A rare case of cystic dilation of the intrahepatic bile ducts, without involvement of the choledochus was complicated by severe gram-negative septicemia, presented with an atypical clinical picture, and had a fulminating course.
Although presenting in adult patients, it is assumed that, in accordance with the literature, this anomaly is congenital in origin. Secondary obstructive and infective factors, however, may influence the picture. The relative ineffectiveness of any treatment in these cases is mentioned, which contrasts with cases of solitary choledochal cyst.
Zer M, Chaimoff C, Dintsman M, Glanz I. Cystic Dilation of Intrahepatic Bile DuctsPresenting as Gram-Negative Septicemia. Arch Surg. 1973;106(2):225-227. doi:10.1001/archsurg.1973.01350140081023