The clinical manifestations and morbid anatomy of congenital agenesis of the interlobular (portal) bile ducts have been described by Ahrens et al.1 and by MacMahon and Thannhauser.2 There are 10 documented cases believed to be congenital agenesis of the interlobular bile ducts.1-7 These 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
ROSENTHAL IM, SPELLBERG MA, McGREW EA, ROZENFELD IH. Absence of Interlobular Bile Ducts: Report of a Case of Probable Intrahepatic Bile Duct Agenesis with Severe Hypercholesterolemia, Xanthomatosis, and Glomerular Lipid Deposition. Am J Dis Child. 1961;101(2):228–237. doi:10.1001/archpedi.1961.04020030092014
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