Congenital absence of the intrahepatic bile ducts.

Routine histological examination of the liver during the acute stages of neonatal obstructive jaundice, whether of biopsy or of autopsy specimens, reveals a variety of different pathological conditions. Congenital absence of the intrahepatic bile ducts appears to be a rare cause of obstructive jaundice in the newborn. The purpose of this paper is to draw attention to the condition by reporting two cases, and by discussing its main clinical and pathological features in the light of these two and eight other cases that have been found in the medical literature. One of the present cases was previously shown at a clinical meeting (Smythe and Dobbs, 1949) as a case of xanthomatosis, and later included in a monograph on this subject by Ahrens, Harris and MacMahon (1951) who considered the underlying pathology to be intrahepatic biliary atresia. Neonatal obstructive jaundice with pale stools and dark urine has been common to all described cases, and in no way differed from that found in, for instance, congenital extrahepatic obstruction of the bile ducts or neonatal hepatitis. But, though the diagnosis was arrived at histologically in seven out of the 10 cases only after the age of 2 years, certain features common to the post-neonatal course of all of them appear to make the condition a recognizable clinical entity at least before the end of the first year of life.