Curr Opin Pediatr 1997 Jun;9(3):265-9

Biliary atresia

Middlesworth W, Altman RP
Division of Pediatric Surgery, College of Physicians and Surgeons, Columbia
University, Babies and Children's Hospital of New York, NY 10022, USA

Abstract
Biliary atresia, a progressive obliterative process involving the bile ducts,
has its onset in the newborn period. It is characterized by worsening
cholestasis, hepatic fibrosis, and cirrhosis, which lead to portal hypertension
and a decline in hepatic synthetic function. Untreated, the outcome is uniformly
fatal. The two major milestones toward improved treatment of this disease have
been the Kasai portoenterostomy and orthotopic liver transplantation. There has
been discussion regarding transplantation as primary therapy, but
portoenterostomy remains the standard of care as first-line intervention.
Hepatic transplantation, done more frequently for biliary atresia than for any
other cause of liver failure in the pediatric population, offers improved
survival and quality of life to those for whom the Kasai operation fails. The
etiology of biliary atresia remains poorly understood. Working toward a better
understanding of this disease, recent investigations target more precise
characterization of the hepatic pathology and seek to identify possible
causative agents and predictors of favorable outcome. Recent advances in the
understanding of biliary atresia published between December 1995 and November
1996 are the focus of this review