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喉气管食管裂

[Laryngotracheoesophageal cleft].

作者信息

Baeza Herrera C, Santos Mariscal F, Pérez Zamudio B, González Carballeda F

出版信息

Bol Med Hosp Infant Mex. 1981 May-Jun;38(3):485-92.

PMID:7271979
Abstract

Laryngotracheoesophageal cleft is a rare congenital anomaly that should be considered in the differential diagnosis of any neonatal respiratory distress aggravated by feeding. Diagnosis is difficult, but can be made by roentgenographic and endoscopic techniques. Roentgenographic evidence of abnormal positioning of nasogastric tubes should arouse suspicion. Repeated bronchoscopy may be necessary to identify the defect. Immediate surgical repair is indicated. Incorporation of part of the esophageal wall into the repair may enhance success. Tracheostomy has been required in all survivors. The embryologic pathogenesis of the anomaly is discussed and it is concluded that the defect is found more often than is reported. We present the fifty-fourth case, the first associated to hypospadias in the world, and the first case in the Mexican literature. This child presented severe respiratory distress. Death occurred on the tenth neonatal day.

摘要

喉气管食管裂是一种罕见的先天性畸形,在鉴别诊断任何因喂养而加重的新生儿呼吸窘迫时均应考虑到。诊断困难,但可通过X线和内镜技术作出。鼻胃管位置异常的X线证据应引起怀疑。可能需要反复进行支气管镜检查以确定缺损。应立即进行手术修复。将部分食管壁纳入修复可能会提高成功率。所有存活者均需行气管切开术。文中讨论了该畸形的胚胎发病机制,并得出结论认为该缺损的实际发现率高于报道。我们报告了第54例病例,这是世界上首例与尿道下裂相关的病例,也是墨西哥文献中的首例。该患儿出现严重呼吸窘迫,于出生后第10天死亡。

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