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混乱:一份胎儿尸检报告。

CHAOS: A fetal autopsy report.

作者信息

Chawla Latika, Grover Nitika, Joshi Prashant, Singh Preeti

机构信息

Department of Obstetrics & Gynecology, AIIMS, Rishikesh, Uttarakhand, India.

出版信息

J Family Med Prim Care. 2020 Aug 25;9(8):4448-4450. doi: 10.4103/jfmpc.jfmpc_792_20. eCollection 2020 Aug.

Abstract

Congenital high airway obstruction syndrome (CHAOS) is a rare congenital malformation, which results from deficient recanalization of the upper airways. Laryngeal atresia is the most common cause, other etiologies being trachea atresia, laryngeal or tracheal webs, subglottic stenosis, obstructing laryngeal cysts, and laryngeal or tracheal agenesis. There is decreased clearance of the fluid produced by fetal lungs due to obstruction leading to increased intratracheal pressure and thereby secondary proliferative lung growth. The heart becomes compressed in the midline due to hyperexpansion of the lungs causing elevated intrathoracic pressure, decreased venous return, and fetal cardiac failure. This sequence causes ascites, placento-megaly, and eventually hydrops fetalis. We present a case of antenatal diagnosis of a fetus with CHAOS corroborated by fetal autopsy.

摘要

先天性高气道梗阻综合征(CHAOS)是一种罕见的先天性畸形,由上呼吸道再通不全引起。喉闭锁是最常见的原因,其他病因包括气管闭锁、喉或气管蹼、声门下狭窄、阻塞性喉囊肿以及喉或气管发育不全。由于梗阻导致胎儿肺产生的液体清除减少,从而引起气管内压力升高,进而导致继发性肺增生性生长。由于肺部过度扩张导致胸腔内压力升高、静脉回流减少和胎儿心力衰竭,心脏在中线处受到压迫。这一系列情况会导致腹水、胎盘肿大,最终导致胎儿水肿。我们报告一例经胎儿尸检证实的CHAOS胎儿产前诊断病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8bc3/7586612/fc77669d526d/JFMPC-9-4448-g001.jpg

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