Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.
Adult ; Bile Duct Neoplasms/*diagnosis/etiology/pathology ; *Bile Ducts, Intrahepatic ; Cholangiocarcinoma/*diagnosis/etiology/pathology ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst/complications/*diagnosis ; Female ; Humans ; Positron-Emission Tomography and Computed Tomography ; Tomography, X-Ray Computed

OBJECTIVETo summarize the experience in diagnosis, prevention and treatment of carcinoma arising from congenital biliary duct cyst.

METHODSThe clinical and pathological data of 185 patients with congenital biliary duct cyst admitted to Chinese PLA General Hospital were analyzed retrospectively.

RESULTSAmong 185 patients, twenty-seven cases had carcinomas arising from congenital biliary duct cyst, and the frequency of malignant transformation was 14.6%, which closely related to the age (P < 0.001). The incidences of malignancy for different age groups were: 0 for 0-9 age group, 5.1% for 0-19, 9.1% for 20-29, 16.2% for 30-39, 26.7% for 40-49, 33.3% for 50-59, and 50% for over 60, respectively. Six patients had the history of cyst-enterostomy. Abdominal pain, fever, jaundice and weight loss were the main clinical manifestations. Abdominal ultrasonography, CT, MRI or magnetic resonance cholangiopancreatography, MRCP and endoscopic retrograde choledochopancreatography (ERCP) were the main diagnostic methods. For twenty patients (74.1%), a definite diagnosis was made preoperatively, but it's hard to make an early diagnosis. Nine patients (33.3%) underwent curative resection.

CONCLUSIONSCongenital biliary duct cyst is a premalignant lesion, and the incidence of carcinogenesis increases remarkably with age. The most effective method for prevention of carcinogenesis in choledochal cyst is complete excision of choledochal cyst during childhood, and the prognosis is poor for patients with biliary malignancy.

Adolescent ; Aged ; Child ; Child, Preschool ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst ; complications ; diagnosis ; surgery ; Common Bile Duct Neoplasms ; diagnosis ; etiology ; surgery ; Early Diagnosis ; Female ; Humans ; Infant ; Male ; Middle Aged ; Retrospective Studies ; Tomography, X-Ray Computed

BACKGROUND: Choledochal cysts are not so rare in far east Asian countries as in western countries and are found mainly in children. Reflux of pancreatic juice to the bile duct through anomalous pancreaticobiliary ductal union (APBDU) is considered to be an important etiologic factor. METHOD: To study the clinical characteristics of choledochal cysts in children and the significance of APBDU in this disease, we reviewed 80 choledochal cyst cases. APBDU is defined as a long common channel (>1.5 cm). RESULTS: Ages ranged from 19 days to 13 years, and the male to female ratio was 1 to 4 which revealed a predominance among females. The symptoms, in order of frequency, were abdominal pain (63%), jaundice (48%), and abdominal mass (26%); classic triads were present in 3 cases (4%). The diagnosis was made mainly with ultrasonography. Todani type I (88%) and IV (11%) were the two most common types of cysts. An operative cholangiography was performed in 80 cases, among which an analysis of the status of the pancreaticobiliary ductal union was possible in 21 cases. A long common channel (>1.5 cm) was found in all 21 cases, 16 cases being of the P- type and 5 cases of the B- type. A cyst excision and hepaticojejunostomy was performed in 79 cases of type I and type IV cysts, and a liver biopsy only was performed in one case of a type V cyst. A common bile duct stone was associated with one case and cholangitis was associated with two cases as postoperative complications. One patient expired due to heart disease. Seventy-ine patients were followed up for a mean period of 7.2 years after the operation. Seventy-ight patients are healthy now, and liver function tests have returned to normal level. One Carolie's disease patient has been followed up outpatiently without bad liver function. An intraoperative liver biopsy was performed in 73 cases. The results were normal in 3 cases; cholestasis was noted in 2 cases, inflammation in 26, fibrosis in 38, and cirrhosis in 4. CONCLUSIONS: APBDU seems to play an important role in the pathogenesis of choledochal cysts, especially type I and type IV cysts. Excision of the cyst is the procedure of choice for treatment because it eliminates the APBDU and prevents both hepatic cirrhosis and the potential for developing a malignancy.
Abdominal Pain ; Asian Continental Ancestry Group ; Bile Ducts ; Biopsy ; Child ; Cholangiography ; Cholangitis ; Choledochal Cyst* ; Cholestasis ; Common Bile Duct ; Diagnosis ; Far East ; Female ; Fibrosis ; Heart Diseases ; Humans ; Inflammation ; Jaundice ; Liver ; Liver Cirrhosis ; Liver Function Tests ; Male ; Pancreatic Juice ; Postoperative Complications ; Ultrasonography

Improvement in prenatal ultrasonography is leading to diagnose choledochal cyst before birth and before onset of classical symptom more frequently. But, there is a controversy about optimal timing for cyst excision of prenatally diagnosed asymptomatic choledochal cyst. To identify the most appropriate timing for surgery in prenatally diagnosed choledochal cysts, we analyzed 6 patients who had operation for choledochal cysts within 30days after birth at the division of Pediatric Surgery, Samsung Medical Center and Inha University School of Medicine, from June 1995 to June 2002. Males were four and females 2, the mean age at operation was 11.2 days, and the median age 8.0 days. The range of gestational ages of the antenatal diagnosis of bile duct dilatation was 24 weeks to 32 weeks, mean was 38.3 weeks, and mean birth weight was 3,298.3 g. After birth, abdominal ultrasonography, hepatobiliary scintigraphy, and magnetic resonance cholangiopancratography (MRCP) were performed. Mean age at operation was 11.2 days. All patients had the cyst excision and Roux- en-Y hepaticojejunostomy. Immediate postoperative complication was not found. During the median follow-up period of 41 months, one patient was admitted due to cholangitis, and the other due to variceal bleeding. Early operative treatment of asymptomatic newborn is safe and effective to prevent developing complications later in life.
Bile Ducts ; Birth Weight ; Cholangitis ; Choledochal Cyst* ; Dilatation ; Esophageal and Gastric Varices ; Female ; Follow-Up Studies ; Gestational Age ; Humans ; Infant, Newborn ; Male ; Parturition ; Postoperative Complications ; Prenatal Diagnosis ; Radionuclide Imaging ; Ultrasonography ; Ultrasonography, Prenatal

OBJECTIVE: The aim of this study was to evaluate the frequency of postoperative biliary stricture and its risk factors in patients undergoing surgery for type I choledochal cyst. MATERIALS AND METHODS: A total of 35 patients with type I choledochal cyst underwent laparoscopic cyst excision and Roux-en-Y hepaticojejunostomy between August 2004 and August 2011. Their medical records and radiologic images (including endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography, pancreatobiliary computed tomography, or ultrasound) were retrospectively analyzed to evaluate the frequency of postoperative biliary stricture and its risk factors. RESULTS: Postoperative biliary stricture was found in 10 (28.6%) of 35 patients. It developed more frequently in patients with type Ia choledochal cyst (53.8%, 7 of 13 patients) than in patients with type Ic choledochal cyst (13.6%, 3 of 22 patients), which was statistically significant (p = 0.011). There were no significant associations between other factors and postoperative biliary stricture. CONCLUSION: Type Ia is a risk factor of postoperative anastomotic stricture. Therefore, preoperative radiologic subclassification of type Ia and Ic may be useful in predicting postoperative outcomes of choledochal cysts.
Adolescent ; Adult ; Anastomosis, Roux-en-Y ; Biliary Tract Surgical Procedures/methods ; Chi-Square Distribution ; Child ; Child, Preschool ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst/*pathology/*surgery ; Female ; Humans ; Infant ; Jejunostomy/methods ; Laparoscopy/*methods ; Male ; Middle Aged ; Postoperative Complications/*diagnosis ; Statistics, Nonparametric ; Tomography, X-Ray Computed ; Treatment Outcome ; Ultrasonography/methods

OBJECTIVE: The aim of this study was to evaluate the frequency of postoperative biliary stricture and its risk factors in patients undergoing surgery for type I choledochal cyst. MATERIALS AND METHODS: A total of 35 patients with type I choledochal cyst underwent laparoscopic cyst excision and Roux-en-Y hepaticojejunostomy between August 2004 and August 2011. Their medical records and radiologic images (including endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography, pancreatobiliary computed tomography, or ultrasound) were retrospectively analyzed to evaluate the frequency of postoperative biliary stricture and its risk factors. RESULTS: Postoperative biliary stricture was found in 10 (28.6%) of 35 patients. It developed more frequently in patients with type Ia choledochal cyst (53.8%, 7 of 13 patients) than in patients with type Ic choledochal cyst (13.6%, 3 of 22 patients), which was statistically significant (p = 0.011). There were no significant associations between other factors and postoperative biliary stricture. CONCLUSION: Type Ia is a risk factor of postoperative anastomotic stricture. Therefore, preoperative radiologic subclassification of type Ia and Ic may be useful in predicting postoperative outcomes of choledochal cysts.
Adolescent ; Adult ; Anastomosis, Roux-en-Y ; Biliary Tract Surgical Procedures/methods ; Chi-Square Distribution ; Child ; Child, Preschool ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst/*pathology/*surgery ; Female ; Humans ; Infant ; Jejunostomy/methods ; Laparoscopy/*methods ; Male ; Middle Aged ; Postoperative Complications/*diagnosis ; Statistics, Nonparametric ; Tomography, X-Ray Computed ; Treatment Outcome ; Ultrasonography/methods