• Advances in pediatric surgery
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• v.5 no.2
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• pp.126-129
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• 1999

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst ($4.5{\times}4.0$ cm in size) in the left hepatic lobe and another tubular dilatation ($2.0{\times}0.8$ cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangiodrainage(PTCD) and cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring $6.6{\times}5.0$ cm in size. Following drainage and administration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks. The patient successfully underwent intrahepatic cystojejunostomy with intraoperative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.4
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• pp.433-436
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• 2023

Forty-five adults with type IV-A choledochal cysts (CDC) who underwent extrahepatic cyst excision from January 2013 to December 2021 were followed up for a median interval of 25 months (range, 2 to 10 years) to observe the long-term complications in the remaining intrahepatic cyst. Late complications in varying combinations were seen in 10 patients, which included cholangitis and/or intrahepatic stones in 9 patients, intrahepatic bile duct stenosis with stones in 2 patients, anastomotic stricture in 6 patients, and left lobar atrophy with intrahepatic stones in 3 patients. Out of 6 patients who required re-do hepaticojejunostomy (HJ), three patients had left lobe atrophy with patent HJ anastomosis and a recurrent attack of cholangitis on follow-up at 3, 8, and 10 years. Complications occur frequently after extrahepatic cyst excision for type IV-A CDC and require a long-term follow-up.

• Advances in pediatric surgery
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• v.15 no.1
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• pp.11-17
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• 2009

Choledochal cyst is a congenital dilatation of the bile duct. Intrahepatic bile duct dilatation of type IVa by Todani's classification at the time of diagnosis resolved spontaneously after cyst excision and hepaticojejunostomy in many cases. It should be distinguished from the true cystic dilatation of the intrahepatic ducts, which tends to persist, albeit after some regression. We therefore studied postoperative intrahepatic duct dilatation changes in choledochal cyst. A total of seventy-six choledochal cysts were managed at the Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center from May 1995 to December 2005. The ratio of males to females was 1:2.8. Preoperative radiologic diagnosis by Todani's classification was Type I (n=52, 68.4 %), II (n=1, 1.3 %), IVa (n=23, 30.3 %). Among fifty-five patients with intrahepatic bile duct dilatation we were able to follow up forty-eight by ultrasonography. Twenty-two patients were type IVa, and twenty-six patients were type I and showed intrahepatic duct dilatation. Mean follow-up duration was 35.3 months (9~105 months). Complete regression of dilated intrahepatic duct was observed in fifteen patients of type IVa and twenty-four patients of type I. Incomplete regression of dilated intrahepatic duct was observed in six patients in type IVa and two patients in type I. Only one patient in type IVa showed no change in ductal dilatation during a follow-up period of 15 months. We conclude that true type IVa is much less frequent than what was diagnosed preoperatively by imaging study. Therefore in type IVa patients who are diagnosed preoperatively the decision to perform liver resection should be carefully considered. Postoperative long term follow up of choledochal cyst with intrahepatic bile duct dilation is needed.

• Clinical and Experimental Pediatrics
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• v.59 no.5
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• pp.239-241
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• 2016

Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a differential diagnosis of a cystic mass regardless of the cyst's size or patient's age, especially in children presenting with abdominal pain, jaundice, and palpable mass. To the best of our knowledge, we report the largest choledochal cyst in infancy.

• 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.

• The Korean Journal of Nuclear Medicine
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• v.21 no.1
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• pp.17-24
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• 1987

Authors classified 161 cases of photon defects due to the gall bladder on hepatic parenchymal scintigraphy on $^{99m}Tc-phytate$ and $^{99m}Tc-DISIDA$ according to the position of the gall bladder, the pattern of photon defects and the hepatobiliary diseases. The results were as follows; 1) Conocordance of $^{99m}Tc-DISIDA$ and $^{99m}Tc-phytate$ hepatic parenchymal images in photon defect due to the gall bladder was 94% of 32 cases. 2) The frequency according to the position of the gall bladder was in order to 68% of the gall bladder of the lower margin of the liver, 30% of the intrahepatic gall bladder and 2% of the extrahepatic gall bladder, and the frequency of the photon defects due to the gall bladder was in order to 81% of the intrahepatic gall bladder, 71% of the gall bladder of the lower margin of the liver and 20% of the extrahepatic gall bladder. 3) The pattern of the photon defects due to the gall bladder was 47% of funnel shape in the intrahepatic gall bladder, 69% of semilunar shape in the gall bladder of the lower margin of the liver and 100% of semilunar shape in the extrahepatic gall bladder. 4) All of 9 cases of the intrahepatic gall bladder at the lateral area of the right lobe and the gall bladder of the lower margin of the liver at the right hepatic angle were associated with liver cirrhosis with the right lobe atrophy and the left lobe hypertrophy, 2 cases of the gall bladder of the lower margin of the liver at just-left side of the porta hepatis with hepatoma in the right lobe and 1 case of the intrahepatic gall bladder at the central portion of the right lobe with choledochal cyst.

• The Korean Journal of Nuclear Medicine
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• v.27 no.1
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• pp.71-80
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• 1993

In order to evaluate the scintigraphic features of choledochal cyst and these diagnostic value, authors investigated the findings of fourteen patients with choledochal cyst undergone hepatobiliary scan with $^{99m}Tc$-DISIDA before surgery. Five cases demonstrated the decreased hepatic uptake at 5-minute image of which four cases revealed severe jaundice. Seven cases demonstrated visualization of the cystic dilated common bile duct within 1 hour after injection. Two cases showed the cyst activity between 1 and 12 hours, but the cyst activity was not visible in five cases. Nonvisualization of the gall bladder was noted in ten cases, while four cases demonstrated visualization of the gall bladder within 1 hour. The time of visualization of gut activity was variably delayed. The intestinal activity was found in three cases within 1 hour and appeared in three cases between 1 and 2 hours and eight cases showed no visible gut activity. In four cases, intrahepatic ductal prominence was visible on the scintigram. Seven cases showed early and persistent accumulation of tracer in the common bile duct. Three cases showed persistent photon-deficient area in the gall bladder region. Two cases showed early photon-deficient area around gall bladder region with progressive accumulation of tracer in the same region. Two cases showed no evidence of activity in the biliary tract but noted late excretion into the small intestine. We concluded that hepatobiliary scan using $^{99m}Tc$-DISIDA is a noninvasive test useful in the evaluation and the diagnosis of choledochal 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.

• 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.

• The Korean Journal of Nuclear Medicine
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• v.14 no.2
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• pp.19-27
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• 1980

Although primary application of radioisotope scanning technics to the liver has been of use in the detection of the intra-hepatic space occupying lesion from the normal functioning liver parenchyme, there has been on increasing awareness of its use in evalution of Liver function. In this study, the diseases of the liver were classified into group A,B,C and D by the liver scanning findings, conventional liver function tests and clinical findings. Following were the results: 1. The colloidal radiogold liver scan appeared normal in the group A, also the albumin in serum, alkaline phosphatase activity and prothrombin time were within normal levels in this group. 2. In the group B, there were acute hepatitis 24(48%), chronic hepatitis 5(10%), toxic hepatitis 3(6%), subacute hepatic necrosis 3(6%), typhoid liver 4(8%), hepatic tuberculosis 2(4%), diabetes mellitus 3(6%) and others 3(6%). In this group, SGOT and SGPT were increased predominantly as compared with group A, and the liver scan showed small amount of mottling of activity and faintly visualized spleen. 3. In the group C, there were postnecrotic liver cirrhosis 30(60%), Laennec cirrhosis 10(20%), cardiac cirrhosis 1(2%), cholangiocarcinoma 1(2%), chronic active hepatitis 6(12%), hepatic milliary tuberculosis 1(2%) and gall bladder cancer 1(2%). In this group, the albumin in serum and prothrombin time were lowered significantly and the liver scan showed severe mottling of activity with extra-hepatic uptake in the spleen and bone marrow along the vertebral column. 4. In the group D, there were primary hepatoma 26(52%), hepatoma with liver cirrhosis 7(14%), metastatic liver cancer 5(10%), liver abscess 10(20%), multiple liver cyst 1(2%) and cystic duct adenoma 1(2%), In this group, the alkaline phosphatase activity was elevated with single or multiple intrahepatic space occupying lesion in the radiogold colloid liver scan.