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伴有左心室憩室的坎特雷尔五联症:一例报告并文献复习

Pentalogy of Cantrell with left ventricular diverticulum: a case report and review of literature.

作者信息

Singh Navdeep, Bera M L, Sachdev Manvinder S, Aggarwal Neeraj, Joshi Raja, Kohli Vikas

机构信息

Pediatric Cardiology and Congenital Cardiac Surgery Unit, Indraprastha Apollo Hospital, New Delhi, India.

出版信息

Congenit Heart Dis. 2010 Sep-Oct;5(5):454-7. doi: 10.1111/j.1747-0803.2009.00375.x.

Abstract

Pentalogy of Cantrell is a rare congenital anomaly involving deficiency of the following structures: anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and associated congenital intracardiac abnormality. We describe a 3-month-old child with this syndrome having left ventricular diverticulum along with omphalocele who presented to us with a pulsating mass in the epigastrium. The defect was evaluated and defined by computed tomography scan. A team of pediatric, cardiac, and plastic surgeons successfully repaired the defects. This case report discusses the review of literature along with management options and concludes that there must be an emphasis on early repair of left ventricular diverticulum to prevent complications. Antenatal ultrasound can also detect the anomaly, and early postnatal diagnosis of the syndrome, followed by immediate surgical repair, can prevent lethal complications.

摘要

坎特雷尔五联症是一种罕见的先天性异常,涉及以下结构的缺损:前膈、脐上腹壁、膈心包、下胸骨以及相关的先天性心脏内异常。我们描述了一名患有此综合征的3个月大儿童,伴有左心室憩室和脐膨出,其因上腹部有搏动性肿块前来就诊。通过计算机断层扫描对缺损进行了评估和界定。一组儿科、心脏和整形外科医生成功修复了这些缺损。本病例报告讨论了文献回顾以及治疗方案,并得出结论,必须强调早期修复左心室憩室以预防并发症。产前超声也可检测到该异常,出生后尽早诊断该综合征并立即进行手术修复可预防致命并发症。

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