Gautier M, Valayer J, Odievre M, Alagille D
J Pediatr Surg. 1984 Jun;19(3):263-8. doi: 10.1016/s0022-3468(84)80182-7.
Clinical, biological, and histological data are described in 20 patients at least 5 years after surgery for extrahepatic biliary atresia. Seventeen had an " uncorrectable " type and underwent hepatoportoenterostomy or hepatoportocholecystostomy , 3 patients had a "correctable" type and underwent cystojejunostomy . Clearing of jaundice was observed in all patients. Portal hypertension developed in 16 out of the 20. Liver specimens were obtained by surgical biopsy in all patients. Definite biliary cirrhosis was seen in all but two. Particular patterns were observed in some cases: biliary structures without bile stasis were absent in 8, while 4 displayed marked cell infiltration in portal areas, and 7 had distended vascular channels. The appearance of liver cells was always normal. No relationship could be clearly established between the degree of fibrosis and the earlier presence of cholangitis.
对20例肝外胆管闭锁手术后至少5年的患者的临床、生物学和组织学数据进行了描述。其中17例为“不可纠正”型,接受了肝门肠吻合术或肝门胆囊吻合术,3例为“可纠正”型,接受了囊肿空肠吻合术。所有患者黄疸均消退。20例中有16例出现门静脉高压。所有患者均通过手术活检获取肝脏标本。除2例患者外,其余均可见明确的胆汁性肝硬化。部分病例观察到特殊模式:8例无胆汁淤积的胆管结构缺失,4例门静脉区有明显细胞浸润,7例血管腔扩张。肝细胞外观始终正常。纤维化程度与早期胆管炎的存在之间未能明确建立联系。