Congenital Biliary Hypoplasia

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Abstract
Hypoplasia of the bile ducts is found in patients with neonatal jaundice. Like atresia, hypoplasia may involve any portion or all of the extra or intrahepatic biliary system, and it may occur with atresia. Unlike atresia, hypoplasia may vary in degree. It may be associated with normal or near normal biliary drainage or with almost complete absence of biliary drainage, and thereby the clinical course may be similar to that of an irremediable biliary atresia. Of 5 patients with hypoplasia of the biliary tract reported 2 seemed to have normal or near normal bile drainage when last seen. Two patients have suffered the results of prolonged partial biliary obstruction, but are able to carry on many normal activities at 6 and 7 years of age. One patient, 26 months old, has severe biliary obstruction, and is following the general course of a patient with biliary atresia. Recognition of the condition of biliary hypoplasia may help to explain the unusual clinical course of certain patients with neonatal jaundice. For example, it may explain the course of patients who recover spontaneously when a patent ductal system has not been identified at operation, or those with signs of neonatal biliary obstruction who survive in reasonably good general condition for years.