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先天性胆囊缺失。

Congenital absence of the gall bladder.

作者信息

Singh B, Satyapal K S, Moodley J, Haffejee A A

机构信息

Department of Surgery, Faculty of Medicine, University of Natal, Durban, South Africa.

出版信息

Surg Radiol Anat. 1999;21(3):221-4. doi: 10.1007/BF01630907.

Abstract

Congenital absence of the gall bladder is an extremely rare embryological aberration with a reported incidence ranging between 0.013 and 0.075%. This report, the first from South Africa, discusses 2 cases of gall bladder agenesis, bringing to 413 the number of cases reported in the literature. In confirming the diagnosis of an agenesis of the gall bladder, it is necessary to exclude the abnormal locations which are intrahepatic, retrohepatic, on the left side, or within the lesser omentum or falciform ligament and retroperitoneal. Patients with gall bladder agenesis are classified into 3 categories: i) Multiple foetal anomaly (12.9%), ii) Asymptomatic (31.6%) and iii) Symptomatic (55.6%). Notwithstanding current diagnostic modalities, this rare condition may still present a dilemma to the abdominal surgeon. Agenesis of the gall bladder is a well-recognised but uncommon congenital abnormality. With the advent of minimal access surgery laparotomy may be avoided as the condition, when suspected, may be confirmed by ERCP and CT scan.

摘要

先天性胆囊缺如是一种极其罕见的胚胎学异常,报告发病率在0.013%至0.075%之间。本报告是来自南非的首例报告,讨论了2例胆囊发育不全病例,使文献报道的病例数增至413例。在确诊胆囊发育不全时,有必要排除肝内、肝后、左侧、小网膜内或镰状韧带及腹膜后等异常位置。胆囊发育不全的患者分为3类:i)多发胎儿异常(12.9%),ii)无症状(31.6%),iii)有症状(55.6%)。尽管有目前的诊断方法,这种罕见情况仍可能给腹部外科医生带来难题。胆囊发育不全是一种公认但不常见的先天性异常。随着微创外科手术的出现,当怀疑有这种情况时,可通过内镜逆行胰胆管造影(ERCP)和CT扫描确诊,从而避免剖腹手术。

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