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先天性膈疝

Congenital diaphragmatic hernia.

作者信息

Ersöz Köse Elçin, Yalçınkaya İrfan

机构信息

Department of Thoracic Surgery, Health Sciences University Istanbul Hamidiye Medical Faculty, Süreyyapaşa Chest Diseases and Thoracic Surgery Hospital, Istanbul, Türkiye.

出版信息

Turk Gogus Kalp Damar Cerrahisi Derg. 2024 Feb 5;32(Suppl1):S89-S97. doi: 10.5606/tgkdc.dergisi.2024.25705. eCollection 2024 Jan.

Abstract

Congenital diaphragmatic hernia (CDH) is a rare developmental defect of the diaphragm, characterized by the herniation of abdominal contents into the chest, resulting in varying degrees of pulmonary hypoplasia and pulmonary hypertension. Significant advances in the prenatal diagnosis and identification of prognostic factors have resulted in the continued refinement of the approach to fetal therapies for CDH. In the postnatal period, protocolized approaches to lung-protective ventilation, nutrition, prevention of infection, and early aggressive management of pulmonary hypertension have led to improved outcomes in infants with CDH. Surgical repair of CDH is not urgent in most circumstances and can be delayed until the pulmonary status of the patient has stabilized. This article provides a comprehensive review of CDH, focusing on the complex pathophysiology, advances in prenatal diagnosis, fetal interventions, and optimal postnatal management of CDH.

摘要

先天性膈疝(CDH)是一种罕见的膈肌发育缺陷,其特征是腹腔内容物疝入胸腔,导致不同程度的肺发育不全和肺动脉高压。产前诊断和预后因素识别方面的重大进展使得先天性膈疝胎儿治疗方法不断完善。在出生后阶段,采用标准化的肺保护性通气、营养、预防感染以及早期积极治疗肺动脉高压的方法,已改善了先天性膈疝婴儿的预后。在大多数情况下,先天性膈疝的手术修复并不紧急,可以推迟到患者的肺部状况稳定后进行。本文对先天性膈疝进行了全面综述,重点关注其复杂的病理生理学、产前诊断进展、胎儿干预措施以及先天性膈疝的最佳出生后管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/793d/10995690/af900a717167/TJTCS-2024-32-10-089-097-F1.jpg

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