• Advances in pediatric surgery
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• v.10 no.2
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• pp.136-141
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• 2004

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease. It has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our institute, 7 (9.6 %) cases of type III BA with EHBC (uncorrectable with cystic dilatation) are reviewed. Clinical findings, laboratory data, radiologic findings, treatment methods and outcomes were reviewed. Female was more prevalent (male to female ratio; 2:5). All cases were type III with EHBC according to the intraoperative cholangiography, and underwent Kasai' s portoenterostomy. The mean age was 57 days at operation. Three patients(42.9 %) are long term survivors. Further evaluation is needed to determine the correlation between prognostic factors and outcome for.

• The Korean Journal of Nuclear Medicine
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• v.28 no.3
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• pp.357-363
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• 1994

We evaluated the diagnostic accuracy of $^{99m}Tc$-DISIDA scintigrauhy as a mean of differentiating biliary atresia from neonatal hepatitis. $^{99m}Tc$-DISIDA scintigraphy was visually interpreted by assessing the presence or absence of radioactivity in the intestine or gall bladder. In patients without intestinal radioactivity, we measured the hepatic retention index and the hepatic uptake index. The hepatic retention Index was expressed as the amount of change of liver activity from 5 minutes to 30 minutes postinjection. The hepatic uptake Index was graded visually with 5 minute images using the following scoring scheme : grade 0(normal hepatic uptake), grade 1(decreased hepatic uptake), grade 2(hepatic uptake equal to cardiac uptake), and grade 3(hepatic uptake less than cardiac uptake). Age, total bilirubin, and hepatic uptake index were compared between the biliary atresia and the neonatal hepatitis group, between neonatal hepatitis patients with and without intestinal radioactivity, and between the biliary atresia and neonatal hepatitis patients with absent intestinal radioactivity. The results were as follows ; 1) None of the 30 biliary atresia patients showed intestinal radioactivity, while 31/40 neonatal hepatitis patients showed intestinal radioactivity. The sensitivity, specificity, and accuracy of the presence of intestinal radioactivity ?or the diagnosis of biliary atresia was 100%, 78%, and 87%, respectively. 2) In patients with absent intestinal radioactivity the mean hepatic retention index was $1.5{\pm}0.6$ in the 16 biliary atresia patients, and $1.1{\pm}0.2$ in the 7 neonatal hepatitis patients(p<0.01). All 7 patients with hepatic retention index over 1.5 had biliary atresia. But there were 9 patients with biliary atresia below 1.5. 3) No significant differences were found in age, total bilirubin, or hepatic uptake Index between biliary atresia and neonatal hepatitis patients. However there were differences in age, total bilirubin, and hepatic uptake index between neonatal hepatitis patients with and without intestinal radioactivity. The hepatic uptake index was significantly lower, age was old, and total bilirubin was low in the group with intestinal radioactivity compared the group without intestinal radioactivity(p<0.05). Relation between total bilirubin and the hepatic uptake index was that total bilirubin was relatively low at normal hepatic uptake index in biliary atresia and neonatal hepatitis patients. 4) When hepatic uptake index and hepatic retention index were high it suggest that biliary atresia is more likely, considered relation between hepatic uptake Index and the hepatic retention index. Thus, we conclude that $^{99m}Tc$-DISIDA scintigraphy is accurate in the differential diagnosis of biliary atresia and neonatal hepatitis. In patients without intestinal radioactivity, the hepatic retention index and hepatic uptake index, along with the patient's age and total bilirubin level may supplement diagnosis and improve diagnostic accuracy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.178-184
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• 1999

Purpose: The aims of this study were to evaluate the clinical manifestations and prognosis of the syndromic and nonsyndromic intrahepatic bile duct paucity (IHBDP). Methods: We studied histology of 42 infants with neonatal cholestasis. Fourteen patients were diagnosed as IHBDP. We evaluated the clinical manifestations, courses and prognosis retrospectively. Results: Underlying disease of the 42 infants with neonatal cholestasis were biliary atresia in 23, intrahepatic bile duct paucity in 14 (Alagille syndrome in 4 and nonsyndromic IHBDP in 10), neonatal hepatitis in 5 infants. The mean ratio of the bile ducts per portal tract was 0.087 (range: 0~0.5). The manifestations in 4 patients with Alagille syndrome demonstrated as follows: characteristic face in 3, chronic cholestasis in 4, posterior embryotoxon in 2, vertebral anomalies in 2, peripheral pulmonary stenosis in 2. One of 4 patients of Alagille syndrome improved cholestasis and the other 3 patients were remained their cholestasis and growth retardation. All patients of the nonsyndromic IHBDP were idiopathic. Seven out of 8 patients of nonsyndromic IHBDP showed improvement of cholestasis, and one patient received liver transplantation due to cirrhosis. Conclusion: This study suggested that IHBDP should be considered in the differential diagnosis of neonatal cholestasis. The outcome of idiopathic IHBDP was better than predicted.

• 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.3 no.1
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• pp.63-67
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• 2000

Purpose: Biliary atresia, one of the major causes of neonatal cholestais, is an idiopathic, serious disorder, affecting the newborn that results in complete obstruction of biliary tract. Successful reestablishment of bile flow is dependent on early surgical intervention, early diagnosis is imperative. The authors evaluate the utility of Tc-99m-labeled diisoprpyliminodiacetic acid (DISIDA) hepatobiliary scintigraphy in the diagnosis of biliary atresia. Methods: From January, 1995 to August, 1999, total 60 patients with neonatal cholestasis underwent Tc-99m DISIDA hepatobiliary scintigraphy at Asan Medical Center. Results: The undelying causes of neonatal cholestasis were biliary atresia in 14, neonatal hepatitis in 33, intrahepatic bile duct paucity in 9, and total parenteral nutrition induced cholestasis in 4. All patient with biliary atresia were interpreted correctely in DISIDA hepatobiliary scintigraphy, showing 100% sensitivity. Of the 46 patients with neonatal hepatitis and other causes, 37 patients had intestinal radioactivity showing 80% specificity. Conclusion: Visualization of DISIDA in the intestinal tract indicates patency of the biliary ducts and excludes the diagnosis of biliary atresia. But the absence of intestinal excretion on the DISIDA hepatobiliary scintigraphy dose not necessarily indicate biliary atresia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.65-69
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• 2008

A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operation were performed. Because the liver biopsy pathology revealed a paucity of intrahepatic bile ducts, the patient was diagnosed with the Alagille syndrome. We report the case of an infant diagnosed with the Alagille syndrome with atresia of the hepatic duct.

• Investigative Magnetic Resonance Imaging
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• v.1 no.1
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• pp.148-153
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• 1997

Purpose: To evaluate the diagnostic role of a three-dimensional MR cholangiopancreatography (MRCP) over endoscopic retrograde cholangio-pancreatography (ERCP) in various extrah-epatic biliary disease. Materials and Methods: MRCP and ERCP were performed in 45 consecutive patients with suspected extrahepatic biliary diseases. MRCP was obtained using a reverse fast imaging with a steady-state free precession (reverse FISP: PSIF) sequence, and then images were reconstructed by standard MIP algorithm. The predictability of biliary dilatation and level of obstruction of MRCP was evaluated using ERCP as a gold standard. The accuracy distinguishing malignant from benign lesions, and overall diagnostic accuracy were compared between MRCP and ERCP. Results: The sensitivity, specificity and accuracy of MRCP in predicting biliary dilatation were 94.6%, 75.0% and 91.1%, respectively. The level of obstruction was accurate in 87.0% with MRCP. The sensitivity, specificity and accuracy of MRCP and ERCP in distinguishing malignant from benign lesions were 76.2%, 87.5% and 82.2% and 71.4%, 83.3% and 77.8%, respectively. The overall diagnostic accuracy was 60.0% with MRCP and 55.6% with ERCP. Conclusion: 3D MRCP shows a good diagnostic value compared to that of ERCP, and can replace a ERCP.

• The Korean Journal of Pancreas and Biliary Tract
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• v.22 no.4
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• pp.165-171
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• 2017

Background/Aim: There is little data on whether plastic stents with a larger diameter are patent for longer than small stents in patients with bile duct cancer. The aim of this study was to compare the stent survival between 7-French (Fr) and 10-Fr plastic stents and evaluate the factors affecting stent survival. Methods: Patients with biliary obstruction due to biliary tract cancer were enrolled at Yonsei University Wonju College of Medicine from January 2010 to October 2014. Results: A total of 215 patients (7-Fr:10-Fr = 89:126 patients) were retrospectively enrolled. The primary tumor sites were common bile duct (n = 111), hilar (n = 45), and ampulla of Vater (n = 59). Rates of stent migration and stent obstruction were not different between the two groups. The median duration of stent survival was 3.3 months in the 7-Fr group and 5.9 months in the 10-Fr group (p = 0.543). The diameter of the stent did not have an effect on stent survival (hazard ratio 1.11, 95% confidence interval 0.71-1.73, p = 0.649). Conclusions: 7-Fr and 10-Fr stents have similar rates of stent migration and stent obstruction. The stent survival of 7-Fr was not inferior to 10-Fr stents in the management of biliary tract cancer.

• 대한핵의학회:학술대회논문집
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• 1986.05a
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• pp.122.1-122.1
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• 1986

• 대한핵의학회:학술대회논문집
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• 1986.05a
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• pp.122.2-123
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• 1986