在喀麦隆雅温德省记录一个特殊的病例

Kamsu Zicfried, Aurore Albane Essomba, L. Makowa, Raïssa Monayong Mendomo, -. SoniaZambouZeba, Ze, Audrey Thérèse Mbang, Christiale Batibonak, Oummy Djamila Ngapout
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引用次数: 1

摘要

OEIS综合征中最复杂形式的泄殖腔外翻以脐膨出、膀胱外翻、肛门闭锁和脊柱裂为特征。我们报告一个特殊的情况下,泄殖腔外翻与OEIS复杂和进一步的出生缺陷。强调了诊断和治疗方法,以及在资源有限的情况下遇到的困难。这是一个1天大的新生儿转介管理多种先天性畸形,包括产前诊断畸形尿病。入院时,临床表现包括:1型脐膨出,肛门直肠畸形伴直肠尿瘘和覆盖腰骶畸形。临床前心脏和经囟门超声检查显示相关的心脏异常和有利于腰骶发育异常的发现。给予支持性护理,并于第20天进行出生缺陷手术重建。总之,在新生儿中,特别是当出现OEIS并发症或综合征时,泄殖腔外翻是一种罕见的先天性多畸形综合征。他们需要及时诊断和产后立即多学科管理,并长期随访,以获得良好的结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cloacal Exstrophy: Documenting a Particular Case in Yaoundé, Cameroon
Cloacal exstrophy in its most complex form with OEIS syndrome is characterized by the existence of an Omphalocele, Exstrophied bladder, Imperforate anus, and Spina bifida. We report a particular case of Cloacal exstrophy with OEIS complex and further birth defects. The diagnostic and therapeutic approaches, as well as difficulties encountered in a limited resource setting, are highlighted. This was a 1-day old neonate referred for the management of multiple congenital malformations, including antenatal diagnosis of malformative uropathy. On admission, the clinical findings included: a type 1 omphalocele, an anorectal malformation with a recto-urinary fistula and a covered lumbosacral dysraphism. Paraclinical examinations with cardiac and trans fontanelle ultrasound revealed associated cardiac anomaly and findings in favor of lombo-sacral dysraphism. Supportive care was given and surgical reconstruction of birth defects on day 20 was done. In conclusion, cloacal exstrophy is a rare morbid congenital polymalformative syndrome in neonates, especially when presenting with OEIS complex or syndrome. They require prompt diagnosis and immediate postnatal multidisciplinary management, with long-term follow-up for a favorable outcome.
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