• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.113-119
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• 2001

Mesenchymal hamartoma is an uncommon benign tumor usually found in childhood, especially during the first two years of life. The tumor consists of a solid component and multiple cyst. Without treatment, these lesions can grow to an enormous size. We experienced a case of mesenchymal hamartoma which was found incidentally, in a 8 month old girl who had ascending cholangitis after Kasai operation due to biliary atresia. On abdominal ultrasonography and CT, there was a well-defined homogeneous small echogenic mass in the right lobe of the liver with cirrhosis. Five months later, she underwent liver transplantation. Gross picture of the resected liver showed a dark greenish pigmented solid mass in the right lobe of the cirrhotic liver. Microscopic findings showed reactive mesenchyma and epithelial overgrowth.

• Radiation Oncology Journal
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• v.21 no.1
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• pp.35-43
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• 2003

Purpose :To analyze the survival period, prognostic factors and complications of patients having undergone high-dose-rate intraluminal brachytherapy (HDR-ILB) as a salvage radiation therapy, while having a catheter, for percutaneous transhepatic billary drainage (PTBD), inserted due to biliary obstruction caused by a secondary malignant biliary tumor Methods and Materials : A retrospective study was performed on 24 patients having undergone HDR-ILB, with PTBD catheter Insertion, be)ween December 1992 and August 2001. Their median age was 58.5, ranging from 35 to 82 years. The primary cancer site were the stomach, gallbladder, liver, pancreas and the colon, with 12, 6, 3, 2 and 1 cases, respectively. Eighteen patients were treated with external beam radiation therapy and HDR-lLB, while slx were treated with HDR-lLB only. The 4otal external beam, and brachytherapy radiations dose were 30$\~$61.2 and 9$\~$30 Gy, with median doses of 50 and 15 Gy, respectively. Results : Of the 24 patients analyzed, 22 died during the follow-up period, with a median survival of 7.3 months. The 6 and 12 months survival rates were 54.2 (13 patients) and 20.8$\%$ (5 patients), respectively. The median survivals for stomach and gailbladder cancers were 7.8 and 10.2 months, respectively, According to the unlvariate analysis, a significant factor affecting survival of over one year was the total radiation dose (over 50 Gy) (o=0.0200), with all )he patients surviving more than one year had been Irradiated with more than 50 Gy. The acute side effects during the radiation therapy were managed with conservative treatment. During the follow-up period, 5 patients showed symptoms of cholangltis due to the radiation therapy Conclusion :An extension to the survival of those patients treated with HDR-ILB is suggested compared to the median historical survival of 4hose patients treated with external biliary drainage. A boost radiation dose could be effectively given, by performing HDR-lLB, which is a prognostic factor In addition, the acute complications of radiation therapy were effectively controlled by conservative management, and It could be regarded as a safe treatment.

• 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.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.111-115
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• 1999

The results of hepatic portojejunostomy in 34 patients with biliary atresia operated upon by one surgeon between May 1989 and December 1997 were analyzed. Eleven (32.3 %) patients were 60 days or younger, 14 patient (41.2 %) were between 60 and 90 days, and 9 (26.5 %) were over 90 days of age. Jaundice cleared in 20 cases (58.8 %). Three patients died of liver insufficiency, 2 were anicteric but died from esophageal variceal bleeding. Three patients died as a result of sepsis, heart failure and left kidney agenesis. Five patients were lost to follow-up. The five-year survival rate was 73.8 %. Two patients over 90 days of age, survived more than 5 years. Survival rates were not significantly related to the age at operation. We conclude that hepatic portojejunostomy should be considered as a primary surgical modality for biliary atresia, even at age 90 days or more. Early detection of esophageal varices and sclerotherapy may be necessary. Liver transplantation is necessary if hepatic failure develops.

• Advances in pediatric surgery
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• v.14 no.2
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• pp.125-133
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• 2008

To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

• Advances in pediatric surgery
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• v.6 no.1
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• pp.19-26
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• 2000

This paper reports our 9-year experience treating 34 infants with biliary atresia utilizing a new non-invasive diagnostic method, ultrasonographic "triangular cord"(TC) sign. The TC sign is present when there is visualization of a triangular or a band-like echogenicity just cranial to the portal vein. The ultrasonographic TC sign appears to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia. Sensitivity is 84 %. Active bile excretion was restored in 90 % of the patients who were treated between 31-60days, 78 % of those between 61-90 days, and 33 % of those being 91days or older. The incidence of postoperative cholangitis was 36 %. Construction of an antireflux valve in the Roux-en-Y loop did not affect the incidence of postoperative cholangitis(p=0.18). Among 34 infants with biliary atresia, 23(68 %) are alive for 2-102 months after operation, and 12 are alive for more than 5 years. Five-year estimate survival by Kaplan-Meier method was 66 %.

• Advances in pediatric surgery
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• v.3 no.2
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• pp.93-97
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• 1997

Extended porta hepatis dissection and hepatic portojejunostomy was performed on 14 biliary atresia patients during last 13 years by a single surgeon. The average age at operation was 68 days(range from 37 days to 98 days). The patients were admitted for 8 weeks postoperatively for administration of parenteral antibiotics. There was one operative mortality due to acute hepatic necrosis. Among 13 patients remaining, 12(92.5 %) became chemically jaundice-free within 36 weeks postoperatively(average 16.8 weeks). the earliest 8 weeks, and in one patients jaundice persisted. Five(38.5 %) patients developed cholangitis after operation. Among jaundice-free patients, one patient died of unrelated disease 2 years after hepatic portojejunostomy, who underwent left lateral segmentectomy because of a biloma. Eleven survivors(78.6 %) are jaundice-free. The oldest one is 13 years old, enjoying a normal life. The mean period of follow-up is 7 years and 3 months.

• Advances in pediatric surgery
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• v.4 no.2
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• pp.125-130
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• 1998

To assess the clinical and nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, MAC, TSF), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure were performed more than 10 years ago at Department of Pediatric Surgery in Seoul National University Hospital. None were below the 5th percentile in height and weight. TSF was above the 75th percentile in all patients and showed good subcutaneous fat deposition. MAC was above the 5th percentile in all patients. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Although some patients who have survived for more than 10 years after Kasai procedure developed protein malnutrition and vitamin deficiencies, growth and development and nutritional status were generally satisfactory. Five patients(42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion of long-term survivors had abnormal liver function and portal hypertension. Continuous and careful follow-up is necessary to determine when liver transplantation may be indicated.

• Advances in pediatric surgery
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• v.3 no.1
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• pp.6-14
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• 1997

To differentiate biliary atresia from intraheaptic cholestasis, Tc-99m DlSIDA hepatobiliary scintigraphies and percutaneous needle biopsies of 60 consecutive infants were evaluated retrospectively. Twenty three patients had biliary atresia and 37 patients intraheaptic cholestasis(neonatal hepatitis 34, TPN induced jaundice 2 and Dubin-Johnson syndrome 1). All sixty patients underwent Tc-99m DlSIDA hepatobiliary scintigraphy with phenobarbital pretreatment. Of 23 patients with biliary atresia, 22 were correctly interpreted showing 96% sensitivity while of 37 patients with intraheaptic cholestasis, only 12 had intestinal excretion of radionuclide showing 32% specificity. Among the forty needle biopsies, 17 of biliary atresia and 23 of intrahepatic cholestasis, 37 were correctly interpreted as either having biliary atresia or intrahepatic cholestasis. The overall diagnostic accuracy was 93%. Of 3 misdiagnosed cases, the histologic findings of two patients with biliary atresia(aged 43 days and 54 days at the first needle biopsy) were essentially the same as those of neonatal hepatitis. Follow-up biopsies, however, showed findings consistent with biliary atresia. The third one(VLBW premie with history of 8 weeks of TPN) showed mild ductal proliferation and portal fibrosis. This was interpreted as suspicious for biliary atresia. Jaundice resolved gradually. In summary, patients who have intestinal excretion of radionuclide on Tc-99m DlSIDA hepatobiliary scintigraphy, biliary atresia can be ruled out. But the patients who do not have intestinal excretion of radionuclide should have further investigation by needle biopsy. Judicious use of Tc-99m DISIDA hepatobiliary scintigraphy and percutaneous needle biopsy can give a diagnostic accuracy of 95% or more in cases of infantile cholestasis.

• Advances in pediatric surgery
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• v.13 no.1
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• pp.1-12
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• 2007

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.