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完全性喉气管食管裂与左肺发育不全的关联:一个自然实验提供的病理生理线索

The association of complete laryngotracheoesophageal cleft with left lung agenesis: pathophysiological clues provided by an experiment of nature.

作者信息

Noorily M J, Farmer D L, Flake A W

机构信息

Department of General Surgery, Children's Hospital of Michigan, Wayne State University, Detroit 48201, USA.

出版信息

J Pediatr Surg. 1998 Oct;33(10):1546-9. doi: 10.1016/s0022-3468(98)90495-x.

Abstract

The authors present a patient with complete laryngotracheoesophageal cleft and concurrent left lung agenesis and microgastria. Prenatal ultrasound scan showed polyhydramnios and a hypertrophic right lung. The authors propose that the combination of right lung hypertrophy, polyhydramnios, and microgastria in the absence of a competent laryngeal mechanism may suggest that the preferential path for swallowed amniotic fluid was into the lung, rather than the normal route through the stomach. This case illustrates the prenatal findings suggestive of complete laryngotracheoesophageal cleft and lung agenesis, and suggests a potential causal relationship between shunting of swallowed amniotic fluid into the bronchial tree and prenatal lung hypertrophy and microgastria.

摘要

作者报告了一名患有完全性喉气管食管裂、并发左肺不发育和小胃畸形的患者。产前超声扫描显示羊水过多和右肺肥大。作者提出,在缺乏有效的喉部机制的情况下,右肺肥大、羊水过多和小胃畸形的组合可能表明,吞咽的羊水的优先路径是进入肺部,而不是通过胃的正常路径。该病例说明了提示完全性喉气管食管裂和肺不发育的产前检查结果,并提示吞咽的羊水进入支气管树与产前肺肥大和小胃畸形之间可能存在因果关系。

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