• Advances in pediatric surgery
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• v.4 no.2
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• pp.100-109
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• 1998

The anomalous pancreaticobiliary duct union (APBDU) might cause the formation of choledochal cyst and malignancies of hepatopancreaticobiliary system. The purpose of this study is to make an experimental animal model of APBDU similar to that of human. One to two-month-old Mongrel dogs (n=12) were divided into two groups; the control group (n=2) had a sham operation performed, and in the experimental group (n=10) the end of distal ' common bile duct (CBD) was anastomosed to the side of the dorsal pancreatic duct making APBDD. Serum was obtained for chemical analysis on the 10th postoperative day. The dogs were sacrificed at the 5th week (n=3), the 6th week (n=3), the 7th week (n=2), the 8th week (n=2) and the 6th month (n=2) after the experimental surgery. With sacrifice, operative cholangiogram was taken, and bile juice was obtained for chemistry and bacterial culture. The en-bloc specimens of the hepatopancreaticobiliary system were removed for microscopic examination. Serum and bile juice amylase levels were elevated in the experimental group(n=10), but not in the control group(n=2). Operative cholangiograms of control group revealed no evidence of bile duct dilatation.. On the other hand, the bile duct in the experimental group was markedly dilated without any evidence of stenosis at the anastomosis site (n=10). Histologic examination of the hepatopancreaticobiliary system in the experimental group resembled the findings of choledochal cyst in human. The APBDU of this animal model can produce bile duct dilatation by pancreaticobiliary reflux. We think that this animal model can be potentially promising for the research about the APBDU associated hepatopancreaticobiliary diseases.

• Advances in pediatric surgery
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• v.9 no.1
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• pp.45-51
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• 2003

A nationwide survey on choledochal cyst was undertaken among 39 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the five year period of 1997 to 2001. Three hundred and forty eight patients were registered from 32 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:3.4. The age of patients on diagnosis was $49.0{\pm}44.4$ months. The geographic distribution was 34.8% in Seoul and Kyoungki-do, 33.3% in Kyoungsang-do, 17.9% in Cholla-do, and 8.5% in Choongchung-do, in order of frequency. The three common clinical presentations were abdominal pain (63.8%), vomiting (35.3%), and jaundice (29.1%). Only seven patients (2%) presented with classic triad, and 25 patients were diagnosed by antenatal ultrasonographic examination. According to the Todani Classification, 238 patients (7l.3%) were type 1, 3 (0.9%) type 11, and 93 (27.8%) type IV. At the time of the operation, three important associated conditions were choledocholithiasis in 45 patients (15.1%), liver fibrosis (Grade 1-4) in 35, and previous operative procedure for biliary diseases in 10. Associated anomalies were observed in 13 patients (3.8%). Three hundred thirty nine (98.8%) of 343 lesions were treated by Cyst excision and Roux-Y hepaticoiejunostomy. One hundred seventy-six patients had an anomalous arrangement of the pancreatobiliary ductal system (APBD): APBD was not in 92 patients, biliary duct joined to the pancreatic duct in 51, and pancreatic duct joined to the biliary duct in 26. There were 8.5% early, and 7.7% late phase operative complications. The major complications were bleeding, anastomotic leakage, and acute pancreatitis. The combination of acute abdomen and choledochal cyst may suggest spontaneous rupture. Because of the development of late intrahepatic bile duct stones, long term follow up after cyst excision and hepaticojejunostomy is required. The optimal time of surgical intervention should also be considered in the situation of routine use of antenatal ultrasonographic examination. This is the first review of the choledochal cyst in Korea and provides baseline data for future comparisons.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1381-1388
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• 2002

Purpose : Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. Methods : From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. Results : A total of 70 patients, consisting of 31 males and 39 females, with a mean age of $2.6{\pm}3.3$ years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. Conclusion : MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.6
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• pp.531-538
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• 2020

Purpose: Our aim was the longitudinal assessment of cyst size in fetuses with biliary cystic malformation (BCM) to explore its etiology and the possibility of antenatal differentiation between biliary atresia (BA) and congenital biliary dilatation (CBD). Methods: We conducted a retrospective review of all patients diagnosed antenatally with BCM from 1994 to 2014 at our institutions. Results: The study cohort comprised of three patients with BA and six with CBD. There were no significant differences in the gestational age and cyst size at the first detection of BCM between the two groups. In fetuses with CBD, the cyst size steadily increased as the gestational age advanced, while it fluctuated around 1.5 cm and remained below 2.1 cm in those with BA. However, the ratio of cystic area to fetal trunk area was approximately constant due to linear fetal growth in fetuses with CBD. Conclusion: Fetuses with BCM <2.1 cm in the late gestation period were more likely to have BA than CBD. Our observation of cyst enlargement with advancing gestational age in the CBD group was attributed solely to fetal growth. Biliary dilatation in fetuses with CBD and BA might be completed at the onset of BCM.

• Investigative Magnetic Resonance Imaging
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• v.20 no.4
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• pp.254-258
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• 2016

Spontaneous perforation of the bile duct without any traumatic or iatrogenic injury is extremely rare. We report a case of abscess formation related to spontaneous perforation of the common bile duct by a gallstone, mimicked a cholecochal cyst.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.3
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• pp.203-208
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• 2018

Purpose: The aim of this study was to describe our treatment experiences with patients who had acute abdomen (AA) with common bile duct (CBD) dilatation. Methods: The treatment outcomes in children with AA and CBD dilatation were retrospectively reviewed. According to the shape of the intrahepatic bile ducts on ultrasonography (US), the origin of the pain was estimated as choledochal cyst (CC) complication or choledocholithiasis in normal CBD. Patients with complicated CC underwent surgery, and patients with choledocholithiasis in a normal appearing CBD underwent symptomatic treatment initially. Results: Of the 34 patients, 30 (88.2%) were female. The mean age of the patients was $6.4{\pm}4.9$ (range, 0.8-17) years. Seventeen (50.0%) patients had CBD stones and 17 (50.0%) did not. Surgical treatment was performed in 20 (58.8%) patients, 2 of whom underwent preoperative stone removal with endoscopic retrograde cholangiopancreatography and an operation. Conservative treatment was applied in 12 (35.3%) patients (8 with and 4 without stones), 1 of whom developed symptom relapse and underwent an operation. Among the 8 patients with CBD stones, 4 (4/17, 23.5%) had complete resolution of the stones and recovery of the CBD diameter after conservative treatment. US findings of patients with stone showed a fusiform or cylindrical shape of the CBD in 14 (82.4%) patients. Conclusion: The presence of stones in the distal CBD and the US features of CBD dilatation may be helpful to diagnose and treat the causes of biliary dilatation. Conservative treatment can be considered as initial therapy in patients with uncomplicated CBD dilatation with stone.

• Journal of Digestive Cancer Research
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• v.3 no.1
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• pp.42-44
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• 2015

Anomalous union of the pancreaticobiliary duct (AUPBD) has been shown to predispose to carcinomas of the biliary tract (bile duct and gallbladder) and pancreatic cancer because of chronic recurrent inflammatory reaction due to pancreatic or bile juice refluxes. However, pancreatic duct would be less affected by the bile because of the relatively higher intraductal pressure of the pancreatic duct. We report a case of metachronous pancreatic cancer in AUPBD patient without choledochal cyst who underwent cholecystectomy because of gallbladder cancer.

• Journal of Yeungnam Medical Science
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• v.32 no.1
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• pp.60-64
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• 2015

A choledochocele is an expanded sac of the duodenal side of the distal common bile duct (CBD), and is categorized as a type III choledochal cyst. Unlike other choledochal cysts, it can be easily overlooked because of its very low prevalence, non-specific clinical symptoms, and lack of distinctive radiological findings. However, a patient having a repeated pancreaticobiliary disorder with an unknown origin, frequent abdominal pain after cholecystectomy, or repeated non-specific gastrointestinal symptoms can be suspected as having a choledochocele, and a more accurate diagnosis can be achieved via endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound. Because it rarely becomes malignant, a choledochocele can be treated via endoscopic sphincterotomy (EST) and surgical treatment. The authors were able to diagnose choledochocele accompanied by a stone in a patient admitted to the authors' hospital due to cholangitis and pancreatitis. The patient's condition was suspected to have been caused by a distal CBD stone detected via multiple detector computed tomography and ERCP, and was successfully treated via EST.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.923-927
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• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.2
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• pp.114-123
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• 2017

Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.