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[胆囊缺如。关于病理生理学及诊断的论述。附2例报告]

[Agenesis of the gallbladder. Comments on physiopathology and diagnosis. Apropos of 2 cases].

作者信息

Odimba B F, Stoppa R, Henry X, Largueche S, Verhaeghe P

出版信息

J Chir (Paris). 1982 Nov;119(11):647-54.

PMID:6818237
Abstract

Initially a simple anatomical curiosity discovered at operation or autopsy, agenesis of the ball bladder has now, and despite its rarity, become of the greatest interest from a pathogenic, physiopathological and diagnostic standpoint. Embryogenesis explains that this a developmental abnormality affecting the hepatic diverticulum which is probably of genetic origin, hence its frequent association with other malformations and the hereditary nature of the condition. The usual finding of a syndrome of blockage of the sphincter of Oddi with dilatation of the common bile duct raises the problem of synergism between the sphincter of Oddi and the gall bladder. The discovery of lithiasis of the hepatic and common bile ducts in agenesis of the gall bladder leads to discussion of the reality of organ lithiasis or the role of the distended wall of the common duct. Cholecystography and echotomography would appear to be the two techniques of investigation to be recommended preoperatively in order to increase support for the presumptive diagnosis once disorders affecting neighbouring structures have been eliminated. The definite diagnosis is made peroperatively by manual exploration, peroperative cholangiography or even coeliac arteriography during the operation. The indication for surgery is based upon clinical symptomatology. This does not treat the agenesis but the associated lesions which are its complications, in particular: lithiasis the common duct, sphincter of Oddi block syndrome and dilatation of the common duct.

摘要

胆囊缺如最初是在手术或尸检时发现的一种简单的解剖学奇象,如今,尽管其罕见,但从发病机制、生理病理学及诊断角度来看,已引起了极大的关注。胚胎发生学解释了这是一种影响肝憩室的发育异常,可能源于遗传,因此它常与其他畸形相关联,且具有遗传性。通常会发现伴有胆总管扩张的Oddi括约肌梗阻综合征,这就引发了Oddi括约肌与胆囊之间协同作用的问题。在胆囊缺如时发现肝内胆管和胆总管结石,引发了关于器官结石的真实性或胆总管扩张壁的作用的讨论。胆囊造影术和超声断层扫描似乎是术前推荐的两种检查技术,以便在排除影响邻近结构的疾病后,增强对推定诊断的支持。术中通过手动探查、术中胆管造影甚至术中腹腔动脉造影来明确诊断。手术指征基于临床症状。手术并非针对胆囊缺如进行治疗,而是针对其并发症,即相关病变,特别是:胆总管结石、Oddi括约肌梗阻综合征和胆总管扩张。

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