Signs & Symptoms
The usual history is a full term infant who appears normal at birth but develops jaundice after the age of 2 to 3 weeks.
The stools are characteristically pale or even white in colour.
What causes it
Biliary atresia is a disorder of uncertain cause.
About the condition
Biliary atresia is a progressive inflammatory destructive process of the bile ducts. There is progressive obliteration of the extrahepatic bile ducts as well as abnormalities of the intrahepatic bile ducts. Left untreated, it will lead to liver failure.
Diagnosis and Treatment Options
The diagnosis is based on blood tests to confirm conjugated hyperbilirubinaemia, ultrasound to confirm an atretic gall bladder, radio-isotope HIDA (iminodiacetic acid) scan to confirm obstruction to bile flow, liver biopsy to look for cirrhosis and eventual surgical exploration to determine feasibility of a bile drainage procedure to relieve obstruction.
Early diagnosis of this disease is very important. If surgery is performed before the baby is 2 months old, success is much more likely. After 3 months, success of the operation is poor. For this reason, all infants who are jaundiced after the age of 4 weeks should be evaluated for biliary atresia.
Currently, the conventional open Kasai porto-enterostomy remains the standard approach for re-establishing biliary drainage. Following Kasai procedure, about 50-70% patients achieve clearance of jaundice. About 21 to 44% children may survive with their native liver for longer than 20 years. Long-term complications following Kasai are cholangitis and portal hypertension.
However, a significant proportion may continue to suffer from insufficient biliary excretion and persistent jaundice. These children may develop decompensated liver disease requiring liver transplantation within the first 2 years of life.
Prior to the development of liver transplantation as a therapeutic option for children with decompensated liver disease, the long-term survival rate for infants with biliary atresia following portoenterostomy was below 50%.
The application of liver transplantation for decompensated liver disease has increased the long-term survival of biliary atresia patients to more than 90%.
For infants with biliary atresia, the current standard of care is to offer Kasai procedure as the initial option so that the child can live with his native liver and as a continuum of treatment liver transplantation may become necessary for chronic decompensated liver disease.
The paediatric liver transplantation unit in NUH provides a comprehensive life-long management of children with biliary atresia. The care begins with the Kasai procedure in infancy. The child is monitored and follow-up continues until adulthood. Liver transplantation may be indicated for those cases who develop decompensated liver disease and its complications. Following liver transplantation survival in these children is more than 85% at one year.
Information correct as at June 2016
1) Lee S, Park H, Moon SB, Jung SM, Kim JM, Kwon CH, Kim SJ, Joh JW, Seo JM, Lee SK.
Long-term results of biliary atresia in the era of liver transplantation.
Pediatr Surg Int. 2013; 29:1297-301
2) Bijl EJ, Bharwani KD, Houwen RH, de Man RA.
The long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review.
Neth J Med. 2013; 71:170-3.
3) Mali VP, Aw M, Quak SH, Loh DL, Prabhakaran K. Transplant Proc. 2012;44:1373-8. Vascular complications in
pediatric liver transplantation; single-center experience from Singapore.