• 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.

• The Korean Journal of Nuclear Medicine
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• v.32 no.2
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• pp.161-167
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• 1998

Purpose: Segmental parenchymal excretion delay on Tc-99m DISIDA scan is caused by intrahepatic bile duct obstruction. However, the diagnostic value for intrahepatic bile duct obstruction is unknown. We conducted this study to assess the positive predictive value of segmental excretion delay for the diagnosis of intrahepatic bile duct obstruction, and additional benefit over other noninvasive radiologic studies. Materials and Methods: The study population consisted of 43 patients (48 scans) who showed segmental parenchymal excretion delay on Tc-99m DISIDA scan. The results of abdominal CT or ultrasonography, which was done within 1 month of Tc-99m DISIDA scan, were compared with scintigraphic findings. Results: The etiology of segmental parenchymal excretion delay was determined by ERC or PTC in 31 scans, and follow-up studies in 13 scans. No causes were identified in 4 scans. The positive predictive value of segmental parenchymal excretion delay for intrahepatic bile ductobstruction was 92% (44/48). On the other hand, 13% (5/38) of CT and 28% (5/18) of ultrasonography were normal. In 18% (7138) of CT and 17% (3/18) of ultrasonography, only intraheipatic bile duct dilatation was noted without any diagnostic findings of intrahepatic bile duct obstruction. Conclusion: Segmental parenchymal excretion delay on Tc-99m DISIDA scan had a high positive predictive value for the diagnosis of intrahepatic bile duct obstruction. Tc-99m DISIDA scan may be useful for the diagnosis of intrahepatic bile duct obstruction, especially in patients with nondiagnostic CT or ultrasonography. The diagnostic usefulness need to be confirmed by further prospective studies.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.46-58
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• 1999

Purpose: The aim of the present study was to evaluate the long-term clinical profile including the underlying etioligy and the prognostic factors of the neonatal cholestasis. Method: We studied the 190 infants presented with neonatal cholestasis for the last 12 years (from 1981 to 1992). The underlying causes, clinical findings and long-term outcomes were evaluated. And the prognostic factors were also analyzed. Result: Underlying disease were neonatal hepatitis in 101 (idiopathic in 77 and infectious in 24), intrahepatic bile duct paucity in 5, biliary atresia in 79, choledochal cyst in 5. Metabolic disease was not observed in this study. The important clinical problems during follow-up were persistent high fever, gastrointestinal bleeding, hepatic encephalopathy and ascites. The main causes of the death were hepatic encephalopathy and gastrointestinal bleeding. While three fourth of infants with idiopathic and infectious neonatal hepatitis recovered usually within a year, five-year survival rate for biliary atresia was just 40%, the mortality observed usually within the first year after Kasai operation and prognostic factor was the time of operation. Underlying disease was the most important prognostic factor of neonatal cholestasis. Conclusion: This study showed that most common causes of neonatal cholestasis were biliary atresia and idiopathic neonatal hepatitis, infectious neonatal hepatitis, choledochal cyst and Alagille syndrome, but few neonatal cholestasis of genetic or metabolic liver disease was observed. The most important long-term prognostic factor of neonatal cholestasis was the underlying disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.208-214
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• 2004

Purpose: The prognosis of neonates with cholestasis is not clear. Some factors, such as high peak bilirubin levels and liver histologic findings have been claimed to affect the prognosis adversely. Our study aims to define which factors influence the prognosis of neonatal intrahepatic cholestasis. Methods: Retrospective reviews of the medical records were performed in 32 cases with neonatal intrahepatic cholestasis, who were admitted to Department of Pediatrics, Pusan National University Hospital from July 1995 to July 2002. Neonates were classified into 2 groups according to the duration of elevated serum alanine aminotransferase (ALT) levels more or less than 6 months. The data, such as biochemical, histopathologic and radiologic findings, were compared in both groups. Biochemical data included mean peak level of serum ALT, total bilirubin, direct bilirubin, and alkaline phosphatase. Histologic parameters related to lobular architecture, fibrosis, inflammatory infiltration and degenerative features of hepatocytes were arbitrary estimated on a scale of 1 to 3. Results: There were 19 males and 13 females, whose mean age was 48 days (14~77 days). The peak serum ALT levels were higher in the poor outcome group. Ductular proliferation and portoportal bridging were more severe in the poor outcome group. But the degree of multinucleated hepatocytes, hepatocellular swelling and canalicular plug did not appear to be significantly related to the long-term outcome. The DISIDA scintigraphy by visualization time of gall bladder and intestine was not useful in predicting outcome of neonatal intrahepatic cholestasis. Conclusion: Neonates who have intrahepatic cholestasis with high serum ALT levels, severe ductular proliferation and portoportal bridging in the liver biopsy specimen should be carefully followed up because they may have a poor prognosis.

• Herbal Formula Science
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• v.6 no.1
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• pp.119-139
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• 1998

This study was to investigate the protective and effects of Ikgukwhan and Ikgukbowhawhan on the liver cirrhosis or fibrosis induced by prolonged bile duct ligation; a new experimental model for cirrhosis and the intraperitoneal injection of dimethylnitrosamine in the rat. The development of fibrosis or cirrhosis and its inhibition by the two prescriptions were examined by the chemical analysis of AST, ALT, and hydroxyproline. The results obtained were as follows. 1. The increase of serum asparate aminotransferase induced by bile ductligation was inhibited by the administration of Ikgukwhan and Ikgukbowhawn extract. 2. The increase of serum alanine aminotransferase induced by bile duct ligation was inhibited by the administration of Ikgukwhan and Ikgukbowhawhan extract. 3. The increase level of serum AST and ALT induced by the intraperitoneal injection of dimethylnitrosamine was inhibited by the administration of Ikgukwhan and Ikgukbowhawhan extract. 4. The increase level of hydroxyproline volume in the damaged liver tissues in the rat was decreased by the oral administration of Ikgukwhan and Ikgukbowhawhan extract. But there were no significant differences in the inhibition rate between the two experimental groups.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.86-90
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• 2003

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.

• Parasites, Hosts and Diseases
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• v.25 no.2
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• pp.110-122
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• 1987

The rabbits were infected with Clonorchis sinensis and were treated with praziquantel at the dose of $50{\;}mg/kg{\times}2회{\times}2$ days after 1,2,4,8 weeks and 7 months from the infection. Their livers were observed histopathologically 1, 4 and 12 weeks after treatment. The findings are summarized as below. 1. The changes of the liver in control rabbits were relatively mild until 2 weeks after infection. However, widening and thickening of bile ducts, proliferation of biliary epithelium and periductal fibrosis were moderate after 4 weeks from infection and those changes were severe after 8 weeks and 7 months. Goblet cell metaplasia was found after 8 weeks from infection. 2. The mild changes of 2-week infection group were completely recovered by 4 weeks after the treatment. In the groups of 4 or more weeks after infection, the changes of bile ducts became milder in the degree after the treatment, but were still found 12 weeks after the treatment. As the infection duration was passed, more severe changes were observed after the treatment. In this context, it is concluded that the liver changes of acute clonorchiasis in the early two weeks are reversible by treatment while chronic biliary epithelial changes are irreversible. Therefore, early treatment should be recommended as possible to minimize the remaining histopathological changes of liver in clonorchiasis.

• Investigative Magnetic Resonance Imaging
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• v.6 no.2
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• pp.129-136
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• 2002

Purpose : To evaluate the usefulness of MRCP in the diagnosis of the variable causes of common bile duct(CBD) dilatation, except stone or tumor Materials and methods : Twenty-six patients(M:F=15:11, mean age; 62 years) with both MRCP and ERCP were included in this study. Dynamic MRCP(n=12) and contrast-enhanced MRI(n=10) of abdomen were also added. Dilatation of CBD, intrahepatic ducts and pancreatic duct was evaluated, including coexistence of intrahepatic ductal stone, pancreatic pseudocyst, and papillary or papillary edema. The criteria of CBD dilatation was over than 7mm(n= 21, without cholecystectomy) or 10 mm(n=5, with cholecystecto-my) in diameter on T2-weighted coronal image. Results : The mean diameter of CBD was 12.7mm without cholecystectomy(9-19 mm) and 13.0 mm with cholecystectomy(10-15mm), respectively(p 〉0.05). Cholangitis(n=11, 42.3%), chronic pancreatitis(n=8, 30.8%), stenosis of distal CBD(n= 6, 23.1%), periampullary diverticulum(n=3, 11.5%), stenosis of ampulla of Vater(n=2, 7.7%), dysfunction of sphincter of Oddi(n=2, 7.7%), acute focal pancreatitis in the pancreatic head(n=2, 7.7%), papillitis(n=1, 3.8%), pseudocyst in the pancre atic head(n = 1, 3.8%), and ascaris in CBD(n=1, 3.8%) were noted. Pancreatic duct dilatation(n=10, 38.5%) and duodenal diverticulum(n=3, 11.5%) were also seen on MRC P. On dynamic MRCP(12 patients), distal CBD was visualized in 2 patients(16.7%), which was not shown on routine MRCP. Only 1 patient(10.0%) showed papillitis with slightly enhancing papilla on contrast-enhanced MRI (10 patients). Conclusion : MRCP was thought to be helpful in the evaluation of the causes of CBD dilatation, not caused by stone or tumor, especially in the cases of stenosis of distal CBD and chronic pancreatitis, dysfunction of sphincter of Oddi on dynamic MRCP and cholangitis and pericholangitic abnormality on contrast-enhanced MRI.

• Pediatric Infection and Vaccine
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• v.8 no.1
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• pp.123-128
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• 2001

Clonorchiasis, known as the parasitic fluke in the intrahepatic bile ducts, occurs throughout Korea, Japan, Taiwan, and Vietnam with high frequency. Most of the infections follow the consumptions of raw or undercooked freshwater fish, which explains its rare occurrence to childhood even in those areas mentioned earlier above. The authors have experienced it with an 8 year old boy. Abdominal ultrasonogram revealed multiple low echogenic nodules in the right lobe of liver and lymph nodes proliferation around the porta hepatis area. And abdominal C.T. scan also found multiple low attenuated nodules with delayed enhancement in liver parenchyma with hepatomegaly and multiple conglomerated lymph nodes in porta hepatis. Both abdominal ultrasonogram and C.T. scan didn't show any dilatation of peripheral intrahepatic bile ducts. In repeated stool examination, Clonorchis sinensis eggs were found in feces and the result of the skin test for Clonorchis sinensis was positive. The patient had a history of having eaten raw pond smelt back in Cheongju a month before the symptom onset. On the basis of the patient's history and the fact that Clonorchis eggs were found in feces 3 weeks after eosinophilia and symptoms were noted, the authors estimated that the findings of the patient's abdominal sonogram and C.T. scan were the radiologic findings of the acute phase of clonorchiasis, of which no report had ever been made before. So the authors report a case of clonorchiasis with atypical radiologic findings in an 8 year old boy.