CONGENITAL SEGMENTAL CYSTIC DILATATION OF BILIARY DUCTAL SYSTEM
CONGENITAL SEGMENTAL CYSTIC DILATATION OF BILIARY DUCTAL SYSTEM
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DOI：
10.1097/00000658-197306000-00009
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发表时间：
1973-01-01
影响因子：
9
通讯作者：
MCSHERRY, CK
中科院分区：
文献类型：
--
作者：
GLENN, F;MCSHERRY, CK
胆管系统的节段性囊性扩张导致胆汁淤积，易导致细菌生长，并通过压迫实质细胞损害肝功能。当这些病变是由于导管壁的发育缺陷时，它们被称为“先天性导管系统节段性囊性扩张”（图2）。1）. 这个术语适用于肝内和肝外胆管。自1985年Caroli详细描述了这种疾病的痛苦过程以来，对181814年以来长期处于休眠状态的患者的历史报告受到了越来越多的关注。我们同意其他人的观点，即作为节段性扩张基础的缺陷是先天性的。154年前，也就是1818年，Todd14在都柏林医院报告了一个患有弥漫性囊性管扩张的病人。1904年（86年后），梅奥-罗布森提请人们注意伦敦亨特里安博物馆的一个标本。他将其描述为“由扩张的肝管构成的肝脏大肿瘤，肝管在肝脏内部形成一系列囊肿。”....主航道上没有障碍物。”Yotuyanagi18在1936年记录了一个病人有一个大的囊性扩张的普通管和右管的肝内囊肿。他将囊肿描述为“苹果大小”，显微镜下发现囊肿壁内有一层上皮。另一个独特而重要的病例报告是McWhorter于192411年和1939.10年报道的患者，一名49岁的女性，接受了大的扩张的共管切除术。行胆囊切除术和胆十二指肠吻合术。她在69岁时去世。尸检显示主要肝内管扩张伴“囊性变性”。
SEGMENTAL CYSTIC DILATATION of the biliary ductal system leads to bile stasis, predisposes to bacterial growth and by compression of parenchymal cells, impairs liver function. When these lesions are due to a develop-mental defect in the duct wall they are referred to as" congenital segmental cystic dilatation of the ductal system"(Fig. 1). This term is applicable to both the intraand extrahepatic bile ducts. Historical reports of patients with this condition, extending back to 181814 and long dormant, have received increasing attention since Caroli in 19585 described in detail the distressing course of this condition. We agree with others3, 4" 12" 15 that the defects that are the basis for segmental dilatation are congenital. One hundred and fifty-four years ago, in 1818, Todd14 reported from the Dublin Hospital a patient with a diffuse cystic dilatation of the common duct. In 1904 (86 years later) Mayo-Robson9 called attention to a specimen in the Hunterian Museum in London. It was described by him as" a large tumor of the liver constituted by dilated hepatic ducts which form a series of cysts within the substance of the liver itself."...." There was no obstruction in the main channel." Yotuyanagi18 in 1936 recorded a patient with a large cystic dilatation of the common duct and an intrahepatic cyst of the right duct. He described the cyst as" the size of an apple" and microscopically as having a single layer of epithelium inside the cyst wall. Another unique and significant case report is that of McWhorter re-ported in 192411 and again in 1939.10 The patient, a woman of 49, underwent resection of a large dilated common duct. A cholecystectomy and choledocho-duodenostomy were performed. She died at age 69. Autopsy revealed dilatation of major intrahepatic ducts with" cystic degeneration."