J Korean Assoc Pediatr Surg.  2004 Dec;10(2):136-141.

Biliary Atresia with Extrahepatic Biliary Cyst

Affiliations
  • 1Department of Surgery, College of Medicine, the Catholic University of Korea, Seoul, Korea. ytong@catholic.ac.kr

Abstract

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.

Keyword

Biliary atresia; Extrahepatic biliary cyst; Uncorrectable type

MeSH Terms

Biliary Atresia*
Cholangiography
Female
Humans
Prognosis
Rare Diseases
Survivors
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