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Of the 187 cases of infantile choledochal cyst treated at our hospitals, we encountered 13 with spontaneous perforation. All cases were under 4 years old. Eight cases were found to have biliary peritonitis and 5 had a sealed perforation. The shape of the extrahepatic bile duct was cystic in 8 and fusiform in 5. The cyst wall around the perforation was filmy and bile was found to be oozing through the thinned wall. Nine perforations were single while 4 cases had multiple perforations. Four of 17 perforations occurred in the posterior part of the cyst wall. Only 1 case of perforation was associated with protein plugs in a common channel, while 7 of the 10 cases of choledochal cyst requiring percutaneous biliary drainage due to signs of raised intrabiliary pressure were found to have protein plugs. We consider that spontaneous perforation of a choledochal cyst is not rare in infancy. The etiology of a perforation must be epithelial irritation of the biliary tract due to refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity due to infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point.

Choledochal cysts are an uncommon anomaly of the biliary system manifested by cystic dilatation of the extra or intrahepatic biliary tree or both. It is most frequently found in Orientals and in females. Endoscopic retrograde cholangiopancreatography is a valuable imaging technique in the diagnosis of choledochal cysts in adults. Additionally, in selected cases, a choledochocele may be effectively managed by endoscopic sphincterotomy. We present clinical and endoscopic findings of six adult patients with choledochal cysts. Clinical symptoms were characterized by abdominal pain, jaundice and cholangitis. Associated hepatobiliary pathologic findings included cholelithiasis, recurrent acute pancreatitis, gallbladder carcinoma, Cystolithiasis, choledocholithiasis, biliary stricture and hepatic abscess.

We report an adult female with a rare giant choledochal cyst. The patient presented following a normal pregnancy with the classical triad of an abdominal mass associated with jaundice and right upper quadrant abdominal pain. The cyst was excised using an intramural technique and biliary reconstruction achieved with a Roux-en-Y hepaticojejunostomy. Our patient has remained well with no evidence of malignancy over a 12 year review period. The aetiology and current management of this condition are discussed.

The clinical features and long-term outcome of 21 children with choledochal cyst treated over a 31-year-period is reviewed. All 7 infants ( < 1-year-old) presented with jaundice. Only 3 older children (14%) presented with the classical triad of pain, jaundice and an abdominal mass. Other forms of presentation included pancreatitis (n = 8), cholangitis (n = 3), biliary peritonitis (n = 2) and biliary cirrhosis (n = 2). An incidental diagnosis of a choledochal cyst was made in 3 patients one each with bilateral ureteroceles, renal hypoplasia and meningitis with hepatitis A infection. An elevated serum amylase (SA: mean = 1005 U/L) and intraoperative bile amylase (BA: mean = 16,902 U/L) was observed in all 8 children with pancreatitis. Complete excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy was the primary operative procedure in 18 patients. The remaining patients underwent cystoduodenostomy (n = 2) and cystojejunostomy (n = 1). Recurrent cholangitis and stricture formation complicated cyst enterostomies. In comparison cyst excision with Roux-en-Y hepaticojejunostomy gave excellent long-term results with minimal complication.

Background and Study Aims: Choledochocele (CDCL) is still regarded as being a rare abnormality, despite the increasing number of case reports published in the last ten years. The aim of this prospective study was to investigate the prevalence rate of CDCL in patients referred for endoscopic retrograde cholangiopancreatography (ERCP), as well as the short-term and long-term complications of endoscopic treatment., Patients and Methods: Over a seven-year period, fifteen symptomatic patients (ten male, five female, age range 47-93 years) were identified as having CDCL out of a total of 1019 ERCP referrals to our unit. The diagnosis of CDCL was made by both duodenoscopy and ERCP., Results: All 15 patients had small (4-25 mm) CDCLs associated with pancreatic (n = 1) or biliary (n = 14) disorders. Ten of the 15 patients (67%) had choledocholithiasis as well. Twelve patients were treated by endoscopic sphincterotomy (ES), and three by surgery. The immediate post-ES complications (2 of 12, 16.7%) were acute cholangitis (n = 1) and acute pancreatitis (n = 1). Eleven (76%) of the 14 patients who had a complete follow-up were symptom-free, with normal liver function tests at a median of 18 months (range 12-97). Two patients reported episodes of acute cholangitis, and one patient died of a misdiagnosed ampullary carcinoma arising in the CDCL., Conclusions: These results suggest that CDCL is probably an overlooked diagnosis in patients with pancreaticobiliary symptoms. It should be readily recognized and treated, preferably by endoscopic sphincterotomy. The endoscopist must be aware that an ampullary carcinoma may develop in a CDCL.

From 1971 to 1990, 18 children were surgically managed for choledochal cysts at two Berlin pediatric surgical units. Eight patients underwent an internal drainage procedure, eight a Roux-en-Y hepaticojejunostomy and one a choledochoplasty. The children were followed-up 2 to 20 years later (median: 8.4 years). Three children died in the early postoperative period, but the immediate postoperative course was uneventful in all other cases. Later, five patients suffered from recurrent gastrointestinal disease, cholangitis and pancreatitis and, in two cases, from progressive liver fibrosis. Four of the five patients with symptoms had undergone internal drainage procedures; one developed cholangitis after a hepaticojejunostomy. In one case of internal drainage, the anastomosis was transformed into a hepaticojejunostomy. No patient had cancer.

During a two years period, four children were operated on because of choledochal cysts. All four patients were girls aged 9, 9, 8 and 6 years at operation. The symptoms at presentation were mainly episodic abdominal pain, mild jaundice and tenderness under the right costal curvature. Ultrasonography of the biliary tract revealed a cystic dilatation of the choledochus. Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the cystic dilatation and, importantly, delineated the junction of the pancreatic duct. The surgical procedure in all four patients consisted of complete excision of the choledochal cyst and extrahepatic biliary tree combined with a Roux-en-Y hepaticojejunostomy. The postoperative course of all patients was uneventful. Ultrasonography of the abdomen should be performed liberally in case of biliary tract symptoms in children.

The resection of a choledochal cyst was performed in two patients (22 and 31 years old, both type Todani IVa). Reconstruction of the biliary-intestinal drainage was performed by interposition of a 15 cm jejunal segment between the liver hilum and duodenum. In both patients, hepatico-jejunostomy could be endoscopically evaluated 5-6 months after the operation, showing patent anastomoses without any signs of reflux or cholangitis.

The radiologic findings in five neonates with choledochal cyst associated with extra-hepatic biliary atresia are described. All five patients (age range, 13-72 days) presented with jaundice and acholic stools. In all four patients who underwent sonographic examination, a cystic structure separate from the gallbladder representing the choledochal cyst was shown. The diagnosis of atresia of the distal common bile duct was made preoperatively in all cases by hepatobiliary scintigraphy. Diagnosis was confirmed by surgical findings and was demonstrated by intraoperative cholangiography in four cases. All patients were successfully treated with surgical intervention within 1 month from the time of diagnosis. Early detection of this rare disorder, which may be distinct from choledochal cyst found in children and adults, is important to prevent fatal complications of biliary obstruction. The combined use of sonography and hepatobiliary scintigraphy can correctly identify this subset of patients with persistent neonatal jaundice and provide valuable information for prompt surgical management.