The classification of choledochoceles as a type of choledochal cyst stems from the 1959 article by Alonso-Lej and colleagues describing 94 choledochal cysts, only 4 of which were choledochoceles. Even then, Alonso-Lej questioned the propriety of including the choledochocele, stating it was unclear "as to whether or not it originates from the same etiologic factors [as other choledochal cysts]". In 1971, Trout and Longmire also questioned the validity of classifying choledochoceles as choledochal cysts, noting the anatomic position article and variant mucosa of the choledochocele. Wearn and Wiot, in an article titled "Choledochocele: not a form of choledochal cyst", cite the differences in clinical presentation, demographics, and histology as reasons why choledochoceles represent separate entities from choledochal cysts. Over the ensuing decades, numerous investigators have questioned the legitimacy of classifying choledochoceles as choledochal cysts. In our recent series (the only one to our knowledge directly comparing patients with choledochocele and other [type I, II, IV, and V] choledochal cysts), patients with choledochoceles differed from patients with choledochal cysts in their age, gender, presenting symptoms, history of previous cholecystectomy, pancreatobiliary ductal anatomy, management, and most importantly, propensity to developing biliary malignancy. Based on the available cases of choledochoceles found in the literature, combined with the recent series from our institution, we conclude that choledochoceles seem to be distinct entities from choledochal cysts.

We report the case of a 63-year-old woman who presented with the rare finding of a distal choledochocele in a pancreas divisum with recurrent abdominal pain and episodes of pancreatitis. She underwent successful resection with choledochectomy, papillectomy and reconstruction with a hepatico-jejunostomy and reinsertion of the uncinate pancreatic duct into the same jejunal loop. Comparable literature findings are discussed with regard to the presented case.

MRCP is rapidly replacing diagnostic ERCP in various pancreaticobiliary diseases. This study was designed to evaluate the accuracy of MRCP in detecting and determining the extent of choledochal cysts, as well as associated ductal anomalies or lesions.

The study design was an 8-year retrospective survey conducted at a tertiary referral center, Asan Medical Center (University of Ulsan College of Medicine, Seoul, Korea). There were 72 patients with choledochal cysts. All patients underwent both MRCP and ERCP. MRCP findings were compared with those of ERCP as the criterion standard.

The overall detection rate of MRCP for choledochal cysts was 96% (69/72). The sensitivity, the specificity, the positive predictive value, and the negative predictive value of MRCP for classifying choledochal cysts according to Todani's classification were 81%, 90%, 86%, and 86% in type I, respectively; 73%, 100%, 100, and 95% in type III, respectively; 83%, 90%, 80%, and 91% in type IVa, respectively; 100%, 100%, 100%, and 100% in type IVb, respectively; and 100%, 100%, 100%, and 100% in type V, respectively. The sensitivity, the specificity, and the accuracy of MRCP for detecting ductal anomalies were 83%, 90%, and 86%, respectively. The detection rate of MRCP for concurrent cholangiocarcinoma and choledocholithiasis was 87% (13/15) and 100% (8/8), respectively.

MRCP showed overall good accuracy in the detection and the classification of choledochal cysts and revealed associated cholangiocarcinoma and choledocholithiasis with excellent accuracy. MRCP, therefore, may supersede the diagnostic role of ERCP for the patients with choledochal cysts. However, MRCP showed limited capacity to detect minor ductal anomalies or small choledochocele.

A retrospective study of 11 cases of choledochal cyst over a period of 10 years is presented. There were 8 females and 3 males in a ratio of 2.67:1. The classical triad of jaundice, mass and abdominal pain was seen in none of our patients. Ultrasonography was diagnostic in 9 out of 11 (80%) patients. Surgical procedures performed were, complete excision of the cyst with Roux en Y hepaticojejunostomy (nine cases), internal drainage of the cyst (one case) and excision of the extrahepatic cyst with Roux en Y hepaticojejunostomy in one case of Type IV choledochal cyst. One patient died in the immediate postoperative period. Others have remained well upto 10 years follow-up. We recommend total cyst excision with hepaticojejunostomy as the treatment of choice.

Choledochal cyst in an uncommon congenital anomaly with classic presentation triad of abdominal pain, jaundice and right upper abdominal mass. Presentation due to biliary peritonitis following cyst rupture is extremely rare. One such case which was successfully treated is being reported.

Twelve infants operated upon for choledochal cyst (CC) are reviewed with emphasis on the operative technique of biliary tract reconstruction, incidence of cholangitis, postoperative hypergastrinemia, biliary excretion, and upper gastrointestinal (GI) motility in a follow-up of 24 to 35 months. In 7 patients biliary reconstruction was performed with a Roux-en-Y hepaticojejunostomy (HJ), and in 5 with a hepaticoantrostomy (HAST). In the HJ group 4 patients had recurrent episodes of cholangitis and intermittent diarrhea and serum gastrin levels were significantly elevated in 5. Hepatobiliary scintigraphy showed unobstructed excretion of labelled bile through bile ducts into the Roux-en-Y loop, but with significantly delayed emptying of bile into the distal jejunum in all patients. Gastric emptying and upper intestinal passage were normal. In the HAST group no episode of cholangitis occurred and serum gastrin levels were within the normal range. Scintigraphically, hepatobiliary excretion, and duodenojejunal passage of labelled bile was normal, except in 1 patient who developed a postoperative stenosis of the left hepatic duct. Upper GI contrast studies demonstrated normal gastric emptying without reflux into the biliary system. These results suggest that biliary reconstruction with HAST can be performed safely with a low incidence of complications HAST offers a more physiologic method of biliary reconstruction after resection of a CC that allows bile to drain directly into the duodenum.

In the long-term follow-up of patients with choledochal cyst, postoperative ascending cholangitis and/or stone formation in the intrahepatic bile ducts (IHBD) owing to anastomotic stricture present serious problems. To prevent the formation of anastomotic strictures, some surgeons recently have performed hepaticoenterostomy at the hepatic hilum, with a wide stoma, in all patients with choledochal cyst. The authors of the present study review the surgical procedures performed on a total of 180 children with choledochal cyst and discuss the treatment of choice, with special reference to the types of hepaticoenterostomy. The medical records and radiographs of all patients treated for choledochal cyst between January 1964 and December 1993 at the authors' institutions were reviewed. A total of 180 patients (mean age at time of surgery, 4.3 years) had follow-up for a mean of 11.1 years; 174 of them had cyst excision and hepaticoenterostomy, and six had cystoenterostomy. Of the 174 patients who underwent cyst excision, 171 had a conventional hepaticoenterostomy; two had an intrahepatic cystoenterostomy, and one had a hepaticoenterostomy at the hepatic hilum. IHBD stones with or without cholangitis developed postoperatively in four (2.3%) of the 171 patients who had conventional hepaticoenterostomy. The age at time hepaticoenterostomy of these four patients was 12, 7, 16, and 6 years. Postoperative IHBD stone formation and cholangitis were not found in 121 patients under 5 years of age. The authors recommend conventional hepaticoenterostomy as the treatment of choice for children with choledochal cyst. Hepaticoenterostomy at the hepatic hilum is indicated in only selected cases.

A cutaneous stoma in the Roux limb of hepaticojejunostomy (hepaticocutaneous jejunostomy) was used for stone extraction in two children who had hepatolithiasis (14.5 and 15.5 years, respectively) after operation for choledochal cysts. In constructing the hepaticocutaneous jejunostomy, a short, straight proximal limb from the skin to the bilioenteric anastomosis is mandatory. It provides a pathway, superior to the T-tube tract, for repeated stone extraction, which can be performed under sedation, thus obviating repeat laparotomies. The stoma allows flexible choledochoscopy, balloon dilatation of intrahepatic duct strictures, and extraction of intrahepatic stones using grasping forceps, baskets, and balloons. The electrohydraulic lithotriptor may be applied to fragment larger stones.

Hospital records of 24 Chinese infants and children (7 males and 17 females) with choledochal cysts were reviewed. The patients were aged 1 day to 17 years. Abdominal pain, palpable abdominal mass and jaundice, either alone or in combination, accounted for most of the initial presentations. Twenty-two patients were classified as type I with surgery, while the other two patients were type V without surgery. Ultrasonography is a rapid and accurate diagnostic method in the initial evaluation. Cyst excision with Roux-en-Y hepaticojejunostomy was the treatment of choice in this series. Chronic cholecystitis with or without inflammation of choledochal cyst was the most common pathological finding. Five patients underwent liver biopsies, showing four biliary cirrhosis and one portal fibrosis. So far, there is no evidence of malignancy in any patient.

The widespread availability of ultrasound imaging has led to more frequent recognition of cystic disease affecting the liver and biliary tract. There is a wide range of possible causes. Cystic disease of infective origin is usually caused by an Echinococcal species, or as the sequel of a treated amoebic or pyogenic abscess. The clinical and radiological features are often then distinctive and will not be dwelt upon in this review, except in respect of their contribution to the differential diagnosis of non-infective disorders. The principal non-infective cysts can be conveniently divided between the simple cyst, the polycystic syndromes (usually with coexistent renal disease), Caroli's syndrome, and choledochal cysts. The overlap between constituent members of these groups, and the association of cystic disease with hepatic fibrosis (especially with congenital hepatic fibrosis) has attracted considerable attention, and it has been suggested that they may all be considered to belong to a hepatobiliary fibrocystic continuum. In addition there are a variety of cystic neoplasms and a miscellany of unusual forms.

A personal follow-up of 52 cases of choledochal cyst managed over a 28-year period has provided a unique opportunity to review the long-term problems and outcome. Eleven patients had internal drainage procedures and at least four have developed cholangitis more than 10 years later, with three requiring reoperation and cyst excision. Of the 41 patients who had cyst excision, 34 were performed personally by the author; 33 have remained completely well, whereas one died subsequently of cholangiocarcinoma. Complete cyst excision can be successfully achieved provided the dissection of the distal part of the cyst is carried out in a clear, bloodless field obtained by working directly on the cyst wall beneath the overlying loose areolar tissue. Partial excision, mucosectomy, and operative cholangiograms have not been found necessary. This study provides conclusive evidence that any anastomosis of cyst wall to the gastrointestinal tract will ultimately result in stricture and cholangitis.

We present 13 patients who were seen at Glasgow hospitals over a 33-year period. Only five (38 per cent) were children. Of the eight adults, two had complications of the cyst at presentation, namely biliary cirrhosis and cholangiocarcinoma respectively. Another adult patient developed a carcinoma in the cyst 9 years after internal drainage. The incidence of malignant change in the series was therefore 15 per cent. Only five patients have remained well after surgery: three had complete excision and two internal drainage. Of the eight other patients, one had complete excision, five had internal drainage, one had partial excision and one had cholecystectomy and (years later) sphincterotomy. We believe that excision of the cyst is the treatment of choice.

Congenital cystic dilation of the biliary tree is rarely considered as cause of cholangitis in the adult patient. Emergency operation in seven adults with unsuspected choledochal cysts resulted in reoperation in all seven. Each patient presented with right upper quadrant pain, a mass, and cholangitis or jaundice. Abdominal ultrasonography incorrectly identified the cyst as a dilated gallbladder in three of the patients. All initial emergency drainage procedures required subsequent modification to cyst excision and Roux-Y reconstruction. From review of the experience presented and the literature to date, we recommend that otherwise stable patients be managed nonsurgically and undergo endoscopic retrograde cholangiopancreatography or percutaneous transhepatic cholangiography to plan primary single-stage excisional operation. Patients requiring emergency decompression should have cholecystostomy or choledochostomy for good control without compromising subsequent operation. At the time of excision, a technical consideration not previously reported is the presence of small daughter cysts in Calot's triangle which must be distinguished from the hepatic bile ducts. The surgical literature has frequently addressed the problems of elective surgery for choledochal cysts; however, emergency complications requiring urgent operative intervention are seldom addressed. We believe emergency intervention should correct the urgent complication without compromising the definitive surgical treatment.