Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.
Child ; Choledochal Cyst ; Common Bile Duct/pathology* ; Diagnosis, Differential ; Humans ; Jaundice, Obstructive/etiology* ; Male ; Rhabdomyosarcoma, Embryonal/diagnosis*

This pictorial review aims to illustrate the magnetic resonance imaging (MRI) findings and presentation patterns of anatomical variations and various benign and malignant pathologies of the duodenum, including sphincter contraction, major papilla variation, prominent papilla, diverticulum, annular pancreas, duplication cysts, choledochocele, duodenal wall thickening secondary to acute pancreatitis, postbulbar stenosis, celiac disease, fistula, choledochoduodenostomy, external compression, polyps, Peutz-Jeghers syndrome, ampullary carcinoma and adenocarcinoma. MRI is a useful imaging tool for demonstrating duodenal pathology and its anatomic relationships with adjacent organs, which is critical for establishing correct diagnosis and planning appropriate treatment, especially for surgery.
Ampulla of Vater/anatomy & histology/radiography ; Choledochal Cyst/pathology/radiography ; Diverticulum/radiography ; Duodenal Diseases/pathology/*radiography ; Duodenum/*anatomy & histology/radiography ; Humans ; *Magnetic Resonance Imaging ; Pancreas/abnormalities/anatomy & histology/radiography ; Pancreatic Diseases/radiography

PURPOSE: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years. METHODS: A total of 113 patients, diagnosed with choledochal cyst and who received an operation in Severance Children's Hospital from January 1988 to May 2013, were included. Medical records were reviewed, including clinical and demographic data, surgical procedures. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography were used as diagnostic tools for evaluation and classification of choledochal cyst and the presence of anomalous pancreaticobiliary ductal union. Todani's classification, and relationship between APBDU and surgical pathology. RESULTS: Among 113 patients, 77 patients (68.1%) presented symptoms such as hepatitis, pancreatitis and/or cholecystitis. Eighty three patients (73.5%) had APBDU, and 94 patients (83.2%) showed inflammatory pathologic changes. APBDU, pathologic inflammation, and serological abnormalities such as hepatitis or pancreatitis showed a statistically significant correlation to one another. CONCLUSION: APBDU is thought to be one of the etiologic factors of choledochal cyst. It is related to the inflammatory changes in bile duct that can lead to the cystic dilatation.
Bile Ducts* ; Child ; Cholangiography ; Cholangiopancreatography, Magnetic Resonance ; Cholecystitis ; Choledochal Cyst* ; Classification ; Common Bile Duct ; Dilatation ; Hepatitis ; Humans ; Inflammation* ; Medical Records ; Pancreatitis ; Pathology ; Pathology, Surgical ; Ultrasonography

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

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

Choledochal cyst is an uncommon premalignant anomaly. The morphology and pathogenesis of the premalignant lesion of cholangiocarcinoma arising from the choledochal cyst has not been well described. Herein, we report a rare case of bile duct adenoma arising from choledochal cyst with anomalous union of pancreaticobiliary duct (AUPBD). 50-year-old woman was admitted to our hospital with the complaint of epigastric pain. She had received common bile duct (CBD) exploration and choledocholithotomy and cholecystectomy 3 months earlier under the diagnosis of multiple CBD stones. Intraoperalive cholangiogram was not remarkable except CBD dilatation at that time. Endoscopic retrograde cholangiopancreatography revealed choledochal cyst with AUPBD and round filling defect which disappeared easily on the balloon cholaniogram. On magnetic resonance cholangiopancreatography, the filling defect was confirmed as 2 cm polypoid mass attached to the distal bile duct wall. At laparotomy, a soft whitish mass was palpable on the lower CBD. On histological examination, adenoma with focal carcinoma change arising from choledochal cyst was diagnosed.
Adenoma, Villous/*diagnosis/pathology/radiography ; Bile Duct Neoplasms/*diagnosis/pathology/radiography ; Cholangiopancreatography, Magnetic Resonance ; Choledochal Cyst/*radiography/secretion/surgery ; Female ; Humans ; Middle Aged ; Tomography, X-Ray Computed

We present a case report to show how manganese-enhanced T1- and T2-weighted MR cholangiography could differentiate cystic parenchymal lesions from cystic abnormalities which communicate with the bile ducts.
Bile Ducts/*pathology ; Cholangiopancreatography, Magnetic Resonance/*methods ; Choledochal Cyst/diagnosis ; Contrast Media/chemistry ; Cysts/diagnosis ; Diagnosis, Differential ; Humans ; Liver/*pathology ; Male ; Manganese/*chemistry ; Middle Aged ; Reproducibility of Results ; Sensitivity and Specificity

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- xisting intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.
Tomography, X-Ray Computed ; Postoperative Complications/ultrasonography ; Male ; Liver Diseases/complications/*radiography/surgery ; Infant, Newborn ; Infant ; Humans ; Female ; Choledochal Cyst/complications/*radiography/surgery ; Cholangiography ; Child, Preschool ; Child ; Bile Ducts/*abnormalities/pathology/surgery

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- xisting intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.
Tomography, X-Ray Computed ; Postoperative Complications/ultrasonography ; Male ; Liver Diseases/complications/*radiography/surgery ; Infant, Newborn ; Infant ; Humans ; Female ; Choledochal Cyst/complications/*radiography/surgery ; Cholangiography ; Child, Preschool ; Child ; Bile Ducts/*abnormalities/pathology/surgery