Idiopathic extrahepatic biliary atresia: recurrence in sibs in two families

Since 1855, 11 instances of familial extrahepatic biliary atresia (EHBA) have been described. Genetic, environmental, vascular, and infectious causes for this disorder have been proposed. We report on 2 families each with 2 sibs with EHBA. We suggest that a genetic susceptibility to an environmental insult to the biliary tree may be responsible for the genesis of this disease.