Wu Z M
First Affiliated Hospital, Sun Yat-sen University of Medical Sciences, Guangzhou.
Zhonghua Wai Ke Za Zhi. 1991 Oct;29(10):623-5, 653.
Forty cases of adult congenital cystic dilatation of the intrahepatic bile ducts were reported in this paper together with a review of the literature. The disease is characterized by repeated attack of cholangitis, and the diagnosis is dependent almost entirely upon cholangiographic findings. The authors believe that basing on the typical cholangiographic findings as well as the tendency of having a longer course, this disease can be differentiated from the acquired dilatation of the intrahepatic bile ducts secondary to hepatolithiasis. The principle of treatment is resection of the diseased focus as well as establishment of an internal drainage. The diagnosis in 50% of the cases in this series was confirmed by histopathology, which has not been reported in the previous literature.
本文报告了40例成人先天性肝内胆管囊状扩张症，并复习了相关文献。该病以胆管炎反复发作为特征，诊断几乎完全依赖胆管造影检查结果。作者认为，基于典型的胆管造影表现以及病程较长的特点，本病可与肝内胆管结石继发的获得性扩张相鉴别。治疗原则是切除病灶并建立内引流。本系列病例中有50%的诊断经组织病理学证实，此前文献中未见报道。
