Prenatal diagnosis and management of congenital diaphragmatic hernia
Prenatal diagnosis and management of congenital diaphragmatic hernia
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DOI：
10.1016/j.bpobgyn.2018.12.010
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发表时间：
2019-07-01
影响因子：
5.5
通讯作者：
Deprest, Jan
中科院分区：
文献类型：
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作者：
Basurto, David;Russo, Francesca Maria;Deprest, Jan
先天性膈疝的特征是横膈膜闭合失败，从而使腹部内脏疝入胸腔，随后干扰正常的肺发育。出生时，肺发育不良导致呼吸功能不全和持续性肺动脉高压（PHT），这在高达32%的患者中是致命的。在个别病例中，可以通过医学成像和先进的基因检测在产前预测结果。在那些预测预后不良的胎儿中，可以提供胎儿镜下腔内气管闭塞。该手术目前正在全球随机临床试验（www.TOTALtrial.欧盟）。我们目前正在研究替代策略，包括经胎盘西地那非给药，以减少持续性PHT的发生。(C)2018由Elsevier Ltd.出版
Congenital diaphragmatic hernia is characterized by failed closure of the diaphragm, thereby allowing abdominal viscera to herniate into the thoracic cavity and subsequently interfering with normal lung development. At birth, pulmonary hypoplasia leads to respiratory insufficiency and persistent pulmonary hypertension (PHT), that is lethal in up to 32% of patients. In isolated cases, the outcome may be predicted prenatally by medical imaging and advanced genetic testing. In those fetuses with a predicted poor outcome, fetoscopic endoluminal tracheal occlusion may be offered. This procedure is currently being evaluated in a global randomized clinical trial (www.TOTALtrial. eu ). We are currently investigating alternative strategies including transplacental sildenafil administration to reduce the occurrence of persistent PHT. (C) 2018 Published by Elsevier Ltd.