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孤立性V形胸骨裂——一种罕见的胸壁畸形

Isolated V-Shaped Sternal Cleft - A Rare Chest Wall Malformation.

作者信息

Gebremariam Dawit Seyoum, Miruts Asmamaw, Desta Kibrom Gebreselassie

机构信息

Department of Pediatrics and Child Health, College of Health Sciences, Mekelle University, Mekelle, Tigray, Ethiopia.

Department Surgery, Pediatric Surgery Unit, College of Health Sciences, Mekelle University, Mekelle, Tigray, Ethiopia.

出版信息

Pediatric Health Med Ther. 2023 Mar 4;14:81-87. doi: 10.2147/PHMT.S397462. eCollection 2023.

Abstract

Sternal cleft is a rare chest wall anomaly resulting from a failure of the lateral mesodermal folds to migrate to the midline, causing a cleft in the early stage of embryological development. This can be a complete or partial defect. It can also occur as an isolated anomaly or in association with other syndromes. Fetal sonographic diagnosis of this defect is possible, but less practiced. After birth, this defect can be easily diagnosed clinically because of the presence of paradoxical chest wall movement. The flexibility of the thorax is maximal and compression of the underlying structures is minimal during the neonatal and early infancy period, and this period is the preferred time for surgical repair. We report a 39-day-old infant who presented with an isolated V-shaped inferior sternal cleft, its surgical primary closure, and postoperative course.

摘要

胸骨裂是一种罕见的胸壁畸形,由外侧中胚层皱襞未能迁移至中线所致,在胚胎发育早期形成裂隙。可为完全性或部分性缺损,也可作为孤立性畸形出现,或与其他综合征相关。胎儿超声检查有可能诊断出这种缺损,但较少应用。出生后,由于存在反常胸壁运动,这种缺损很容易通过临床诊断。在新生儿期和婴儿早期,胸廓的柔韧性最大,对其下方结构的压迫最小,此期是手术修复的最佳时机。我们报告一例39日龄婴儿,其表现为孤立的V形低位胸骨裂、首次手术闭合及术后病程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8348/9994661/1314d5561b8c/PHMT-14-81-g0001.jpg

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