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Previous research has confirmed that patients with choledochal cyst have an elevated risk of cholangiocarcinoma and gallbladder carcinoma. Current data suggest a risk of malignancy of 6 to 30% in adults with choledochal cyst. Malignancy has also occasionally been identified in children and adolescents. Multiple factors, including the age of the patient, cyst type, histological findings, and localization, have an impact on the prognosis. Information on long-term outcomes after cyst excision is limited. However, recent data suggest a lifelong elevated risk of up to 4% of cancer development following operation. This paper presents a review of the literature on cancer in patients with choledochal cyst before and after excision. A postoperative follow-up concept that consists of annual controls of CA19-9 and abdominal ultrasound is introduced., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background/aims: Choledochal cysts are rare congenital anomalies of the pancreaticobiliary system, whose etiology remains unknown. We aimed to review patients with choledochal cysts and to compare our results with current literature., Methodology: Twenty-three patients diagnosed as having choledochal cysts between January 2004 and July 2010 were evaluated retrospectively., Results: Thirteen patients had type I (56.5%), 3 patients type II (13%), 3 patients type III (13%), 1 patient type IV-A (8.3%) and the remaining 3 patients had type V (13%) choledochal cysts. All patients with type I cysts underwent cyst excision with Roux-en-Y hepaticojejunostomy. Two patients with type II cysts underwent cyst excision with choledochoduodenostomy, whereas cyst excision with T-tube drainage was applied to the other. Endoscopic unroofing was performed type III cysts. The patient with type IV-A cyst was not eligible for surgery due to low cardiopulmonary performance status but ERCP was applied successfully more than 3 times for the extraction of the stones which fell from the intrahepatic ducts into the common bile duct. Patients with Type V cysts underwent left hepatectomy, choledocoduodenostomy and cadaveric liver transplantation, respectively. Wound infection developed in 5 patients and anastomotic leakage occurred in 3; one died from sepsis., Conclusions: Choledochal cysts are rare congenital malformations. Although treatment varies depending on the type of the cysts, complete excision of the cysts should be performed if possible.

Background/aims: Congenital choledochal cyst is almost always associated with pancreaticobiliary maljunction and is sometimes associated with biliary carcinoma. This study aimed to investigate the clinicopathological features and surgical treatment of choledochal cyst associated with carcinoma arising in the cyst wall., Methodology: Relationship of the clinicopathological findings and surgical treatment of 8 patients with histologically proven carcinoma that had developed in the choledochal cyst wall were examined to determine mode of tumor spread, multicentric tumor origins, and coincidence with other neoplastic lesions., Results: Papillary adenocarcinoma (n = 5) had different clinicopathological features than tubular adenocarcinoma (n = 3). Radiologically, papillary adenocarcinoma presented as an eccentrically located polypoid mass in the cyst, while with tubular adenocarcinoma, there was evidence of bile duct stenosis with irregular thickening of the bile duct wall. Papillary adenocarcinoma was associated with extensive superficial spread (n = 1), synchronous (n = 2), or metachronous (n = 2) multicentric tumors. Pancreatoduodenectomy with (n = 1) or without (n = 1) hepatic lobectomy, and repeated resection by hepatic lobectomy (n = 2) were performed for these circumstances of tumors. Extensive or repeated resections allowed 3 patients to live longer than 2 years. Tubular adenocarcinoma was associated with scirrhously infiltrative spread and a poor prognosis., Conclusions: Papillary adenocarcinoma frequently occurs in the choledochal cyst wall. Since aggressive resection offers survival benefits in papillary adenocarcinoma arising in the choledochal cyst wall, the presence of superficial spread and multicentric tumors should be identified and taken into account when planning surgery. Patients require close monitoring so that recurrent carcinoma of the remnant bile duct can be identified early.

Methods: The study population was divided into 4 groups: 12 infants with choledochal cyst, aged 4 m to 12 m, were classified as the infant choledochal cyst (ICC) group; 36 children, aged 1 y to 14 y, were classified as the children with choledochal cyst (CCC) group; while 18 patients, aged 2 m to 5 m, with biliary atresia (BA) were included as positive controls; and 14 infants, aged 1 d to 3 y, who died from non-liver diseases served as negative controls (CON). Liver specimens were examined using H&E sections to score fibrosis by means of Ohkuma's classification, and immunohistochemical sections were evaluated by counting the cells positive for cytokeratin (CK) and human leukocyte antigen-DR (HLA-DR) to discover the pathogenic factors of fibrosis., Results: Most ICC patients had clinical biliary obstruction. The liver fibrosis score was highest in the BA group (2.9 +/- 0.7). The fibrosis score in the ICC group was higher (2.5 +/- 0.9) than that of the CCC (1.5 +/- 1.2; p < 0.05) and of the CON (0.1 +/- 0.4; p < 0.01) groups. The densities of CK-positive cells were 164 +/- 80/HP, 253 +/- 165/HP, 70 +/- 57/HP and 23 +/- 12/HP in the BA, ICC, CCC and CON groups, respectively, and differed significantly (p < 0.01) with the exception of the ICC vs. the BA group. The densities of HLA-DR positive cells were 130 +/- 72/HP, 98 +/- 54/HP, 96 +/- 50/HP and 36 +/- 13/HP in the portal area in the BA, ICC, CCC and CON groups, respectively. The density was lowest in the CON group (p < 0.01)., Conclusion: In patients with choledochal cyst, liver fibrosis is more common and severe in infants than in children. Obstruction of the bile duct and proliferation of bile duct cells were the main pathogenic factors for fibrosis, while HLA-DR mediating immuno-injury may play a limited role.

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.

Background/aims: Choledochocele is a rare abnormality of cystic or diverticular dilatation of the terminal intramural portion of the common bile duct. Because its anatomic form does not fit the criteria for pancreaticobiliary maljunction, reflux of bile or pancreatic juice has not been discussed in relation to choledochocele. Considering the flow of bile and pancreatic juice, the authors developed a new classification of choledochocele, and investigated associated pancreaticobiliary diseases., Methodology: We reviewed 82 cases of choledochocele, and classified them into three groups on cholangiopancreatographic findings; Type A (n=27): cystic dilatation of the common channel, Type B (n=31): cystic dilatation of the intramural bile duct forming the common channel, Type C (n=24): cystic dilatation of the intramural bile duct opening into the duodenum separately from the pancreatic duct., Results: Biliary carcinoma was associated with 2 cases of Type A and 1 case of Type B choledochocele. Acute pancreatitis occurred in 10 Type A and 6 Type B choledochoceles. Biliary pancreatic reflux was reported in 4 cases of Type A and 1 case of Type B. Marked elevation of amylase in the bile was reported in 5 Type A and 5 Type B choledochoceles., Conclusions: In choledochoceles forming a common channel (Type A and B), two-way regurgitation may occur, and induce acute pancreatitis and biliary carcinoma. This classification of choledochocele is useful for analysis of pathophysiology and determination of therapeutic strategy.

Background/aims: The clinical features of pancreatitis in patients with an anomalous union of the pancreatobiliary duct (AUPBD) are unclear. This study analyzed the clinical features, pathogenic mechanisms, risk factors, and clinical courses after treatment in AUPBD patients., Methodology: The medical records and cholangiopancreatograms of 58 patients with AUPBD and a choledochal cyst diagnosed between 1982 and 2001 were retrospectively reviewed., Results: Sixteen (27.6%) out of the 58 AUPBD patients experienced pancreatitis. The incidence of pancreatitis was significantly higher in those patients with a long (>21mm) and wide (>5mm) common channel, a wide diameter of the proximal pancreatic duct (>2.5mm), the presence of a filling defect in the common channel, and the presence of a pancreatic duct anomaly (p<0.05). A bile duct diversion from the pancreatic duct by a choledochal cyst excision prevented the recurrence of pancreatitis in most cases. A duodenopancreatectomy was required in some cases., Conclusions: The morphological characteristics of the common channel and pancreatic duct contribute to the development pancreatitis in AUPBD patients and bile reflux into the pancreatic duct might be the main mechanism of pancreatitis in these patients.

Purpose: To investigate the changes of pancreatic isoamylases in the serum of children with choledochal cyst., Methods: Serum and bile from 20 children with choledochal cyst were studied for total amylase activity and isoamylases. Using electrophoresis, the assay of pancreatic isoamylases was expressed by peak appearance rate (PAR) and ratio of isoenzyme activity (RIA). Serum from 20 healthy children and 12 adults with acute pancreatitis served as a comparison., Results: The total amylase activity in the serum of children with choledochal cyst was slightly higher than that of healthy controls, but dramatically lower than that of acute pancreatitis. The serum of children with choledochal cyst showed 5 pancreatic peaks (PAR: P1, 100 %; P2, 100 %; P3, 100 %; P4, 75 %; P5, 45 %) without any major peak. In healthy controls, only P1 and P2 were present (PAR: P1, 40 %; P2, 100 %) with the latter being a major peak (RIA: 81.8 +/- 25.3 %). These results in children with choledochal cyst differed from those of patients with acute pancreatitis who presented with the less common P4 and no P5 (PAR: P1, 100 %; P2, 100 %; P3, 91.6 %; P4, 8.3 %). The pattern of pancreatic isoenzymes in bile was similar to that in the serum from patients with choledochal cyst. These abnormal pancreatic isoamylases gradually disappeared after operation., Conclusions: Abnormal pancreatic isoamylases, especially P4 and P5, are found in the serum of patients with choledochal cyst, which would point to a reflux of pancreatic juice into the biliary tract.

Background/aims: Malignancy in choledochal cysts is a rare condition. This study presents our experience with this condition, with emphasis on the clinical presentation, management and outcome., Methodology: Subjects included 80 adults with choledochal cysts treated from January 1979 to December 1995. Of these patients, eight were found to have malignancy in the cyst and formed the basis of this study., Results: Four patients had synchronous and four had metachronous carcinoma lesions arising in the choledochal cyst. The clinical presentations were: biliary tract infection in 5 patients, gastric outlet obstruction in two and right upper quadrant pain and body weight loss in one. Operations for bile duct malignancy included total excision in two patients, choledochotomy with T-tube drainage in two patients, gastrojejunostomy in two patients, percutaneous transhepatic biliary drainage and gastrojejunostomy in one patient and metastatic lymph node biopsy only in one. One patient died due to septic shock within 30 days of operation (operative mortality). Postoperative survival time ranged from 4 to 9 months with a mean of 6.2 months. One of these patients was still alive at the time of writing (7 months)., Conclusions: The frequency of malignancy in the choledochal cysts was 10% in our series. Malignancy in the choledochal cyst should be highly suspected in patients with cholangitis symptoms, body weight loss and anemia. Prognosis in this disease entity is poor.

The case of a 16-year-old male with expansion of the gallbladder and dilatation of the common bile duct is reported. Ultrasonography and computed tomography imaging showed expansion of the gallbladder and eminent cystic dilatation in the common bile duct and the left intrahepatic bile duct. Endoscopic retrograde cholangiopancreatography indicated expansion-like beads of the bilateral hepatic ductus and the left intrahepatic bile duct, including anomalies of the pancreaticobiliary ductal junction. Because relative stenosis of the membranous diaphragm was revealed in the porta hepatis, we diagnosed this case as a type IV-A choledochal cyst, using Todani's classification. Intraoperative cholangiography and cholangiofiberscopy showed a pinhole stricture and re-expansion of the tip of the left intrahepatic bile duct. As the narrow segment could not be expanded though we put proper pressure there, left hepatectomy was performed as a preventive measure in addition to extended biliary tract excision and cholangiojejunostomy. Hepatectomy seems to be an appropriate choice in a case of intrahepatic stenosis to help increase the patient's postoperative quality of life.

Anomalous pancreaticobiliary junction often leads to biliary tract carcinoma but only rarely to pancreatic carcinoma. We report three cases of pancreatic carcinoma associated with anomalous pancreaticobiliary junction. All three were female with a mean age of 68 years. Carcinomas were located in the pancreatic head (n = 2) or body (n = 1). None had choledochal cyst and one had experienced recurrent acute pancreatitis. All carcinomas were at an advanced stage with a poor prognosis. No unique imaging or histologic findings of the carcinomas could be identified. Attention should be paid to the possibility of pancreatic carcinoma in patients with anomalous pancreaticobiliary junction, particularly in aged patients. Early diagnosis and treatment of anomalous pancreaticobiliary junction may prevent development of pancreatic carcinoma.

We here report a 53-year-old woman who had undergone resection of a choledochal cyst and hepaticojejunostomy three years before. She was readmitted because of intermittent fever, and abdominal computed tomography revealed a 4-cm tumor in the head of the pancreas. We performed pancreatoduodenectomy, and examination of the resected specimen showed well-differentiated papillary adenocarcinoma. Only 5 cases of carcinoma occurring after the resection of a choledochal cyst have been reported, and to our knowledge, this is the second case of carcinoma of the head of the pancreas.

Recurring complaints of unknown origin in the upper abdomen during childhood are not frequent. Choledochoceles, or type III choledochal cysts, are rare congenital abnormalities of unclear aetiology. They are rarely considered in the differential diagnosis of upper abdominal symptoms in childhood. Only 130 cases of choledochoceles have been reported in world literature to date. Only 20% of these patients were children or adolescents. The symptoms do not specifically suggest a choledochocele, and are usually attributed to other disorders of the upper intestinal tract, frequently resulting in a delayed diagnosis. We present the case of a 10-year old girl in whom a congenital choledochocele was diagnosed by gastro-intestinal studies and endoscopic retrograde cholangiopancreatography (ERCP) following a 2-years history of recurrent pancreatitis. Various types of choledochoceles are presented in the literature, classified according to morphology and histology. Most authors consider them to be a form of choledochal cyst. Their embryonic development is related to that of congenital duodenal diverticula. Although congenital intraluminal duodenal dilatations are of known oncogenic significance, only two cases of adenocarcinoma arising from a choledochocele have been described to date. Established treatment of large choledochoceles in childhood is transduodenal marsupialization.

Herein, we report an extremely rare case of early cancer localized in the cystic intrahepatic duct, which is associated with the choledochal cyst including both the intra- and extrahepatic duct (type IV-A cyst). A small polypoid lesion in the cyst was detected by computed tomography. Extensive resection of the choledochal cyst with lobectomy was performed on the tumor in a 23-year-old woman. Tubular adenocarcinoma localized in the wall of the cystic left hepatic duct was histologically confirmed. Awareness of the diagnostic and treatment modalities, with a focus on potential malignancy in the cystic intrahepatic duct, in cases with anomalous arrangement of the pancreatobiliary duct is necessary.

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.

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.