Extra hepatic biliary atresia (EHBA) is a neonatal obstructive cholangiopathy, affecting at least 1 in 20000 live born infants. Bile drainage can be restored by portoenterostomy with a striking improvement in prognosis. Surgery must be done before all the intra hepatic ducts leading to porta hepatis are destroyed, usually by 8 weeks of age or else the prognosis is poor with most children dying before 2 yrs of age.

In India there is a long delay in parents seeking medical attention for these infants (average during 4.5 weeks). The average age of presentation to a specialized center is 3.5 months. Inadequate follow up, misdiagnosis, repeated reassurances that the jaundice is physiological and inadequate or unnecessary investigations are some of the reasons for delayed identification of the problem. Infants with EHBA are often healthy otherwise and this results in a false sense of security both on the part of treating physician and parents who tend to overlook the jaundice, mistaking it to be physiological.

Any neonate with persistent jaundice beyond 2 weeks of age should have a total and direct S. bilirubin estimation done and in case of the direct fraction being more than 20% of total should be investigated. A prompt referral to a specialized unit at this point is warranted. Fifteen to twenty percent of infants with EHBA may continue to pass cholic stools hence too much credence should not be given to this finding. However, dark yellow (mustard colour) urine should be taken as a pointer towards EHBA.

Infants with EHBA should be managed and operated at center's with experience in handling such cases (atleast 5 cases per year), as this has been shown to improve outcomes. A prompt ultrasound and post priming HIDA scan along with liver biopsy and preoperative cholangiogram help in identifying 99% of the cases.

In conclusion, serum bilirubin estimation (total/direct) in all children with jaundice beyond 2 weeks of age can help identify most children with EHBA. A prompt referral to a well equipped and experienced center is a must for desirable outcome. It is important to realize that even though EHBA is the most common indication for liver transplantation in childhood, its role in biliary atresia is secondary to that of portoenterostomy.

Pedgastro 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 March 2006(Supplement 3)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
Biliary_Atresia.asp