Absence of Interlobular Bile Ducts

Abstract

The clinical manifestations and morbid anatomy of congenital agenesis of the interlobular (portal) bile ducts have been described by Ahrens et al.¹and by MacMahon and Thannhauser.²There are 10 documented cases believed to be congenital agenesis of the interlobular bile ducts.^1-7These cases have been recently reviewed by Haas and Dobbs.⁷ One of the striking features of congenital agenesis of the interlobular ducts is the appearance in some cases of cutaneous xanthomata during the course of the illness. Of the reported cases of congenital agenesis of the interlobular ducts, 6 have had cutaneous xanthomata. The appearance of cutaneous xanthomata in this disease is always associated with severe and prolonged hypercholesterolemia. It is the purpose of this paper to report an additional case believed to be congenital agenesis of the interlobular bile ducts. The case is of interest for several reasons. Cutaneous xanthomata appeared in this