Gorelick F S, Dobbins J W, Burrell M, Riely C A
Dig Dis Sci. 1982 Sep;27(9):815-20. doi: 10.1007/BF01391375.
Arteriohepatic dysplasia is a congenital syndrome associated with life-long cholestasis. Because of symptoms suggesting extrahepatic biliary tract obstruction, we studied three patients with this syndrome by endoscopic retrograde cholangiopancreatogram (ERCP). All patients showed a decrease in the number of intrahepatic ducts. In addition, the intrahepatic ducts show attenuation with focal areas of dilatation. In one subject, this latter finding appeared to be a localized process. The extrahepatic ducts were also narrowed. One patient in this series was found to have gallstones and another cirrhosis. Although the relationship of these anomalies to the cholestasis seen in these patients is unclear, arteriohepatic dysplasis can be added to the list of processes associated with biliary tract abnormalities.
动脉肝发育不良是一种与终身胆汁淤积相关的先天性综合征。由于有提示肝外胆道梗阻的症状，我们通过内镜逆行胰胆管造影（ERCP）对3例该综合征患者进行了研究。所有患者肝内胆管数量均减少。此外，肝内胆管呈变细并伴有局灶性扩张区域。在1例患者中，后一种表现似乎是一个局限性病变。肝外胆管也变窄。该组中有1例患者发现有胆结石，另1例有肝硬化。虽然这些异常与这些患者中所见胆汁淤积的关系尚不清楚，但动脉肝发育不良可被列入与胆道异常相关的病变之列。
