• Journal of Yeungnam Medical Science
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• v.39 no.2
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• pp.161-167
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• 2022

Congenital web formations are extremely rare anomalies of the extrahepatic biliary tree. We herein report a case of common bile duct septum combined with multiple intrahepatic bile duct strictures in a 74-year-old female patient who was successfully treated with radiological intervention. The patient initially visited the hospital because of upper abdominal pain. Imaging studies revealed multifocal strictures with dilatation in both intra- and extrahepatic ducts; the final clinical diagnosis was congenital common bile duct web combined with multiple intrahepatic duct strictures. Surgical treatment was not indicated because multiple biliary strictures were untreatable, and the disease was clinically diagnosed as benign. The multiple strictures were extensively dilated twice through bilateral percutaneous transhepatic biliary drainage (PTBD) for 2 months. After 1 month of observation, PTBD catheters were successfully removed. The patient is doing well at 6 months after completion of the radiological intervention, with the maintenance of normal liver function. Congenital web of the bile duct is very rare, and its treatment may vary depending on the patterns of biliary stenosis. In cases where surgical intervention is not indicated for congenital web and its associated disease, radiological intervention with balloon dilatation can be a viable therapeutic option.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.964-970
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• 2021

Extrahepatic duct duplication is an extremely rare congenital anomaly. Hilar cholangiocarcinoma with extrahepatic bile duct duplication was reported; however, intraductal papillary neoplasm of the bile duct (IPNB) with extrahepatic bile duct duplication has not been reported to the best of our knowledge. We report a rare case of IPNB with extrahepatic bile duct duplication of a 64-year-old female. The patient underwent extended right hepatectomy, and the results of a subsequence histopathological examination were consistent with an IPNB with extrahepatic bile duct duplication. We report this rare case with radiologic imaging findings and a brief review of the current literature.

• The Korean Journal of Nuclear Medicine
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• v.30 no.1
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• pp.126-129
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• 1996

Spontaneous perforation of CBD in infant is a rare but fatal disease. We report a case of bile leakage from common bile duct in 11 months old girl with progressive abdominal distension and vomiting, preoperatively diagnosed by hepatobiliary scan with 99mTc-DISIDA, which was confirmed by surgery, Operative cholangiogram showed a small perforation at the confluence of cystic duct and common bile duct with mild fusiform dilatation, and no definite abnormality in confluence of the common bile duct and pancreatic duct. Simple drainage of the free peritoneal bilous fluid and T-tube drainage were performed without any evidence of the complication. Patient was inevitable for 6 months OPD follow-up examination.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.3
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• pp.180-186
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• 2015

Purpose: Bile duct dilatation is a relatively common sonographic finding; nevertheless, its clinical significance in children is controversial because little research has been done in the area. Therefore, we investigated the natural course and clinical significance of biliary duct dilatation in children. Methods: We performed a retrospective study of 181 children (range, 1-day-old to 17-year-old) in whom dilatation of the intrahepatic duct and/or common hepatic duct and/or common bile duct was detected by abdominal ultrasonography at the Severance Children's Hospital between November 2005 and March 2014. We reviewed and analyzed laboratory test results, clinical manifestations, and clinical course in these patients. Results: Pediatric patients (n=181) were enrolled in the study and divided into two groups. The first group included 59 subjects, without definitive cause of bile duct dilatation, who did not require treatment; the second group included 122 subjects, with definitive cause of bile duct dilatation or underlying biliary disease, who did require treatment. In the first group, 24 patients (40.7%) showed spontaneous resolution of bile duct dilatation, 20 patients (33.9%) showed no change, and 15 patients (25.4%) were lost to follow-up. In the second group, 31 patients were diagnosed with choledochal cysts, and 91 patients presented with biliary tract dilatations due to secondary causes, such as gallbladder or liver disease, post-operative complications, or malignancy. Conclusion: Biliary dilatation in pediatric patients without symptoms, and without laboratory and other sonographic abnormalities, showed a benign clinical course. No pathologic conditions were noted on follow-up ultrasonography.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.284-288
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• 2004

Spontaneous perforation of the biliary duct is a rare disease that must be treated immediately when diagnosed in infancy. This disease must be suspected in a previously healthy infant who suddenly has abdominal distension, ascites, and intermittent jaundice. The best way to diagnose the leakage of bile in a clinically stable infant is to use radionuclide hepatobiliary scan. In most cases, however, the diagnosis of the perforation of common bile duct is frequently made during the procedure of operative cholangiogram. The prognosis is usually good with early diagnosis. We experienced an infant of 4 months of age who presented with sudden abdominal distension, jaundice and acholic stool, and confirmed the diagnosis of perforation of common bile duct through operative cholangiogram with t-tube insertion. We report a case of spontaneous perforation in an infant with review of the literatures.

• Advances in pediatric surgery
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• v.9 no.2
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• pp.81-88
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• 2003

Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani's classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type 1 consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.108-112
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• 2001

Nonsyndromic intrahepatic bile duct paucity is known to be associated with several kinds of etiology such as infection, chromosomal anomaly, metabolic disease and idiopathic. We report a rare case of intrahepatic bile duct paucity with congenital bilateral vocal cord paralysis and 13q deletion.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.2
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• pp.121-124
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• 2014

Omega (${\omega}$)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nutrition-associated liver disease, and several mechanisms were proposed for this observation. An 8-week-old male infant with cholestasis and acholic stool was diagnosed non-syndromic intrahepatic interlobular bile duct paucity by open-wedge liver biopsy. Initially he was treated with usual supportive medical therapy, including ursodeoxycholic acid. However, the clinical status and laboratory tests did not improve. Omega (${\omega}$)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including aminotransferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effectiveness of ${\omega}$-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease.

• Journal of Digestive Cancer Research
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• v.2 no.2
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• pp.78-81
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• 2014

Intraductal papillary neoplasm of the bile duct (IPN-B) is a rare disease characterized by multicentric proliferation of adenomatous epithelium within the bile ducts. It has better prognosis because of slow disease progression and high probability of early diagnosis, but also has potential for malignant transformation. Therefore early treatment such as surgical resection has been recommended. There are few reports about nature course of IPN-B without treatment. We report a case of papillary adenoma of intrahepatic duct that transformed into intrahepatic cholangiocarcinoma with 8 years of follow up.

• Clinical Endoscopy
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• v.51 no.6
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• pp.541-546
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• 2018

Recent advances in cholangiopancreatoscopy technology permit image-enhanced endoscopy (IEE) for pancreatobiliary diseases. There are limitations in endoscopy performance and in the study of the clinical role of IEE in bile duct or pancreatic duct diseases. However, currently available IEEs during cholangiopancreatoscopy including traditional dye-aided chromoendoscopy, autofluorescence imaging, narrow-band imaging, and i-Scan have been evaluated and reported previously. Although the clinical role of IEE in pancreatobiliary diseases should be verified in future studies, IEE is a useful promising tool in the evaluation of bile duct or pancreatic duct mucosal lesions.