Ruffa G, Lucigrai G, Bartocci M, Tomà P, Bruschettini P
II Clinica Pediatrica G Gaslini, Instituto Giannina Gaslini, Università degli Studi, Genova.
Minerva Pediatr. 1996 Jul-Aug;48(7-8):337-40.
We describe a case of congenital non-obstructive dilatation of the hepatic bile ducts (Caroli disease). The accuracy of ultrasound for the diagnosis of this syndrome is pointed out. Ultrasound scan can be carried out easily and with a remarkable imaging precision. The ultrasonographic patterns consist of both evident dilatation of the ducts and characteristic protrusions connecting the ductal walls to intraluminal portal branches (bridging). These sonographic findings confirm the etiopathogenetic hypothesis according to which Caroli disease is a result of the blockage of the bile ducts development. Moreover, we underline the efficacy of ursodeoxycholic acid in the treatment of the chronic cholestasis, always present in this disease.
我们描述了一例先天性肝内胆管非梗阻性扩张(卡罗利病)的病例。指出了超声诊断该综合征的准确性。超声检查操作简便,成像精度高。超声图像表现为胆管明显扩张以及连接胆管壁与管腔内门静脉分支的特征性凸起(桥接)。这些超声检查结果证实了病因发病学假说,即卡罗利病是胆管发育受阻的结果。此外,我们强调了熊去氧胆酸在治疗该病中始终存在的慢性胆汁淤积方面的疗效。