Biliary atresia registry, 1976 to 1989.
The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.
Karrer
F M
FM
Department of Surgery, University of Colorado School of Medicine, Denver 80262.
Lilly
J R
JR
Stewart
B A
BA
Hall
R J
RJ
D000367:Age Factors; D000704:Analysis of Variance; D000713:Anastomosis, Roux-en-Y; D001656:Biliary Atresia / Q000473:pathology / Q000601:surgery*; D002170:Canada; D002758:Cholangiography; D005260:Female; D006723:Hong Kong; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008800:Mexico; D011171:Portoenterostomy, Hepatic / Q000379:methods; D011379:Prognosis; D044469:Racial Groups; D012042:Registries; D013514:Surgical Procedures, Operative / Q000379:methods; D015996:Survival Rate; D014481:United States