autosomal recessive polycystic kidney disease (ARPKD) reflecting the mulstisystemic nature of the disease. The associating biliary ductal disease led the authors to think that all these entities are a continuum and different reflections of the same underlying pathophysiological process. Although, conventional method of diagnosis of CHF is the liver biopsy the advent of imaging technologies and modalities, today, may permit the correct diagnosis in a non-invasive manner. Characteristic imaging features are generally present and recognition of these findings may obviate liver biopsy while preserving the diagnostic accuracy. In this article, it is aimed to increase the awareness of the practising radiologists to the imaging findings of this uncommon clinical disorder and trail the blaze for future articles relating to this issue.

  1. Heterotopic gastric mucosa involving the gallbladder and biliary tree

    Energy Technology Data Exchange (ETDEWEB)

    Madrid, Carmen; Berrocal, Teresa; Gorospe, Luis; Prieto, Consuelo [Department of Paediatric Radiology, Hospital Infantil ' ' La Paz' ' , Paseo de la Castellana 261, 28046 Madrid (Spain); Gamez, Manuel [Department of Paediatric Surgery, Hospital Infantil ' ' La Paz' ' , Madrid (Spain)

    2003-02-01

    A case of heterotopic gastric mucosa in the common bile duct, cystic duct and gallbladder is reported in a 3-year-old girl with abdominal pain and jaundice. Abdominal US and CT showed dilatation of the biliary tree and a well-defined mass in the common bile duct that narrowed its lumen. The gallbladder was contracted in both examinations. The common bile duct and the gallbladder were resected and a choledochojejunostomy was performed. Although gastric heterotopy has been described throughout the entire length of the gastrointestinal tract, it is a very uncommon finding in the gallbladder and extremely rare in the biliary tree. A firm diagnosis of gastric heterotopia is based on the presence of fundal mucosa replete with parietal and chief cells. To our knowledge, this is the fifth reported case of heterotopic gastric tissue within the common bile duct, and the first to describe the US and CT findings. A relevant literature review and brief outline of the histological and radiological features are included in the discussion. (orig.)

  2. Heterotopic gastric mucosa involving the gallbladder and biliary tree

    International Nuclear Information System (INIS)

    Madrid, Carmen; Berrocal, Teresa; Gorospe, Luis; Prieto, Consuelo; Gamez, Manuel

    2003-01-01

    A case of heterotopic gastric mucosa in the common bile duct, cystic duct and gallbladder is reported in a 3-year-old girl with abdominal pain and jaundice. Abdominal US and CT showed dilatation of the biliary tree and a well-defined mass in the common bile duct that narrowed its lumen. The gallbladder was contracted in both examinations. The common bile duct and the gallbladder were resected and a choledochojejunostomy was performed. Although gastric heterotopy has been described throughout the entire length of the gastrointestinal tract, it is a very uncommon finding in the gallbladder and extremely rare in the biliary tree. A firm diagnosis of gastric heterotopia is based on the presence of fundal mucosa replete with parietal and chief cells. To our knowledge, this is the fifth reported case of heterotopic gastric tissue within the common bile duct, and the first to describe the US and CT findings. A relevant literature review and brief outline of the histological and radiological features are included in the discussion. (orig.)

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

    Directory of Open Access Journals (Sweden)

    Fuqiang Ye

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

  4. Effect of ursodeoxycholic acid on bile acid profiles and intestinal detoxification machinery in primary biliary cirrhosis and health.

    Science.gov (United States)

    Dilger, Karin; Hohenester, Simon; Winkler-Budenhofer, Ursula; Bastiaansen, Barbara A J; Schaap, Frank G; Rust, Christian; Beuers, Ulrich

    2012-07-01

    Ursodeoxycholic acid (UDCA) exerts anticholestatic, antifibrotic and antiproliferative effects in primary biliary cirrhosis (PBC) via mechanisms not yet fully understood. Its adequate biliary enrichment is considered mandatory for therapeutic efficacy. However, precise determination of biliary enrichment of UDCA is not possible in clinical practice. Therefore, we investigated (i) the relationship between biliary enrichment and plasma pharmacokinetics of UDCA, (ii) the effect of UDCA on plasma and biliary bile acid composition and conjugation patterns, and (iii) on the intestinal detoxification machinery in patients with PBC and healthy controls. In 11 PBC patients and 11 matched healthy subjects, cystic bile and duodenal tissue were collected before and after 3 weeks of administration of UDCA (15 mg/kg/day). Extensive pharmacokinetic profiling of bile acids was performed. The effect of UDCA on the intestinal detoxification machinery was studied by quantitative PCR and Western blotting. The relative fraction of UDCA and its conjugates in plasma at trough level[x] correlated with their biliary enrichment[y] (r=0.73, p=0.0001, y=3.65+0.49x). Taurine conjugates of the major hydrophobic bile acid, chenodeoxycholic acid, were more prominent in bile of PBC patients than in that of healthy controls. Biliary bile acid conjugation patterns normalized after treatment with UDCA. UDCA induced duodenal expression of key export pumps, BCRP and P-glycoprotein. Biliary and trough plasma enrichment of UDCA are closely correlated in PBC and health. Taurine conjugation may represent an adaptive mechanism in PBC against chenodeoxycholic acid-mediated bile duct damage. UDCA may stabilize small intestinal detoxification by upregulation of efflux pumps. Copyright © 2012. Published by Elsevier B.V.

  5. [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.

  6. Assessment of MRI and MRCP in diagnosis of biliary cystadenoma and cystadenocarcinoma

    International Nuclear Information System (INIS)

    Lewin, Maite; Arrive, Lionel; Tubiana, Jean-Michel; Mourra, Najat; Flejou, Jean-Francois; Honigman, Isabelle; Parc, Rolland

    2006-01-01

    Magnetic resonance imaging (MRI) and magnetic resonance cholangio-pancreatography (MRCP) features were analyzed in the diagnosis of seven surgically resected hepatobiliary cystic tumors with reference to histopathological data. Homogeneity, size, location, signal intensity, presence or absence of septa and/or nodules and MRCP features of the lesions were studied. Histological evidence demonstrated six biliary cystadenoma (BCA) including four pseudo-ovarian stroma (POS) and one biliary cystadenocarcinoma (BCAC). Cystic lesions (3-15 cm in diameter) were homogeneous in the six BCA, heterogeneous in the one BCAC, and were located in the left and right liver, respectively. On T2-weighted images all lesions were hyperintense. On T1-weighted images hypointensity was found in three BCA (all serous fluid, including one POS), isointensity was found in the three others (two mucinous and one hemorrhagic fluid, including three POS) and in the one BCAC (containing mucinous fluid). Septas were present in all cases and nodules only in the one BCAC. On MRCP a hyperintense cystic lesion was found in all cases and a bile ducts dilatation in two BCA and the one BCAC. Gadolinium-enhanced MRI in combination with MRCP is a valuable tool for the diagnosis of BCA or BCAC. However, no specific information is gained for POS detection. (orig.)

  7. Biliary Innate Immunity: Function and Modulation

    Directory of Open Access Journals (Sweden)

    Kenichi Harada

    2010-01-01

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

  8. CT findings and differential diagnosis of cystic neck masses

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    Lee, Ji Yeon; Lee, Kil Jun; Jeong, Seong Ki; Han, Seong Nim; Tae, Seok; Shin, Kyoung Ja; Lee, Sang Chun [Seoul Red Cross Hospital, Seoul (Korea, Republic of)

    1995-10-15

    The purpose of this study is to analyze the CT features of the cystic masses in the neck and to review differential diagnosis. We retrospectively reviewed and analyzed the CT findings of 22 histopathologically proved, cystic neck masses in regard to the location in fascial plane and relationship with adjacent organ. Of 22 cases, ten congenital cysts two ranulas, seven inflammatory lesions, and three solid tumors were included. Ten congenital cystic masses were located in typical locations as branchial cleft cyst (5) in mandibular angle, thyroglossal duct cyst (3) in visceral space embeded within the strap muscles, cystic hygroma (1) and cavernous hemangioma (1) in posterior cervical space with insinuating appearance. Two cases of ranula included one simple ranula localized in sublingual space and a plunging ranula extending to adjacent submandibular space. Seven cases of inflammatory lesions were characterized by multispatial locations and good contrast-enhancement of walls and adjacent tissue. Solid masses of low density mimicking cyst were two pleomorphic adenomas of submandibular gland and one neurilemmoma. It is considered that thorough analysis of the CT findings with attention to typical location, CT appearance, and the relationship with the adjacent structures usually leads to the correct diagnosis.

  9. Primary biliary cirrhosis: natural history.

    Science.gov (United States)

    Tornay, A S

    1980-03-01

    All patients seen with primary biliary cirrhosis during this decade were reviewed. Sixty per cent were diagnosed during the earliest asymptomatic stage of this illness and only 20% progressed symptomatically during a mean follow-up period of 52 months. Those patients presenting with persistent jaundice followed a classic downhill course. These data establish the previously predicted trend toward earlier diagnosis and raise questions about the concept of universal progression of this disease and its time course.

  10. [Extrahepatic biliary atresia: diagnostic methods].

    Science.gov (United States)

    Cauduro, Sydney M

    2003-01-01

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

  11. [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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2000-02-01

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

  13. 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.)

  14. Biliary atresia: the Canadian experience.

    Science.gov (United States)

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

    2007-12-01

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

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

    Directory of Open Access Journals (Sweden)

    Madhavi Nori

    2013-01-01

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

  16. Understanding the Mechanical forces of Self-Expandable Metal Stents in the Biliary Ducts.

    Science.gov (United States)

    Isayama, Hiroyuki; Nakai, Yousuke; Hamada, Tsuyoshi; Matsubara, Saburo; Kogure, Hirofumi; Koike, Kazuhiko

    2016-12-01

    Self-expandable metallic stent (SEMS) was an effective biliary endoprosthesis. Mechanical properties of SEMS, radial and axial force (RF, AF), may play important roles in the bile duct after placement. RF was well known dilation force and influenced on the occurrence of migration. AF, newly proposed by this author, was defined as the recovery force when the SEMS vended. AF was related with the cause of bile duct kinking, pancreatitis, and cholecystitis due to the compression of the bile duct, orifice of the cystic duct, and pancreatic orifice. Ideal SEMS may show high RF and low AF.

  17. Challenges in the prenatal and post-natal diagnosis of mediastinal cystic hygroma: a case report

    Directory of Open Access Journals (Sweden)

    Nazir Sarfraz

    2008-08-01

    Full Text Available Abstract Introduction Cystic hygroma is a benign congenital neoplasm that mostly presents as a soft-tissue mass in the posterior triangle of the neck. Pure mediastinal lesions are uncommon; the vast majority are asymptomatic and are an incidental finding in adulthood. The diagnosis is often made intra- or postoperatively. Prenatal identification is exceptional and post-natal diagnosis also proves challenging. Case presentation We report one such case that was mistaken for other entities in both the prenatal and immediate post-natal period. Initial and follow-up antenatal ultrasound scans demonstrated a multicystic lesion in the left chest, and the mother was counselled about the possibility of her baby having a congenital diaphragmatic hernia. Initial post-natal chest radiographs were reported as normal. An echocardiogram and thoracic computed tomography scan confirmed a complex multiloculated cystic mediastinal mass. The working diagnoses were of a mediastinal teratoma or congenital cystic adenomatous malformation. At operation, the lesion was compressed by the left lung and was found to be close to the left phrenic nerve, which was carefully identified and preserved. After excision, histopathological examination of the mass confirmed the diagnosis of cystic hygroma. Postoperative dyspnoea was observed secondary to paradoxical movement of the left hemidiaphragm and probable left phrenic neuropraxia. This settled conservatively with excellent recovery. Conclusion Despite the fact that isolated intrathoracic cystic hygroma is a rare entity, it needs to be considered in the differential diagnosis of foetal and neonatal mediastinal masses, particularly for juxtadiaphragmatic lesions. The phrenic nerve is not identifiable on prenatal ultrasound imaging, and it is therefore understandable that a mass close to the diaphragm may be mistaken for a congenital diaphragmatic hernia because of the location, morphology and potential phrenic nerve compression

  18. Congenital amusias.

    Science.gov (United States)

    Tillmann, B; Albouy, P; Caclin, A

    2015-01-01

    In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." © 2015 Elsevier B.V. All rights reserved.

  19. Role of Fully Covered Self-Expandable Metal Stent for Treatment of Benign Biliary Strictures and Bile Leaks

    Energy Technology Data Exchange (ETDEWEB)

    Pausawasadi, Nonthalee [Department of Medicine, Siriraj Hospital, Mahidol University, Bangkok (Thailand); Soontornmanokul, Tanassanee; Rerknimitr, Rungsun [Department of Medicine, Chulalongkorn University, Bangkok (Thailand)

    2012-02-15

    Endoscopic therapy by balloon dilation and placement of multiple large-bore plastic stents is the treatment of choice for benign biliary stricture. This approach is effective but it typically requires multiple endoscopic sessions given the short duration of stent patency. The endoscopic approach for treatment of bile leak involves the placement of a stent with or without biliary sphincterotomy. The self-expandable metal stent (SEMS) has traditionally been used for palliation of malignant biliary strictures given the long duration of stent patency owing to their larger stent diameter. Recently, SEMS has been used in a variety of benign biliary strictures and leaks, especially with the design of the covered self-expandable metal stent (CSEMS), which permits endoscopic-mediated stent removal. The use of CSEMS in benign biliary stricture could potentially result in a decrease in endoscopic sessions and it is technically easier when compared to placement of multiple plastic stents. However, complications such as cholecystitis due to blockage of cystic duct, stent migration, infection and pancreatitis have been reported. The potential subsegmental occlusion of contralateral intrahepatic ducts also limits the use of CSEMS in hilar stricture. Certain techniques and improvement of stent design may overcome these challenges in the future. Thus, CSEMS may be appropriate in only highly selected conditions, such as refractory benign biliary stricture, despite multiple plastic stent placement or difficult to treat bile duct stricture from chronic pancreatitis, and should not be used routinely. This review focuses on the use of fully covered self-expandable metal stent for benign biliary strictures and bile leaks.

  20. Role of Fully Covered Self-Expandable Metal Stent for Treatment of Benign Biliary Strictures and Bile Leaks

    International Nuclear Information System (INIS)

    Pausawasadi, Nonthalee; Soontornmanokul, Tanassanee; Rerknimitr, Rungsun

    2012-01-01

    Endoscopic therapy by balloon dilation and placement of multiple large-bore plastic stents is the treatment of choice for benign biliary stricture. This approach is effective but it typically requires multiple endoscopic sessions given the short duration of stent patency. The endoscopic approach for treatment of bile leak involves the placement of a stent with or without biliary sphincterotomy. The self-expandable metal stent (SEMS) has traditionally been used for palliation of malignant biliary strictures given the long duration of stent patency owing to their larger stent diameter. Recently, SEMS has been used in a variety of benign biliary strictures and leaks, especially with the design of the covered self-expandable metal stent (CSEMS), which permits endoscopic-mediated stent removal. The use of CSEMS in benign biliary stricture could potentially result in a decrease in endoscopic sessions and it is technically easier when compared to placement of multiple plastic stents. However, complications such as cholecystitis due to blockage of cystic duct, stent migration, infection and pancreatitis have been reported. The potential subsegmental occlusion of contralateral intrahepatic ducts also limits the use of CSEMS in hilar stricture. Certain techniques and improvement of stent design may overcome these challenges in the future. Thus, CSEMS may be appropriate in only highly selected conditions, such as refractory benign biliary stricture, despite multiple plastic stent placement or difficult to treat bile duct stricture from chronic pancreatitis, and should not be used routinely. This review focuses on the use of fully covered self-expandable metal stent for benign biliary strictures and bile leaks.

  1. Role of Fully Covered Self-Expandable Metal Stent for Treatment of Benign Biliary Strictures and Bile Leaks

    Science.gov (United States)

    Pausawasadi, Nonthalee; Soontornmanokul, Tanassanee

    2012-01-01

    Endoscopic therapy by balloon dilation and placement of multiple large-bore plastic stents is the treatment of choice for benign biliary stricture. This approach is effective but it typically requires multiple endoscopic sessions given the short duration of stent patency. The endoscopic approach for treatment of bile leak involves the placement of a stent with or without biliary sphincterotomy. The self-expandable metal stent (SEMS) has traditionally been used for palliation of malignant biliary strictures given the long duration of stent patency owing to their larger stent diameter. Recently, SEMS has been used in a variety of benign biliary strictures and leaks, especially with the design of the covered self-expandable metal stent (CSEMS), which permits endoscopic-mediated stent removal. The use of CSEMS in benign biliary stricture could potentially result in a decrease in endoscopic sessions and it is technically easier when compared to placement of multiple plastic stents. However, complications such as cholecystitis due to blockage of cystic duct, stent migration, infection and pancreatitis have been reported. The potential subsegmental occlusion of contralateral intrahepatic ducts also limits the use of CSEMS in hilar stricture. Certain techniques and improvement of stent design may overcome these challenges in the future. Thus, CSEMS may be appropriate in only highly selected conditions, such as refractory benign biliary stricture, despite multiple plastic stent placement or difficult to treat bile duct stricture from chronic pancreatitis, and should not be used routinely. This review focuses on the use of fully covered self-expandable metal stent for benign biliary strictures and bile leaks. PMID:22563290

  2. 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

  3. 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

  4. Biliary tract enhancement in gadoxetic acid-enhanced MRI correlates with liver function biomarkers

    International Nuclear Information System (INIS)

    Noda, Yoshifumi; Goshima, Satoshi; Kajita, Kimihiro; Kawada, Hiroshi; Kawai, Nobuyuki; Koyasu, Hiromi; Matsuo, Masayuki; Bae, Kyongtae T.

    2016-01-01

    Purpose: To evaluate the association between gadoxetic-acid-enhanced magnetic resonance (MR) imaging measurements and laboratory and clinical biomarkers of liver function and fibrosis. Materials and methods: One hundred thirty nine consecutive patients with suspected liver disease or liver tumor underwent gadoxetic-acid-enhanced MR imaging. MR imaging measurements during the hepatobiliary phase included biliary tract structure-to-muscle signal intensity ratio (SIR). These measurements were compared with Child-Pugh classification, end-stage liver disease (MELD) score, and aspartate aminotransferase-to-platelet ratio index (APRI). Results: The SIRs of cystic duct and common bile duct were significantly correlated with Child-Pugh classification (P = 0.012 for cystic duct and P < 0.0001 for common bile duct), MELD score (P = 0.0016 and P = 0.0033), and APRI (P = 0.0022 and P = 0.0015). The sensitivity, specificity, and area under the receiver-operating-characteristic curve were: (74%, 88%, 0.86) with the SIR of common bile duct for the detection of patients with Child-Pugh class B or C; (100%, 87%, 0.94) with the SIR of cystic duct for MELD score (>10); (65%, 76%, 0.70) with the SIR of common bile duct for APRI (>1.5). Conclusion: Gadoxetic-acid contrast enhancement of cystic duct and common bile duct could be used as biomarkers to assess liver function.

  5. Biliary tract enhancement in gadoxetic acid-enhanced MRI correlates with liver function biomarkers

    Energy Technology Data Exchange (ETDEWEB)

    Noda, Yoshifumi [Department of Radiology, Gifu University Hospital, 1-1 Yanagido, Gifu, 501-1194 (Japan); Goshima, Satoshi, E-mail: gossy@par.odn.ne.jp [Department of Radiology, Gifu University Hospital, 1-1 Yanagido, Gifu, 501-1194 (Japan); Kajita, Kimihiro [Radiology Services, Gifu University Hospital, 1-1 Yanagido, Gifu 501-1194 (Japan); Kawada, Hiroshi; Kawai, Nobuyuki; Koyasu, Hiromi; Matsuo, Masayuki [Department of Radiology, Gifu University Hospital, 1-1 Yanagido, Gifu, 501-1194 (Japan); Bae, Kyongtae T. [Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, PA (United States)

    2016-11-15

    Purpose: To evaluate the association between gadoxetic-acid-enhanced magnetic resonance (MR) imaging measurements and laboratory and clinical biomarkers of liver function and fibrosis. Materials and methods: One hundred thirty nine consecutive patients with suspected liver disease or liver tumor underwent gadoxetic-acid-enhanced MR imaging. MR imaging measurements during the hepatobiliary phase included biliary tract structure-to-muscle signal intensity ratio (SIR). These measurements were compared with Child-Pugh classification, end-stage liver disease (MELD) score, and aspartate aminotransferase-to-platelet ratio index (APRI). Results: The SIRs of cystic duct and common bile duct were significantly correlated with Child-Pugh classification (P = 0.012 for cystic duct and P < 0.0001 for common bile duct), MELD score (P = 0.0016 and P = 0.0033), and APRI (P = 0.0022 and P = 0.0015). The sensitivity, specificity, and area under the receiver-operating-characteristic curve were: (74%, 88%, 0.86) with the SIR of common bile duct for the detection of patients with Child-Pugh class B or C; (100%, 87%, 0.94) with the SIR of cystic duct for MELD score (>10); (65%, 76%, 0.70) with the SIR of common bile duct for APRI (>1.5). Conclusion: Gadoxetic-acid contrast enhancement of cystic duct and common bile duct could be used as biomarkers to assess liver function.

  6. Ultrasound Imaging of Cystic Nephroma

    Directory of Open Access Journals (Sweden)

    Federico Greco

    2017-07-01

    Full Text Available Cystic nephroma is a rare, benign multicystic lesion of the kidney. This tumor occurs both in children and in adults. In children, it is highly prevalent in males; in adults, it is more frequent in women. The term “cystic nephroma” represents two apparently different entities: pediatric cystic nephroma, a benign form thought to originate from metanephric tissue, and adult cystic nephroma, considered as a lesion of mixed epithelial stromal tumor. The clinical presentation may be a palpable mass or nonspecific symptoms such as abdominal pain, hematuria, and urinary tract infections. In this review, we summarize the ultrasound imaging features of cystic nephroma and describe the characteristics of the most common renal cystic lesions and the differential diagnosis of cystic nephroma with other renal cystic lesions.

  7. 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

  8. Biliary complications after orthotopic liver transplantation

    NARCIS (Netherlands)

    Karimian, Negin; Westerkamp, Andrie C.; Porte, Robert J.

    Purpose of reviewThe incidence, pathogenesis and management of the most common biliary complications are summarized, with an emphasis on nonanastomotic biliary strictures (NAS) and potential strategies to prevent NAS after liver transplantation.Recent findingsNAS have variable presentations in time

  9. 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)

  10. 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

  11. Ultrasound findings in biliary system

    International Nuclear Information System (INIS)

    Park, Won Sik; Lee, Yong Woo; Cheung, Hwan

    1986-01-01

    In the liver and biliary system ultrasound has emerged as one of the most useful imaging techniques. It is usually the first radiological procedure selected and is often sufficient alone to enable a clinical decision to be made. Good result with ultrasound depend critically on expert scanning technique coupled with an understanding of tomographic anatomy and, of course, an appreciation of the clinical significance of any findings. In addition to we'd like to stress on the ultrasonical anatomy and for the technologist and also discuss about pathological part

  12. Congenital double bile duct presenting as recurrent cholangitis in a child

    Directory of Open Access Journals (Sweden)

    K.D. Chakravarty

    2015-12-01

    Full Text Available Double common bile duct (DCBD is a rare congenital anomaly. Most of these bile duct anomalies are associated with bile duct stones, anomalous pancreaticobiliary junction (APBJ, pancreatitis and bile duct or gastric cancers. Early detection and treatment is important to avoid long term complications. Surgical resection of the anomalous bile duct and reconstruction of the biliary enteric anastomosis is the treatment of choice. We report a rare case of DCBD anomaly in a girl, who presented with recurrent cholangitis. She had type Va DCBD anomaly. She underwent successful resection of the bile duct and reconstruction of the biliary enteric anastomosis. Preoperative imaging and diagnosis of the congenital biliary anomaly is very important to avoid intraoperative bile duct injury. Review of the literature shows very few cases of type Va DCBD, presenting with either bile duct stones or APBJ.

  13. Congenital Hydrocephalus.

    Science.gov (United States)

    Estey, Chelsie M

    2016-03-01

    There are several types of hydrocephalus, which are characterized based on the location of the cerebrospinal fluid (CSF) accumulation. Physical features of animals with congenital hydrocephalus may include a dome-shaped skull, persistent fontanelle, and bilateral ventrolateral strabismus. Medical therapy involves decreasing the production of CSF. The most common surgical treatment is placement of a ventriculoperitoneal shunt. Postoperative complications may include infection, blockage, drainage abnormalities, and mechanical failure. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Congenital diplopodia

    International Nuclear Information System (INIS)

    Brower, Jason S.; Wootton-Gorges, Sandra L.; Costouros, John G.; Boakes, Jennette; Greenspan, Adam

    2003-01-01

    Diplopodia, or duplicated foot, is a rare congenital anomaly. It differs from polydactyly in that supernumerary metatarsal and tarsal bones are present as well as extra digits. Only a few cases of this anomaly have been reported in the literature to date. We present a newborn male without intrauterine teratogen exposure who was born with a duplicate foot of the left lower extremity and imperforate anus. (orig.)

  15. Mature Cystic Renal Teratoma

    International Nuclear Information System (INIS)

    Yavuz, Alpaslan; Ceken, Kagan; Alimoglu, Emel; Akkaya, Bahar

    2014-01-01

    Teratomas are rare germline tumors that originate from one or more embryonic germ cell layers. Teratoma of the kidney is extremely rare, and less than 30 cases of primary intrarenal teratomas have been published to date. We report the main radiologic features of an unusual case of mature cystic teratoma arising from the left kidney in a two-year-old boy. A left-sided abdominal mass was detected on physical examination and B-Mod Ultrasound (US) examination revealed a heterogeneous mass with central cystic component. Computed tomography (CT) demonstrated a lobulated, heterogeneous, hypodense mass extending craniocaudally from the splenic hilum to the level of the left iliac fossa. Nephrectomy was performed and a large, fatty mass arising from the left kidney was excised. The final pathologic diagnosis was confirmed as cystic renal teratoma

  16. Congenital toxoplasmosis.

    Science.gov (United States)

    Kieffer, François; Wallon, Martine

    2013-01-01

    Congenital toxoplasmosis results from the transplacental transmission of the parasite Toxoplasma gondii after a maternal infection acquired in pregnancy. Prevalence of congenital infection ranges from 0.1 to 0.3 per 1000 live births. The maternal-fetal transmission rate increases with gestational age at maternal seroconversion, from less than 15% at 13 weeks of gestation to over 70% at 36 weeks. Conversely, the later the maternal infection, the lower the risk of symptomatic congenital infection (infections acquired during the third trimester are most often asymptomatic at birth). Prenatal diagnosis is currently performed by PCR analysis in amniotic fluid. Antenatal management and treatment vary considerably among countries. In some European countries, maternal infections are detected through serological screening allowing a prompt treatment with spiramycin, which is expected to reduce the risk of vertical transmission. If PCR analysis in amniotic fluid is positive or if maternal infection was acquired in the third trimester of pregnancy, a combination with pyrimethamine and sulphonamide is given until delivery. Benefits of antenatal treatments remain controversial. Infected newborns are prescribed pyrimethamine and sulphonamide for 12 months. Despite antenatal and postnatal treatment, chorioretinitis can occur at any age (prevalence>20% at 10 years of age): long-term ophthalmological follow-up remains necessary. Copyright © 2013 Elsevier B.V. All rights reserved.

  17. Cystic pancreatic lymphangioma

    Directory of Open Access Journals (Sweden)

    Alihan Gurkan

    2012-04-01

    Full Text Available Lymphangioma of the pancreas is a rare benign tumor of lymphatic origin. Retroperitoneal lymphangiomas account for 1% of all lymphangiomas. Herein, we report a case of cystic pancreatic lymphangioma diagnosed in 34 year-old female patient who was hospitalized for a slight pain in the epigastrium and vomiting. Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum. During the laparoscopic surgery, a well circumscribed polycystic tumor was completely excised preserving the pancreatic duct. The patient made a complete recovery and is disease-free 12 months postoperatively.

  18. Emergencies in neonatal management: jaundice and biliary atresia

    OpenAIRE

    Clemente, Maria Grazia; Dessanti, Antonio

    2016-01-01

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

  19. Hypertension and Biliary Ductopenia in a Patient with Duplication of Exon 6 of the Gene

    Directory of Open Access Journals (Sweden)

    J. Uberos

    2012-01-01

    Full Text Available We describe a neonatal patient with biliary ductopenia featuring duplication of exon 6 of the JAG1 gene. Facial alterations were observed, consisting of a prominent forehead, sunken eyes, upward slanting palpebral fissures, hypertelorism, flat nasal root and prominent chin. From birth, these were accompanied by the development of haematuria and renal failure and by renal Doppler findings indicative of peripheral renal artery stenosis. JAG1 gene mutations on chromosome 20 have been associated with various anomalies, including biliary cholestasis, vertebral abnormalities, eye disorders, heart defects and facial dysmorphia. This syndrome, first described by Alagille, is an infrequent congenital disorder caused by a dominant autosomal inheritance with variable expressivity. Anatomopathological effects include the destruction and disappearance of hepatic bile ducts (ductopenia. The duplication of exon 6 of JAG1 has not previously been described as an alteration related to the Alagille syndrome with peripheral renal artery stenosis.

  20. Ursodeoxycholic acid for cystic fibrosis-related liver disease.

    Science.gov (United States)

    Cheng, Katharine; Ashby, Deborah; Smyth, Rosalind L

    2017-09-11

    Abnormal biliary secretion leads to the thickening of bile and the formation of plugs within the bile ducts; the consequent obstruction and abnormal bile flow ultimately results in the development of cystic fibrosis-related liver disease. This condition peaks in adolescence with up to 20% of adolescents with cystic fibrosis developing chronic liver disease. Early changes in the liver may ultimately result in end-stage liver disease with people needing transplantation. One therapeutic option currently used is ursodeoxycholic acid. This is an update of a previous review. To analyse evidence that ursodeoxycholic acid improves indices of liver function, reduces the risk of developing chronic liver disease and improves outcomes in general in cystic fibrosis. We searched the Cochrane CF and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. We also contacted drug companies and searched online trial registries.Date of the most recent search of the Group's trials register: 09 April 2017. Randomised controlled trials of the use of ursodeoxycholic acid for at least three months compared with placebo or no additional treatment in people with cystic fibrosis. Two authors independently assessed trial eligibility and quality. The authors used GRADE to assess the quality of the evidence. Twelve trials have been identified, of which four trials involving 137 participants were included; data were only available from three of the trials (118 participants) since one cross-over trial did not report appropriate data. The dose of ursodeoxycholic acid ranged from 10 to 20 mg/kg/day for up to 12 months. The complex design used in two trials meant that data could only be analysed for subsets of participants. There was no significant difference in weight change, mean difference -0.90 kg (95% confidence interval -1.94 to 0.14) based on 30

  1. Cystic tumours of the pancreas

    Energy Technology Data Exchange (ETDEWEB)

    Itai, Y. [Dept. of Radiology, Inst. of Clinical Medicine, Tsukuba Univ. (Japan); Ohtomo, K. [Univ. of Tokyo Hospital, Tokyo (Japan)

    1996-12-01

    In this pictorial essay we present the typical appearances of cystic pancreatic tumours, the wide spectrum of their features, and differential features among cystic pancreatic masses with an emphasis on CT. Pseudocysts are the most common cystic lesion in the pancreas and can be induced by pancreatitis, trauma or surgery. Pseudocysts appear as a round cystic mass with a definite wall. However, they can mimic cystic tumours associated with internal septation and/or necrotic mass of various shapes. Conversely, cystic tumours can appear as a simple cyst lacking any thickening of wall, septation or mural nodule. Pancreatic carcinoma not infrequently induces secondary cysts upstream of the obstructed pancreatic duct. The cysts are pseudocysts or retention cysts in nature. When cysts are formed in the pancreatic parenchyma or adjacent to pancreatic carcinoma they may mimic cystic tumour. (orig./VHE)

  2. Cystic tumours of the pancreas

    International Nuclear Information System (INIS)

    Itai, Y.; Ohtomo, K.

    1996-01-01

    In this pictorial essay we present the typical appearances of cystic pancreatic tumours, the wide spectrum of their features, and differential features among cystic pancreatic masses with an emphasis on CT. Pseudocysts are the most common cystic lesion in the pancreas and can be induced by pancreatitis, trauma or surgery. Pseudocysts appear as a round cystic mass with a definite wall. However, they can mimic cystic tumours associated with internal septation and/or necrotic mass of various shapes. Conversely, cystic tumours can appear as a simple cyst lacking any thickening of wall, septation or mural nodule. Pancreatic carcinoma not infrequently induces secondary cysts upstream of the obstructed pancreatic duct. The cysts are pseudocysts or retention cysts in nature. When cysts are formed in the pancreatic parenchyma or adjacent to pancreatic carcinoma they may mimic cystic tumour. (orig./VHE)

  3. 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.

  4. 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.

  5. Cholecystokinin cholescintigraphic findings in the cystic duct syndrome

    International Nuclear Information System (INIS)

    Fink-Bennett, D.; DeRidder, P.; Kolozsi, W.; Gordon, R.; Rapp, J.

    1985-01-01

    Fourteen patients with a cystic duct syndrome (CDS) underwent cholecystokinin (CCK) cholescintigraphy. All patients presented with persistent postprandial right upper quadrant pain and biliary colic. None of the patients had an abnormal oral cholecystography, gallbladder (GB) ultrasound exam or upper GI series. Each patient received 5 mCi of technetium-99m disofenin. When the GB maximally filled, 0.02 microgram/kg CCK was administered (3 min) intravenously. Background corrected gallbladder ejection fractions (GBEFs) were determined every 5 min X 4 by rationing the pre-CCK GB counts minus post-CCK GB counts to pre-CCK GB counts. GBEFs were: 12% (3 patients), 17% (2), 0%, 1.3%, 3%, 4%, 6%, 11%, 14%, 18.5%, and 22% (1 each). All patients underwent a surgical exploration and all had macro- or microscopically abnormal cystic ducts with (12 patients) or without (2 patients) concomitant chronic cholecystitis. No patient with a partially occluded cystic duct with or without concomitant chronic cholecystitis had an ejection fraction that exceeded 22%. In an appropriate clinical setting, a low EF response to CCK should alert the physician to the presence of either chronic acalculous cholecystitis, CDS, or the combination of both

  6. A huge cystic craniopharyngioma

    International Nuclear Information System (INIS)

    Takamura, Seishi; Fukumura, Akinobu; Ito, Yoshihiro; Itoyama, Yoichi; Matsukado, Yasuhiko.

    1986-01-01

    The findings of computed tomography (CT) of a huge cystic craniopharyngioma in a 57-year-old woman are described. Cyst density varied from low to high levels in a short duration. Follow-up CT scans were regarded as important to diagnose craniopharyngioma. The mechanism of increment of cyst density was discussed. (author)

  7. Giant abdominal cystic lymphangioma

    International Nuclear Information System (INIS)

    Vazquez, V.; Florencio, I.; Boluda, F.

    1996-01-01

    We present a case of giant abdominal cystic lymphangioma in a 10-year-old boy. Despite numerous consultations with physicians to identify the underlying problem, it had originally been attributed to ascites of unknown cause. We review the characteristics of this lesion and the diagnostic features that aid in differentiating it from ascites

  8. Cystic hemispheric medulloepithelioma

    African Journals Online (AJOL)

    2015-12-09

    Dec 9, 2015 ... A right parieto-occipital craniotomy with subtotal resection was performed. Histological sections. (Figure 3) showed a primitive neuro-ectodermal tumour with a heterogeneous appearance and cystic change. Areas of primitive embryonal cells alternated with areas showing trabecular, papillary and.

  9. Three-dimensional reconstruction of the biliary tract using spiral computed tomography. Three-dimensional cholangiography

    International Nuclear Information System (INIS)

    Gon, Masanori; Ogura, Norihiro; Uetsuji, Shouji; Ueyama, Yasuo

    1995-01-01

    In this study, 310 patients with benign biliary diseases, 20 with gallbladder cancer, and 8 with biliary tract carcinoma underwent spiral CT (SCT) scanning at cholangiography. Depiction rate of the shape of the conjunction site of the gallbladder and biliary tract was 27.5% by conventional intravenous cholangiography (DIC), 92.5% by ERC, and 90.0% by DIC-SCT. Abnormal cystic duct course was admitted in 14.1%. Multiplanar reconstruction by DIC-SCT enabled identification of the common bile duct and intrahepatic bile duct stone. Three-dimensional reconstruction of DIC-SCT was effective in evaluating obstruction of the anastomosis or passing condition of after hepatico-jejunostomy. Two-dimensional SCT images through PTCD tube enabled degree of hepatic invasion in bile duct cancer, and three-dimensional images were useful in grasping the morphology of the bile duct branches near the obstruction site. DIC-SCT is therefore considered a useful procedure as non-invasive examination of bile duct lesions. (S.Y.)

  10. Congenital syphilis

    International Nuclear Information System (INIS)

    Lee, Sang Wook; Kim, Kyung Soo; Hur, Don

    1983-01-01

    In recent years, marked increase in incidence of congenital syphilis has occurred throughout the world due to changes in social norms and development of penicillin-resistant strains. Early diagnosis plays an important role in congenital syphilis as the clinical manifestations may simulate many other conditions in the paediatric age group. The authors analyzed 52 cases of congenital syphilis admitted to the department of paediatrics, Chosun University Hospital, clinically and radiologically. Among them, 18 cases were born in this hospital and 34 cases were admitted from OPD, during the period of 8 years from January, 1975 to December, 1982. The results obtained were as follows; 1. In 28 of 34 cases (82%), the first clinical manifestations were below the age of 3 months. 2. Among the 52 cases, a male predominance was observed with a male to female ratio of 2 : 1. 3. The serologic test (VDRL) of the 52 studied cases showed reactive response in 49 cases (94%), and that of syphilitic mothers except 6 cases, reactive in all studied cases. 4. The major manifestations of the 52 cases were bone tenderness (12%) and swelling of the joints (7%) in skeletal system, hepatosplenomegaly (79%) and skin lesions (73%) in extraskeletal one. 5. The radiological skeletal changes were detected in 45 of 52 cases (87%), and the commonest findings were detected in 45 of 52 cases (87%), and the commonest findings were metaphysitis (83%) and periostitis (81%). The most characteristic type of metaphysitis were transverse trophic line (74%) and zone of rarefaction (65%). 6. The commonest bones to be affected were growing metaphyses of the long bones, particulary about the wrist and the knee. The order of frequency were radius (80%), uina (80%), tibia (77%), femur (69%) and humerus (40%)

  11. Congenital transmesenteric hernia presenting as neonatal ascites

    Directory of Open Access Journals (Sweden)

    Andreia Felizes

    2018-01-01

    Full Text Available Transmesenteric hernia is an internal hernia without sac, which forms through a congenital mesentery defect. Mostly diagnosed intraoperatively, it as a variable prognosis, which can lead to high morbidity and mortality.The authors describe a case of transmesenteric hernia that presented as isolated fetal ascites. A 34 week preterm baby was delivered by forceps, with respiratory compromise due to abdominal distension. Orotracheal intubation and evacuation paracentesis were performed. After excluding major causes of neonatal ascites and persistent bowel loop distension, the newborn underwent an exploratory laparotomy, where a transmesenteric hernia and pellets of meconium were identified. Hernia reduction, enterectomy and enterostomies were performed, with good outcome. Cystic fibrosis was diagnosed during post-operatory period.This is the first reported case of transmesenteric hernia presenting as fetal ascites, without associated morbidity or mortality due to an early intervention. Keywords: Transmesenteric hernia, Fetal ascites, Cystic fibrosis

  12. 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.)

  13. 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.

  14. Adult-onset cystic hygroma: A case report of rare entity.

    Science.gov (United States)

    Bahl, Sumit; Shah, Vandana; Anchlia, Sonal; Vyas, Siddharth

    2016-01-01

    Cystic hygroma is a benign congenital malformation of the lymphatic system that occurs in infant or children younger than 2 years of age. Although cystic hygroma is well recognized in pediatric practice, it seldom presents de novo in adulthood. These are commonly present in head and neck but can be present anywhere. Cystic hygroma is very rare in adults, but it should be considered in the differential diagnosis of adult neck swellings. Patients presenting with a painless, soft, fluctuant, and enlarging neck mass should have a careful history and physical examination along with radiological imaging to assist with diagnosis. Surgical intervention is the treatment of choice for this rare condition. Here, we are reporting a case of cystic hygroma in a 32-year-old male patient in the neck region. The objectives of this case report are to discuss the clinical presentation, diagnosis, histopathological findings and management of this malformation.

  15. 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.

  16. 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.

  17. The gallbladder and biliary ducts

    International Nuclear Information System (INIS)

    Amberg, J.R.; Juhl, J.H.; Univ. of California, San Diego, Medical Center, Veterans Administration Hospital, La Jolla, CA)

    1987-01-01

    There is an extensive menu for investigating the hepatobiliary area. From the simplicity of the plain film to the expense of magnetic resonance imaging to the invasiveness of percutaneous transhepatic cholangiography, all modalities can contribute to the goal of a correct diagnosis. Not all are needed in each patient; thus a careful evaluation of the clinical needs is required before proceeding. It is also apparent that changes are occurring rapidly. The current importance of gallbladder ultrasonography and computerized tomography and the decline of oral cholecystography and intravenous cholangiography in biliary tract diagnosis was impossible to anticipate a decade ago. Because not all modalities are available in all communities, it is important to tailor the diagnostic algorithm to local skills and equipment

  18. 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.

  19. 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.

  20. Bisphosphonates for osteoporosis in primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Rudic, Jelena; Giljaca, Vanja; Krstic, Miodrag N

    2011-01-01

    Bisphosphonates are widely used for treatment of postmenopausal osteoporosis. Patients with primary biliary cirrhosis often have osteoporosis - either postmenopausal or secondary to the liver disease. No systematic review or meta-analysis has assessed the effects of bisphosphonates for osteoporosis...

  1. 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...

  2. [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.

  3. Abdominal manifestations of cystic fibrosis in children

    International Nuclear Information System (INIS)

    Chaudry, Gulraiz; Navarro, Oscar M.; Levine, Daniel S.; Oudjhane, Kamaldine

    2006-01-01

    Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities. (orig.)

  4. Abdominal manifestations of cystic fibrosis in children

    Energy Technology Data Exchange (ETDEWEB)

    Chaudry, Gulraiz; Navarro, Oscar M.; Levine, Daniel S.; Oudjhane, Kamaldine [University of Toronto, Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)

    2006-03-15

    Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities. (orig.)

  5. 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.

  6. Sealing of the cystic and appendix arteries with monopolar electrocautery during laparoscopic combined cholecystectomy and appendectomy.

    Science.gov (United States)

    Liu, Gui-Bao; Mao, Yuan-Yuan; Yang, Chang-Ping; Cao, Jin-Lin

    2018-03-01

    The best method to ligate the arteries during laparoscopic cholecystectomy or appendectomy remains controversy. The aim of this study is to introduce a new approach during laparoscopic combined cholecystectomy and appendectomy using a monopolar electrocautery to seal the cystic and appendix arteries. We retrospectively reviewed data from 57 patients who underwent laparoscopic combined cholecystectomy and appendectomy between December 2006 and June 2016. Each laparoscopic combined cholecystectomy and appendectomy was performed by coagulating and sealing the cystic and appendix arteries. Absorbable clip or coils were then used to ligate the proximal of cystic duct and the stump of appendix. The other side of the cystic duct and appendix which subsequently were to be removed from abdomen were used titanium clips or silk ligature. Of the 57 patients, 3 patients (5.3%) were converted to open surgery due to severe abdominal adhesions or gallbladder perforation. The mean operative time was 56 minutes (range, 40-80 minutes). Mean blood loss was 12 mL (range, 5-120 mL), and the mean postoperative hospital stay was 3.0 days (range, 2-5 days). No postoperative bleeding, biliary leakage, infection, or mortality occurred. Monopolar electrocautery to seal the cystic and appendix arteries is a safe, effective, and economical surgical procedure during laparoscopic combined cholecystectomy and appendectomy. Further randomized controlled clinical trials are required to validate our findings.

  7. Congenital amusia.

    Science.gov (United States)

    Williamson, Victoria J; Stewart, Lauren

    2013-01-01

    For most people, music, like language, is acquired effortlessly in early life. But a few percent of the population have lifelong difficulties in the perception and production of music. In this chapter we discuss psycho-acoustic and behavioral studies that have attempted to delineate the nature of the auditory perceptual deficits in this group and consider whether these difficulties extend outside the musical domain. Finally, we review structural imaging studies in this group which point to subtle anomalies in temporal and frontal areas. We suggest that amusia can be considered a disorder of neural development, which has relatively specific consequences at the behavioral level. Studies of congenital amusia provide a unique window on the neurocognitive architecture of music processing. Copyright © 2013 Elsevier B.V. All rights reserved.

  8. Cystic pulmonary hydatidosis

    Directory of Open Access Journals (Sweden)

    Malay Sarkar

    2016-01-01

    Full Text Available Cystic echinococcosis (CE is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2012-12-15

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

  10. 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.

  11. Prenatal Diagnosis of Congenital Cystic Adenomatoid Malformations: Evolution and Outcome

    Directory of Open Access Journals (Sweden)

    Wei-Shiu Chen

    2009-09-01

    Conclusion: The outcomes of the prenatally detected CCAMs were good in our cases. If the CCAM is not complicated by hydrops fetalis, maintaining the pregnancy with continuing management seems to be a reasonable recommendation. Despite antenatal resolution of CCAM on ultrasound, postnatal examination with chest radiography and computed tomography scan is necessary.

  12. Biventricular heart failure secondary to a pericardial cystic mass: case report

    Energy Technology Data Exchange (ETDEWEB)

    Nizzero, A. [Sudbury Regional Hospital, Dept. of Diagnostic Imaging, Sudbury, Ontario (Canada); Dobranowski, J. [St. Joseph' s Hospital, Dept. of Radiology, Hamilton, Ontario (Canada); Tanser, P. [St. Joseph' s Hospital, Dept. of Cardiology, Hamilton, Ontario (Canada)

    2000-07-01

    Cystic masses of the pericardium causing symptoms due to cardiac compression are very unusual. Such cysts may be congenital, or they may occur secondary to inflammatory processes or hemorrhage, similar to cysts seen in the pleura or peritoneum. Echocardiography, computed tomography (CT) and magnetic resonance imaging (MRI) are useful for noninvasive investigation of the pericardium, although in the remote past, definitive diagnosis was possible only with thoracotomy. We present a case of biventricular cardiac failure secondary to a calcified pericardial cystic mass in a patient with constrictive pericarditis. Because of the extensive calcification, echocardiography was not helpful. CT and MRI allowed excellent delineation of the nature and effects of this abnormality. (author)

  13. Cholangitis following percutaneous biliary drainage

    International Nuclear Information System (INIS)

    Audisio, R.A.; Bozzetti, F.; Cozzi, G.; Severini, A.; Belloni, M.; Friggerio, L.F.

    1989-01-01

    The binomial PTBD-cholangitis often stands under different and sometimes even opposite relations. Among its indications the procedure lists, the treatment of cholangitis which, on the other hand, may be itself a complication of biliary drainage. The present work proposes a critical review of cholangitis-PTBD correlations, from an ordinary clinical-radiological point of view. Different pathogenetic hypothesis of cholangitis (inflammation, cholestasis, surgical manipulation) are discussed together with risk factors (impaired macrophagic-phagocytic system, immunosuppresion, wide neoplastic liver involvement, multiple intrahepatic ductal obstructions, chronic liver diseases, aged patients, etc.). The authors also report about prevention and treatment of septic complications which must be carried out following technical and therapeutic strategies, such as chemoprophylaxis and focused antibiotic therapy according to coltural samples, slow injection of small amounts of contrast medium, peripheral branches approach, gentle handling of catheters and guidewires, flushing with saline solutions and brushing of the catheter itself, and finally use of large gauge catheters in the presence of bile sludge

  14. Diagnostic imaging of digestive tract involvement in cystic fibrosis. Part 1: hepatobiliary disease

    International Nuclear Information System (INIS)

    Miralles, M.; Gonzalez, G.; Serrano, C.; Manzanares, J.; Berrocal, T.

    1998-01-01

    Cystic fibrosis is a severe hereditary disease characterized by epithelial chloride channel dysfunction, leading to the production of abnormally thick secretions. The abnormal gene is located on the long arm of chromosome 7. Hepatobiliary involvement derives from ductal obstruction causing cholestasis, steatosis, cirrhosis and portal hypertension. Biliary sludge, cholelithiasis and gallbladder sclerosis and atrophy are common findings. As the correlation between the hepatobiliary changes and their clinical and analytical impact is very limited, imaging techniques are essential in this disease. Ultrasound is the basic imaging tool, both for initial evaluation and follow-up of the hepatic and biliary involvement, although other techniques such as radionuclide imaging, magnetic resonance and computed tomography can be highly useful. Given the long-term, chronic nature of this disease, the use of aggressive techniques or irradiation should be carefully weighed. (Author) 38 refs

  15. 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

  16. [Congenital hypothyroidism].

    Science.gov (United States)

    Castilla Peón, María Fernanda

    Congenital hypothyroidism (CH) is a cause of preventable mental retardation; therefore, timely diagnosis and treatment by the primary care physician is very important. CH screening must be performed between the second and fifth days of life with capillary blood done with a heel prick and must be confirmed by measurement of thyroid hormones in venous blood. The most common cause of CH is thyroid dysgenesis, which may be identified by a thyroid scan carried out before initiating treatment. Treatment should be with levothyroxine (10-15μg/kg/day) and should not be delayed or suspended during the first 3 years of life due to the deleterious effect on neurodevelopment in case of low thyroid hormones during this time. Preterm or sick infants or those with Down syndrome require special consideration. This article provides diagnostic and therapeutic algorithms for CH. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  17. Percutaneous transhepatic biliary drainage through the normal duct in patients with post-operative bile leakage

    International Nuclear Information System (INIS)

    Lee, Hyun; Kim, Young Hwan; Kim, Yong Joo

    2004-01-01

    To evaluate the technical feasibility and clinical efficacy of percutaneous transhepatic biliary drainage (PTBD) through the normal duct in patients with post-operative bile leakage. From January 1998 to December 2003, fourteen patients (male: 12, female: 2, mean age: 56) with biliary leak after laparoscopic cholecystectomy (n = 5), T-tube removal (n = 5), choledochojejunostomy due to small bowel perforation (n = 1), right lobectomy (n = 1), laparoscopic adrenalectomy (n = 1), and subtotal gastrectomy (n = 1) were treated by means of PTBD; this was performed with the two-step approach. The central bile duct was cannulated using a 21-G Chiba needle to map the intrahepatic biliary tree. An 8.5-F drainage catheter tip was positioned at the CBD after puncturing peripheral bile duct with an additional Chiba needle. We evaluated the technical feasibility, the procedure-related complications, clinical efficacy and the duration of catheter placement. PTBD of the normal duct with the two-step approach was successful in all but two cases. In these two cases, the two-step approach was failed due to the rapid disappearance of the targeted peripheral duct, and this was the result caused by biloportal fistula. PTBD was performed through the central bile duct in one patient, and through the remnant cystic duct in one patient. There were no procedure-related complications except for mild abdominal pain in seven patients. Bile leakage was demonstrated on cholangiogram in 10 of 14 patients; this occurred at the T-tube exit site (n = 4), cystic duct stump (n = 2), choledochojejunostomy site (n = 1), resection margin of liver (n = 1), caudate lobe (n = 1), and GB bed (n = 1). In 13 patients, the biliary leak stopped after drainage (mean duration: 32.1 days). In one patient, surgical management was performed one day after PTBD due to the excessive amount of bile leakage. PTBD is a technically feasible and clinically efficacious treatment for post-operative bile leakage, and it can

  18. [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.

  19. 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.

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

    International Nuclear Information System (INIS)

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

    2000-01-01

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

  1. Imaging from cystic fibrosis

    International Nuclear Information System (INIS)

    Schmidt, H.; Posselt, H.G.

    2008-01-01

    Cystic fibrosis (CF) is the most frequent metabolic disorder with autosomal recessive inheritance in the Caucasian population. The gene defect is located on the long arm of chromosome 7. In Germany today, the actual median survival is 37 years. The genetic defect caused by chloride anion disturbances affects multiple body systems but the morbidity and mortality is due to lung disease. The secretion of highly viscous mucus promotes viral and bacterial pulmonary infections leading to airway obstruction and consecutive destruction of the lung parenchyma. This article will review and discuss both the clinical aspects of the disease and the diagnostic methods, referring in particular to new imaging strategies. (orig.)

  2. 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

  3. 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.

  4. 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.

  5. Update on Primary Biliary Cirrhosis

    Directory of Open Access Journals (Sweden)

    Jenny Heathcote

    2000-01-01

    Full Text Available The diagnosis of primary biliary cirrhosis (PBC is most often made in the asymptomatic phase, sometimes before the development of abnormal liver biochemistry. The antimitochondrial antibody remains the predominant hallmark, although not all patients test positive, even when the most sensitive techniques are used. The etiology of PBC remains elusive; studies suggest that the interlobular bile duct destruction is immune based, and associated autoimmune diseases are common. There are no surrogate markers that predict outcome in asymptomatic patients, whose chance of survival is less than that of age- and sex-matched populations but much better than the median survival of eight years in patients with symptomatic PBC. Symptoms common in this disease are fatigue, pruritus and xanthelasma, as well as complications of portal hypertension and osteoporosis. Treatment includes symptomatic and preventive measures, as well as specific therapeutic measures. Immunosuppressive therapy has yielded disappointing results in the long term management of PBC, and the only therapy shown to improve survival is the hydrophobic dihydroxy bile acid ursodeoxycholic acid. Treatment at a dose of 13 to 15 mg/kg/day is optimal, given in separate doses or as a single dose at least 4 h from giving the oral anion exchange resin cholestyramine, which may be used to control pruritus. However, liver transplantation remains the only cure for this disease, and the best postoperative survival is seen in patients whose serum bilirubin does not exceed 180 µmol/L at the time of liver transplantation. Recurrence takes place but is rarely symptomatic and does not deter from the benefits of transplantation.

  6. Prenatal diagnosis of congenital ranula: Case Report

    Directory of Open Access Journals (Sweden)

    Aytül Çorbacıoğlu Esmer

    2013-12-01

    Full Text Available Congenital ranula is a mucous retention cyst which originates from the base of the oral cavity and is caused by sublingual or submandibular canal atresia or canalization defect. As with the other tumors in the oral cavity it may cause upper airway obstruction and hypoxia immediately after delivery. In the following stages of life, apart from the respiratory problems it may cause speach, chewing and swallowing disorders. The prenatal diagnosis of congenital sublingual ranula is very rare, and it presents as an avascular and anechoic cystic mass displacing the tongue upwards on fetal ultrasonography. Polyhydramnios can develop due to the obstruction and stomach may not be visualized. The differential diagnosis includes lymphatic malformations, epulis, epignatus, tyroglossal canal cyst and hemangioma. In this paper, we present a case of ranula which was diagnosed during prenatal ultrasonographic examination. The detailed fetal ultrasound scan performed at the 31th week of gestation revealed a non-septated anechoic cyst in the oral cavity measuring 20 x 17 x 15 mm. The cyst had well-defined regular borders with no solid component. Doppler ultrasonograhy did not show any vascularization within the cyst. After the delivery at the 38th week of gestation, a sublingual cystic mass with regular borders measuring 1.5 x1.5 cm was detected in the mouth of the neonate. Entubation was not required due to the absence of respiratory distress. In order to prevent difficutlies in feeding, the cystic mass was aspirated just after the delivery. A diagnosis of sublingual ranula was made based on the localization and the mucoid consistency of the cyst.

  7. [Tracheobronchial and pulmonary parenchymatous congenital abnormalities requiring surgical treatment in adults].

    Science.gov (United States)

    Mordant, P; De Dominicis, F; Berna, P; Riquet, M

    2012-04-01

    Most tracheobronchial and parenchymatous congenital abnormalities of the respiratory system are diagnosed in early life. However, some lesions may be initially silent and diagnosed only in adulthood. These cases included congenital abnormalies of the tracheobronchial tract (tracheal and/or bronchial stenosis, bronchogenic cysts, bronchial atresia, oesotracheal fistula, oesobronchial fistula, and tracheal diverticulum), and lung parenchyma itself (pulmonary sequestration, congenital cystic adenomatoïd malformation, lobar emphysema, lobar or lung hypoplasia). To avoid dreadful complications, these rare cases deserve surgical management, and must be known by chest physicians and surgeons. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  8. Congenital platelet function defects

    Science.gov (United States)

    ... pool disorder; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital ... Congenital platelet function defects are bleeding disorders that cause reduced platelet function. Most of the time, people with these disorders have ...

  9. Congenital Heart Information Network

    Science.gov (United States)

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  10. Congenital heart disease

    Science.gov (United States)

    Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth. ... Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, ... Sabiston Textbook of Surgery: The Biological Basis of Modern ...

  11. 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)

  12. 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.)

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

    Directory of Open Access Journals (Sweden)

    Dipanjan Panda

    2016-06-01

    Full Text Available We present a case report of broncho-biliary fistula that developed due to the blockage of biliary stent placed during the management of pancreatic neuroendocrine tumor (pNET; diagnosed on high clinical suspicion, percutaneous cholangiogram and contrast enhanced computed tomography (CECT; and successfully treated with percutaneous transhepatic biliary drainage (PTBD.

  14. 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)

  15. Congenital Intrahepatic Portosystemic Shunts

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Woong Hee; Kim, Young Tong; Jou, Sung Shick; Shin, Hyeong Cheol [Soonchunhyang University, Asan (Korea, Republic of)

    2008-12-15

    Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/IVC, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult

  16. Cystic tumors of the pancreas

    International Nuclear Information System (INIS)

    Brambs, H.J.; Juchems, M.

    2008-01-01

    Cystic lesions of the pancreas encompass a broad spectrum of benign, premalignant, and malignant tumors which are primarily cystic or result from cystic necroses of solid neoplasms. Because of the wide use of cross-sectional imaging techniques they are increasingly being identified in asymptomatic patients as well as in patients presenting with abdominal pain, jaundice or pancreatitis. Among these lesions, intraductal papillary mucinous neoplasms, serous cystic neoplasms and mucinous cystic neoplasms represent the majority of cases. With increasing experience with these tumors, a refinement of our understanding of their morphology and of their natural course has emerged. It is important to be familiar with the CT and MR imaging features of these lesions to differentiate these tumors and to orient the diagnosis towards benign or malignant forms. Because characterization of cystic tumors of the pancreas can sometimes be difficult due to overlapping imaging features, additional criteria such as clinical symptoms, localization, age and gender have to be taken into account. If appropriately treated, these tumors can usually be cured by resection and the decreasing risk of pancreatic surgery has led to an increasing number of resections of pancreatic tumors. The management of cystic tumors of the pancreas has not yet been standardized and the correct evaluation and subsequent management of the disease in asymptomatic patients have not been fully defined. (orig.) [de

  17. 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.

  18. Gastrointestinal Manifestations of Cystic Fibrosis

    Science.gov (United States)

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  19. Cholecystokinin (CCK) functional cholescintigraphic findings in patients with a partial cystic duct obstruction - the cystic duct syndrome (CDS)

    International Nuclear Information System (INIS)

    Fink-Bennett, D.; DeRidder, P.; Kolozsi, W.; Gordon, R.

    1984-01-01

    Fourteen patients (pts.) with a CDS underwent CCK functional cholescintigraphy (FC). All pts. presented with persistent post-prandial right upper quadrant pain and biliary colic. None had an abnormal OCG, gallbladder (GB) ultrasound exam or upper G.I. series. All had macro- or microscopically abnormal cystic ducts (5 fibrotic, 7 elongated and narrow, 2 kinked) with (12) or without (2) concomitant chronic cholecystitis. Each pt. (NPO after 12 A.M.) received 5 mCi of technetium (TC)-99m Hepatolite. When the GB max. filled, .02 ug/kg CCK was administered (3 min.) I.V. Background corrected GBEFs were determined q.5 min. x 4 by ratioing the pre-CCK GB cts. minus post-CCK GB cts. to pre-CCK GB cts. GB EFRs were: 3 (12%), 2 (17%), and 1 each 0%, 1.3%, 3%, 4%, 6%, 11%, 14%, 18.5% and 22%. No pt. with a partially occluded cystic duct with or without concomitant chronic cholecystitis had an ejection fraction that exceeded 22%. In an appropriate clinical setting, a low ejection fraction response to CCK should alert the physician to the presence of either chronic acalculous cholecystitis, CDS, or the combination of both

  20. Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue

    International Nuclear Information System (INIS)

    Waelti, Stephan L.; Garel, Laurent; Rypens, Francoise; Dubois, Josee; Dal Soglio, Dorothee; Messerli, Michael

    2017-01-01

    The differential diagnosis for primary lung masses in neonates includes a variety of developmental abnormalities; it also consists of the much rarer congenital primary lung tumors: cystic pleuropulmonary blastoma (cystic PPB), fetal lung interstitial tumor (FLIT), congenital peribronchial myofibroblastic tumor (CPMT), and congenital fibrosarcoma. Radiologic differentiation between malformations and tumors is often very challenging. The objective was to establish distinctive features between developmental pulmonary abnormalities and primary lung tumors. We conducted a retrospective study of 135 congenital lung lesions at a university mother and child center over a period of 10 years (2005-2015). During this time, we noted four tumors (two cystic PPBs and two FLITs) and 131 malformations. We recorded the following parameters: timing of conspicuity in utero (mid-second trimester, third trimester, or not seen prenatally), presence of symptoms at birth, prenatal and perinatal radiologic findings, and either histological diagnoses by pathology or follow-up imaging in non-operated cases. All lesions except the four tumors were detected during mid-second-trimester ultrasound. In none of the tumors was any pulmonary abnormality found on the mid-second-trimester sonogram, contrary to the developmental pulmonary abnormalities. The timing of conspicuity in utero appears to be a key feature for the differentiation between malformations and tumors. Lesions that were not visible at the mid-second-trimester ultrasound should be considered as tumor. A cystic lung lesion in the context of a normal mid-second-trimester ultrasound is highly suggestive of a cystic PPB. Differentiating the types of solid congenital lung tumors based upon imaging features is not yet feasible. (orig.)

  1. Neonatal congenital lung tumors - the importance of mid-second-trimester ultrasound as a diagnostic clue

    Energy Technology Data Exchange (ETDEWEB)

    Waelti, Stephan L.; Garel, Laurent; Rypens, Francoise; Dubois, Josee [University of Montreal, Department of Medical Imaging, Sainte-Justine Hospital, Quebec (Canada); Dal Soglio, Dorothee [University of Montreal, Department of Pathology, Sainte-Justine Hospital, Quebec (Canada); Messerli, Michael [University Hospital Zurich, University of Zurich, Department of Nuclear Medicine, Zurich (Switzerland)

    2017-12-15

    The differential diagnosis for primary lung masses in neonates includes a variety of developmental abnormalities; it also consists of the much rarer congenital primary lung tumors: cystic pleuropulmonary blastoma (cystic PPB), fetal lung interstitial tumor (FLIT), congenital peribronchial myofibroblastic tumor (CPMT), and congenital fibrosarcoma. Radiologic differentiation between malformations and tumors is often very challenging. The objective was to establish distinctive features between developmental pulmonary abnormalities and primary lung tumors. We conducted a retrospective study of 135 congenital lung lesions at a university mother and child center over a period of 10 years (2005-2015). During this time, we noted four tumors (two cystic PPBs and two FLITs) and 131 malformations. We recorded the following parameters: timing of conspicuity in utero (mid-second trimester, third trimester, or not seen prenatally), presence of symptoms at birth, prenatal and perinatal radiologic findings, and either histological diagnoses by pathology or follow-up imaging in non-operated cases. All lesions except the four tumors were detected during mid-second-trimester ultrasound. In none of the tumors was any pulmonary abnormality found on the mid-second-trimester sonogram, contrary to the developmental pulmonary abnormalities. The timing of conspicuity in utero appears to be a key feature for the differentiation between malformations and tumors. Lesions that were not visible at the mid-second-trimester ultrasound should be considered as tumor. A cystic lung lesion in the context of a normal mid-second-trimester ultrasound is highly suggestive of a cystic PPB. Differentiating the types of solid congenital lung tumors based upon imaging features is not yet feasible. (orig.)

  2. Large Omental Cystic Lymphangioma Masquerading as Mucinous Ovarian Neoplasia in an 8-Year-Old Premenarchal Girl: The Findings from Diagnostic Imaging and Laparoscopic-Assisted Excision.

    Science.gov (United States)

    Takeda, Akihiro; Ito, Hiroaki; Nakamura, Hiromi

    2017-12-01

    Omental cystic lymphangioma is an extremely rare abdominal mass caused by congenital malformation. An 8-year-old premenarchal girl reported abdominal pain. Diagnostic imaging revealed a large multicystic mass measuring 22 cm in diameter, which occupied the entire abdominal cavity with ascites. Emergency laparoscopy revealed a ruptured large cystic mass originating from the greater omentum; this was followed by successful laparoscopic-assisted excision. The pathological diagnosis was omental cystic lymphangioma. The present findings show that omental cystic lymphangioma masquerading as mucinous ovarian neoplasia was a rare cause of acute abdominal events in a young girl. The present case shows that minimally invasive surgery can be a feasible option, which might achieve a favorable outcome in a young patient with an omental cystic lymphangioma. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  3. A role of computed tomography or magnetic resonance imaging for cystic lung diseases in children

    International Nuclear Information System (INIS)

    Yanagihara, Jun; Shimotake, Takashi; Deguchi, Eiichi; Tokiwa, Kazuaki; Iwai, Naomi

    1995-01-01

    Fourteen children with cystic lung diseases were imaged using computed tomography (CT) and/or magnetic resonance imaging (MRI). These patients studied included 7 patients with bronchogenic cyst of lung, 4 with pulmonary sequestration, 1 with congenital cystic adenomatoid malformation, 1 with lung abscess, and 1 with cystic bronchoectasis. MRI identified all lesions seen on the chest radiographs. It was particularly valuable delineating contents of bronchogenic cyst without the need for contrast material enhancement. However, MRI was not quite as sensitive as CT that detected very small abnormalities, because MRI had more artifacts than those of CT. In conclusion, we believe that both CT and MRI are needed for differential diagnosis of lung diseases in children. (author)

  4. A role of computed tomography or magnetic resonance imaging for cystic lung diseases in children

    Energy Technology Data Exchange (ETDEWEB)

    Yanagihara, Jun; Shimotake, Takashi; Deguchi, Eiichi; Tokiwa, Kazuaki; Iwai, Naomi [Kyoto Prefectural Univ. of Medicine (Japan)

    1995-06-01

    Fourteen children with cystic lung diseases were imaged using computed tomography (CT) and/or magnetic resonance imaging (MRI). These patients studied included 7 patients with bronchogenic cyst of lung, 4 with pulmonary sequestration, 1 with congenital cystic adenomatoid malformation, 1 with lung abscess, and 1 with cystic bronchoectasis. MRI identified all lesions seen on the chest radiographs. It was particularly valuable delineating contents of bronchogenic cyst without the need for contrast material enhancement. However, MRI was not quite as sensitive as CT that detected very small abnormalities, because MRI had more artifacts than those of CT. In conclusion, we believe that both CT and MRI are needed for differential diagnosis of lung diseases in children. (author).

  5. OK432 (picibanil) efficacy in an adult with cystic cervical lymphangioma. A case report.

    Science.gov (United States)

    Alonso, Juan; Barbier, Luis; Alvarez, Julio; Romo, Laura; Martín, Jesús C; Arteagoitia, Iciar; Santamaría, Joseba

    2005-01-01

    Cervical cystic lymphangioma (CCL) is a rare and benign tumour involving congenital and cystic abnormalities derived from lymphatic vessels. The most accepted treatment continues to be surgical excision. However, when this infiltrates vital neurovascular neck structures, complete excision is difficult and if only partial, the recurrence rate is very high. The most frequently used alternative treatment is to inject sclerosants into the lesion. The use of these techniques has reported good results in children; however, there are few references thereof with regard to adults. We are reporting on a cervical cystic lymphangioma in a male aged 22, treated with an intra-lesion injection of 20 cc with 0.01 mg/cc dilution of OK-432 (picibanil) in physiological serum. Sole complications were fever and local reaction where the solution was injected. One month after treatment the lymphangioma had totally remitted and sixteen months later continues in remittance.

  6. Percutaneous Transcholecystic Biliary Interventions Using Gallbladder Anchors: Feasibility Study in the Swine

    International Nuclear Information System (INIS)

    Lopera, Jorge E.; Kirsch, David; Qian Zhong; Ruiz, Bernardo; Brazzini, Augusto; Gonzales, Arturo; Castaneda-Zuniga, Wilfrido

    2005-01-01

    The purpose of this study was to report our initial experience with a swine model for biliary interventions by using a percutaneous transcholecystic access after suture anchor of the gallbladder. Telepaque tablets were given to five pigs to opacify the gallbladder. Under fluoroscopy, the opacified gallbladder was punctured percutaneously and three suture anchors were used to fix the anterior wall of the gallbladder to the abdominal wall. Two weeks later, the gallbladder was punctured and access into the distal common bile was obtained through the cystic duct. Balloon expandable stents were deployed into the distal common bile duct. Follow-up cholangiograms were obtained at 1 and 2 weeks. Necropsy was performed after 2 weeks to evaluate the relationship between the gallbladder and abdominal wall. Suture anchor placement was successful in all five pigs. One pig with a deep and highly positioned gallbladder developed fever, anorexia, and vomiting secondary to excessive stretch of the gallbladder. Placement of the guidewire through the extremely tortuous and small cystic ducts proved to be the most challenging step of the procedure. Metallic stents were successfully deployed in all four pigs in which it was attempted. Four animals tolerated the procedures without changes in their clinical conditions and no symptoms. Successful follow-up cholangiograms were performed at 1 and 2 weeks post-stent deployment without complications. All stents remained patent during the follow-up period. Necropsy demonstrated close attachment and adherence of the gallbladders to the antero-lateral abdominal wall in all four animals. Suture anchoring of the gallbladder is feasible in most pigs with superficially located gallbladders. This technique allows a safe and repeat access into the biliary system using a transcholecystic approach

  7. Clinical significance of magnetic resonance cholangiopancreatography for the diagnosis of cystic tumor of the pancreas compared with endoscopic retrograde cholangiopancreatography and computed tomography

    International Nuclear Information System (INIS)

    Mera, Kiyomi; Tajiri, Hisao; Muto, Manabu

    1999-01-01

    Cystic tumor of the pancreas has been investigated by a variety of imaging techniques. Magnetic resonance cholangiopancreatography (MRCP) is being widely used as a non-invasive diagnostic modality for investigation of the biliary tree and pancreatic duct system. The purpose of this study was to compare MRCP images with those of endoscopic retrograde cholangiopancreatography (ERCP) and computed tomography (CT) in order to clarify the diagnostic efficacy of MRCP for cystic tumor of the pancreas. We retrospectively studied 15 patients with cystic tumor of the pancreas that had been surgically resected and histopathologically confirmed. There were five cases of intraductal papillary adenocarcinoma, five of intraductal papillary adenoma, two of serous cyst adenoma, two of retention cyst associated with invasive ductal adenocarcinoma and one of solid cystic tumor. In all cases MRCP correctly identified the main pancreatic duct (MPD) and showed the entire cystic tumor and the communication between the tumor and the MPD. On the other hand, the detection rate by ERCP of the cystic tumor and the communication between the cystic tumor and the MPD was only 60%. Although the detection rates by CT for the septum and solid components inside the cystic tumor were 100 and 90.0%, respectively, those of MRCP for each were 58.3 and 20.0%. MRCP is capable of providing diagnostic information superior to ERCP for the diagnosis of cystic tumor of the pancreas. Although MRCP may provide complementary information about the whole lesion of interest, the characteristic internal features of cystic tumor of the pancreas should be carefully diagnosed in combination with CT. (author)

  8. Sonographic Finding of Scrotal Cystic Lymphangioma with Hemorrhage Caused by Percutaneous Needle Aspiration: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jae Woon; Cho, Jae Ho [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2011-03-15

    Lymphangioma is a hamartoma, which is a benign tumor caused by congenital malformation of the lymphatic system. These tumors usually occur in the neck and axilla, and occasionally in the mediastinum, mesenterium, retroperitoneum and thigh, The scrotum and perineum are the least frequent sites. We report here on an uncommon case of cystic lymphangioma that presented as focal hemorrhage caused by percutaneous needle aspiration, and we briefly review the radiologic finding and the relevant literature

  9. 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.

  10. 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)

  11. Fatal liver gas gangrene after biliary surgery

    Directory of Open Access Journals (Sweden)

    Yui Miyata

    2017-01-01

    Discussion: Liver gas gangrene is rare and has a high mortality rate. This case seems to have arisen from an immunosuppressive state after major surgery with biliary reconstruction for bile duct cancer and subsequent gastrointestinal bleeding, leading to gas gangrene of the liver.

  12. Primary biliary cirrhosis: Diagnostic and therapeutic aspects

    NARCIS (Netherlands)

    E.M.M. Kuiper (Edith)

    2010-01-01

    textabstractPrimary Biliary Cirrhosis (PBC) is a relatively rare cholestatic liver disease. The first case was described by Addison and Gull in 1851. The name PBC is generally accepted, however in fact this is a misnomer since cirrhosis is found in a minority of patients. PBC is one of the most

  13. 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

  14. Giant cystic craniopharyngiomas

    International Nuclear Information System (INIS)

    Young, S.C.; Zimmerman, R.A.; Nowell, M.A.; Bilaniuk, L.T.; Hackney, D.B.; Grossman, R.I.; Goldberg, H.I.

    1987-01-01

    Three cases of giant cystic craniopharyngiomas with large areas of extension beyond the suprasellar area are presented. The magnetic resonance (MR) appearance in one case is described. These giant tumors had large, multilobulated cysts that comprised the bulk of the tumors. In one case, there was an unusual extension of the large tumor cyst into the lateral ventricle. In two cases, the tumors extended to the level of the foramen magnum. On CT, the cyst contents of these two tumors were hyperdense and became hypodense postoperatively. All three tumors harbored calcifications in the form of clumps in the suprasellar region and rim calcifications around the cysts. None of the tumors exhibited contrast enhancement. A literature review of the radiographic features of craniopharyngiomas is discussed. (orig.)

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

    Science.gov (United States)

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

    1999-01-01

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

  16. Profile of cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Mona M. El-Falaki

    2014-09-01

    Full Text Available It was generally believed that Cystic fibrosis (CF is rare among Arabs; however, the few studies available from Egypt and other Arabic countries suggested the presence of many undiagnosed patients. The aim of the present study was to determine the frequency of CF patients out of the referred cases in a single referral hospital in Egypt. A total of 100 patients clinically suspected of having CF were recruited from the CF clinic of the Allergy and Pulmonology Unit, Children’s Hospital, Cairo University, Egypt, throughout a 2 year period. Sweat chloride testing was done for all patients using the Wescor macroduct system for collection of sweat. Quantitative analysis for chloride was then done by the thiocyanate colorimetric method. Patients positive for sweat chloride (⩾60 mmol/L were tested for the ΔF508 mutation using primer specific PCR for cystic fibrosis transmembrane conductance regulator (CFTR gene. Thirty-six patients (36% had a positive sweat chloride test. The main clinical presentations in patients were chronic cough in 32 (88.9%, failure to thrive in 27 (75%, steatorrhea in 24 (66.7%, and hepatobiliary involvement in 5 (13.9%. Positive consanguinity was reported in 50% of CF patients. Thirty-two patients were screened for ΔF508 mutation. Positive ΔF508 mutation was detected in 22 (68.8% patients, 8 (25% were homozygous, 14 (43.8% were heterozygous, and 10 (31.3% tested were negative. CF was diagnosed in more than third of patients suspected of having the disease on clinical grounds. This high frequency of CF among referred patients indicates that a high index of suspicion and an increasing availability of diagnostic tests lead to the identification of a higher number of affected individuals.

  17. Percutaneous transcholecystic approach for an experiment of biliary stent placement: an experimental study in dogs

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Tae Seok [Medical School of Gachon, Inchon (Korea, Republic of); Song, Ho Young; Lim, Jin Oh; Ko, Gi Young; Sung, Kyu Bo; Kim, Tae Hyung; Lee, Ho Jung [College of Medicine, Ulsan Univ., Seoul (Korea, Republic of)

    2002-06-01

    To determine, in an experimental study of biliary stent placement, the usefulness and safety of the percutaneous transcholecystic approach and the patency of a newly designed biliary stent. A stent made of 0.15-mm-thick nitinol wire, and 10 mm in diameter and 2 cm in length, was loaded in an introducer with an 8-F outer diameter. The gallbladders of seven mongrel dogs were punctured with a 16-G angiocath needle under sonographic guidance, and cholangiography was performed. After anchoring the anterior wall of the gallbladder to the abdominal wall using a T-fastener, the gallbladder body was punctured again under fluoroscopic guidance. The cystic and common bile ducts were selected using a 0.035-inch guide wire and a cobra catheter, and the stent was placed in the common bile duct. Post-stenting cholangiography was undertaken, and an 8.5-F drainage tube was inserted in the gallbladder. Two dogs were followed-up and sacrificed at 2,4 and 8 weeks after stent placement, respectively, and the other expired 2 days after stent placement. Follow-up cholangiograms were obtained before aninmal was sacrified, and a pathologic examination was performed. Stent placement was technically successful in all cases. One dog expired 2 days after placement because of bile peritonitis due to migration of the drainage tube into the peritoneal cavity, but the other six remained healthy during the follow-up period. Cholangiography performed before the sacrifice of each dog showed that the stents were patent. Pathologic examination revealed the proliferation of granulation tissue at 2 weeks, and complete endothelialization over the stents by granulation tissue at 8 weeks. Percutaneous transcholecystic biliary stent placement appears to be safe, easy and useful. After placement, the stent was patent during the follow-period.

  18. Ultrasonographic Findings of Fetal Congenital Intracranial Teratoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hak Jong [Seoul National University College of Medicine, Seoul (Korea, Republic of); Lee, Young Ho; Song, Mi Jin; Cho, Jeong Yeon; Min, Jee Yeon; Moon, Min Hwan; Kim, Jeong Ah [Seoul National University Bundang Hospital, Seongnam (Korea, Republic of)

    2005-06-15

    To evaluate the sonographic findings of fetal congenital intracranial teratoma. From 1994 to 2002, of the 11 fetuses which had been diagnosed with fetal intracranial tumors after second level fetal ultrasonography, the six that were confirmed after autopsy as congenital intracranial teratomas were included in our study. The sonographic findings, including size, homogeneity, echogenicity compared with surrounding normal brain tissues, cystic components, and tumor related calcification, were retrospectively evaluated. The incidence of fetal congenital intracranial teratoma out of all fetal intracranial tumors was 54.5% (6 of 11 cases) during the 8-year period. The mean mass size was 7.4 cm (3.0-15.0 cm). Two thirds of (4/6) of the teratoma cases showed high echogenicity compared with normal brain tissues, and two thirds (4/6) showed heterogeneous echogenicity. Four teratoma cases (67%) showed cysts in the mass with a mean size of 1.9cm. One third (2/6) showed calcifications within the tumor. Out of the six cases, two had oropharyngeal teratoma with extension into the intracranial portion (so called epignathus) and showed homogenous mass without any cysts or calcifications. The typical sonographic appearance of intracranial teratoma was a heterogeneous, hyperechoic mass with cysts. In the epignathus cases, the sonographic appearances differed somewhat from the others. An understanding of the sonographic findings of fetal intracranial teratoma will help in the timely counseling of the parents and in obstetric decision making

  19. Breakdown in Breathing: The Complexities of Cystic Fibrosis

    Science.gov (United States)

    ... Healthier Lungs in Kids Wise Choices Living with Cystic Fibrosis In between checkups, practice good self-care and ... Links What Is Cystic Fibrosis? Learning About Cystic Fibrosis NIH Cystic Fibrosis Fact Sheet Genetic and Rare Diseases Information ...

  20. Congenital Cytomegalovirus Hepatitis in the XXI Century is not Uncommon!

    Directory of Open Access Journals (Sweden)

    N.P. Skorodumova

    2014-08-01

    Full Text Available The article presents features of early clinical and laboratory diagnosis of congenital cytomegalovirus (CMV hepatitis. The analysis of the causes of late diagnosis of chronic hepatitis is carried out. Some children have repeatedly appealed to the doctor and treated for gastroduodenitis, cholecystitis, biliary dyskinesia, ulcerative colitis. Chart review allowed to assume that the extrahepatic manifestations of chronic hepatitis and liver cirrhosis district pediatricians interpreted as allergy (recurrent urticaria, juvenile rheumatoid arthritis (arthritis, vegetovascular dystonia (ECG changes; endocrine violations (delayed menstruation, striae on the abdominal skin — as the feature of puberty; signs of hypo- or hyperthyroidism — as endemic pathology. A sequence of doctor’s actions in diagnosing congenital CMV infection, mainly affecting the liver, is provided.

  1. Over-the-catheter precut to gain access to the biliary duct during ERCP rendezvous.

    Science.gov (United States)

    Manes, Gianpiero; Baratti, Cinzia; Ardizzone, Sandro; Ferla, Fabio; Spiropoulos, Jean; Corsi, Fabio; Foschi, Diego; Trabucchi, Emilio; Bianchi Porro, Gabriele

    2008-10-01

    Endoscopic retrograde cholangiopancreatography (ERCP) rendezvous during laparoscopic cholecystectomy is an efficient and safe method to treat cholecystocholedocholithiasis. Advancing a guidewire through the cystic duct into the duodenum and withdrawing it in the accessory channel of duodenoscope may be, however, laborious. Moreover, rendezvous performed in the typical manner needs the use of several costly accessories. We herein describe a simpler and cheaper method to gain access to the biliary duct at rendezvous. Twenty-four consecutive patients undergoing ERCP rendezvous during laparoscopic cholecystectomy were considered. A catheter was introduced in the cystic duct and advanced into the duodenum. Access to the bile duct was than achieved by means of a precut sphincterotomy performed over the catheter emerging from the papilla. Cannulation was successful in all but two patients, in whom ERCP was performed in the conventional manner. The only complication was a case of mild post-sphincterotomy bleeding. In comparison with the typical rendezvous technique our procedure allowed savings of about 250, since its performance only requires a catheter and a knife sphincterotome. Over-the-catheter precut during ERCP rendezvous is a feasible and safe method which avoids the need for the manipulation of several accessories and guidewires, and thus results in money and time savings.

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

    Science.gov (United States)

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

    2018-03-01

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

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. Human cystic echinococcosis in South Africa

    NARCIS (Netherlands)

    Mogoye, Benjamin; Menezes, Colin N.; Grobusch, Martin P.; Wahlers, Kerstin; Frean, John

    2012-01-01

    Cystic echinococcosis (CE) is caused by the tapeworm, Echinococcus granulosus. The tapeworms resides in the small intestines of canids and the lifecycle involves both intermediate and definitive hosts. Humans are accidental intermediate hosts. Cystic echinococcosis is an economically important

  8. Computed tomography of cystic pancreatic fibrosis

    International Nuclear Information System (INIS)

    Brachlow, M.; Zaunbauer, W.; Haertel, M.

    1984-01-01

    The computer tomographic appearances of atrophic and lipomatous degeneration of the pancreas in cystic pancreatic fibrosis are described. CT exploration of the pancreas in recommended, particularly in differential diagnostic aspects of cystic fibrosis. (orig.) [de

  9. Cytomegalovirus Congenital Cataract

    Directory of Open Access Journals (Sweden)

    Ridha Wahyutomo

    2011-06-01

    Full Text Available Cytomegalovirus congenital infection is an infection caused by the the subfamily â Herpesviridae, during pregnancy. The incidence of infections among newborn infants is 1 %. One of the effects of congenitally acquired infection is the congenital cataract. A 6-year-old child complained to have a blurred vision diagnosed with cytomegalovirus congenital cataract. The diagnosis was confirmed by a positive serology testing for Ig M and Ig G CMV. The laboratory test using Giemsa staining to find inclusion bodies and a faster PCR could not be carried out (Sains Medika, 3(1:84-88.

  10. 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

  11. Arterial Complications of Percutaneous Transhepatic Biliary Drainage

    International Nuclear Information System (INIS)

    L'Hermine, Claude; Ernst, Olivier; Delemazure, Olivier; Sergent, Geraldine

    1996-01-01

    Purpose: To report on the frequency and treatment of arterial complications due to percutaneous transhepatic biliary drainage (PTBD).Materials: Lesions of the intrahepatic artery were encountered in 10 of 525 patients treated by PTBD (2%). Hemobilia followed in 9 patients and subcapsular hematoma in 1. Seven patients had a benign biliary stenosis and 3 had a malignant stenosis.Results: The bleeding resolved spontaneously in 3 patients. In 7 it required arterial embolization, which was successfully achieved either through the percutaneous catheter (n= 3) or by arteriography (n= 4).Conclusion: Arterial bleeding is a relatively rare complication of PTBD that can easily be treated by selective arterial embolization when it does not resolve spontaneously. In this series its frequency was much higher (16%) when the stenosis was benign than when it was malignant (0.6%)

  12. Genetics Home Reference: congenital hypothyroidism

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Congenital hypothyroidism Congenital hypothyroidism Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Congenital hypothyroidism is a partial or complete loss of function ...

  13. Open resections for congenital lung malformations

    Directory of Open Access Journals (Sweden)

    Mullassery Dhanya

    2008-01-01

    Full Text Available Aim: Pediatric lung resection is a relatively uncommon procedure that is usually performed for congenital lesions. In recent years, thoracoscopic resection has become increasingly popular, particularly for small peripheral lesions. The aim of this study was to review our experience with traditional open lung resection in order to evaluate the existing "gold standard." Materials and Methods: We carried out a retrospective analysis of all children having lung resection for congenital lesions at our institution between 1997 and 2004. Data were collected from analysis of case notes, operative records and clinical consultation. The mean follow-up was 37.95 months. The data were analyzed using SPSS. Results: Forty-one children (13 F/28 M underwent major lung resections during the study period. Their median age was 4.66 months (1 day-9 years. The resected lesions included 21 congenital cystic adenomatoid malformations, 14 congenital lobar emphysema, four sequestrations and one bronchogenic cyst. Fifty percent of the lesions were diagnosed antenatally. Twenty-six patients had a complete lobectomy while 15 patients had parenchymal sparing resection of the lesion alone. Mean postoperative stay was 5.7 days. There have been no complications in any of the patients. All patients are currently alive, asymptomatic and well. None of the patients have any significant chest deformity. Conclusions: We conclude that open lung resection enables parenchymal sparing surgery, is versatile, has few complications and produces very good long-term results. It remains the "gold standard" against which minimally invasive techniques may be judged.

  14. Antenatal sonographic appearance of a large orbital encephalocele: a case report and differential diagnosis of orbital cystic mass.

    Science.gov (United States)

    Ahmed, Ahmed; Noureldin, Rehab; Gendy, Mohamed; Sakr, Sharif; Abdel Naby, Mahmoud

    2013-06-01

    Orbital meningoceles and encephaloceles are rare extracranial extensions of the brain and meninges with or without direct communication between the central nervous system and the abnormal mass. We reported a rare case of large fetal orbital encephalocele; the diagnosis was suspected initially by prenatal ultrasound and confirmed by postnatal MRI and CT scans. The differential diagnosis of an intrauterine fetal cystic orbital mass includes orbital teratoma, epidermoid inclusion cysts, hemangioma or lymphangioma, congenital cystic eye, dacryocystocele, and orbital cephalocele. Copyright © 2012 Wiley Periodicals, Inc.

  15. Ultrasonographic findings of type IIIa biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

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

    2014-12-15

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

  16. Cystic Lesions in Autoimmune Pancreatitis

    Directory of Open Access Journals (Sweden)

    Macarena Gompertz

    2015-11-01

    Full Text Available Autoimmune pancreatitis (AIP can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases.

  17. Mitochondrial disorders in congenital myopathies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.

  18. Lung Ultrasound Findings in Congenital Pulmonary Airway Malformation.

    Science.gov (United States)

    Yousef, Nadya; Mokhtari, Mostafa; Durand, Philippe; Raimondi, Francesco; Migliaro, Fiorella; Letourneau, Alexandra; Tissières, Pierre; De Luca, Daniele

    2018-05-01

    Congenital pulmonary airway malformation (CPAM) is a group of rare congenital malformations of the lung and airways. Lung ultrasound (LU) is increasingly used to diagnose neonatal respiratory diseases since it is quick, easy to learn, and radiation-free, but no formal data exist for congenital lung malformations. We aimed to describe LU findings in CPAM neonates needing neonatal intensive care unit (NICU) admission and to compare them with a control population.  A retrospective review of CPAM cases from three tertiary academic NICUs over 3 years (2014-2016) identified five patients with CPAM who had undergone LU examination. LU was compared with chest radiograms and computed tomography (CT) scans that were used as references.  CPAM lesions were easily identified and corresponded well with CT scans; they varied from a single large cystic lesion, multiple hypoechoic lesions, and/or consolidation. The first two LU findings have not been described in other respiratory conditions and were not found in controls.  We provide the first description of LU findings in neonates with CPAM. LU may be used to confirm antenatally diagnosed CPAM and to suspect CPAM in infants with respiratory distress if cystic lung lesions are revealed. Further studies are necessary to define the place of LU in the management of CPAM. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  19. 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)

  20. Ultrasonography and surgery of canine biliary diseases.

    Science.gov (United States)

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

    2001-01-01

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

  1. Malignant Biliary Obstruction: Evidence for Best Practice

    Directory of Open Access Journals (Sweden)

    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.

  2. Functional Self-Expandable Metal Stents in Biliary Obstruction

    Science.gov (United States)

    Kwon, Chang-Il; Ko, Kwang Hyun; Hahm, Ki Baik

    2013-01-01

    Biliary stents are widely used not only for palliative treatment of malignant biliary obstruction but also for benign biliary diseases. Each plastic stent or self-expandable metal stent (SEMS) has its own advantages, and a proper stent should be selected carefully for individual condition. To compensate and overcome several drawbacks of SEMS, functional self-expandable metal stent (FSEMS) has been developed with much progress so far. This article looks into the outcomes and defects of each stent type for benign biliary stricture and describes newly introduced FSEMSs according to their functional categories. PMID:24143314

  3. Microbubbles in macrocysts - Contrast-enhanced ultrasound assisted sclerosant therapy of a congenital macrocystic lymphangioma: a case report.

    Science.gov (United States)

    Menendez-Castro, Carlos; Zapke, Maren; Fahlbusch, Fabian; von Goessel, Heiko; Rascher, Wolfgang; Jüngert, Jörg

    2017-07-06

    Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these lesions by intracavitary injection of OK-432 (Picibanil®), a lyophilized mixture of group A Streptococcus pyogenes, is a common therapeutical option. For an appropriate application of OK-432, a detailed knowledge about the structure and composition of the congenital cystic lymphangioma is essential. SonoVue® is a commercially available contrast agent commonly used in sonography by intravenous and intracavitary application. Here we report the case of 2 month old male patient with a large thoracic congenital cystic lymphangioma. Preinterventional imaging of the malformation was performed by contrast-enhanced ultrasound after intracavitary application of SonoVue® immediately followed by a successful sclerotherapy with OK-432. Contrast agent-enhanced ultrasound imaging offers a valuable option to preinterventionally clarify the anatomic specifications of a congenital cystic lymphangioma in more detail than by single conventional sonography. By the exact knowledge about the composition and especially about the intercystic communications of the lymphangioma sclerosant therapy becomes safer and more efficient.

  4. 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.

  5. Traumatic pulmonary pseudocysts mimicking a congenital malformation of the lung

    Directory of Open Access Journals (Sweden)

    Alexander Ngoo

    2018-02-01

    Full Text Available Traumatic Pulmonary Pseudocysts (TPPs are a rare consequence of thoracic trauma that is seen disproportionately in young adults and paediatric populations. In this case report, we detail a case of a TPP initially misdiagnosed on imaging as a Congenital Cystic Adenomatoid Malformation (CCAM. Conservative management and monitoring for resolution of TPP is highly effective as in most cases it self-resolves without issue or need for intervention. This contrasts with the surgical treatment often indicated for symptomatic CCAMs. Clearly, failure to recognize TPP as a rare but important differential of pulmonary cystic lesions in the context of trauma can lead to significant distress for the patient's family, over investigation and unnecessary or harmful interventions. We also review in this article the literature surrounding the radiological appearances, clinical features and management of both conditions.

  6. Congenital heat disease

    International Nuclear Information System (INIS)

    Higgins, C.B.; Silverman, N.H.; Kersting-Somerhoff, B.A.

    1990-01-01

    The book covers the tomographic anatomy of the normal and congenitally malformed heart and tomographic imaging of the normal heat. It then compares echocardiographic evaluation and the use of MR imaging in the diagnosis and evaluation of individual congenital cardiac malformations

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

    Science.gov (United States)

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

    2012-01-01

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

  8. Prenatal diagnosis of meconium ileus and meconium peritonitis: Indications for cystic fibrosis testing

    Directory of Open Access Journals (Sweden)

    Egić Amira

    2011-01-01

    Full Text Available Introduction. More recently, the regions of increased abdominal echogenicity such as echogenic bowel, meconium ileus and meconium peritonitis have been associated with an increased prevalence of a variety of unfavourable outcomes including chromosomal abnormalities, cytomegalovirus infection, intestinal obstruction, anorectal malformations and cystic fibrosis. Earlier prenatal examinations of these severe autosomal recessive diseases had been suggested only to families with history of cystic fibrosis. Recently, systemic examination has been introduced by ultrasound with bowel hyperechogenicity where the fetus is the index case for genetic disease. Risk for cystic fibrosis with this ultrasonography findings ranges from 0-33%. Outline of Cases. Two patients are presented, aged 24 and 29 years, both primigravide. The first one had ultrasonography finding of meconium peritonitis revealed at the 37th week of gestation and the other meconium ileus revealed on ultrasonography at the 29th week of gestation. Both patients had prenatal testing of foetal blood obtained by cordocenthesis, both had normal kariotype and were negative for cytomegalovirus infection. Parental DNA testing for the 2nd patient showed that parents were not carriers for the 29 most frequent mutations. Both neonates had intestinal obstruction, underwent surgery and early postoperative course was normal. Hystopathological finding suggested a possibility of cystic fibrosis for the 1st patient, but parents did not want to be tested and for the 2nd one congenital bowel stenosis as a cause of intestinal obstruction. Conclusion. Ultrasonographic echogenic bowel is an indication for invasive procedures for foetal blood testing for chromosomal abnormalities, congenital infections and parental testing for cystic fibrosis. Only if parental heterozygosity is proven foetus should be tested.

  9. Cystic fibrosis in adults

    Directory of Open Access Journals (Sweden)

    C. Damas

    2007-05-01

    Full Text Available The authors reviewed adult cystic fibrosis patients followed in the Pulmonology Unit from 1994-2004 (n = 8, five female and three male, aged 20-34 years old (median = 27 years. Patients were diagnosed at 18 months - 31 years old by sweat testing (positive in six patients and genotyping (four patients homozygous for ΔF508 mutation.Respiratory involvement was characterised by sinusitis and bronchiectasis. Pulmonary involvement was accompanied by functional abnormalities and gas exchange impairment in the majority of the patients. Bronchial tree was colonised permanently in five patients: Pseudomonas aeruginosa in four and Staphilococcus aureus in four (three patients affected by both agents simultaneously.The main causes of exacerbation were respiratory infections and haemoptysis.Non-respiratory involvement was variable. Four patients had digestive involvement (one with hepatic cirrhosis, one had renal failure and only one had a sperm count to document infertility. Four patients had osteopaenia.Treatment included chest physiotherapy, bronchodilators, dornase alfa, mucolytics, digestive enzymes, vitamins, antibiotics and oxygen therapy.At review, one had left follow-up, one had died, one was awaiting lung transplant and the others evidenced no difference in clinical characteristics.In this group of patients the severity of the pulmonary disease was not related to a late diagnosis. It can be explained by the diversity of cystic fibrosis presentation in adults Resumo: Os autores efectuaram uma revisão de doentes adultos com fibrose quística (FQ, seguidos na consulta de Pneumologia no período de 1994-2004 (n = 8: cinco mulheres e três homens, com idades compreendidas entre 20 e 34 anos (mediana  =  27 anos, cuja idade de diagnóstico variou entre os 18 meses e os 31 anos.O diagnóstico foi obtido por prova de suor (positiva em seis doentes e estudo genético (homozigotia para a mutação ΔF508 em

  10. MRI of cystic pituitary tumors

    Energy Technology Data Exchange (ETDEWEB)

    Tokunaga, Hitoshi; Hoshi, Seiichiro; Sunada, Souichi; Sunami, Kenro [Kawatetsu Chiba Hospital (Japan); Saeki, Naokatsu; Yamaura, Akira

    1998-11-01

    We retrospectively reviewed MRI findings of 17 patients with 3 histologically proven cystic pituitary tumors. They consisted of 10 cystic pituitary adenomas, 4 craniopharyngiomas and 3 Rathke`s cleft cysts. We analyzed the following MRI parameters such as cyst wall appearance, enhancement pattern of cyst wall, location of residual pituitary gland and location of tumor. They were clinically significant parameters for histological differentiation. Even though combinations of such MRI parameters helped for more accurate preoperative diagnosis, the differentiation between craniopharyngioma and Rathke`s cleft cyst was difficult in some cases. (author)

  11. MRI of cystic pituitary tumors

    International Nuclear Information System (INIS)

    Tokunaga, Hitoshi; Hoshi, Seiichiro; Sunada, Souichi; Sunami, Kenro; Saeki, Naokatsu; Yamaura, Akira

    1998-01-01

    We retrospectively reviewed MRI findings of 17 patients with 3 histologically proven cystic pituitary tumors. They consisted of 10 cystic pituitary adenomas, 4 craniopharyngiomas and 3 Rathke's cleft cysts. We analyzed the following MRI parameters such as cyst wall appearance, enhancement pattern of cyst wall, location of residual pituitary gland and location of tumor. They were clinically significant parameters for histological differentiation. Even though combinations of such MRI parameters helped for more accurate preoperative diagnosis, the differentiation between craniopharyngioma and Rathke's cleft cyst was difficult in some cases. (author)

  12. Cystic meningiomas in 2 dogs

    International Nuclear Information System (INIS)

    Bagley, R.S.; Kornegay, J.N.; Lane, S.B.; Thrall, D.L.; Page, R.L.

    1996-01-01

    Two dogs with signs of forebrain disease had hypodense lesions on computed tomography evaluation. Magnetic resonance imaging of the first dog showed a hypointense lesion on the T1-weighted scan and a hyperintense lesion on T2-weighted scanning. At surgery, both dogs had a primary cystic intracranial lesion, and the abnormal tissue adjacent to the cyst had histological features of meningioma. Each dog underwent whole brain irradiation after surgery, and 1 dog lived for 3 years after treatment. While uncommon, meningioma should be considered as a differential diagnosis in dogs with cystic intracranial lesions

  13. Cystic neuroblastoma: a case report

    International Nuclear Information System (INIS)

    Duran, A.; Lorente, M.L.; Fernandez, C.

    1997-01-01

    Neuroblastoma is the most common neonatal malignant tumor. Hemorrhage and necrosis are usual features of this lesion, but it rarely presents a totally cyst form. We report a case of cystic neuroblastoma detected on prenatal ultrasound and stress the need to include it in the differential diagnosis of cystic abdominal masses in the newborn. Ultrasound is the method of choice for assessing abdominal masses in children. However, magnetic resonance has been shown to be more advantageous for the study and follow-up of neuroblastomas. (Author) 16 refs

  14. Key aspects congenital infection

    Directory of Open Access Journals (Sweden)

    Yu. V. Lobzin

    2014-01-01

    Full Text Available The key questions to solve the problem of congenital infection in the Russian Federation are: using in national practice over world accepted terminology adapted to the recommendations of the World Health Organization; representation of the modern concepts of an infectious process in the classification of congenital infections; scientific development and introducing in clinical practice the «standard case definitions», applied to different congenital infections; optimization of protocols and clinical guidelines for diagnosis, treatment and prevention of congenital infections; improvement a knowledge in the infectious disease for all  pecialists involved in the risk assessment of congenital infections, manage pregnancy and children. Based on our experience and analysis of publications, the authors suggest possible solutions.

  15. 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.

  16. CT diagnosis of cystic echinococcosis in pelvis

    International Nuclear Information System (INIS)

    Liu Wenya; Li Li; Xing Yan; Xie Jingxia

    2003-01-01

    Objective: To analyze the CT findings of pelvic cystic echinococcosis, and to assess the value of CT examination. Methods: Twenty-one cases of pelvic cystic echinococcosis, confirmed by clinical and pathological results, underwent CT scanning. CT characters were analyzed and compared with pathology. Results: Simple cystic echinococcosis appeared as single or multiple cystic lesions with round or oval shape, smooth boundary, and no enhancement after contrast medium administration in 3 cases; Different number and size of daughter cysts were detected inside the lesion in 17 cases; Ruptured lesions showed 'double wall', 'water snake', or 'flow ribbon' signs in 4 cases; Increase density of contents and enhanced cystic wall were demonstrated in 2 cases with companied infections. In 8 cases, calcification occurred on the cystic wall or extended inside the content. Conclusion: CT could accurately demonstrate the location, appearance, internal structure, and adjacent situation of the cystic echinococcosis, providing valuable information for correct diagnosis and treatment

  17. Congenital cervical cysts, sinuses, and fistulae in pediatric surgery.

    Science.gov (United States)

    LaRiviere, Cabrini A; Waldhausen, John H T

    2012-06-01

    Congenital cervical anomalies are essential to consider in the clinical assessment of head and neck masses in children and adults. These lesions can present as palpable cystic masses, infected masses, draining sinuses, or fistulae. Thyroglossal duct cysts are most common, followed by branchial cleft anomalies and dermoid cysts. Other lesions reviewed include median ectopic thyroid, cervical teratomas, and midline cervical clefts. Appropriate diagnosis and management of these lesions requires a thorough understanding of their embryology and anatomy. Correct diagnosis, resolution of infectious issues before definitive therapy, and complete surgical excision are imperative in the prevention of recurrence. Copyright © 2012 Elsevier Inc. All rights reserved.

  18. 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

  19. 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 ...

  20. 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.

  1. 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

  2. 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.)

  3. 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.

  4. 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

  5. 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.

  6. 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.)

  7. [Genetic counseling in cystic fibrosis].

    Science.gov (United States)

    Julia, S; Bieth, E

    2000-08-01

    Genetic counseling is an important part of health care in patients with cystic fibrosis or respiratory diseases associated with the CFTR (cystic fibrosis transmembrane conductance regulator) gene, including certain types of allergic bronchopulmonary aspergilloses or bronchial diseases (diffuse bronchiectasia). The basic goal is to provide patients with information on the transmission of cystic fibrosis and to asses the risk of recurrence. This risk is determined from molecular biology analyses examining the CFTR gene. Genotyping is the only means of screening for the heterozygous state, frequent in the French population (about 1/30). Because of the large number of mutated alleles not covered entirely by the genetic tests, there remains a question of probability expressed as a residual risk of a heterozygous state. A prenatal genotype diagnosis should be proposed to heterozygous couples who have a 25% risk of having a diseased child. Technically, this is almost always possible and the results are highly reliable. Nevertheless, there remains the risks related to sample taking and the ethical issue about which the patients must be informed. Management of these at risk couples who desire a child must be based on a multidisciplinary approach, particularly important when one of the parents has overt cystic fibrosis.

  8. Cystic echinococcosis of the liver

    DEFF Research Database (Denmark)

    Branci, Sonia; Ewertsen, Caroline; Thybo, Søren

    2012-01-01

    Cystic echinococcosis (CE) of the liver can be treated with ultrasound-guided puncture, aspiration, injection, and re-aspiration (PAIR), with surgery and with benzimidazole derivatives. The aim of this study was to review available data concerning treatment modality and outcome for patients treated...

  9. 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.

  10. 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

  11. 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.

  12. [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.

  13. Congenital Splenic Cyst Treated with Percutaneous Sclerosis Using Alcohol

    International Nuclear Information System (INIS)

    Anon, Ramon; Guijarro, Jorge; Amoros, Cirilo; Gil, Joaquin; Bosca, Marta M.; Palmero, Julio; Benages, Adolfo

    2006-01-01

    We report a case of successful percutaneous treatment of a congenital splenic cyst using alcohol as the sclerosing agent. A 14-year-old female adolescent presented with a nonsymptomatic cystic mass located in the spleen that was believed to be congenital. After ultrasonography, a drainage catheter was placed in the cavity. About 250 ml of serous liquid was extracted and sent for microbiologic and pathologic studies to rule out an infectious or malignant origin. Immediately afterwards, complete drainage and local sclerotherapy with alcohol was performed. This therapy was repeated 8 days later, after having observed 60 ml of fluid in the drainage bag. One year after treatment the cyst has practically disappeared. We believe that treatment of splenic cyst with percutaneous puncture, ethanolization, and drainage is a valid option and it does not rule out surgery if the conservative treatment fails

  14. Magnetic resonance imaging of the brain in congenital cytomegalovirus infection

    International Nuclear Information System (INIS)

    Boesch, C.; Issakainen, J.; Kewitz, G.; Kikinis, R.; Martin, E.; Boltshauser, E.

    1989-01-01

    The children (age 2 months to 8 years) with a congenital cytomegalovirus (CMV) infection were studied by magnetic resonance imaging (MRI) using a 2.35 Tesla magnet. CMV infection was confirmed by serological investigations and virus culture in the neonatal period. Nine children had severe mental retardation and cerebral palsy, 1 patient suffered from microcephaly, ataxia and deafness. The cranial MRI examination showed the following abnormalities (N): Dilated lateral ventricles (10) and subarachnoid space (8), oligo/pacgyria (8), delayed/pathological myelination (7), paraventricular cysts (6), intra-cerebral calcification (1). This lack of sensitivity for calcification is explainable by the basic principles of MRI. The paraventricular cystic lesions were adjacent ot the occipital horns of the lateral ventricles and separated only by a thin membrane. This finding might represent a 'new sign' for congenital CMV infection in MRI examinations, being characteristic but nevertheless nonspecific, like calcification in CT. (orig.)

  15. Congenital symmastia revisited

    DEFF Research Database (Denmark)

    Sillesen, Nanna H; Hölmich, Lisbeth R; Siersen, Hans Erik

    2012-01-01

    Symmastia is defined as medial confluence of the breast. The term 'symmastia' is modified from Greek (syn meaning 'together', and mastos meaning 'breast') and was first presented by Spence et al. in 1983. Two forms of symmastia exist: an iatrogenic and a congenital version. Congenital symmastia...... is a rare condition in which web-like soft tissue traverses the sternum to connect the breasts medially. The literature on congenital symmastia is limited, few cases have been published, and knowledge about ideal treatment is still insufficient....

  16. 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

  17. Percutaneous biliary drainage in patients with cholangiocarcinoma

    International Nuclear Information System (INIS)

    Mehta, A.C.; Gobel, R.J.; Rose, S.C.; Hayes, J.K.; Miller, F.J.

    1990-01-01

    This paper determines whether radiation therapy (RT) is a risk factor for infectious complications (particularly hepatic abscess formation) related to percutaneous biliary drainage (PBD). The authors retrospectively reviewed the charts of 98 consecutive patients who had undergone PBD for obstruction. In 34 patients with benign obstruction, three infectious complications occurred, none of which were hepatic abscess or fatal sepsis. In 39 patients who had malignant obstruction but did not have cholangiocarcinoma, 13 infectious complications occurred, including two hepatic abscesses and three cases of fatal sepsis. Of the 25 patients with cholangiocarcinoma, 15 underwent RT; in these 15 patients, 14 infectious complications occurred, including six hepatic abscesses and two cases of fatal sepsis

  18. Risk of gastrointestinal cancers in patients with cystic fibrosis: a systematic review and meta-analysis.

    Science.gov (United States)

    Yamada, Akihiro; Komaki, Yuga; Komaki, Fukiko; Micic, Dejan; Zullow, Samantha; Sakuraba, Atsushi

    2018-04-26

    82-30·14; pcystic fibrosis compared with the general population: small bowel cancer (pooled SIR 18·94, 95% CI 9·37-38·27; pcystic fibrosis had a significantly increased risk of gastrointestinal cancer compared with the general population, including small bowel, colon, biliary tract, and pancreatic cancers. These findings highlight the need to develop individualised screening strategies for site-specific gastrointestinal cancers in patients with cystic fibrosis. None. Copyright © 2018 Elsevier Ltd. All rights reserved.

  19. Congenital generalized lipodystrophia: a case report; Lipodistrofia generalizada congenita: relato de um caso

    Energy Technology Data Exchange (ETDEWEB)

    Malheiros, N.R.; Marchiori, E.; Praxedes, M.C.; Machado, D.M.; Carvalho, A.A.V. [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia

    1995-01-01

    Congenital generalized lipodystrophia is a rare genetic disorder, transmitted as an autosomal recessive trait and is prevalent on female. This paper presents a case of a man, 36-year-old, suffering from congenital generalized lipodystrophia with clinical features of diabetes mellitus and dyspnea. Radiographic findings have shown cystic areas in the skeleton, interstitial pulmonary fibrosis and paucity of abdominal fat. Radiological and anatomo-pathological aspects are presented as well as a review of the medical literature about the case. (author). 8 refs, 4 figs.

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

    Directory of Open Access Journals (Sweden)

    Andrew W. Wang

    2017-01-01

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

  1. Chest CT features of cystic fibrosis in Korea: Comparison with non-cystic fibrosis diseases

    International Nuclear Information System (INIS)

    Yang, So Yeon; Lee, Kyung Soo; Kim, Tae Jung; Kim, Tae Sung; Cha, Min Jae; Yoon, Hyun Jung

    2017-01-01

    Cystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF. From November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study. A diagnosis of CF was made with the CF gene study. CT findings were evaluated for the presence and distribution of parenchymal abnormalities including bronchiectasis, tree-in-bud (TIB) pattern, mucus plugging, consolidation, and mosaic attenuation. Of the 13 patients, 7 (median age, 15 years) were confirmed as CF, 4 (median age, 19 years) had primary ciliary dyskinesia, 1 had bronchiectasis of unknown cause, and 1 had chronic asthma. CT of patients with CF showed bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging in all patients, with upper lung predominance (57%). In CT of the non-CF patients, bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging were also predominant features, with lower lung predominance (50%). Korean patients with CF showed bilateral bronchiectasis, cellular bronchiolitis, mucus plugging, and mosaic attenuation, which overlapped with those of non-CF patients. CF gene study is recommended for the definitive diagnosis of CF in patients with these clinical and imaging features

  2. Chest CT features of cystic fibrosis in Korea: Comparison with non-cystic fibrosis diseases

    Energy Technology Data Exchange (ETDEWEB)

    Yang, So Yeon; Lee, Kyung Soo; Kim, Tae Jung; Kim, Tae Sung [Dept. of Radiology, and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of); Cha, Min Jae [Dept. of Radiology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul (Korea, Republic of); Yoon, Hyun Jung [Dept. of Radiology, Hanyang University Hospital, Hanyang University College of Medicine, Seoul (Korea, Republic of)

    2017-01-15

    Cystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF. From November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study. A diagnosis of CF was made with the CF gene study. CT findings were evaluated for the presence and distribution of parenchymal abnormalities including bronchiectasis, tree-in-bud (TIB) pattern, mucus plugging, consolidation, and mosaic attenuation. Of the 13 patients, 7 (median age, 15 years) were confirmed as CF, 4 (median age, 19 years) had primary ciliary dyskinesia, 1 had bronchiectasis of unknown cause, and 1 had chronic asthma. CT of patients with CF showed bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging in all patients, with upper lung predominance (57%). In CT of the non-CF patients, bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging were also predominant features, with lower lung predominance (50%). Korean patients with CF showed bilateral bronchiectasis, cellular bronchiolitis, mucus plugging, and mosaic attenuation, which overlapped with those of non-CF patients. CF gene study is recommended for the definitive diagnosis of CF in patients with these clinical and imaging features.

  3. Congenital Constriction Band Syndrome

    OpenAIRE

    Rajesh Gupta, Fareed Malik, Rishabh Gupta, M.A.Basit, Dara Singh

    2008-01-01

    Congenital constriction bands are anomalous bands that encircle a digit or an extremity. Congenitalconstriction band syndrome is rare condition and is mostly associated with other musculoskeletaldisorders.We report such a rare experience.

  4. Congenital anterior urethral diverticulum

    International Nuclear Information System (INIS)

    Jung, Hyun Sub; Chung, Young Sun; Suh, Chee Jang; Won, Jong Jin

    1985-01-01

    Two cases of congenital anterior urethral diverticular which have occurred in a 4 year old and one month old boy are presented. Etiology, diagnostic procedures, and its clinical results are briefly reviewed

  5. Congenital giant megaureter associated with ipsilateral multicystic dysplastic kidney in newborn

    Directory of Open Access Journals (Sweden)

    Rajendran Ramaswamy

    2016-01-01

    Full Text Available Congenital giant megaureter presents as abdominal mass and impose diagnostic difficulties. It can be associated with other upper urinary tract anomalies. A female newborn with antenatal diagnosis of polycystic kidneys was admitted at birth due to lower abdominal mass. Ultrasound and CT scans diagnosed a multiloculated cystic lesion in the mid and lower abdomen along with right side multicystic kidney. At laparotomy, an extaperitoneal, lobulated cystic swelling was found due to rightside giant megaureter. Its lower end was of normal caliber and orthotopic. End cutaneous ureterostomy was done. Intravenous urogram and isotope renograms showed nonfunctioning right kidney. She also had grade II vesicoureteral reflux on left side. Child suffered urinary infection twice. At 9m age, right nephroureterectomy was done. Histopathologic examination was consistent with cystic renal dysplasia and dilated ureter. This is the first case report of giant megaureter associated with ipsilateral multicystic dysplastic kidney in newborn.

  6. Postoperative follow-up studies in congenital dilatation of the common bile duct using hepatobiliary scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Hirobe, Seiichi; Ishida, Haruo; Hayashi, Akira; Kamagata, Shoichiro; Fuchimoto, Yasufumi; Mizuno, Dai; Yano, Tsunehiro [Tokyo Metropolitan Kiyose Children`s Hospital (Japan); Ishii, Katsumi

    1995-03-01

    Follow-up studies in congenital dilatation of the common bile duct were done in 20 cases ranging 3 to 20 years after operation. Operative cholangiography had shown dilatation of the intrahepatic bile ducts in 15 patients (75%), of these, 7 patients had cystic dilatation. Five cases of these 7 patients showed prolonged stasis of the nuclide in the cystic dilated intrahepatic bile ducts by {sup 99m}Tc-PMT hepatobiliary scintigraphy in the follow-up studies even at 10 years after operation. But none of 20 cases had complication such as intrahepatic lithiasis, cholangitis, and liver dysfunction. Hepatobiliary scintigraphy could provide moderate anatomic and excellent functional information which were useful for these follow-up studies and careful follow-up should be done in the case of cystic dilatation of the intrahepatic bile ducts. (author).

  7. Congenital chylous ascites

    International Nuclear Information System (INIS)

    Romanska-Kita, J.; Borszewska-Kornacka, M. K.; Rudzinska, I.; Wawrzoniak, T.; Dobrzanska, A.; Czech-Kowalska, J.

    2011-01-01

    Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity. (authors)

  8. Multifocal Congenital Hemangiopericytoma.

    Science.gov (United States)

    Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

    2017-01-01

    Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported. © 2016 Wiley Periodicals, Inc.

  9. Percutaneous dilatation of biliary benign strictures

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Hyung; Choi, Byung Ihn; Sung, Kyu Bo; Han, Man Chung; Park, Yong Hyun; Yoon, Yong Bum [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1986-06-15

    Percutaneous biliary dilation was done in 3 patients with benign strictures. The first case was 50-year-old male who had multiple intrahepatic stones with biliary stricture. The second 46-year-old female and the third 25-year-old male suffered from recurrent cholangitis with benign stricture of anastomotic site after choledocho-jejunostomy. In the first case, a 6mm diameter Grunzing dilatation balloon catheter was introduced through the T-tube tract. In the second case, the stricture was dilated with two balloons of 5mm and 8mm in each diameter sequentially through the U-loop tract formed by surgically made jejunostomy and percutaneous transhepatic puncture. In the third case, the dilatation catheter was introduced through the percutaneous transhepatic tract. Dilatation was made with a pressure of 5 to 10 atmospheres for 1 to 3 minutes duration for 3 times. In all 3 cases, the strictures were successfully dilated and in second and third cases internal stent was left across the lesion for prevention of restenosis.

  10. Recommendations for quality improvement in genetic testing for cystic fibrosis European Concerted Action on Cystic Fibrosis

    NARCIS (Netherlands)

    Dequeker, E; Cuppens, H; Dodge, J; Estivill, [No Value; Goossens, M; Pignatti, PF; Scheffer, H; Schwartz, M; Schwarz, M; Tummler, B; Cassiman, JJ

    These recommendations for quality improvement of cystic fibrosis genetic diagnostic testing provide general guidelines for the molecular genetic testing of cystic fibrosis in patients/individuals. General strategies for testing as well as guidelines for laboratory procedures, internal and external

  11. Congenital pulmonary airway malformation with mucoepidermoid carcinoma: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Narayanappa Harini

    2012-01-01

    Full Text Available Congenital cystic adenomatoid malformations (CCAM are rare developmental anomalies of the lung characterized by cysts of varying sizes and/or adenomatoid proliferation. Type I CCAM, the most frequent subtype, is associated with an increased incidence of malignant transformation, principally bronchioloalveolar carcinoma, with a reported incidence of around 1%. We report the first case of mucoepidermoid carcinoma arising in a type 1 CCAM.

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

    Science.gov (United States)

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

    2017-10-01

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

  13. 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

  14. Effect of Covered Metallic Stents Compared With Plastic Stents on Benign Biliary Stricture Resolution

    Science.gov (United States)

    Coté, Gregory A.; Slivka, Adam; Tarnasky, Paul; Mullady, Daniel K.; Elmunzer, B. Joseph; Elta, Grace; Fogel, Evan; Lehman, Glen; McHenry, Lee; Romagnuolo, Joseph; Menon, Shyam; Siddiqui, Uzma D.; Watkins, James; Lynch, Sheryl; Denski, Cheryl; Xu, Huiping; Sherman, Stuart

    2017-01-01

    IMPORTANCE Endoscopic placement of multiple plastic stents in parallel is the first-line treatment for most benign biliary strictures; it is possible that fully covered, self-expandable metallic stents (cSEMS) may require fewer endoscopic retrograde cholangiopancreatography procedures (ERCPs) to achieve resolution. OBJECTIVE To assess whether use of cSEMS is noninferior to plastic stents with respect to stricture resolution. DESIGN, SETTING, AND PARTICIPANTS Multicenter (8 endoscopic referral centers), open-label, parallel, randomized clinical trial involving patients with treatment-naive, benign biliary strictures (N = 112) due to orthotopic liver transplant (n = 73), chronic pancreatitis (n = 35), or postoperative injury (n = 4), who were enrolled between April 2011 and September 2014 (with follow-up ending October 2015). Patients with a bile duct diameter less than 6 mm and those with an intact gallbladder in whom the cystic duct would be overlapped by a cSEMS were excluded. INTERVENTIONS Patients (N = 112) were randomized to receive multiple plastic stents or a single cSEMS, stratified by stricture etiology and with endoscopic reassessment for resolution every 3 months (plastic stents) or every 6 months (cSEMS). Patients were followed up for 12 months after stricture resolution to assess for recurrence. MAIN OUTCOMES AND MEASURES Primary outcome was stricture resolution after no more than 12 months of endoscopic therapy. The sample size was estimated based on the noninferiority of cSEMS to plastic stents, with a noninferiority margin of −15%. RESULTS There were 55 patients in the plastic stent group (mean [SD] age, 57 [11] years; 17 women [31%]) and 57 patients in the cSEMS group (mean [SD] age, 55 [10] years; 19 women [33%]). Compared with plastic stents (41/48, 85.4%), the cSEMS resolution rate was 50 of 54 patients (92.6%), with a rate difference of 7.2% (1-sided 95% CI, −3.0% to ∞; P stents was rejected. The mean number of ERCPs to achieve resolution

  15. 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.

  16. 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.

  17. Cystic form of rheumatoid arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Dijkstra, P.F.; Gubler, F.M.; Maas, A.

    1988-10-01

    A nonerosive form of rheumatoid arthritis (R.A.) was found in 62 patients out of 660 patients with R.A.. These 62 patients exhibit slowly progressive cystic changes in about the same joints in which usually erosions develop in classic R.A.. The E.S.R. is often low, half of the patients remained seronegative and there are 35 males and 27 females in the group. A smaller group of 15 out of these patients could be followed from a stage wherein the radiographs were normal to a stage of extensive cystic changes, over a period of at least 6 years. An attempt is made to delineate this group within the rheumatoid arthritis disease entity.

  18. MRI in mucoviscidosis (cystic fibrosis)

    International Nuclear Information System (INIS)

    Eichinger, M.; Puderbach, M.; Kauczor, H.-U.; Heussel, C.-P.

    2006-01-01

    Cystic fibrosis (CF) is a multi-systemic disease with major impact on the lungs. Pulmonary manifestation is crucial for the prognosis and life expectancy of patients. Imaging modalities and lung function tests reflect the pulmonary status in these patients. The standard imaging modality for diagnosis and follow-up of pulmonary changes is chest x-ray. The gold standard for the detection of parenchymal lung changes remains high resolution computed tomography (HRCT), but this is not used routinely for CF-patients due to radiation exposure. Magnetic resonance imaging (MRI) used to be of no importance in monitoring cystic fibrosis lung disease, as shown in studies from the 1980s and early 1990s. The continuing improvement of MRI techniques, however, has allowed for an adequate application of this non-radiation method in diagnosing the major pulmonary findings in CF, in addition to the assessment of lung function. (orig.) [de

  19. [Historical compilation of cystic fibrosis].

    Science.gov (United States)

    Navarro, Salvador

    2016-01-01

    Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future. Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

  20. 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).

  1. Lactate in cystic fibrosis sputum

    DEFF Research Database (Denmark)

    Bensel, Tobias; Stotz, Martin; Borneff-Lipp, Marianne

    2011-01-01

    Antibiotic therapy is thought to improve lung function in patients with cystic fibrosis (CF) by decreasing neutrophil-derived inflammation. We investigated the origin and clinical significance of lactate in the chronically inflamed CF lung. Methods Lactate was measured in sputa of 18 exacerbated...... and 25 stable CF patients via spectrophotometry and gaschromatography. Lung function was assessed via spirometry. Seven patients with chronic obstructive pulmonary disease (COPD) and three patients with acute lung inflammation served as control groups. Neutrophil and bacterial lactate production...

  2. Anorexia nervosa in cystic fibrosis.

    Science.gov (United States)

    Linkson, Lynette; Macedo, Patricia; Perrin, Felicity M R; Elston, Caroline M

    2018-03-01

    This article explores the challenges associated with diagnosing and managing eating disorders such as anorexia nervosa amongst adolescents and adults with cystic fibrosis. It reviews the known risk factors, generic verses disease specific eating disorder risk screening tools and considers the ethical dilemmas associated with critically low body mass indices. A case review is included to illustrate the complexities of managing both conditions in the context of declining respiratory function. Copyright © 2017. Published by Elsevier Ltd.

  3. Otorhinolaryngologic manifestations of cystic fibrosis: literature review

    Directory of Open Access Journals (Sweden)

    Carvalho, Carolina Pimenta

    2008-12-01

    Full Text Available Introduction: Cystic Fibrosis is the most common recessive autosomic genetic disease among Caucasians. It's caused by mutations in the gene that decodes regulatory protein for transmembrane conductance, resulting in defective transport of chlorine. Objective: Review the literature about Cystic Fibrosis, with emphasis on otorhinolaryngologic manifestations. Method: The online Pub Med databases were researched and we applied the following search terms Fibrosis Cystic and Sinusitis, and Mucoviscidosis and Sinusitis. Conclusions: Although it is not the main cause of death, the otorhinolaryngologic manifestations of the Cystic Fibrosis bring important morbidity to these patients.

  4. 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)

  5. 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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1988-09-01

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

  7. 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.

  8. 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

  9. [Recurrent benign cystic peritoneal mesothelioma].

    Science.gov (United States)

    Stroescu, C; Negulescu, Raluca; Herlea, V; David, L; Ivanov, B; Nitipir, Cornelia; Popescu, I

    2008-01-01

    The benign cystic peritoneal mesothelioma (BCPM) is a rare neoplasm affecting mainly females at reproductive age. The natural history and physiopathology of the BCPM are not entirely known. It is mainly characterized by the lack of malignant elements, no tendency to metastasis and by a pervasive tendency to generate local recurrences after surgical removal. The clinical manifestations are insidious, uncharacteristic; the benign cystic peritoneal mesothelioma is often discovered during a surgical procedure addressing another condition. Imaging tests can raise the suspicion of BCPM but the diagnostic can only be confirmed by histopathological examination corroborated with an immunohistochemical analysis. There are no long term studies dictating a single therapeutic attitude but a high risk of local recurrences and the possibility of transformation into malignant mesothelioma have lead to the current tendency towards an aggressive treatment of the tumor. We present the case of a recurrent benign cystic peritoneal mesothelioma in a 40 years old female patient, emphasizing the therapeutic approach and the role of radical surgery in the treatment of BPCM.

  10. Cystic Fibrosis-Related Diabetes

    Directory of Open Access Journals (Sweden)

    Kayani Kayani

    2018-02-01

    Full Text Available Cystic fibrosis (CF is the most common autosomal recessive disorder in Caucasian populations. Individuals with CF have seen significant increases in life expectancy in the last 60 years. As a result, previously rare complications are now coming to light. The most common of these is cystic fibrosis-related diabetes (CFRD, which affects 40–50% of CF adults. CFRD significantly impacts the pulmonary function and longevity of CF patients, yet a lack of consensus on the best methods to diagnose and treat CFRD remains. We begin by reviewing our understanding of the pathogenesis of CFRD, as emerging evidence shows the cystic fibrosis transmembrane conductance regulator (CFTR also has important roles in the release of insulin and glucagon and in the protection of β cells from oxidative stress. We then discuss how current recommended methods of CFRD diagnosis are not appropriate, as continuous glucose monitoring becomes more effective, practical, and cost-effective. Finally, we evaluate emerging treatments which have narrowed the mortality gap within the CF patient group. In the future, pharmacological potentiators and correctors directly targeting CFTR show huge promise for both CFRD and the wider CF patient groups.

  11. The Sociology and Entrenchment. A Cystic Fibrosis Test for Everyone?

    DEFF Research Database (Denmark)

    Koch, Lene; Stemerding, Dirk

    1994-01-01

    Socialmedicine, genetic screening, cystic fibrosis, ethics, political regulation, sociology of technology......Socialmedicine, genetic screening, cystic fibrosis, ethics, political regulation, sociology of technology...

  12. 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.

  13. Ruptured hydatid cyst in biliary tract

    International Nuclear Information System (INIS)

    Hafeez, M.; Hussain, T.; Salamat, A.

    2012-01-01

    A lady aged 26 years reported with a 2 months history of fever, upper abdominal pain and weight loss. Her abdominal ultrasonographic scan revealed a complex cystic mass in left lobe of liver suggestive of hydatid cyst that was confirmed on magnetic resonance imaging of abdomen and magnetic resonance cholangiopancreatogram. With strong suspicion of a hydatid cyst, endoscopic retrograde cholangiogram was performed which confirmed the diagnosis. During the procedure, hydatid membranes protruding from the papilla were removed after sphincterotomy. She was put on albendazole 400 mg twice daily after the procedure and showed a remarkable clinical improvement. (author)

  14. [Congenital mucocele of the ventral face of the tongue].

    Science.gov (United States)

    Nohuz, E; Gallot, D; Rousset, C; Brunel, A; Albaut, M; Bayeh, S; Vendittelli, F; Laurichesse-Delmas, H; Lemery, D

    2016-03-01

    Congenital cystic lesions of the oral cavity are an extremely rare occurrence. Their prenatal diagnosis is essential since they can impede respiratory and swallowing functions. We describe a case that was detected prenatally and discuss its management. A 21-year-old primigravida patient who was 23 weeks pregnant was referred to our obstetrics and gynecology center after fetal ultrasonography showed a cystic lesion of the oral cavity. She had no family history of any congenital anomalies. Ultrasonography showed a male fetus with an anechoic mass measuring 21×11 mm encompassing the entire oral cavity, evoking either a mucocele or a cystic hygroma. Magnetic resonance imaging (MRI) showed a fetus with a wide-open mouth, due to a well-demarcated protruding cystic mass with no solid component, suggestive of a mucocele. A prenatal sonographically guided percutaneous needle aspiration of mucous fluid was performed at 33 gestational weeks. Although the mucocele decreased significantly in size, it nevertheless continued to expand progressively. After an uncomplicated pregnancy, the patient had spontaneous onset of labor at 39 weeks of gestation. An iterative aspiration was performed in the same manner in utero, resulting in a complete collapse of the mucocele. If needed, intubation could be considered. A 3030-g male was born by vaginal delivery, without respiratory distress. Clinical examination showed the extremely opened mouth and confirmed the presence of a large cystic mass approximately 4 cm in diameter, of sublingual origin and encompassing the entire oral cavity. The continuous protrusion of the tongue was responsible for the infant's inability to close the mouth and be breastfed. After insertion of a feeding tube, the newborn had maxillofacial surgery consisting in marsupialization of the cyst at 2 days of age. The mucocele decreased in size and the postoperative course was uneventful. No recurrence was observed at 6 months' follow-up. Congenital mucoceles of the

  15. Imaging of congenital mesoblastic nephroma with pathological correlation

    International Nuclear Information System (INIS)

    Chaudry, Gulraiz; Perez-Atayde, Antonio R.; Ngan, Bo Yee; Gundogan, Munire; Daneman, Alan

    2009-01-01

    There are a variety of imaging findings for congenital mesoblastic nephroma (CMN) and two main pathological variants: classic and cellular. To determine whether imaging findings in children can predict the likely pathological variant. We reviewed imaging in children with pathology-proven CMN. Imaging findings correlated with gross and histological appearance. In 15 boys and 15 girls with CMN, US was performed in 27, CT in 19, and MRI in 7. Cystic components were readily identified on US; central hemorrhage was better differentiated on CT. MRI demonstrated high sensitivity for both. Histology confirmed classic CMN in 13 children, cellular CMN in 14 and ''mixed'' CMN in 3. Age at presentation was significantly higher in children with the cellular variant. Of 15 solid or predominantly solid tumors and 10 lesions with a hypoechoic ring, 12 and 7, respectively, had pathology consistent with classic CMN. In contrast, five of seven with intratumoral hemorrhage and all with a large cystic/necrotic component had pathology consistent with the cellular variant. The imaging appearance of CMN is often determined by the pathological type of tumor. Findings suggestive of the classic variant include a peripheral hypoechoic ring or large solid component. In comparison, cystic/necrotic change and hemorrhage is much more common in cellular CMN. (orig.)

  16. The association of congenital neuroblastoma and congenital heart disease

    International Nuclear Information System (INIS)

    Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

    1989-01-01

    Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

  17. Congenital malaria in China.

    Directory of Open Access Journals (Sweden)

    Zhi-Yong Tao

    2014-03-01

    Full Text Available BACKGROUND: Congenital malaria, in which infants are directly infected with malaria parasites from their mother prior to or during birth, is a potentially life-threatening condition that occurs at relatively low rates in malaria-endemic regions. It is recognized as a serious problem in Plasmodium falciparum-endemic sub-Saharan Africa, where recent data suggests that it is more common than previously believed. In such regions where malaria transmission is high, neonates may be protected from disease caused by congenital malaria through the transfer of maternal antibodies against the parasite. However, in low P. vivax-endemic regions, immunity to vivax malaria is low; thus, there is the likelihood that congenital vivax malaria poses a more significant threat to newborn health. Malaria had previously been a major parasitic disease in China, and congenital malaria case reports in Chinese offer valuable information for understanding the risks posed by congenital malaria to neonatal health. As most of the literature documenting congenital malaria cases in China are written in Chinese and therefore are not easily accessible to the global malaria research community, we have undertaken an extensive review of the Chinese literature on this subject. METHODS/PRINCIPAL FINDINGS: Here, we reviewed congenital malaria cases from three major searchable Chinese journal databases, concentrating on data from 1915 through 2011. Following extensive screening, a total of 104 cases of congenital malaria were identified. These cases were distributed mainly in the eastern, central, and southern regions of China, as well as in the low-lying region of southwest China. The dominant species was P. vivax (92.50%, reflecting the malaria parasite species distribution in China. The leading clinical presentation was fever, and other clinical presentations were anaemia, jaundice, paleness, diarrhoea, vomiting, and general weakness. With the exception of two cases, all patients

  18. Congenital cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Katarina Rednak-Paradiž

    2006-11-01

    Full Text Available Background: CMV is the most common agent that causes congenital virus infection. Only 10 % of infected children have symptomatic infection immediately after birth. Signs of central nervous system damage, neurosensory deafness and delayed psychomotor development may manifest as a result of asimptomatic congenital infection later in childhood. In the article we present basic properties of CMV; we describe clinical picture of the congenital infection and possibilities of diagnose and its treatment. We present five children with symptomatic congenital CMV infection that were hospitalized for the period 1992–2002 at the Neonatal department in the University Children’s Hospital in Ljubljana.Conclusions: Identification of infected neonates, especially those with asimptomatic congenital CMV infection, is difficult. Latest incidence of infection in Slovenia is unknown. With new investigations the efficiency of antiviral therapy was discovered but exact indications for therapy are not yet known. CMV vaccine, once available, may ultimately be the best control strategy for this important public health problem. Proper educating women in childbearing age about the risks of CMV and how to avoid disease transmission during pregnancy (hand washing, avoiding mouth-to-mouth contact with preschool children, usage of gloves especially when handling dipers or respiratory secretions are the only control strategies available.

  19. Prevalence of congenital amusia.

    Science.gov (United States)

    Peretz, Isabelle; Vuvan, Dominique T

    2017-05-01

    Congenital amusia (commonly known as tone deafness) is a lifelong musical disorder that affects 4% of the population according to a single estimate based on a single test from 1980. Here we present the first large-based measure of prevalence with a sample of 20 000 participants, which does not rely on self-referral. On the basis of three objective tests and a questionnaire, we show that (a) the prevalence of congenital amusia is only 1.5%, with slightly more females than males, unlike other developmental disorders where males often predominate; (b) self-disclosure is a reliable index of congenital amusia, which suggests that congenital amusia is hereditary, with 46% first-degree relatives similarly affected; (c) the deficit is not attenuated by musical training and (d) it emerges in relative isolation from other cognitive disorder, except for spatial orientation problems. Hence, we suggest that congenital amusia is likely to result from genetic variations that affect musical abilities specifically.

  20. The effect of morphine on biliary dynamics

    International Nuclear Information System (INIS)

    Pedersen, S.A.; Oester-Joergensen, E.; Kraglund, K.

    1987-01-01

    The effect of morphine on biliary dynamics was studied by cholescintigraphy with 99m Tc-HIDA. Among 30 normals without morphine injection 3 did not demonstrate intestinal radioactivity after 1 h, whereas all visualized the gallbladder. Eight normals with morphine injection did not demonstrate intestinal radioactivity after 2 h, but all had gallbladder visualization very early. Variables of the time-activity curves from liver areas did not point to impaired uptake or excretion. Morphine-induced increase in resistance to passage from the common duct to the intestines in normals is of a magnitude that forces the total amount of bile to accumulate in the gallbladder. Results from 11 patients after cholecystectomy indicate that the increase in pressure is less than the maximal secretory pressure of the liver. The resorptive capacity and the compliance of the gallbladder enable these events to take place without signs of secondary liver impairment

  1. Toward precision medicine in primary biliary cholangitis.

    Science.gov (United States)

    Carbone, Marco; Ronca, Vincenzo; Bruno, Savino; Invernizzi, Pietro; Mells, George F

    2016-08-01

    Primary biliary cholangitis is a chronic, cholestatic liver disease characterized by a heterogeneous presentation, symptomatology, disease progression and response to therapy. In contrast, clinical management and treatment of PBC is homogeneous with a 'one size fits all' approach. The evolving research landscape, with the emergence of the -omics field and the availability of large patient cohorts are creating a unique opportunity of translational epidemiology. Furthermore, several novel disease and symptom-modifying agents for PBC are currently in development. The time is therefore ripe for precision medicine in PBC. In this manuscript we describe the concept of precision medicine; review current approaches to risk-stratification in PBC, and speculate how precision medicine in PBC might develop in the near future. Copyright © 2016 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  2. Molecular diagnostic testing for primary biliary cholangitis.

    Science.gov (United States)

    Gatselis, Nikolaos K; Dalekos, George N

    2016-09-01

    A reliable liver autoimmune serology for the diagnosis of primary biliary cholangitis (PBC) is of particular importance. Recognition of patients at early stages and prompt treatment initiation may alter the outcome, slow progression, delays liver failure, and improves survival. In this review, we summarize and discuss the published data obtained from literature searches from PubMed and The National Library of Medicine (USA) and our own experience on the current and potential molecular based approaches to the diagnosis of PBC. Expert commentary: Standardization of liver diagnostic serology and clinical governance are two major points as antimitochondrial antibodies are the diagnostic hallmark of the disease and PBC-specific antinuclear antibodies could assist in the diagnosis and estimation of prognosis. New biomarkers such as novel autoantibodies, genetic polymorphisms, metabolomic profiling, micro-RNA and epigenetics may assist to the understanding, diagnosis and management of the disease.

  3. Thyroid storm precipitated by acute biliary pancreatitis

    Directory of Open Access Journals (Sweden)

    Mehrdad Karimi

    2017-01-01

    Full Text Available Thyroid storm is an acute, life-threatening exacerbation and sudden releasing large amounts of thyroid hormone in a short period of time. Nevertheless, critical aggravation of hyperthyroidism typically resulted from concurrent disorder. Synchronous management of thyroid storm along with its precipitant, such as infection is recommended. We described the case of an acute biliary pancreatitis complicated with a thyroid storm. The patient was successfully managed with a quick surgical intervention and further critical care for thyroid storm. Although it is widely believed that pancreatitis is seldom concurrent with thyrotoxicosis, thyroid storm can be precipitated by a variety of factors, including intra-abdominal infections such as acute pancreatitis or perforated peptic ulcer. In conclusion, acute pancreatitis in patients with thyrotoxicosis seems to be extremely rare, but such patients should be managed intensively against underlying thyroid disorders as well as pancreatitis.

  4. The post-traumatic biliary cyst

    International Nuclear Information System (INIS)

    Stellamor, K.; Hruby, W.; Stelzer, P.

    1982-01-01

    The case report communicated here illustrates a late complication subsequent to an emergency operation for liver rupture. This complication may result from ligating a branch of the hepatic artery to stop bleeding. If the necessary second operation is omitted for any reason, the hepatic sequester can develop into an abscess or a biliary cyst. It is to be expected that these late complications will occur more frequently in future owing to a probable increase in the incidence of the so-called blunt abdominal traumas. The reason for this is, on the one hand, the growing number of traffic accidents and, on the other hand, the increasing use of safety seat belts which tend to promote the occurrence of ruptures of the liver as part of the blunt abdominal traumas. However, we must emphasize that it is the use of safety seat belts only which ensures the traffic victims' chance of survival. (orig.) [de

  5. MR imaging evaluation of pancreas and hepatobiliary system in cystic fibrosis

    International Nuclear Information System (INIS)

    Murayama, S.; Robinson, A.E.; Stallworth, J.; Mulvihill, D.; Beckerman, R.; Davis, S.

    1988-01-01

    The pancreas and hepatobiliary system of 20 patients with cystic fibrosis were analyzed with a 1.5-T magnet. T1-weighted, T2-weighted, and proton-density images were obtained, and the T1 and T2 values of the pancreas were calculated. Signal intensity ratios were calculated for the signal intensities of the pancreas and the liver relative to that of muscle and fat on each pulse sequence. Fatty replacement of the pancreas and regenerating nodules of biliary cirrhosis were readily identified on T1-weighted images. The T1 values for pancreatic tissue showed promise as a good marker for pancreatic disease progression. Pancreas-to-fat signal intensity ratios on 300/20 (repetition time msec/echo time msec) and 2,000/75 images were considered to be a good standard for evaluating the level of pancreatic involvement

  6. MRI findings of intracranial cystic meningiomas

    International Nuclear Information System (INIS)

    Zhang, D.; Hu, L.-B.; Zhen, J.W.; Zou, L.-G.; Feng, X.-Y.; Wang, W.-X.; Wen, L.

    2009-01-01

    Aim: To report the magnetic resonance imaging (MRI) features of intracranial cystic meningiomas and compare these features in intra- and peritumoural cyst groups. Materials and methods: Fourteen cases of peritumoural cystic meningiomas were compared with 18 cases of intratumoural cystic meningiomas. All patients were examined using non-enhanced and contrast-enhanced MRI. Tumour location, tumour size, signal intensity, enhancement characteristics, and cystic changes were assessed. The MRI features were compared between the intra- and peritumoural cyst groups. Results: Most cystic meningiomas comprised two or more cysts. The solid parts of the tumours showed moderate or marked enhancement after the injection of contrast material. An enhanced cyst wall was found in six out of 14 cases in the peritumoural cyst group, but not in the intratumoural cyst group. Peritumoural cystic meningiomas were predominately located in the cerebral falx, whereas the intratumoural cystic meningiomas were predominantly found in frontal convexity (X 2 = 7.434, p = 0.024). The cysts were larger in the peritumoural cyst group than in the intratumoural cyst group (t = 5.274, p = 0.0258). Peritumoural oedema was more commonly found in the intratumoural cyst group (X 2 = 6.863, p = 0.008). Cystic meningiomas with solid parts located inside the cyst are reported for the first time. Conclusion: Cystic meningiomas, although uncommon, should be differentiated from other cystic intracranial lesions. Peri- and intratumoural cystic meningiomas have distinct MRI features. The present study provides the first report of two lesions with solid parts located inside the cyst, as well as one lesion with a calcified solid nodule and haemorrhage within the cyst.

  7. 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.

  8. 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...

  9. 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.

  10. Hormone replacement for osteoporosis in women with primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Rudic, Jelena S; Poropat, Goran; Krstic, Miodrag N

    2011-01-01

    Women with primary biliary cirrhosis often suffer from postmenopausal osteoporosis due to their age, or osteoporosis secondary to their liver disease, or treatments provided for their liver disease. Hormone replacement increases bone mineral density and reduces fractures in postmenopausal women...

  11. 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.

  12. 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...

  13. 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.

  14. Nitrofurantoin and congenital abnormalities

    DEFF Research Database (Denmark)

    Czeizel, A.E.; Rockenbauer, M.; Sørensen, Henrik Toft

    2001-01-01

    or fetuses with Down’s syndrome (patient controls), 23 (2.8%) pregnant women were treated with nitrofurantoin. The above differences between population controls and cases may be connected with recall bias, because the case-control pair analysis did not indicate a teratogenic potential of nitrofurantoin use......Objective: To study human teratogenic potential of oral nitrofurantoin treatment during pregnancy. Materials and Methods: Pair analysis of cases with congenital abnormalities and matched population controls in the population-based dataset of the Hungarian Case-Control Surveillance of Congenital...... during the second and the third months of gestation, i.e. in the critical period for major congenital abnormalities. Conclusion: Treatment with nitrofurantoin during pregnancy does not present detectable teratogenic risk to the fetus....

  15. Congenital syphilis: literature review

    Directory of Open Access Journals (Sweden)

    Eduardo Chaida Sonda

    2013-01-01

    Full Text Available Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher than the ones spent with a baby without this infection. The treatment is generally carried out with penicillin and must be extended to sexual partners. Inadequate or lack of treatment of congenital syphilis can result in miscarriage, premature birth, acute complications and other fetal sequelae. KEYWORDS: Congenital syphilis. Treponema pallidum. Vertical transmission.

  16. Congenital cataract screening

    Directory of Open Access Journals (Sweden)

    Zhale Rajavi

    2016-01-01

    Full Text Available Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  17. 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

  18. Outcome in cystic fibrosis liver disease.

    LENUS (Irish Health Repository)

    Rowland, Marion

    2011-01-01

    Evidence suggests that cystic fibrosis liver disease (CFLD) does not affect mortality or morbidity in patients with cystic fibrosis (CF). The importance of gender and age in outcome in CF makes selection of an appropriate comparison group central to the interpretation of any differences in mortality and morbidity in patients with CFLD.

  19. Induction of ovarian cystic follicles in sheep.

    Science.gov (United States)

    Christman, S A; Bailey, M T; Head, W A; Wheaton, J E

    2000-10-01

    Cystic follicles are a significant cause of infertility in women, dairy cattle and sheep. Sheep were used as a model to identify factors that may elicit formation of cystic follicles. Insulin resistance and elevated LH activity were tested in overweight ewes because of associations among these factors and the formation of cystic follicles. Sheep were synchronized using a progesterone-releasing pessary and insulin resistance was induced during the synchronization period through administration of bovine somatotropin. Following removal of pessaries follicular growth was stimulated by treatment with eCG or eCG and hCG (PG-600). Follicular growth was monitored via daily transrectal ultrasonography and blood samples were collected for hormonal analyses. Six of 18 ewes had a subnormal or absent preovulatory gonadotropin surge and developed cystic follicles. Neither insulin resistance nor elevated LH activity were associated with formation of cystic follicles. Ewes that developed cystic follicles were heavier (93 +/- 4 kg) than ewes that ovulated (81 +/- 3 kg; P = 0.02). Furthermore, following pessary removal and initiation of daily ultrasonography, ewes that developed cystic follicles lost body weight (-3 +/- 1%), while ovulatory ewes continued to gain body weight (1 +/- 1%; P = 0.005). It is speculated that in heavy ewes metabolic factors associated with acute body weight loss inhibit the positive feedback of estradiol and thereby suppress the preovulatory gonadotropin surge leading to formation of cystic follicles.

  20. Cystic malformations of the neck in children

    Energy Technology Data Exchange (ETDEWEB)

    Koch, Bernadette L. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States)

    2005-05-01

    The most common cystic malformations of the neck are the result of abnormal embryogenesis involving the thyroglossal duct (TGD), lymphatic primordia and the branchial apparatus. When the basic embryology of these structures is considered, a reasonable differential diagnosis - and in some cases a definitive diagnosis - can be achieved based on the location and the imaging characteristics of the cystic mass. (orig.)

  1. Cystic malformations of the neck in children

    International Nuclear Information System (INIS)

    Koch, Bernadette L.

    2005-01-01

    The most common cystic malformations of the neck are the result of abnormal embryogenesis involving the thyroglossal duct (TGD), lymphatic primordia and the branchial apparatus. When the basic embryology of these structures is considered, a reasonable differential diagnosis - and in some cases a definitive diagnosis - can be achieved based on the location and the imaging characteristics of the cystic mass. (orig.)

  2. Laparoscopic cholecystectomy in adult cystic fibrosis.

    LENUS (Irish Health Repository)

    McGrath, D S

    2012-02-03

    Two female patients with Cystic Fibrosis, attending the Adult Regional Cystic Fibrosis centre at the Cork University Hospital, were investigated for upper abdominal pain and found to have gallstones at ultrasonography. Laparoscopic cholecystectomy was performed successfully and, without complication, in both patients.

  3. Neurofibromas as bilateral cystic chest wall swellings.

    African Journals Online (AJOL)

    secondary to an infection, usually parasitic infections. [6,7]. However, cystic tumours of the chest wall result- ing from degenerative changes in peripheral nerves of its layers are rare, and we did not see any in the pub- lished literature. We are reporting a single case of bilat- eral cystic degenerative changes in neurofibromas ...

  4. Unusual growth rate during cystic echinococcosis.

    Science.gov (United States)

    Valour, Florent; Khenifer, Safia; Della-Schiava, Nellie; Cotte, Eddy; Guibert, Benoit; Wallon, Martine; Durupt, Stéphane; Durieu, Isabelle

    2014-04-01

    Cystic echinococcosis is a world wild zoonosis caused by Echinococcus granulosus, leading to hepatic and lung cysts with a usually slight growth rate. We report the case of an 82year-old Algerian woman with hepatic and lung cystic echinococcosis with a 10-fold size increase in 6months. Copyright © 2013. Published by Elsevier Ireland Ltd.

  5. Self-management education for cystic fibrosis.

    LENUS (Irish Health Repository)

    Savage, Eileen

    2011-01-01

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted.

  6. Congenital maxillary double lip

    Directory of Open Access Journals (Sweden)

    Dinesh Singh Chauhan

    2012-01-01

    Full Text Available Double lip, also referred to as "macrocheilia," is a rare anomaly which affects the upper lip more commonly than the lower lip. It consists of a fold of excess or redundant hypertrophic tissue on the mucosal side of the lip. The congenital double lip is believed to be present at birth and becomes more prominent after eruption of teeth. It affects esthetics and also interferes with speech and mastication. Simple surgical excision produces good functional and cosmetic results. We report a case of a non-syndromic congenital maxillary double lip in a 21-year-old male patient.

  7. Congenital hearing impairment

    Energy Technology Data Exchange (ETDEWEB)

    Robson, Caroline D. [Children' s Hospital and Harvard Medical School, Division of Neuroradiology, Department of Radiology, Boston, MA (United States)

    2006-04-15

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  8. Congenital spinal malformations

    International Nuclear Information System (INIS)

    Ertl-Wagner, B.B.; Reiser, M.F.

    2001-01-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [de

  9. Congenital hearing impairment

    International Nuclear Information System (INIS)

    Robson, Caroline D.

    2006-01-01

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  10. Congenital laryngeal anomalies,

    Directory of Open Access Journals (Sweden)

    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  11. Congenital Heart Defects and CCHD

    Science.gov (United States)

    ... and more. Stony Point, NY 10980 Close X Home > Complications & Loss > Birth defects & other health conditions > Congenital heart defects and ... in congenital heart defects. You have a family history of congenital heart ... syndrome or VCF. After birth Your baby may be tested for CCHD as ...

  12. 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

  13. 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.

  14. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial): study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Schepers, N.J.; Bakker, O.J.; Besselink, M.G.; Bollen, T.L.; Dijkgraaf, M.G.; Eijck, C.H. van; Fockens, P.; Geenen, E.J. van; Grinsven, J. van; Hallensleben, N.D.; Hansen, B.E.; Santvoort, H.C. van; Timmer, R.; Anten, M.P.; Bolwerk, C.J.; Delft, F. von; Dullemen, H.M. van; Erkelens, G.W.; Hooft, J.E. van; Laheij, R.; Hulst, R.W. van der; Jansen, J.M.; Kubben, F.J.; Kuiken, S.D.; Perk, L.E.; Ridder, R.J. de; Rijk, M.C. de; Romkens, T.E.; Schoon, E.J.; Schwartz, M.P.; Spanier, B.W.; Tan, A.C.; Thijs, W.J.; Venneman, N.G.; Vleggaar, F.P.; Vrie, W. van de; Witteman, B.J.; Gooszen, H.G.; Bruno, M.J.

    2016-01-01

    BACKGROUND: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with concomitant

  15. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial): Study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    N.J. Schepers (Nicolien); O.J. Bakker (Olaf ); M.G. Besselink (Marc); T.L. Bollen (Thomas); M.G.W. Dijkgraaf (Marcel); C.H.J. van Eijck (Casper); P. Fockens (Paul); E-J.M. Geenen (Erwin-Jan); J. van Grinsven (Janneke); N.D.L. Hallensleben (Nora D.L.); B.E. Hansen (Bettina); H.C. van Santvoort (Hjalmar); R. Timmer (Robin); M.-P.G.F. Anten (Marie-Paule G.F.); C.L. Bolwerk (Clemens); F. van Delft (Foke); H.M. van Dullemen (Hendrik); G.W. Erkelens (G.Willemien); J.E. van Hooft (Jeanin); C. Laheij (Claudia); R.W.M. van der Hulst (René); J.M. Jansen (Jeroen); F.J. Kubben; S.D. Kuiken (Sjoerd D.); L.E. Perk (Lars E.); R. de Ridder (Rogier); M.C.M. Rijk; T.E.H. Römkens; E.J. Schoon (Erik); M.P. Schwartz (Matthijs P.); B.W.M. Spanier (Marcel); A.C. Tan (Adriaan); W.J. Thijs; N.G. Venneman (Niels); F.P. Vleggaar (Frank); W. van de Vrie (Wim); B.J.M. Witteman (Ben); H.G. Gooszen (Hein); M.J. Bruno (Marco)

    2016-01-01

    textabstractBackground: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with

  16. Biliary obstruction caused by intra-biliary tumor growth from recurred hepatocellular carcinoma after radiofrequency ablation: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Yi, Ji Hyun; Kim, Jae Won [Dept. of Radiology, Yeungnam University College of Medicine, Daegu (Korea, Republic of)

    2014-04-15

    A 59-year-old man with a known central hepatocellular carcinoma (HCC) underwent a trans-arterial-chemo-embolization (TACE) and a post-TACE percutaneous radiofrequency ablation (PRFA). Two months after the PRFA, the patient presented jaundice and an abdominal computed tomography was obtained. An arterial enhancing mass adjacent to the ablated necrotic lesion with a continuously coexisting mass inside the right hepatic duct, suggestive of a HCC recurrence with a direct extension to the biliary tract was found. Finally a biliary tumor obstruction has been developed and a percutaneous transhepatic biliary drainage was performed. This case of biliary obstruction caused by directly invaded recurred HCC after PRFA will be reported because of its rare occurrence.

  17. Early biliary decompression versus conservative treatment in acute biliary pancreatitis (APEC trial) : Study protocol for a randomized controlled trial

    NARCIS (Netherlands)

    Schepers, Nicolien J.; Bakker, Olaf J.; Besselink, Marc G H; Bollen, Thomas L.; Dijkgraaf, Marcel G W; van Eijck, Casper H J; Fockens, Paul; van Geenen, Erwin J M; van Grinsven, Janneke; Hallensleben, Nora D L; Hansen, Bettina E.; van Santvoort, Hjalmar C.; Timmer, Robin; Anten, Marie Paule G F; Bolwerk, Clemens J M; van Delft, Foke; van Dullemen, Hendrik M.; Erkelens, G. Willemien; van Hooft, Jeanin E.; Laheij, Robert; van der Hulst, René W M; Jansen, Jeroen M.; Kubben, Frank J G M; Kuiken, Sjoerd D.; Perk, Lars E.; de Ridder, Rogier J J; Rijk, Marno C M; Römkens, Tessa E H; Schoon, Erik J.; Schwartz, Matthijs P.; Spanier, B. W Marcel; Tan, Adriaan C I T L; Thijs, Willem J.; Venneman, Niels G.; Vleggaar, Frank P.; van de Vrie, Wim; Witteman, Ben J.; Gooszen, Hein G.; Bruno, Marco J.

    2016-01-01

    Background: Acute pancreatitis is mostly caused by gallstones or sludge. Early decompression of the biliary tree by endoscopic retrograde cholangiography (ERC) with sphincterotomy may improve outcome in these patients. Whereas current guidelines recommend early ERC in patients with concomitant

  18. Lung Cancers Associated with Cystic Airspaces: Underrecognized Features of Early Disease.

    Science.gov (United States)

    Sheard, Sarah; Moser, Joanna; Sayer, Charlie; Stefanidis, Konstantinos; Devaraj, Anand; Vlahos, Ioannis

    2018-01-01

    Early lung cancers associated with cystic airspaces are increasingly being recognized as a cause of delayed diagnoses-owing to data gathered from screening trials and encounters in routine clinical practice as more patients undergo serial imaging. Several morphologic subtypes of cancers associated with cystic airspaces exist and can exhibit variable patterns of progression as the solid elements of the tumor grow. Current understanding of the pathogenesis of these malignancies is limited, and the numbers of cases reported in the literature are small. However, several tumor cell types are represented in these lesions, with adenocarcinoma predominating. The features of cystic airspaces differ among cases and include emphysematous bullae, congenital or fibrotic cysts, subpleural blebs, bronchiectatic airways, and distended distal airspaces. Once identified, these cystic lesions pose management challenges to radiologists in terms of distinguishing them from benign mimics of cancer that are commonly seen in patients who also are at increased risk of lung cancer. Rendering a definitive tissue-based diagnosis can be difficult when the lesions are small, and affected patients tend to be in groups that are at higher risk of requiring biopsy or resection. In addition, the decision to monitor these cases can add to patient anxiety and cause the additional burden of strained departmental resources. The authors have drawn from their experience, emerging evidence from international lung cancer screening trials, and large databases of lung cancer cases from other groups to analyze the prevalence and evolution of lung cancers associated with cystic airspaces and provide guidance for managing these lesions. Although there are insufficient data to support specific management guidelines similar to those for managing small solid and ground-glass lung nodules, these data and guidelines should be the direction for ongoing research on early detection of lung cancer. © RSNA, 2018.

  19. Increased hepatic Th2 and Treg subsets are associated with biliary fibrosis in different strains of mice caused by Clonorchis sinensis.

    Directory of Open Access Journals (Sweden)

    Bei-Bei Zhang

    Full Text Available Previous studies showed that CD4+T cells responses might be involved in the process of biliary fibrosis. However, the underlying mechanism resulting in biliary fibrosis caused by Clonorchis sinensis remains not yet fully elucidated. The objectives of the present study were to investigate the different profiles of hepatic CD4+T cell subsets (Th1, Th2, Th17 and Treg cells and their possible roles in the biliary fibrosis of different strains of mice (C57BL/6, BALB/c and FVB mice induced by C. sinensis infection. C57BL/6, BALB/c and FVB mice were orally gavaged with 45 metacercariae. All mice were sacrificed on 28 days post infection in deep anesthesia conditions. The leukocytes in the liver were separated to examine CD4+T cell subsets by flow cytometry and the left lobe of liver was used to observe pathological changes, collagen depositions and the concentrations of hydroxyproline. The most serious cystic and fibrotic changes appeared in FVB infected mice indicated by gross observation, Masson's trichrome staining and hydroxyproline content detection. In contrast to C57BL/6 infected mice, diffuse nodules and more intensive fibrosis were observed in the BALB/c infected mice. No differences of the hepatic Th1 subset and Th17 subset were found among the three strains, but the hepatic Th2 and Treg cells and their relative cytokines were dramatically increased in the BALB/c and FVB infected groups compared with the C57BL/6 infected group (P<0.01. Importantly, increased Th2 subset and Treg subset all positively correlated with hydroxyproline contents (P<0.01. This result for the first time implied that the increased hepatic Th2 and Treg cell subsets were likely to play potential roles in the formation of biliary fibrosis in C. sinensis-infected mice.

  20. 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.

  1. 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...

  2. Muscular cystic hydatidosis: case report

    Directory of Open Access Journals (Sweden)

    Naspetti Riccardo

    2007-03-01

    Full Text Available Abstract Background Hydatidosis is a zoonosis caused by Echinococcus granulosus, and ingesting eggs released through the faeces from infected dogs infects humans. The location of the hydatid cysts is mostly hepatic and/or pulmonary, whereas musculoskeletal hydatidosis is very rare. Case presentation We report an unusual case of primary muscular hydatidosis in proximity of the big adductor in a young Sicilian man. The patient, 34 years old, was admitted to the Department of Infectious and Tropical Diseases for ultrasonographic detection, with successive confirmation by magnetic resonance imaging, of an ovular mass (13 × 8 cm in the big adductor of the left thigh, cyst-like, and containing several small cystic formations. Serological tests for hydatidosis gave negative results. A second drawing of blood was done 10 days after the first one and showed an increase in the antibody titer for hydatidosis. The patient was submitted to surgical excision of the lesion with perioperatory prophylaxis with albendazole. The histopathological examination of the bioptic material was not diriment in the diagnosis, therefore further tests were performed: additional serological tests for hydatidosis for the evaluation of IgE and IgG serotype (Western Blot and REAST, and molecular analysis of the excised material. These more specific serological tests gave positive results for hydatidosis, and the sequencing of the polymerase chain reaction products from the cyst evidenced E. granulosus DNA, genotype G1. Any post-surgery complications was observed during 6 following months. Conclusion Cystic hydatidosis should always be considered in the differential diagnosis of any cystic mass, regardless of its location, also in epidemiological contests less suggestive of the disease. The diagnosis should be achieved by taking into consideration the clinical aspects, the epidemiology of the disease, the imaging and immunological tests but, as demonstrated in this case, without

  3. Internal irradiation for cystic craniopharyngioma

    International Nuclear Information System (INIS)

    Kobayashi, Tatsuya; Kageyama, Naoki

    1979-01-01

    Internal irradiation with P-32 chromic phosphate and Au-198 colloid was used to treat cystic craniopharyngioma. A newly developed dosimetric formula, by which the radiation dose can be calculated simultaneously at the cyst wall and at a point far from the radioactive source and the untoward effect of irradiation on surrounding brain tissue can be eliminated, especially in cases in which the wall is thin and can be penetrated by beta emission, was used. Radioactive phosphate or gold was injected into eight craniopharyngioma cysts throught the Ommaya reservoir and a tube inserted at the first craniotomy. All cysts were effectively treated for 3 to 33 months, to eliminate fluid retention or collapse. A collapsed cyst was removed at the second craniotomy and irradiation was histologically shown to be effective. Oculomotor palsy, a side effect of irradiation, occurred 10 days after the injection of 5 mc of P-32 chromic phosphate only in a case of small cysts (5.0 ml) in the supra- and intracellular regions. The thickness of the cyst wall was less than 0.5 mm and the oculomotor nerves were thought to adhere to the wall. Not only the amount of wall dose but also the thickness of the wall and localization of the cyst are important factors in internal irradiation. Sufficient and safer doses which kill tumor cells in the wall and have no side effects, are 9,000 to 30,000 rad. Internal irradiation can be used to treat large cysts of more than 10 ml which are supposedly difficult to remove radically and or multiple cysts. It is effective not only for cystic craniopharyngioma but also for intracrania cystic tumors other than craniopharyngioma, if dosimetry is accurate. (J.P.N.)

  4. 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

  5. MR imaging appearance of laryngeal atresia (congenital high airway obstruction syndrome): unique course in a fetus

    Energy Technology Data Exchange (ETDEWEB)

    Kuwashima, Shigeko; Kitajima, Kazuhiro; Kaji, Yasushi [Dokkyo Medical University, Department of Radiology, Mibu, Shimotsuga-gun, Tochigi (Japan); Watanabe, Hiroshi [Dokkyo Medical University, Department of Obstetrics and Gynecology, Mibu (Japan); Watabe, Yoshiyuki; Suzumura, Hiroshi [Dokkyo Medical University, Department of Pediatrics, Mibu (Japan)

    2008-03-15

    Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening syndrome. Most cases are diagnosed prenatally by US. We report a fetus with this syndrome that showed a unique course revealed on MRI. Ultrasonography at 22 weeks demonstrated that the fetus had ascites and bilaterally enlarged hyperechoic lungs. Congenital infection, congenital cystic adenomatoid malformation or CHAOS was suspected. Subsequent MRI performed at 24 weeks demonstrated bilaterally enlarged high-signal lungs, dilated bronchi, massive ascites, subcutaneous oedema and polyhydramnios. MRI confirmed the diagnosis of CHAOS. A second MRI at 35 weeks showed that the bilateral lung enlargement, ascites, oedema and polyhydramnios had resolved, but that the appearance of the airway was unchanged. The infant was delivered by caesarean section at 38 weeks of gestation and immediate tracheostomy was performed. This spontaneous regression was explained by a tracheo-oesophageal fistula that may have decreased the intrathoracic pressure. (orig.)

  6. MR imaging appearance of laryngeal atresia (congenital high airway obstruction syndrome): unique course in a fetus

    International Nuclear Information System (INIS)

    Kuwashima, Shigeko; Kitajima, Kazuhiro; Kaji, Yasushi; Watanabe, Hiroshi; Watabe, Yoshiyuki; Suzumura, Hiroshi

    2008-01-01

    Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening syndrome. Most cases are diagnosed prenatally by US. We report a fetus with this syndrome that showed a unique course revealed on MRI. Ultrasonography at 22 weeks demonstrated that the fetus had ascites and bilaterally enlarged hyperechoic lungs. Congenital infection, congenital cystic adenomatoid malformation or CHAOS was suspected. Subsequent MRI performed at 24 weeks demonstrated bilaterally enlarged high-signal lungs, dilated bronchi, massive ascites, subcutaneous oedema and polyhydramnios. MRI confirmed the diagnosis of CHAOS. A second MRI at 35 weeks showed that the bilateral lung enlargement, ascites, oedema and polyhydramnios had resolved, but that the appearance of the airway was unchanged. The infant was delivered by caesarean section at 38 weeks of gestation and immediate tracheostomy was performed. This spontaneous regression was explained by a tracheo-oesophageal fistula that may have decreased the intrathoracic pressure. (orig.)

  7. Tumor disease and associated congenital abnormalities on prenatal MRI

    International Nuclear Information System (INIS)

    Nemec, Stefan F.; Horcher, Ernst; Kasprian, Gregor; Brugger, Peter C.; Bettelheim, Dieter; Amann, Gabriele; Nemec, Ursula; Rotmensch, Siegfried; Rimoin, David L.; Graham, John M. Jr.; Prayer, Daniela

    2012-01-01

    Objective: Fetal tumors can have a devastating effect on the fetus, and may occur in association with congenital malformations. In view of the increasing role of fetal magnetic resonance imaging (MRI) as an adjunct to prenatal ultrasonography (US), we sought to demonstrate the visualization of fetal tumors, with regard to congenital abnormalities, on MRI. Materials and methods: This retrospective study included 18 fetuses with tumors depicted on fetal MRI after suspicious US findings. An MRI standard protocol was used to diagnose tumors judged as benign or malignant. All organ systems were assessed for tumor-related complications and other congenital malformations. Available US results and histopathology were compared with MRI. Results: There were 13/18 (72.2%) benign and 5/18 (27.8%) malignant tumors diagnosed: a cerebral primitive neuroectodermal tumor in 1/18, head–neck teratomas in 4/18; ventricular rhabdomyomas in 4/18; a cardiac teratoma in 1/18; a hepatoblastoma in 1/18; neuroblastomas in 2/18; a cystic hemorrhagic adrenal hyperplasia in 1/18; a pelvic leiomyoma in 1/18; sacrococcygeal teratomas in 3/18. Tumor-related complications were present in 13/18 (72.2%) cases; other congenital abnormalities in 3/18 (16.7%). MRI diagnosis and histology were concordant in 8/11 (72.7%) cases. In 6/12 (50%) cases, US and MRI diagnoses were concordant, and, in 6/12 (50%) cases, additional MRI findings changed the US diagnosis. Conclusion: Our MRI results demonstrate the visualization of fetal tumors, with frequently encountered tumor-related complications, and other exceptional congenital abnormalities, which may provide important information for perinatal management. Compared to prenatal US, MRI may add important findings in certain cases.

  8. Tumor disease and associated congenital abnormalities on prenatal MRI.

    Science.gov (United States)

    Nemec, Stefan F; Horcher, Ernst; Kasprian, Gregor; Brugger, Peter C; Bettelheim, Dieter; Amann, Gabriele; Nemec, Ursula; Rotmensch, Siegfried; Rimoin, David L; Graham, John M; Prayer, Daniela

    2012-02-01

    Fetal tumors can have a devastating effect on the fetus, and may occur in association with congenital malformations. In view of the increasing role of fetal magnetic resonance imaging (MRI) as an adjunct to prenatal ultrasonography (US), we sought to demonstrate the visualization of fetal tumors, with regard to congenital abnormalities, on MRI. This retrospective study included 18 fetuses with tumors depicted on fetal MRI after suspicious US findings. An MRI standard protocol was used to diagnose tumors judged as benign or malignant. All organ systems were assessed for tumor-related complications and other congenital malformations. Available US results and histopathology were compared with MRI. There were 13/18 (72.2%) benign and 5/18 (27.8%) malignant tumors diagnosed: a cerebral primitive neuroectodermal tumor in 1/18, head-neck teratomas in 4/18; ventricular rhabdomyomas in 4/18; a cardiac teratoma in 1/18; a hepatoblastoma in 1/18; neuroblastomas in 2/18; a cystic hemorrhagic adrenal hyperplasia in 1/18; a pelvic leiomyoma in 1/18; sacrococcygeal teratomas in 3/18. Tumor-related complications were present in 13/18 (72.2%) cases; other congenital abnormalities in 3/18 (16.7%). MRI diagnosis and histology were concordant in 8/11 (72.7%) cases. In 6/12 (50%) cases, US and MRI diagnoses were concordant, and, in 6/12 (50%) cases, additional MRI findings changed the US diagnosis. Our MRI results demonstrate the visualization of fetal tumors, with frequently encountered tumor-related complications, and other exceptional congenital abnormalities, which may provide important information for perinatal management. Compared to prenatal US, MRI may add important findings in certain cases. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  9. Pulmonary complications of cystic fibrosis

    International Nuclear Information System (INIS)

    Ng, M.Y.; Flight, W.; Smith, E.

    2014-01-01

    The life expectancy of patients with cystic fibrosis (CF) has steadily increased over recent decades with a corresponding increase in the frequency of complications of the disease. Radiologists are increasingly involved with managing and identifying the pulmonary complications of CF. This article reviews the common manifestations of CF lung disease as well as updating radiologists with a number of less well-known complications of the condition. Early and accurate detection of the pulmonary effects of CF are increasingly important to prevent irreversible lung damage and give patients the greatest possibility of benefiting from the new therapies becoming available, which correct the underlying defect causing CF

  10. Endocrine Disorders in Cystic Fibrosis.

    Science.gov (United States)

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

    Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Severe congenital neutropenia

    DEFF Research Database (Denmark)

    Borregaard, Niels

    2014-01-01

    In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutr...... neutropenia (SCN)....

  12. Giant Congenital Melanocytic Nevus

    DEFF Research Database (Denmark)

    Rasmussen, Bo Sonnich; Henriksen, Trine Foged; Kølle, Stig-Frederik Trojahn

    2015-01-01

    Giant congenital melanocytic nevi (GCMN) occur in 1:20,000 livebirths and are associated with increased risk of malignant transformation. The treatment of GCMN from 1981 to 2010 in a tertiary referral center was reviewed evaluating the modalities used, cosmetic results, associated complications...

  13. Congenital neck masses.

    Science.gov (United States)

    Rosa, Peter A; Hirsch, David L; Dierks, Eric J

    2008-08-01

    Congenital neck lesions reflect abnormal embryogenesis in head and neck development. A thorough knowledge of embryology and anatomy is critical in the diagnosis and treatment of these lesions. The appropriate diagnosis of these lesions is necessary to provide appropriate treatment and long-term follow up, because some of these lesions may undergo malignant transformation or be harbingers of malignant disease.

  14. Congenital extracranial meningioma

    International Nuclear Information System (INIS)

    Wong, H.F.; Ng, S.H.; Wai, Y.Y.; Wan, Y.L.; Kong, M.S.

    1995-01-01

    The authors report a case of congenital meningioma in a newborn. This tumour is extremely rare and only six cases have been reported in the literature. Those reported cases were mainly intracranial. This is the first case of a neonatal extracranial meningioma that was evaluated preoperatively by computed tomography and magnetic resonance imaging. (orig.)

  15. Pseudoamblyopia in Congenital Cyclotropia

    Directory of Open Access Journals (Sweden)

    Antonio Frattolillo

    2017-01-01

    Full Text Available Purpose. To study the effect of surgery on amblyopia and suppression associated with congenital cyclovertical strabismus. Methods. The fixation pattern was investigated with microperimetry before and soon after surgery in ten consecutive children operated for congenital superior oblique palsy at the S. Martino Hospital, Belluno, Italy, between September 2014 and December 2015. Changes in visual performance in terms of best-corrected visual acuity (BCVA and stereopsis between the day before and one week after surgery were also evaluated. No other amblyopia treatment has been administered during the time study. Results. Surgical correction of the excyclodeviation in congenital SO palsy determined monocular and binocular sensory consequences: monocularly, in the cyclodeviated amblyopic eye, BCVA (0.46–0.03 LogMAR; p<0.0001 and the fixation pattern improved, as demonstrated by microperimetry examination. Binocularly, stereopsis improved or emerged while suppression at the Worth four-dot test disappeared. Conclusions. In the absence of further amblyopic factors such as coexisting constant vertical and/or horizontal deviation and anisometropia, the amblyopia encountered in congenital SO palsy may resolve soon after the surgical alignment. Therefore, it may be considered and defined “pseudoamblyopia.”

  16. Identification of congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    . The study evaluated the assessment procedure of 190 children and adults found to be congenitally deafblind. Among the 190 individuals 76 percent were determined using functional assessment in addition to medical examination. A case example involving a 12-year-old child is also presented to illustrate...

  17. Congenital tracheobiliary fistula diagnosed with contrast-enhanced CT and 3-D reformation

    Energy Technology Data Exchange (ETDEWEB)

    Chawla, Soni C. [Olive View-UCLA Medical Center, Department of Radiological Sciences, Sylmar, CA (United States); University of California, Los Angeles (UCLA), Department of Radiological Sciences, Sylmar, CA (United States); Jha, Priyanka; Breiman, Richard; Gooding, Charles [University of California, San Francisco (UCSF), Department of Radiological Sciences, San Francisco, CA (United States); Farmer, Diana [University of California, San Francisco (UCSF), Department of Pediatric Surgery, San Francisco, CA (United States)

    2008-09-15

    Congenital tracheobiliary fistula (CTBF) is a rare malformation. So far 24 cases have been reported in the English language literature. The imaging techniques used in diagnosis have been bronchography, fistulography, cholangiography, hepatobiliary nuclear imaging and MRI. We report a newborn patient who presented with right lung consolidation and biliptysis. The radiographic diagnosis of tracheobiliary fistula was made on multidetector CT scan of the chest and abdomen. Multiple 3-D volume-rendered reformations were performed. An abnormal air-filled tract was seen connecting the posteroinferior aspect of the carina and left biliary system, which was successfully treated surgically. (orig.)

  18. Helicobacter species are associated with possible increase in risk of biliary lithiasis and benign biliary diseases

    Directory of Open Access Journals (Sweden)

    Pandey Manoj

    2007-08-01

    Full Text Available Abstract Background Hepato-biliary tract lithiasis is common and present either as pain or as asymptomatic on abdominal ultrasonography for other causes. Although the DNA of Helicobacter species are identified in the gallbladder bile, tissue or stones analyzed from these cases, still a causal relationship could not be established due to different results from different geographical parts. Methods A detailed search of pubmed and pubmedcentral was carried out with key words Helicobacter and gallbladder, gallstones, hepaticolithiasis, cholelithiasis and choledocholithiasis, benign biliary diseases, liver diseases. The data was entered in a data base and meta analysis was carried out. The analysis was carried out using odds ratio and a fixed effect model, 95% confidence intervals for odds ratio was calculated. Chi square test for heterogeneity was employed. The overall effect was calculated using Z test. Results A total of 12 articles were identified. One study used IgG for diagnosis while others used the PCR for Ure A gene, 16 S RNA or Cag A genes. A couple of studies used culture or histopathology besides the PCR. The cumulative results show a higher association of Helicobacter with chronic liver diseases (30.48%, and stone diseases (42.96%(OR 1.77 95% CI 1.2–2.58; Z = 2.94, p = 0.003, the effect of each could not be identified as it was difficult to isolate the effect of helicobacter due to mixing of cases in each study. Conclusion The results of present meta analysis shows that there is a slight higher risk of cholelithiasis and benign liver disease (OR 1.77, however due to inherent inability to isolate the effect of stone disease from that of other benign lesions it is not possible to say for sure that Helicobacter has a casual relationship with benign biliary disease or stone disease or both.

  19. Efficacy of percutaneous intraductal radiofrequency ablation combined with biliary stenting in treatment of malignant biliary obstruction

    Directory of Open Access Journals (Sweden)

    ZHANG Kai

    2015-05-01

    Full Text Available ObjectiveTo investigate the safety and feasibility of percutaneous intraductal radiofrequency ablation (palliative therapy in the treatment of malignant obstructive jaundice. MethodsThis study included 20 patients with unresectable malignant obstructive jaundice, who were treated in the First Affiliated Hospital of Zhengzhou University from January 2013 to March 2014. Nine of them (test group underwent percutaneous intraductal radiofrequency ablation plus metallic biliary stent placement. The other 11 similar cases (control group underwent metallic biliary stent placement alone after successful percutaneous transhepatic cholangiography. Operative complications and remission of jaundice were observed, and the stent patency after at 3 and 6 months after operation was evaluated and compared between the two groups. ResultsAll patients were followed completely by outpatient or telephone. The stent patency rate at 3 months after operation was 9/9 in the test group and 8/11 in the control group (χ2=2.888, P=0.218, and the stent patency rates at 6 months were 7/8 and 3/11, respectively (χ2=6.739, P=0.02. During follow-up, one case in the test group died of gastrointestinal bleeding at 113 d after operation; one case in the control group died of liver failure at 57 d after operation and one case died of disseminated intravascular coagulation at 142 d. ConclusionPercutaneous intraductal radiofrequency ablation is safe and feasible in the treatment of malignant biliary obstruction, and the preliminary efficacy in prolonging the patency of self-expanding metallic stent is satisfactory. However, this therapy needs to be further verified via large-sample randomized controlled studies.

  20. Intracranial cystic lesions; Intrakranielle zystische Laesionen

    Energy Technology Data Exchange (ETDEWEB)

    Ahlhelm, F.; Goetschi, S. [Kantonsspital Baden AG, Abteilung fuer Neuroradiologie, Baden (Switzerland); Shariat, K. [Kantonsspital Winterthur, Klinik fuer Neurochirurgie, Winterthur (Switzerland); Ulmer, S. [Universitaetsklinikum Schleswig-Holstein, Klinik fuer Radiologie und Neuroradiologie, Kiel (Germany)

    2018-02-15

    Intracerebral cysts are common findings in imaging of the neurocranium and are not always clinically significant. The pathological spectrum of intracerebral cysts is, however, very broad and in addition to incidental findings includes developmental disorders, malformation tumors, primary and secondary neoplasms and infectious etiologies, such as cerebral abscess formation, cysticercosis or residuals after congenital cytomegalovirus infections. Intracerebral cystic defects may be caused by inflammatory central nervous system (CNS) diseases, such as multiple sclerosis as well as by mitochondriopathies, leukodystrophy, electrolyte disturbances or osmotic demyelination syndrome or brain infarctions, e.g. after lacunar infarctions or as encephalomalacic changes after severe traumatic brain injury. In addition to the radiological findings of cysts in magnetic resonance imaging (MRI) or in computed tomography (CT), the localization, patient age, patient medical history and laboratory diagnostics are helpful for the differential diagnostics. In addition to the morphological assessment, advanced MRI techniques, such as diffusion-weighted imaging for epidermoids or the use of MR spectroscopy, can provide valuable information for the differential diagnosis. Intracranial cysts can be subdivided into intraventricular and periventricular cysts, intra-axial cysts and cysts in the external fluid-filled spaces. Associated tumor nodules and the contrast medium behavior of the cyst walls and/or associated soft tissue components as well as the reaction of the adjacent parenchyma are helpful for the diagnosis and assessment. (orig.) [German] Bei der Bildgebung des Neurokraniums sind intrazerebrale Zysten haeufig und haben nicht immer einen Krankheitswert. Das Spektrum der intrazerebralen Zysten ist jedoch sehr gross und beinhaltet neben Inzidentalbefunden auch Entwicklungsstoerungen, Missbildungstumoren, primaere und sekundaere Neoplasien sowie infektiologische Ursachen, wie z. B

  1. Congenital cervical bronchogenic cyst: A case report

    Directory of Open Access Journals (Sweden)

    Kiralj Aleksandar

    2015-01-01

    Full Text Available Introduction. Bronchogenic cysts are rare congenital anomalies of the embryonic foregut. They are caused by abnormal budding of diverticulum of the embryonic foregut between the 26th and 40th day of gestation. Bronchogenic cysts can appear in the mediastinum and pulmonary parenchyma, or at ectopic sites (neck, subcutaneous tissue or abdomen. So far, 70 cases of cervical localization of bronchogenic cysts have been reported. Majority of bronchogenic cysts have been diagnosed in the pediatric population. Bronchogenic cysts of the cervical area are generally asymptomatic and symptoms may occur if cysts become large or in case of infection of the cyst. The diagnosis is made based on clinical findings, radiological examination, but histopathologic findings are essential for establishing the final diagnosis. Treatment of cervical bronchogenic cyst involves surgical excision. Case Outline. Authors present a case of a 6-year-old female patient sent by a pediatrician to a maxillofacial surgeon due to asymptomatic lump on the left side of the neck. The patient had frequent respiratory infections and respiratory obstructions. Magnetic resonance imaging (MRI of the neck was performed and a well-circumscribed cystic formation on the left side of the neck was observed, with paratracheal location. The complete excision of the cyst was made transcervically. Histopathological findings pointed to bronchogenic cyst. Conclusion. Cervical bronchogenic cysts are rare congenital malformations. Considering the location, clinical findings and the radiological features, these cysts resemble other cervical lesions. Surgical treatment is important because it is both therapeutic and diagnostic. Reliable diagnosis of bronchogenic cysts is based on histopathological examination.

  2. Vitamin D deficiency as a risk factor for cystic fibrosis-related diabetes in the Scandinavian Cystic Fibrosis Nutritional Study

    DEFF Research Database (Denmark)

    Pincikova, T; Nilsson, Kristine Kahr; Moen, I E

    2011-01-01

    Many cystic fibrosis patients are vitamin D-insufficient. Cystic fibrosis-related diabetes is a major complication of cystic fibrosis. The literature suggests that vitamin D might possess certain glucose-lowering properties. We aimed to assess the relationship between vitamin D and cystic fibrosis...

  3. Internal irradiation for cystic craniopharyngioma

    International Nuclear Information System (INIS)

    Kobayashi, T.; Kageyama, N.; Ohara, K.

    1981-01-01

    The authors report the results of internal irradiation with labeled chromic phosphate (32P) and gold-198 (198Au) colloid in eight cases of cystic craniopharyngiomas. They used a newly developed dosimetric formula, by which the radiation dose at the cyst wall and at any point far from the radioactive source can be calculated. Ten courses of irradiation in eight patients were carried out by injection of either 32P or 198Au colloid into the cyst through an Ommaya drainage system that had been placed at craniotomy. Follow-up studies ranging from 13 to 156 months revealed that all cysts were effectively treated, with elimination of fluid or collapse of the cyst. This was confirmed by Conray cystography and/or computerized tomography. Not only the dose delivered to the wall but also the thickness of the cyst wall and the location of the cyst are important factors in planning internal irradiation. A safe and adequate dose to the cyst wall could range between 9000 to 30,000 rads for craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to remove radically, recurrent cysts resistant to previous treatment, or multiple cysts. Internal irradiation may also be applicable in other cystic intracranial tumors if dosimetry is calculated accurately

  4. 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

  5. Intralobar bronchopulmonary sequestration in the newborn - a congenital malformation

    International Nuclear Information System (INIS)

    Laurin, S.; Haegerstrand, I.

    1999-01-01

    Background. Intralobar sequestration (ILS) has been suggested to be an acquired lesion. However, we have observed several young infants who had ILS. Objectives. Since this fact seems to indicate a congenital origin, we reviewed our experience. Material and methods. A retrospective review of bronchopulmonary sequestration from the Departments of Radiology and Pathology in Lund between 1964 and 1997. Results. We identified seven infants or young children with a diagnosis of intralobar sequestration. In each patient, the ILS was present before recurrent infection developed. Five had chest X-rays as neonates, one at 3 months and one at 11 months of age. All but one showed an abnormality on their first chest X-ray, consistent with sequestration. Six of the ILS were verified at angiography; all seven were surgically removed. Two of the children with ILS also had congenital cystic adenomatoid malformation (CCAM). Three children had both ILS and scimitar syndrome. Conclusions. The fact that ILS was present in seven newborn and young infants indicates that this lesion is, at least in some patients, a congenital malformation. (orig.)

  6. Acute appendicitis mimicking intestinal obstruction in a patient with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Chun-Han Chen

    2012-10-01

    Full Text Available Cystic fibrosis (CF is an inherited disease of the secretory glands caused by mutations of the cystic fibrosis transmembrane regulator (CFTR gene. The clinical manifestations of CF are repetitive lung infections, biliary cirrhosis, pancreatic abnormalities, and gastrointestinal disorders. We report a 21-year-old Taiwanese man with CF who had abdominal pain for 2 days. The diagnosis of CF had been confirmed by peripheral blood analysis of the CFTR gene 5 years before admission. He presented to the emergency department with nausea, vomiting, abdominal distension, and crampy abdominal pain, which is atypical for acute appendicitis. The physical examination and a series of studies revealed intestinal obstruction, but acute appendicitis could not be ruled out. After conservative treatment, together with empiric antibiotics, the refractory abdominal pain and leukocytosis with a left-shift warranted surgical intervention. A diagnostic laparoscopy revealed a swollen, hyperemic appendix, a severely distended small intestine, and serous ascites. The laparoscopic procedure was converted to a laparotomy for open disimpaction and appendectomy. He was discharged on the eighth postoperative day. The histologic examination of the appendix was consistent with early appendicitis. In conclusion, acute abdominal pain in adult CF patients is often associated with intestinal obstruction syndrome. The presentation of concurrent appendicitis may be indolent and lead not only to diagnostic difficulties, but also a number of therapeutic choices.

  7. Acute appendicitis mimicking intestinal obstruction in a patient with cystic fibrosis.

    Science.gov (United States)

    Chen, Chun-Han; Chang, Cheng-Chih; Yang, Bor-Yau; Lin, Paul Y; Wang, Chia-Siu

    2012-10-01

    Cystic fibrosis (CF) is an inherited disease of the secretory glands caused by mutations of the cystic fibrosis transmembrane regulator (CFTR) gene. The clinical manifestations of CF are repetitive lung infections, biliary cirrhosis, pancreatic abnormalities, and gastrointestinal disorders. We report a 21-year-old Taiwanese man with CF who had abdominal pain for 2 days. The diagnosis of CF had been confirmed by peripheral blood analysis of the CFTR gene 5 years before admission. He presented to the emergency department with nausea, vomiting, abdominal distension, and crampy abdominal pain, which is atypical for acute appendicitis. The physical examination and a series of studies revealed intestinal obstruction, but acute appendicitis could not be ruled out. After conservative treatment, together with empiric antibiotics, the refractory abdominal pain and leukocytosis with a left-shift warranted surgical intervention. A diagnostic laparoscopy revealed a swollen, hyperemic appendix, a severely distended small intestine, and serous ascites. The laparoscopic procedure was converted to a laparotomy for open disimpaction and appendectomy. He was discharged on the eighth postoperative day. The histologic examination of the appendix was consistent with early appendicitis. In conclusion, acute abdominal pain in adult CF patients is often associated with intestinal obstruction syndrome. The presentation of concurrent appendicitis may be indolent and lead not only to diagnostic difficulties, but also a number of therapeutic choices. Copyright © 2012. Published by Elsevier B.V.

  8. Prognostic factors after percutaneous transhepatic biliary drainage

    International Nuclear Information System (INIS)

    Castoldi, M.C.; Cozzi, G.; Severini, A.; Pisani, P.; Ideo, G.; Bellomi, M.

    1991-01-01

    The authors reviewed the clinical charts and the radiographic files of 93 patients with obsructive jaundice -in 86 cases due to neoplasms -treated with PTBD. The test of differences from survival curves was used to identify the clinical parameters predictive of short survival after PTBD. The difference in survival curves was significant relative to serum indirect bilirubin (cut point: 7.6 mg%), to serum cholinesterase (cut point: 1290 mU/ml), to white blood cells counts (cut point: 8600/mm 3 ), to blood urea nitrogen (BUN) levels (cut point: 60 mg%). Because of the market negative prognostic value of high BUN levels, our data seemto indicate that PTBD should not be performed when severe renal insufficiency is present. Other parameters correlated with a short survival after PTBD were the histotype of metastasis (in comparison with the other ones) and in large neoplastic volume (in comparison with a small and medium ones). Through pre-PTBD radiological and laboratory data analysis, a group of patients can be selected in whom the procedure will increase neither well-being nor survival, as plotted against those patients who are likely to benefit from biliary drainage

  9. 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.

  10. [Pancreas and biliary tract: recent developments].

    Science.gov (United States)

    de-Madaria, Enrique

    2014-09-01

    Acute pancreatitis (AP) is a common disease that is associated with significant morbidity and considerable mortality. In this article, developments relating to this disease that were presented in DDW 2014 are reviewed. Pancreatic steatosis could be a cause of recurrent AP. Patients with DM have an increased incidence of AP and pancreatic cancer. The use of anti-TNF drugs in inflammatory bowel disease may protect against the occurrence of AP. The presence of pancreas divisum protects against acute biliary pancreatitis. The PANCODE system for describing local complications of AP has good interobserver agreement, when the new definitions of the revised Atlanta classification are applied. The use of prophylactic antibiotics in early-stage AP predisposes the development of intra-abdominal fungal infections. Fluid sequestration in AP is linked with young age, alcoholism and indicators of systemic inflammatory response syndrome. The most common cause of mortality in AP is early onset of multiple organ failure, not pancreatic necrosis infection. Patients with AP and vitamin D deficiency could benefit from taking vitamin D supplements. Moderate fluid administration in emergencies (500-1000 mL) could be associated with better AP development. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  11. Vitamin A supplementation for cystic fibrosis.

    Science.gov (United States)

    Bonifant, Catherine M; Shevill, Elizabeth; Chang, Anne B

    2014-05-14

    People with cystic fibrosis and pancreatic insufficiency are at risk of fat soluble vitamin deficiency as these vitamins (A, D, E and K) are co-absorbed with fat. Thus, some cystic fibrosis centres routinely administer these vitamins as supplements but the centres vary in their approach of addressing the possible development of deficiencies in these vitamins. Vitamin A deficiency causes predominantly eye and skin problems while supplementation of vitamin A to excessive levels may cause harm to the respiratory and skeletal systems in children. Thus a systematic review on vitamin A supplementation in people with cystic fibrosis would help guide clinical practice. To determine if vitamin A supplementation in children and adults with cystic fibrosis:1. reduces the frequency of vitamin A deficiency disorders;2. improves general and respiratory health;3. increases the frequency of vitamin A toxicity. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 07 April 2014. All randomised or quasi-randomised controlled trials comparing all preparations of oral vitamin A used as a supplement compared to either no supplementation (or placebo) at any dose and for any duration, in children or adults with cystic fibrosis (defined by sweat tests or genetic testing) with and without pancreatic insufficiency. No relevant studies for inclusion were identified in the search. No studies were included in this review. As there were no randomised or quasi-randomised controlled trials identified, we cannot draw any conclusions on the benefits (or otherwise) of regular administration of vitamin A in people with cystic fibrosis. Until further data are available, country or region specific guidelines on the use of

  12. Voice Disorder in Cystic Fibrosis Patients

    Science.gov (United States)

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  13. CYSTIC DEGENERATION OF THE DUODENUM ASSOCIATED WITH CHRONIC PANCREATITIS

    Directory of Open Access Journals (Sweden)

    A. G. Kriger

    2016-01-01

    Full Text Available Inflammatory cystic lesion of the descending part of the duodenum, mainly in the field of small duodenal papilla in chronic pancreatitis (CP is described as “duodenal dystrophy” (DD. The pathogenesis of this condition has not been studied and treatment strategy is not defined.Purpose. Investigation of cystic inflammatory transformation of duodenal wall pathogenesis in patients with CP, described as a DD, and evaluate the clinical ef ficiency of surgical treatment.Material and methods. Eighty two patients with DD were retrospectively included over 12 years. The diagnosis of DD was established by transabdominal ultrasound, CT, MRI and endosonography. Initially, all patients were treated conservatively. 74 patients required surgical treatment subsequently after conservative treatment with a median duration of 2 years. 34 patients underwent pancreaticoduodenectomy (PD, 21 patients underwent duodenum resection, 15 – duodenum preserving pancreatic head resection of (DPPHR. 4 patients underwent palliative operations. Diagnosis of CP and DD was verified by histological study of surgical specimens. Not operated patients (8 are under observation. Long-term results of surgical treatment were evaluated in 47 patients with a median follow-up was 49.9 months.Results. Histological examination resulted that in 69.9% of DD was related with groove pancreatitis, with ectopic pancreatic tissue – in 30.1%. DD was associated with CP in 92.6% of cases. Clinical presentation of DD was not related with etiology and showed typical symptoms of CP: abdominal pain occurred in 98.8% of patients, body weight loss – 61.7%, duodenal obstruction – 35.8%, biliary hypertension – 34.1%. The overall morbidity was 35.1%. Overall postoperative mortality was 1.37% (1 patient. 66% of patients had no clinical symptoms postoperatively, a significantimprovement – 32%, no effect – 2%.Conclusion. The most cases of DD is related with groove pancreatitis, less

  14. CT diagnosis of cystic ovarian lesions

    International Nuclear Information System (INIS)

    Iio, Kazuto; Shinmura, Ryoji; Arima, Naomi; Yamada, Eiichiro; Ohkubo, Koichi; Nagata, Yukihiro

    1985-01-01

    CT was undertaken and CT numbers were measured in 47 patients with cystic ovarian lesions. CT features particularly for chocolate cyst revealed the uniform thickness of the whole cystic wall, findings suggesting adhesion to the surrounding organs, circular or oval shape, and higher CT numbers within the cyst than those in the other cystic ovarian lesions. However, because these features are not always observed in cases of chocolate cyst, one should not rely solely on CT findings in the diagnosis of chocolate cyst. (Namekawa, K.)

  15. Ovarian Mature Cystic Teratoma Containing Multiple Mobile

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Hyun Sun; Yoon, Seong Eon; Lee, Young Hwan; Kim, Hye Won; Yoon, Kwon Ha [Wonkwang University Hospital, Iksan (Korea, Republic of); Park, Seong Hoon [Asan Medical Center, Seoul (Korea, Republic of)

    2006-12-15

    A 48-year-old woman was admitted to our hospital with a palpable mass in her lower abdomen. A left ovarian, cystic mass containing multiple mobile globules was seen on CT and MR images. The outer portion of the globules showed fat components on CT and fat-saturated T1-weighted MR images. Ultrasonography showed multiple echogenic, mobile globules with some sound attenuation and hyper echoic lines and dots within the cystic mass, which corresponded with the presence of lipid globules and hair shafts of ovarian mature cystic teratoma, respectively

  16. Ovarian Mature Cystic Teratoma Containing Multiple Mobile

    International Nuclear Information System (INIS)

    Cho, Hyun Sun; Yoon, Seong Eon; Lee, Young Hwan; Kim, Hye Won; Yoon, Kwon Ha; Park, Seong Hoon

    2006-01-01

    A 48-year-old woman was admitted to our hospital with a palpable mass in her lower abdomen. A left ovarian, cystic mass containing multiple mobile globules was seen on CT and MR images. The outer portion of the globules showed fat components on CT and fat-saturated T1-weighted MR images. Ultrasonography showed multiple echogenic, mobile globules with some sound attenuation and hyper echoic lines and dots within the cystic mass, which corresponded with the presence of lipid globules and hair shafts of ovarian mature cystic teratoma, respectively

  17. Usefulness of a Biliary Manipulation Catheter in Percutaneous Transhepatic Biliary Drainage

    Energy Technology Data Exchange (ETDEWEB)

    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.

  18. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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

  1. 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)

  2. 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

  3. Inhaled mannitol for cystic fibrosis.

    Science.gov (United States)

    Nevitt, Sarah J; Thornton, Judith; Murray, Clare S; Dwyer, Tiffany

    2018-02-09

    Several agents are used to clear secretions from the airways of people with cystic fibrosis. Mannitol increases mucociliary clearance, but its exact mechanism of action is unknown. The dry powder formulation of mannitol may be more convenient and easier to use compared with established agents which require delivery via a nebuliser. Phase III trials of inhaled dry powder mannitol for the treatment of cystic fibrosis have been completed and it is now available in Australia and some countries in Europe. This is an update of a previous review. To assess whether inhaled dry powder mannitol is well tolerated, whether it improves the quality of life and respiratory function in people with cystic fibrosis and which adverse events are associated with the treatment. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic databases, handsearching relevant journals and abstracts from conferences.Date of last search: 28 September 2017. All randomised controlled studies comparing mannitol with placebo, active inhaled comparators (for example, hypertonic saline or dornase alfa) or with no treatment. Authors independently assessed studies for inclusion, carried out data extraction and assessed the risk of bias in included studies. The quality of the evidence was assessed using GRADE. Six studies (reported in 50 publications) were included with a total of 784 participants.Duration of treatment in the included studies ranged from 12 days to six months, with open-label treatment for an additional six months in two of the studies. Five studies compared mannitol with control (a very low dose of mannitol or non-respirable mannitol) and the final study compared mannitol to dornase alfa alone and to mannitol plus dornase alfa. Two large studies had a similar parallel design and provided data for 600 participants, which could be pooled where data for a particular outcome and time point were

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

    Science.gov (United States)

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

    2001-11-01

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

  5. The cost-effectiveness of neonatal screening for Cystic Fibrosis: an analysis of alternative scenarios using a decision model

    Directory of Open Access Journals (Sweden)

    Tu Karen

    2005-08-01

    Full Text Available Abstract Background The use of neonatal screening for cystic fibrosis is widely debated in the United Kingdom and elsewhere, but the evidence available to inform policy is limited. This paper explores the cost-effectiveness of adding screening for cystic fibrosis to an existing routine neonatal screening programme for congenital hypothyroidism and phenylketonuria, under alternative scenarios and assumptions. Methods The study is based on a decision model comparing screening to no screening in terms of a number of outcome measures, including diagnosis of cystic fibrosis, life-time treatment costs, life years and QALYs gained. The setting is a hypothetical UK health region without an existing neonatal screening programme for cystic fibrosis. Results Under initial assumptions, neonatal screening (using an immunoreactive trypsin/DNA two stage screening protocol costs £5,387 per infant diagnosed, or £1.83 per infant screened (1998 costs. Neonatal screening for cystic fibrosis produces an incremental cost-effectiveness of £6,864 per QALY gained, in our base case scenario (an assumed benefit of a 6 month delay in the emergence of symptoms. A difference of 11 months or more in the emergence of symptoms (and mean survival means neonatal screening is both less costly and produces better outcomes than no screening. Conclusion Neonatal screening is expensive as a method of diagnosis. Neonatal screening may be a cost-effective intervention if the hypothesised delays in the onset of symptoms are confirmed. Implementing both antenatal and neonatal screening would undermine potential economic benefits, since a reduction in the birth incidence of cystic fibrosis would reduce the cost-effectiveness of neonatal screening.

  6. Congenital imprinting disorders

    DEFF Research Database (Denmark)

    Eggermann, Thomas; Netchine, Irène; Temple, I Karen

    2015-01-01

    Imprinting disorders (IDs) are a group of eight rare but probably underdiagnosed congenital diseases affecting growth, development and metabolism. They are caused by similar molecular changes affecting regulation, dosage or the genomic sequence of imprinted genes. Each ID is characterised...... by specific clinical features, and, as each appeared to be associated with specific imprinting defects, they have been widely regarded as separate entities. However, they share clinical characteristics and can show overlapping molecular alterations. Nevertheless, IDs are usually studied separately despite...... EUCID.net (European network of congenital imprinting disorders) now aims to promote better clinical care and scientific investigation of imprinting disorders by establishing a concerted multidisciplinary alliance of clinicians, researchers, patients and families. By encompassing all IDs and establishing...

  7. Congenital intestinal lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Popović Dušan Đ.

    2011-01-01

    Full Text Available Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  8. [Congenital intestinal lymphangiectasia].

    Science.gov (United States)

    Popović, Dugan D j; Spuran, Milan; Alempijević, Tamara; Krstić, Miodrag; Djuranović, Srdjan; Kovacević, Nada; Damnjanović, Svetozar; Micev, Marjan

    2011-03-01

    Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortuous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and supportive therapy. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  9. Congenital diaphramatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Fetal Care Center of Cincinnati, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States)

    2012-01-15

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  10. Congenital Hepatic Cyst

    Directory of Open Access Journals (Sweden)

    Aldo Recinos

    2017-04-01

    Full Text Available Congenital hepatic cyst is a rare and nonsymptomatic condition in infants and children. Its incidence is 2.5% in the postnatal life with a much lower incidence in the prenatal period. Incidental finding on antenatal imaging is the most common presentation. We present a case of a newborn in whom fetal ultrasound detected a cyst within the fetal liver. Postnatal imaging revealed a liver cyst in the right lobe of the liver, with no other intrahepatic structure affected. Liver function tests were abnormal, but the patient was asymptomatic. Posterior follow-up imaging showed a minor decrease in size. Management of congenital hepatic cyst is usually conservative, done with periodic ultrasound monitoring. However, surgical treatment is the mainstay of treatment when hydrops, progressive enlargement, hemorrhage, torsion, or compression of adjacent structures occurs. Malignant transformation can occur, but it is extremely rare. Partial or total removal of the cyst is the preferred treatment in neonates with a large lesion.

  11. Congenital diaphramatic hernia

    International Nuclear Information System (INIS)

    Kline-Fath, Beth M.

    2012-01-01

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  12. Characterizing Congenital Amusia

    OpenAIRE

    Stewart, Lauren

    2011-01-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of ...

  13. The heart: Congenital disease

    International Nuclear Information System (INIS)

    Higgins, C.B.

    1987-01-01

    The most important diagnostic requirement in congenital heart disease (CHD) is definition of cardiovascular pathoanatomy. The considerable success in operative correction of even the most complex anomalies in recent years compels ever increasing precision in preoperative demonstration of these anomalies. Early experience with magnetic resonance imaging (MRI) at several institutions indicated that this modality is an effective noninvasive technique for evaluation of CHD. Indeed, MRI seems to have some advantage over other techniques, including angiography, for definitive diagnosis of congenital anomalies of the heart and great arteries and veins. The absence of ionizing radiation and contrast medium in MRI is an additional advantage; the former is particularly important for children, who, up to this time, have frequently been subjected to enormous radiation burdens from multiple cineangiograms during initial diagnosis and follow-up. This chapter describes the MRI appearance of cardiovascular anatomy im the segmental fashion proposed for analysis of complex CHD. Likewise, MRI demonstration of congenital cardiovascular lesions is organized into abnormalities situated at the four segmental cardiovascular levels: great vessels, atria, ventricles, and visceroatrial relationship. The role of MRI in evaluation of complex ventricular anomalies such as single ventricle and thoracic aortic abnormalities is specifically described

  14. Cystic mesothelioma of the peritoneum.

    Science.gov (United States)

    Datta, R V; Paty, P B

    1997-10-01

    A 48-year-old man presented with a 3-month history of weight loss and progressive right lower quadrant abdominal pain. His medical history was notable for appendectomy at age 17. Ultrasonography and computed tomography of the abdomen revealed a 12 cm multicystic mass in the right paracolic space. At laparotomy a large serous cyst was found arising from the lateral wall of the cecum, and four additional small cysts were found on the small bowel mesentery, greater omentum, liver capsule, and right hemi-diaphragm. Complete removal of the tumor was accomplished by right colectomy with extraperitoneal dissection of the large cyst and simple excision of the four smaller cysts. Final pathology with immunohistochemical staining confirmed cystic mesothelioma of the peritoneum. In this report we discuss the diagnostic workup and treatment of this rare disease.

  15. European Cystic Fibrosis Society Standards of Care

    DEFF Research Database (Denmark)

    Stern, Martin; Bertrand, Dominique Pougheon; Bignamini, Elisabetta

    2014-01-01

    Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful ...... to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons.......Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful...... therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference...

  16. Nutrition in Cystic Fibrosis: Macro- and Micronutrients

    NARCIS (Netherlands)

    Oudshoorn, Johanna Hermiena

    2006-01-01

    Cystic fibrosis (CF) is the most common life-threatening autosomal recessive inherited disease in Caucasians, and is characterized by progressive lung disease, pancreatic insufficiency, malnutrition, hepatobiliary disease and elevated sweat electrolyte levels. The increased survival of CF patients

  17. Pregnancy Complicated by Portal Hypertension Secondary to Biliary Atresia

    Directory of Open Access Journals (Sweden)

    O. E. O'Sullivan

    2013-01-01

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

  18. 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.

  19. Radiology of congenital heart disease

    International Nuclear Information System (INIS)

    Amplatz, K.

    1986-01-01

    This is a text on the radiologic diagnosis of congenital heart disease and its clinical manifestations. The main thrust of the book is the logical approach which allows an understanding of the complex theory of congenital heart disease. The atlas gives a concise overview of the entire field of congenital heart disease. Emphasis is placed on the understanding of the pathophysiology and its clinical and radiological consequences. Surgical treatment is included since it provides a different viewpoint of the anatomy

  20. Festival food coma in cystic fibrosis.

    Science.gov (United States)

    Pandit, Chetan; Graham, Christie; Selvadurai, Hiran; Gaskin, Kevin; Cooper, Peter; van Asperen, Peter

    2013-07-01

    Children with cystic fibrosis liver disease and portal hypertension are at risk of developing acute hepatic encephalopathy. Even in the presence of normal synthetic liver function these children may have porto-systemic shunting. We report a case of an adolosecent who had cystic fibrosis liver disease and presented with life threatening hepatinc encephalopathy. This case illustrates that it is necessary to consider an appropriate dietary regimen in adolosecents with liver disease to prevent hepatic decompensation. Copyright © 2012 Wiley Periodicals, Inc.

  1. 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.

  2. Early detection of congenital syphilis

    Directory of Open Access Journals (Sweden)

    Nagalakshmi Chowdhary

    2014-01-01

    Full Text Available Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson′s teeth.

  3. CT findings in skeletal cystic echinococcosis

    Energy Technology Data Exchange (ETDEWEB)

    Tuezuen, M.; Hekimoglu, B. [Social Security Hospital, Ankara (Turkey). Dept. of Radiology

    2002-09-01

    Purpose: To evaluate the CT findings of skeletal cystic echinococcosis. Material and Methods: CT findings of 7 patients with pathologically confirmed skeletal cystic echinococcosis were evaluated. Results: There were 4 men and 3 women, aged 36-75 years. Hydatid cysts were located in the spine (n=2), a rib (n=3), the pelvis and a vertebra (n=1), the pelvis and the left femur (n=1). The size of the lesions varied from 1 cm to 15 cm. CT showed well defined, single or multiple cystic lesions with no contrast enhancement, no calcification, no daughter cysts, and no germinal membrane detachment. The cystic lesion had a honeycomb appearance in 2 cases, there was pathologic fracture in 2 cases, bone expansion in 5 cases, cortical thinning in 6 cases, cortical destruction in 6 cases, bone sclerosis in 1 case, and soft tissue extension in 6 cases. Conclusion: Preoperative differential diagnosis of skeletal cystic lesions should include cystic echinococcosis, especially in endemic areas, since this diagnosis may easily be missed unless kept in mind.

  4. Renal cystic disease: A practical overview

    International Nuclear Information System (INIS)

    Hartman, D.S.

    1987-01-01

    Renal cystic disease includes a group of lesions with extremely diverse clinical, radiographic, and pathologic findings. The recent development of multiple imaging systems to study renal cystic disease has resulted in considerable interest in correlating the images obtained by different modalities with each other and with the underlying gross pathology. A thorough knowledge of the disturbed morphology and natural history of these diseases will lead to a better understanding of their appearance on radiologic imaging. This refresher course correlates disturbed morphology with appearances on diagnostic imaging, urography, US, angiography, CT, and MR imaging. The advantages and limitations of each imaging method are detailed. A practical classification emphasizing differential features is presented. The presentation is divided into two parts. In the first part typical and atypical cystic masses, including acquired cystic disease (from dialysis), Von Hippel-Lindau disease, and the cystic disease of tuberous sclerosis are discussed. In the second part, polycystic kidney disease (dominant and recessive), medullary cystic disease, medullary sponge kidney, multicycle-dysplastic kidney, renal sinus cysts (peripelvic), and pluricystic kidney disease are discussed

  5. Genetics Home Reference: congenital mirror movement disorder

    Science.gov (United States)

    ... Health Conditions Congenital mirror movement disorder Congenital mirror movement disorder Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Congenital mirror movement disorder is a condition in which intentional movements ...

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

    International Nuclear Information System (INIS)

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

    1995-01-01

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

  7. BACTERIOLOGICAL INVESTIGATION ON Fasciola hepatica AND CATTLE BILIARY DUCTS

    Directory of Open Access Journals (Sweden)

    A. Panebianco

    2009-03-01

    Full Text Available The aim of this work was to investigate the occurrence of bacteria in Fasciola hepatica and into cattle biliary ducts containing the parasite. A total of 24 liver and 58 F. hepatica samples were analysed. In all biliary ducts and in 62,06% of parasite Enterobacteriaceae were isolated. The bacterial specie more frequently isolated from parasite were Citrobacter freundii (34%, Proteus mirabilis (18%, Providencia rettgeri (12%, Staphylococcus spp. (18%, Enterobacter spp. (12%. There doesn’t appear to be a correlation between bacterial specie from parasite and ducts. The Authors conclude with some related inspective consideration.

  8. A case of biliary Fascioliasis by Fasciola gigantica in Turkey.

    Science.gov (United States)

    Goral, Vedat; Senturk, Senem; Mete, Omer; Cicek, Mutallib; Ebik, Berat; Kaya, Beşir

    2011-03-01

    A case of Fasciola gigantica-induced biliary obstruction and cholestasis is reported in Turkey. The patient was a 37- year-old woman, and suffered from icterus, ascites, and pain in her right upper abdominal region. A total of 7 living adult flukes were recovered during endoscopic retrograde cholangiopancreatography (ERCP). A single dose of triclabendazole was administered to treat possible remaining worms. She was living in a village of southeast of Anatolia region and had sheeps and cows. She had the history of eating lettuce, mallow, dill, and parsley without washing. This is the first case of fascioliasis which was treated via endoscopic biliary extraction during ERCP in Turkey.

  9. Mechanisms of Biliary Plastic Stent Occlusion and Efforts at Prevention

    Directory of Open Access Journals (Sweden)

    Chang-Il Kwon

    2016-03-01

    Full Text Available Biliary stenting via endoscopic retrograde cholangiopancreatography has greatly improved the quality of patient care over the last 30 years. Plastic stent occlusion limits the life span of such stents. Attempts to improve plastic stent patency duration have mostly failed. Metal stents (self-expandable metal stents [SEMSs] have therefore replaced plastic stents, especially for malignant biliary strictures. SEMS are at least 10 times more expensive than plastic stents. In this focused review, we will discuss basic mechanisms of plastic stent occlusion, along with a systematic summary of previous efforts and related studies to improve stent patency and potential new techniques to overcome existing limitations.

  10. Biliary atresia and cerebellar hypoplasia in polysplenia syndrome

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-09-01

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

  11. Trends in congenital anomalies in Europe from 1980 to 2012

    Science.gov (United States)

    Springett, Anna L.; Greenlees, Ruth; Loane, Maria; Addor, Marie-Claude; Arriola, Larraitz; Barisic, Ingeborg; Bergman, Jorieke E. H.; Csaky-Szunyogh, Melinda; Dias, Carlos; Draper, Elizabeth S.; Garne, Ester; Gatt, Miriam; Khoshnood, Babak; Klungsoyr, Kari; Lynch, Catherine; McDonnell, Robert; Nelen, Vera; Neville, Amanda J.; O'Mahony, Mary; Pierini, Anna; Queisser-Luft, Annette; Randrianaivo, Hanitra; Rankin, Judith; Rissmann, Anke; Kurinczuk, Jennifer; Tucker, David; Verellen-Dumoulin, Christine; Wellesley, Diana; Dolk, Helen

    2018-01-01

    Background Surveillance of congenital anomalies is important to identify potential teratogens. Methods This study analysed the prevalence of 61 congenital anomaly subgroups (excluding chromosomal) in 25 population-based EUROCAT registries (1980–2012). Live births, fetal deaths and terminations of pregnancy for fetal anomaly were analysed with multilevel random-effects Poisson regression models. Results Seventeen anomaly subgroups had statistically significant trends from 2003–2012; 12 increasing and 5 decreasing. Conclusions The annual increasing prevalence of severe congenital heart defects, single ventricle, atrioventricular septal defects and tetralogy of Fallot of 1.4% (95% CI: 0.7% to 2.0%), 4.6% (1.0% to 8.2%), 3.4% (1.3% to 5.5%) and 4.1% (2.4% to 5.7%) respectively may reflect increases in maternal obesity and diabetes (known risk factors). The increased prevalence of cystic adenomatous malformation of the lung [6.5% (3.5% to 9.4%)] and decreased prevalence of limb reduction defects [-2.8% (-4.2% to -1.5%)] are unexplained. For renal dysplasia and maternal infections, increasing trends may be explained by increased screening, and deceases in patent ductus arteriosus at term and increases in craniosynostosis, by improved follow up period after birth and improved diagnosis. For oesophageal atresia, duodenal atresia/stenosis and ano-rectal atresia/stenosis recent changes in prevalence appeared incidental when compared with larger long term fluctuations. For microcephaly and congenital hydronephrosis trends could not be interpreted due to discrepancies in diagnostic criteria. The trends for club foot and syndactyly disappeared once registries with disparate results were excluded. No decrease in neural tube defects was detected, despite efforts at prevention through folic acid supplementation. PMID:29621304

  12. 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.

  13. A case of biliary stones and anastomotic biliary stricture after liver transplant treated with the rendez - vous technique and electrokinetic lithotritor

    Institute of Scientific and Technical Information of China (English)

    Marta Di Pisa; Mario Traina; Roberto Miraglia; Luigi Maruzzelli; Riccardo Volpes; Salvatore Piazza; Angelo Luca; Bruno Gridelli

    2008-01-01

    The paper studies the combined radiologic and endoscopic approach (rendezvous technique) to the treatment of the biliary complications following liver transplant. The "rendez-vous" technique was used with an electrokinetic lithotripter, in the treatment of a biliary anastomotic stricture with multiple biliary stones in a patient who underwent orthotopic liver transplant. In this patient, endoscopic or percutaneous transhepatic management of the biliary complication failed. The combined approach, percutaneous transhepatic and endoscopic treatment (rendez-vous technique) with the use of an electrokinetic lithotritor, was used to solve the biliary stenosis and to remove the stones.Technical success, defined as disappearance of the biliary stenosis and stone removal, was obtained in just one session, which definitively solved the complications.The combined approach of percutaneous transhepatic and endoscopic (rendez-vous technique) treatment, in association with an electrokinetic lithotritor, is a safe and feasible alternative treatment, especially after the failure of endoscopic and/or percutaneous trans-hepatic isolated procedures.

  14. Endoscopic Ultrasound-Guided Biliary Drainage Using Self-Expandable Metal Stent for Malignant Biliary Obstruction

    Directory of Open Access Journals (Sweden)

    Lei Lu

    2017-01-01

    Full Text Available Purpose. Endoscopic ultrasound-guided biliary drainage (EUS-BD has been increasingly reported worldwide. However, studies concerning EUS-BD from Mainland China are sporadic. This study aims to investigate the feasibility, efficacy, and safety of EUS-BD using SEMS in a single center from Mainland China. Methods. Between November 2011 and August 2015, 24 patients underwent EUS-BD using a standardized algorithm. Results. Three patients underwent rendezvous technique (RV, 4 underwent hepaticogastrostomy (HGS, and 17 underwent choledochoduodenostomy (CDS. The technical and clinical success rates were 95.8% (23/24 and 100% (23/23, respectively. Mean procedure time for the CDS group (35.9 ± 5.0 min or HGS group (39.3 ± 5.0 min was significantly shorter than that for the RV group (64.7 ± 9.1 min (P<0.05. Complications (13% included (1 cholangitis and (2 postprocedure hemorrhage. During the follow-up periods (mean 6.4 months, 22 (91.7% patients died of tumor progression with mean stent patency of 5.8 ± 2.2 months. Stent occlusion occurred in 2 (8.7% patients. Conclusion. EUS-BD using SEMS is a feasible, effective, and safe alternative for biliary decompression after failed ERCP. EUS-RV may not be the first-line choice for EUS-BD in a medium volume center. Further evaluation and experience of this method are needed.

  15. A review of congenital lung malformations with a simplified classification system for clinical and research use.

    Science.gov (United States)

    Seear, Michael; Townsend, Jennifer; Hoepker, Amy; Jamieson, Douglas; McFadden, Deborah; Daigneault, Patrick; Glomb, William

    2017-06-01

    Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.

  16. Fitness to Fly Testing in Patients with Congenital Heart and Lung Disease.

    Science.gov (United States)

    Spoorenberg, Mandy E; van den Oord, Marieke H A H; Meeuwsen, Ted; Takken, Tim

    2016-01-01

    During commercial air travel passengers are exposed to a low ambient cabin pressure, comparable to altitudes of 5000 to 8000 ft (1524 to 2438 m). In healthy passengers this causes a fall in partial pressure of oxygen, which results in relative hypoxemia, usually without symptoms. Patients with congenital heart or lung disease may experience more severe hypoxemia during air travel. This systematic review provides an overview of the current literature focusing on whether it is safe for patients with congenital heart or lung disease to fly. The Pubmed database was searched and all studies carried out at an (simulated) altitude of 5000-8000 ft (1524-2438 m) for a short time period (several hours) and related to patients with congenital heart or lung disease were reviewed. Included were 11 studies. These studies examined patients with cystic fibrosis, neonatal (chronic) lung disease and congenital (a)cyanotic heart disease during a hypoxic challenge test, in a hypobaric chamber, during commercial air travel, or in the mountains. Peripheral/arterial saturation, blood gases, lung function, and/or the occurrence of symptoms were listed. Based on the current literature, it can be concluded that air travel is safe for most patients. However, those at risk of hypoxia can benefit from supplemental in-flight oxygen. Therefore, patients with congenital heart and lung disease should be evaluated carefully prior to air travel to select the patients at risk for hypoxia using the current studies and guidelines.

  17. 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.

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

    DEFF Research Database (Denmark)

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

    2015-01-01

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

  19. Infected Congenital Epicardial Cyst Presenting as Acute Abdomen.

    Science.gov (United States)

    Dribin, Timothy; Files, Matthew D; Rudzinski, Erin R; Kaplan, Ron; Stone, Kimberly P

    2016-12-01

    A previously healthy 3-year-old boy presented to the emergency department with abdominal pain, fever, and emesis. Laboratory and radiologic evaluation for causes of acute abdomen were negative; however, review of the abdominal x-ray demonstrated cardiomegaly with the subsequent diagnosis of pericardial cyst by echocardiogram and computed tomography. The patient underwent surgical decompression and attempted removal of the cystic structure revealing that the cyst originated from the epicardium. His abdominal pain and fever resolved postoperatively and he completed a 3-week course of ceftriaxone for treatment of Propionibacterium acnes infected congenital epicardial cyst. Emergency department physicians must maintain a broad differential in patients with symptoms of acute abdomen to prevent complications from serious cardiac or pulmonary diseases that present with symptoms of referred abdominal pain.

  20. Molecular and Genetic Studies of Congenital Myopathies

    Science.gov (United States)

    2018-03-21

    Central Core Disease; Centronuclear Myopathy; Congenital Fiber Type Disproportion; Multiminicore Disease; Myotubular Myopathy; Nemaline Myopathy; Rigid Spine Muscular Dystrophy; Undefined Congenital Myopathy

  1. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... with mental retardation Muscular dystrophy, congenital, Fukuyama type Muscular dystrophy, congenital, with central nervous system involvement Polymicrogyria with muscular dystrophy Related Information How ...

  2. 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.

  3. The diagnostic value of MRI and MRCP in congenital duct cysts and complications

    International Nuclear Information System (INIS)

    Xiao Fang; Huang Suiqiao; Hu Tao

    2009-01-01

    Objective: To investigate the value of MRI and MRCP in diagnosis of congenital duct cysts and their complications. Methods: The MRI and MRCP of 29 cases of congenital duct cyst verified by pathological findings were analyzed retrospectively. Results: In 29 cases of Congenital choledochocele, 10 of which are type lb and 7 were Ic, appearing as the focal choledochal ecstasis, which were connected with the bile duct tree and displayed by MRCP. 10 cases were type IV and 2 are type V, appearing as the multiple cholangiectasis extrahepaticly and/or intrahepaticly and displayed by MRCP as the multiple cystoid with different sizes. There were 7 cases with complication of calculus, 4 in type Ib and 3 in type IV, appearing as single or multiple non-enhanced lesion with clear border. There were 3 cases with complication of tumors, 1 cholangiocarcinoma in type IV and type V respectively, appearing as the enhanced nodus within the wall of dilatant bile duct, 1 gallbladder carcinoma in type lb with multiple liver metastasis. The interruption of distal choledochocele was demonstrated in the complication of biliary atresia in 1 of type V. There was pancreatitis in 1 of type IV. The diagnosis and typing of choledochocele can be accurately made by MRI combined with MRCP, and complications were easily and clearly showed. The accuracy rate for location of choledochocele was 100%. Conclusion: There is an important value for MRI and MRCP examination in diagnosis of congenital bile duct cysts and their complications. (authors)

  4. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  5. Percutaneous transhepatic biliary drainage for hilar cholangiocarcinoma

    International Nuclear Information System (INIS)

    Qian Xiaojun; Jin Wenhui; Dai Dingke; Yu Ping; Gao Kun; Zhai Renyou

    2007-01-01

    Objective: To evaluate the effect of PTBD in treating malignant biliary obstruction caused by hilar cholangiocarcinoma. Methods: We retrospectively analyzed the data of 103 patients(M:62,F:41)with malignant obstructive jaundice caused by hilar cholangiocarcinoma. After taking percutaneous transhepatic cholangiography, metallic stent or plastic external catheter or external-internal catheter for drainage was deployed and then followed up was undertaken with clinical and radiographic evaluation and laboratory. examination. Results: All patients went though PTBD successfully (100%). According to Bismuth classification, all 103 cases consisted of I type(N=30), II type (N=30), III type (N=26) and IV type (N=17). Thirty-nine cases were placed with 47 stents and 64 eases with drainage tubes. 4 cases installed two stems for bilateral drainage, 2 cases installed two stents because of long segmental strictures with stent in stent, 1 case was placed with three stents, and 3 cases installed stent and plastic catheter together. Sixty-four cases received plastic catheters in this series, 35 cases installed two or more catheters for bilateral drainage, 28 cases installed external and internal drainage catheters, 12 eases installed external drainage catheters, and 24 eases installed both of them. There were 17 patients involving incorporative infection before procedure, 13 cases cured after procedure, and 15 new patients got inflammation after procedure. 13 cases showed increase of amylase (from May, 2004), 8 eases had bloody bile drainage and 1 case with pyloric obstruction. Total serum bilirubin reduced from (386 ± 162) μmol/L to (161 ± 117) μmol/L, (P<0.01) short term curative effect was related with the type of hilar cholangiocarcinoma. The survival time was 186 days(median), and 1, 3, 6, 12 month survival rate were 89.9%, 75.3%, 59.6%, 16.9%, respectively. Conclusion: Percutaneous transhepatic bile drainage is a safe and effective palliative therapy of malignant

  6. 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.

  7. 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

  8. 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

  9. Congenital Midureteric Stricture

    Directory of Open Access Journals (Sweden)

    Singh Shalinder

    2001-01-01

    Full Text Available Congenital midureteric obstruction is a rare entity which can be caused by either ureteric valves or strictures. We report our experience with four patients with midureteric obstruction due to stricture over a six-year period. The condition needs to be differentiated from obstruction of the pelviureteric and vesicoureteric junctions. Obstruction can be initially screened by ultrasound and confirmed by a radionuclide scan with furosemide challenge. Retrograde ureteropyelography and/or intravenous urography may be required to define the level of the stricture.

  10. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  11. 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)

  12. Gene therapy in cystic fibrosis.

    Science.gov (United States)

    Flotte, T R; Laube, B L

    2001-09-01

    Theoretically, cystic fibrosis transmembrane conductance regulator (CFTR) gene replacement during the neonatal period can decrease morbidity and mortality from cystic fibrosis (CF). In vivo gene transfers have been accomplished in CF patients. Choice of vector, mode of delivery to airways, translocation of genetic information, and sufficient expression level of the normalized CFTR gene are issues that currently are being addressed in the field. The advantages and limitations of viral vectors are a function of the parent virus. Viral vectors used in this setting include adenovirus (Ad) and adeno-associated virus (AAV). Initial studies with Ad vectors resulted in a vector that was efficient for gene transfer with dose-limiting inflammatory effects due to the large amount of viral protein delivered. The next generation of Ad vectors, with more viral coding sequence deletions, has a longer duration of activity and elicits a lesser degree of cell-mediated immunity in mice. A more recent generation of Ad vectors has no viral genes remaining. Despite these changes, the problem of humoral immunity remains with Ad vectors. A variety of strategies such as vector systems requiring single, or widely spaced, administrations, pharmacologic immunosuppression at administration, creation of a stealth vector, modification of immunogenic epitopes, or tolerance induction are being considered to circumvent humoral immunity. AAV vectors have been studied in animal and human models. They do not appear to induce inflammatory changes over a wide range of doses. The level of CFTR messenger RNA expression is difficult to ascertain with AAV vectors since the small size of the vector relative to the CFTR gene leaves no space for vector-specific sequences on which to base assays to distinguish endogenous from vector-expressed messenger RNA. In general, AAV vectors appear to be safe and have superior duration profiles. Cationic liposomes are lipid-DNA complexes. These vectors generally have been

  13. Congenital Syphilis Masquerading as Leukemia

    OpenAIRE

    Lee, Tiffany; Bell, Stephanie; Scimeme, Jason; Maraqa, Nizar

    2017-01-01

    As of late, the incidence of congenital syphilis in the United States is increasing. Each new case represents a failure of preventing, diagnosing, and treating syphilis in pregnant women. Pediatricians should confirm that all women have received adequate screening for and management of syphilis during pregnancy. Congenital syphilis is easily treatable but may be a diagnostic challenge with high morbidity and mortality.

  14. What Are Congenital Heart Defects?

    Science.gov (United States)

    ... a baby with a congenital heart defect. Family history and genetics Congenital heart disease is not usually passed along ... you or your child to a specialist in genetic testing. Cardiac MRI to diagnose a ... Factors to review family history, smoking, and medicines that increase your risk of ...

  15. 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 ...

  16. Petechiae and vasculitis in asymptomatic primary biliary cirrhosis

    DEFF Research Database (Denmark)

    Diederichsen, H; Sørensen, P G; Mickley, H

    1985-01-01

    Recurrent petechiae of the lower legs and signs of asymptomatic primary biliary cirrhosis have been found in three women. Large immune complexes were identified in the serum of three and cryoglobulin/cryofibrinogen in two. Histological examination of skin biopsies revealed a leukocytoclastic vasc...

  17. 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.

  18. 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.

  19. Biliary sludge and recurrent ketoacidosis: a case report

    Directory of Open Access Journals (Sweden)

    Kalra Sanjay

    2009-12-01

    Full Text Available Abstract A five year old boy, weighing 14 kg with no family history of diabetes, presented in frank diabetic ketoacidosis. He recovered, but continued to have episodes of ketoacidosis. He was diagnosed to have biliary sludge, which recovered with insulin treatment.

  20. Malfunctioning Plastic Biliary Endoprosthesis: Percutaneous Transhepatic Balloon Pulling Technique

    Directory of Open Access Journals (Sweden)

    Umberto G. Rossi

    2013-01-01

    Full Text Available Percutaneous transhepatic removal techniques for malfunctioning plastic biliary endoprosthesis are considered safe and efficient second-line strategies, when endoscopic procedures are not feasible. We describe the percutaneous transhepatic balloon pulling technique in a patient with an unresectable malignant hilar cholangiocarcinoma.

  1. 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)

  2. 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...

  3. 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

  4. [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.

  5. A scanning electron microscopic study of biliary stent materials

    NARCIS (Netherlands)

    van Berkel, A. M.; van Marle, J.; van Veen, H.; Groen, A. K.; Huibregtse, K.

    2000-01-01

    Clogging of biliary stents remains an important problem. In vitro studies have shown less sludge formation in Teflon stents. Recently, clinical studies with Teflon stents have produced contradictory results. The aim of this study was to investigate whether the surface properties of the

  6. Hepaticoduodenostomy as a technique for biliary anastomosis in ...

    African Journals Online (AJOL)

    Objective The aim of this study was to investigate the efficacy and complications of hepaticoduodenostomy in the treatment of choledochal cyst in children. Summary background data The conventional treatment of choledochal cyst includes Roux-en-Y hepaticojejunostomy for biliary reconstruction. This procedure, however ...

  7. Hepaticoduodenostomy as a technique for biliary anastomosis in ...

    African Journals Online (AJOL)

    Hepaticoduodenostomy as a technique for biliary anastomosis in children with choledochal cyst: ... anastomotic technique in cases of choledochal cyst in children. Ann Pediatr Surg 13:78–80 c 2017 .... versus hepaticojejunostomy after resection of choledochal cyst: a systematic review and meta-analysis. J Pediatr Surg ...

  8. 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

  9. Percutaneous transhepatic biliary drainage: analysis of 175 cases

    Energy Technology Data Exchange (ETDEWEB)

    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.

  10. 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

  11. Malignant biliary obstruction: percutaneous use of self-expandable stents

    NARCIS (Netherlands)

    Laméris, J. S.; Stoker, J.; Nijs, H. G.; Zonderland, H. M.; Terpstra, O. T.; van Blankenstein, M.; Schütte, H. E.

    1991-01-01

    A total of 83 self-expandable metallic stents were placed percutaneously in 69 patients for palliation of malignant biliary obstruction. Stent diameter was 1 cm; length, 3.5-10.5 cm. Of the 41 patients with common bile duct obstruction, 27 died 0.2-12 months (median, 3.2 months) after stent

  12. Biliary System Architecture: Experimental Models and Visualization Techniques

    Czech Academy of Sciences Publication Activity Database

    Sarnová, Lenka; Gregor, Martin

    2017-01-01

    Roč. 66, č. 3 (2017), s. 383-390 ISSN 0862-8408 R&D Projects: GA MŠk(CZ) LQ1604; GA ČR GA15-23858S Institutional support: RVO:68378050 Keywords : Biliary system * Mouse model * Cholestasis * Visualisation * Morphology Subject RIV: EB - Genetics ; Molecular Biology OBOR OECD: Cell biology Impact factor: 1.461, year: 2016

  13. Primary biliary cirrhosis following lactobacillus vaccination for recurrent vaginitis

    NARCIS (Netherlands)

    Bogdanos, Dimitrios; Pusl, Thomas; Rust, Christian; Vergani, Diego; Beuers, Ulrich

    2008-01-01

    Background/Aims:Antimitochondrial antibodies directed against the E2 subunit of the pyruvate dehydrogenase complex, PDC-E2, and other mitochondrial 2-oxoacid dehydrogenases (AMA-M2) are the hallmark for diagnosis (of primary biliary cirrhosis (PBC). AMA-M2 formation as an early step in the

  14. 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...

  15. 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.

  16. Radioanatomy and physiology of liver and biliary tract

    International Nuclear Information System (INIS)

    Antochanu, V.A.

    1988-01-01

    Peculiarities of the structure and functioning of liver and biliary tract are considered. Comparison with anatomy and physiology of these healthy organs is carried out. The know ledge of radioanatomy of the given organs is shown to be the necessary condition of right interpretation of roentgenological data

  17. 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

  18. 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.

  19. [Neonatal tumours and congenital malformations].

    Science.gov (United States)

    Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

    2008-06-01

    The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data

  20. Congenital nystagmus and negative electroretinography

    Directory of Open Access Journals (Sweden)

    Roussi M

    2011-04-01

    Full Text Available Mirella Roussi, Hélène Dalens, Jean Jacques Marcellier, Franck BacinDepartment of Ophthalmology, Clermont-Ferrand University, Clermont-Ferrand, FranceAbstract: Congenital nystagmus is a pathologic oculomotor state appearing at about three to four months of age. The precise diagnosis requires detailed clinical examination and electrophysiological findings. This case report presents two male patients with congenital nystagmus examined longitudinally from the age of six months until 17-18 years of age. Clinical and electrophysiological protocols were detailed. The first results showed electronegative electroretinography in the two cases and examination combined with electroretinographic findings helped us to make the diagnosis of Congenital Night Stationary Blindness (CSNB. This diagnosis was confirmed by genetic studies. CSNB is interesting to study because through electrophysiological findings, it enables a better understanding of the physiology of neural transmission in the outer part of the retina.Keywords: Congenital nystagmus, negative electroretinography, congenital night stationary blindness