• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1003-1013
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• 2022

Biliary atresia is an unknown etiology of extrahepatic bile duct obstruction with a 'fibrous ductal remnant,' which represents the obliterated ductal remnant in the porta hepatis. The sonographic 'triangular cord' (TC) sign has been reported to indicate a fibrous ductal remnant in the porta hepatis. In this review, we discuss the correlations among surgicopathological and sonographic findings of the porta hepatis and the definition, objective criteria, diagnostic accuracy, and differential diagnosis of the TC sign in biliary atresia.

• 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 %.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.991-1002
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• 2022

Biliary atresia is a rare but significant cause of neonatal cholestasis. An early and accurate diagnosis is important for proper management and prognosis. To diagnose biliary atresia, various imaging studies using ultrasonography, MRI, hepatobiliary scans, and cholangiography can be performed, although ultrasonography is more important for initial imaging studies. In this article, we review the findings of biliary atresia from various imaging modalities, including ultrasonography, MRI, hepatobiliary scans, and cholangiography. The known key imaging features include abnormal gallbladder size and shape, periportal thickening visible as a 'triangular cord' sign, invisible common bile duct, increased hepatic arterial flow, and combined anomalies. Aside from the imaging findings of biliary atresia, we also reviewed the diagnostic difficulty in the early neonatal period and the role of imaging in predicting hepatic fibrosis. We hope that this review will aid in the diagnosis of biliary atresia.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.143-155
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• 2002

A survey on biliary atresia was made among 26 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and a case registration form for each patient during the twentyone-year period of 1980-2000. Three hundred and eighty patients were registered from 18 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:1.3. The age of patients on diagnosis with biliary atresia was on average $65.4{\pm} 36.2$ days old. The national distribution was 32.8% in Seoul, 25.3% in Gyoungki-Do, 21.6% in Gyoungsang-Do, 9.27% in Choongchung-Do, etc. in order. The most common clinical presentation was jaundice (98.4%) and change of stool color (86.2%) was second. Two hundred eighty (74.7%) of 375 patients were operated by 80 days of age. Three hundred thirty six (9 1.9%) of 366 patients were operated on by the original Kasai procedure, and 305 (84.3%) of 362 patients were observed by bile-drainage postoperatively. The overall postoperative complication rate was 18.5% and the overall postoperative mortality rate was 6.8%. The associated anomalies were observed in 72 cases (22.5%). One hundred ninty five (64.7%) of 302 patients have been alive in follow-up and 49 (25.1%) have survived over 5 years without problem after operation. Ascending cholangitis, varices and ascites affected survival significantly, and the important long-term prognostic factor was the occurrence of complications.