Extrahepatic Choledochal Cyst Research Articles

Multiple Choledochal Cysts: A Case Report

Background: Choledochal cysts (CC) are congenital dilatations of the biliary tree, characterized by varying degrees of cystic dilatation at various segments of the biliary tract (extrahepatic or intrahepatic), may be detected at any age and in any portion of the bile duct. This study was aimed to present the management of multiple choledochal cyst case.
 Case presentation: A 9 year old boy was brought by his mother to the emergency department at Dr. M. Djamil General Hospital Padang with chief complaint abdominal pain that’s getting worse 2 weeks before admission. Abdominal pain first complained by the patient since age of 4, the pain was felt all over stomach area, not localized, not radiated, not corelated with meal time, colicky, relieved with paracetamol. USG results: Choledochal cyst, Helicobacter pylori infection, elevated liver enzymes due to suspect suppression of choledochal cyst. Abdominal CT-Scan with contrast results: Intrahepatic and extrahepatic choledochal cyst (type IV A).
 Conclusion: Patients was diagnosed with multiple choledochal cysts, diffuse peritonitis, Helicobacter pylori infection, and familial short stature.

Transabdominal Ultrasonography in Symptomatic Choledocholithiasis - Usefulness in Settings with Limited Resources.

Objective:Ultrasonography remains the initial imaging modality in the management of biliary disease. This study is designed to evaluate the accuracy of transabdominal ultrasonography in diagnosing biliary pathology in patients with choledocholithiasis.Methods:This was a retrospective study of a continuous sample of patients over a period of 3 years ending in January 2016; these patients were referred for endoscopic management of choledocholithiasis to a tertiary care hospital in Colombo, Sri Lanka. Ultrasound reporting was carried out by different consultant radiologists at both the index and the referring hospitals. The findings of endoscopic retrograde cholangiograms were compared with the ultrasound scan (USS) results.Results:A total of 247 patients were included in the study. USS was 97.4% accurate in detecting intrahepatic duct dilatation (IHDD). Stone counts and the location of stone(s) in the USSs correlated strongly with the number of stones delivered during endoscopic removal and their location in cholangiograms (P < 0.001). The difference in mean diameter of the common bile duct (CBD) of patients with choledochal cysts (CCs) (18.57 mm) and of patients without them (12.39 mm) is statistically significant (P < 0.001). At 14.5 mm, the negative predictive value for a CC is 99.02%.Conclusion:Ultrasonography is a reliable tool in predicting IHDD, stone count, and the location of stones in the biliary tree, particularly in a resource-poor setting. A CBD diameter of 14.5 mm in transabdominal ultrasound scan can be used as a cutoff for predicting extrahepatic CC.

Planned hepatectomy for the"central type"intrahepatic and extrahepatic choledochal cysts

Objective To retrospectively study the clinical value and the advantages inplanned hepatectomyfor thecentral typeintrahepatic and extrahepatic choledochal cysts. Methods The clinical data of 7 patients with thecentral typeof intrahepatic and extrahepatic choledochal cysts which were treated withplanned hepatectomyfrom January 2014 through April 2017 at the Department of Biliary Tract Surgery of the Eastern Hepatobiliary Surgery Hospital, Second Military Medical University were retrospectively analyzed. Results All the patients completed radical resection of the intrahepatic and extrahepatic choledochal cysts in accordance with theplanned hepatectomy. The operations included 6 patients who were treated with percutaneous transhepatic cholangial drainage (PTCD) and 5 patients with portal vein embolization (PVE) prior to the surgical excision. Combined right liver resection was performed in 6 patients, and combined left liver resection in one patient. All the 7 patients had a history of chronic cholangitis. Liver volume tests demonstrated that the hemiliver volume to be removed (the embolized hemiliver) significantly decreased after PVE, whereas the hemilivers to be persevered were remarkably enlarged. No complication associated with PTCD and PVE occurred. The mean postoperative hospitalization was 12 days. Liver function tests suggested all the patients recovered well. No postoperative complication of bleeding, infection or liver function failure was observed, except in one patient who experienced pleural and abdominal effusion. Conclusions Combined subtotal hepatectomy may increase the risk of complications associated with thecentral typeintrahepatic and extrahepatic choledochal cysts. The surgical strategy in planned hepatectomy can be used effectively to treat thecentral typeof intrahepatic and extrahepatic choledochal cysts, with improved surgical safety, decrease in incidences of postoperative liver function failure and residual choledochal cysts. Key words: Intrahepatic and extrahepatic bile duct cysts; Congenital cystic dilatation of bile duct; Planned hepatectomy; Percutaneous transhepatic cholangial drainage (PTCD); Portal vein embolization (PVE)

Pancreatic heterotropia in wall of extra-hepatic choledochal cysts: A retrospective analysis of thirteen of such cases from north India

IntroductionHeterotopic pancreas (HP) has rarely been identified in the wall of choledochal cyst (CC). MethodsRetrospectively we screened 200 excised specimens of CC received at our Institute over a period of last eight years and looked for presence of HP rests in them. All the specimens were processed in their entirety. ResultHP was identified in the wall of 13 (6.5%) CCs, out of which 11 were Heinrich Type 2, and two were Heinrich Type 1. In half of the cases peribiliary mucous glands were observed intermingled with the HP rests. Features of chronic fibrosing pancreatitis were identified in these rests, with ulceration of overlying cyst lining. ConclusionsHP rests in the wall of CC though rare; their coexistence with peribiliary glands may possibly indicate their common embryonic origin. As a common site of inflammation, HP rest may be one of the common causes of CC.

Type IV-A Choledochal Cyst with Intrahepatic Bile Duct Stricture

Choledochal cysts are rare congenital anomalies of the bile ducts. They consist of cystic dilatations of the extrahepatic biliary tree, intrahepatic radicles, or both. The standard treatment of choledochal cyst is complete excision of cyst and drainage procedure. I report a case of 48-year-old women who was diagnosed with type IV-A choledochal cyst with intrahepatic bile duct stricture and large stones in extrahepatic choledochal cyst. Sixteen years ago, she received a cholecystectomy and Reux-en Y cystojejunostomy due to choledochal cyst. Because of the intrahepatic bile duct stricture I performed a left hepatectomy, complete cyst excision and hepaticojejunostomy. I report this case with a review of the literature.

Hepatobiliary scintigraphy in the diagnosis of extrahepatic choledochal cyst with biliary atresia

Choledochal cyst, a cystic dilatation of the biliary tree, is an uncommon cause of conjugated hyperbilirubinemia and may be associated with biliary atresia in the neonate. We present a 2-month-old infant with neonatal jaundice and an abdominal mass, where hepatobiliary scintigraphy identified a choledochal cyst with extrahepatic biliary atresia.

Solitary liver cysts in children: not always so simple

Aim Liver cysts in children are uncommon. Many are simple and solitary and do not require intervention. However, this series demonstrates a broad range of potential pathologies, some of which are life-threatening. Methods All children referred to our unit during an 8-year period (1998-2005) and found to have a solitary liver cyst were prospectively recorded. Clinical, radiologic, and pathologic features were analyzed. Children with an isolated extrahepatic choledochal cyst and polycystic disease were excluded. Results Twenty-one children with a liver cyst were identified. Two had undergone unsuccessful surgical intervention before referral. There were 11 prenatally detected cysts. Median gestational age at detection was 22 weeks (19-35 weeks); only 1 was specifically characterized as a liver cyst prenatally. Six of these required surgery: 2 large simple cysts, 2 intrahepatic choledochal cysts, 1 giant complex biliary cyst causing respiratory distress, and 1 ciliated hepatic foregut cyst. Of the 5 cysts remaining under ultrasound surveillance, 4 decreased in size or resolved. In 10 children presenting between birth and 15.8 years, a liver cyst was diagnosed postnatally: 3 huge cystic mesenchymal hamartomas, 1 type V choledochal cyst, 1 hydatid cyst, and 5 simple cysts. Four of these required surgical resection. Simple cysts tended to be small and could be distinguished from other pathologies using a combination of imaging techniques (ultrasound, magnetic resonance imaging/magnetic resonance cholangiopancreatography [MRCP], radionuclide scan). Only 2 of 12 children with “simple” cysts required surgery for symptoms. However, a wide range of other cyst pathologies were found in 9 children, and although none was malignant, some were life-threatening and 7 required resection. Conclusions Simple solitary nonparasitic liver cysts rarely cause symptoms or require surgery, but the pediatric surgeon should be aware of the wide range of other types of liver cyst in children to ensure appropriate treatment.

Spontaneous Rupture of Extrahepatic Choledochal Cyst: Two Pediatric Cases and Literature Review

Spontaneous rupture of extrahepatic choledochal cysts is very rare. Neonatal cases generally present with biliary ascites, and older children with acute abdomen. Although the cause is unclear, accumulation of protein plugs in an anomalous pancreaticobiliary junction, irritation of the cyst wall due to reflux of pancreatic juice, and weakness due to a developmental error such as common channel syndrome have all been suggested to play contributing roles. The treatment of choice is complete excision of the cyst and hepatico-jejunostomy. In newborns who are in a poor condition, the primary treatment should be simple peritoneal drainage.

A genetic screen in zebrafish identifies the mutantsvps18, nf2andfoie grasas models of liver disease

Hepatomegaly is a sign of many liver disorders. To identify zebrafish mutants to serve as models for hepatic pathologies, we screened for hepatomegaly at day 5 of embryogenesis in 297 zebrafish lines bearing mutations in genes that are essential for embryonic development. Seven mutants were identified, and three have phenotypes resembling different liver diseases. Mutation of the class C vacuolar protein sorting gene vps18 results in hepatomegaly associated with large, vesicle-filled hepatocytes, which we attribute to the failure of endosomal-lysosomal trafficking. Additionally, these mutants develop defects in the bile canaliculi and have marked biliary paucity, suggesting that vps18 also functions to traffic vesicles to the hepatocyte apical membrane and may play a role in the development of the intrahepatic biliary tree. Similar findings have been reported for individuals with arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome, which is due to mutation of another class C vps gene. A second mutant, resulting from disruption of the tumor suppressor gene nf2, develops extrahepatic choledochal cysts in the common bile duct, suggesting that this gene regulates division of biliary cells during development and that nf2 may play a role in the hyperplastic tendencies observed in biliary cells in individuals with choledochal cysts. The third mutant is in the novel gene foie gras, which develops large, lipid-filled hepatocytes, resembling those in individuals with fatty liver disease. These mutants illustrate the utility of zebrafish as a model for studying liver development and disease, and provide valuable tools for investigating the molecular pathogenesis of congenital biliary disorders and fatty liver disease.

Experience of Congenital Choledochal Cyst in Adults: Treatment, Surgical Procedures and Clinical Outcome in the Second Affiliated Hospital of Harbin Medical University

This study was undertaken to analyze and evaluate the diagnosis and principal treatment methods for congenital choledochal cyst, focusing on various surgical procedures and clinical outcome. A comprehensive, retrospective study was conducted on 72 adult patients who presented with choledochal cyst from 1985 to 2002. Surgical procedures were cyst excision with hepaticojejunostomy in 25 cases for type I or type IV-B, extrahepatic cyst excision with hepaticojejunostomy in 8 cases for type IV-A, extrahepatic cyst excision with modified hepaticojejunostomy in 2 cases for type IV-B, non-cyst excision with or without hepaticojejunostomy in 27 cases for types I, II, IV-A, IV-B. The early postoperative morbidity and mortality rate were 16.1% (9/62) and 6.5% (4/62) respectively, and the complication rate related to surgical procedure was 30.6% (19/62). The incidence of cholangiocarcinoma with non-cyst excision or non-operated congenital choledochal cyst was 10.8% (4/37). One patient died of primary hepatocellular carcinoma after cyst excision with hepatojejunostomy. In conclusion, our results showed that complete exci-sion of choledochal cyst for types I, II, and IV-B and complete excision of extra-hepatic choledochal cyst from the hepatic hilum in type IV-A with hepaticojejunostomy or modified hepaticojejunostomy are the treatment of choice for choledochal cyst in adult patients.

Successful use of an internal biliary stent in Caroli's disease

Congenital dilatation of the intrahepatic bile ducts is a rare disorder first described by Caroli et al. in 1958.1 In the majority of cases, it is associated with other abnormalities such as congenital hepatic fibrosis,2 renal tubular ectasia,3 or extrahepatic choledochal cyst.4 In about 15 of cases, the abnormality is limited to the intrahepatic bile ducts.5 The long-term management of these patients is controversial, but most frequently involves treatment of recurrent episodes of cholangitis with eventual surgical intervention, including partial resection or hepatic transplantation.