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临床问答——库拉里诺综合征背景下的罕见肛管重复畸形病例

Clinical Quiz-A Rare Case of Anal Canal Duplication in the Context of Currarino Syndrome.

作者信息

Connor Sean Jared, Brisighelli Giulia, Patel Nirav, Levitt Marc A

机构信息

Department of Paediatric Surgery, Chris Hani Baragwanath Hospital, Johannesburg, Gauteng, South Africa.

University of the Witwatersrand School of Clinical Medicine, Johannesburg, Gauteng, South Africa.

出版信息

European J Pediatr Surg Rep. 2021 Nov 10;9(1):e68-e71. doi: 10.1055/s-0041-1735595. eCollection 2021 Jan.

Abstract

Currarino syndrome (CS) is a rare condition that presents with any combination of a sacral defect, a presacral mass, and an anorectal malformation. This collection, referred to as Currarino's triad, may not necessarily present as all three abnormalities in the diagnosis of the syndrome. Anal canal duplication (ACD) is an even rarer occurrence. We present a case that lies on the CS spectrum with an associated ACD and discuss a complex surgical challenge that necessitated a customized management plan, devised through a multidisciplinary approach.

摘要

库拉里诺综合征(CS)是一种罕见病症,表现为骶骨缺损、骶前肿块和肛门直肠畸形的任意组合。这种组合,即所谓的库拉里诺三联征,在该综合征的诊断中不一定会同时出现所有三种异常情况。肛管重复畸形(ACD)则更为罕见。我们报告一例属于CS谱系且伴有ACD的病例,并讨论了一个复杂的手术挑战,这需要通过多学科方法制定定制化的管理方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/62dd/8580735/a9e4b712e086/10-1055-s-0041-1735595-i210591-1.jpg

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