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[胎儿镜手术作为孤立性先天性膈疝的产前干预手段]

[Fetoscopic surgery as prenatal intervention for isolated congenital diaphragmatic hernia].

作者信息

Mayer S, Kluth D, Till H, Deprest J

机构信息

Universität Leipzig, Klinik und Poliklinik für Kinderchirurgie, Leipzig, Deutschland.

出版信息

Zentralbl Chir. 2009 Dec;134(6):502-6. doi: 10.1055/s-0029-1224604. Epub 2009 Dec 17.

Abstract

Congenital diaphragmatic hernia (CDH) occurs sporadically with an incidence of 1:2,500 live births. Despite the progress in neonatal intensive care, CDH remains associated with a mortality of at least 30 % in isolated cases. The in essence surgically correctable defect of the diaphragm enables the prenatal herniation of abdominal organs into the thoracic cavity. The resulting abnormal development of the airways and pulmonary vessels causes neonatal respiratory insufficiency and persistent pulmonary hypertension. The condition can be diagnosed prenatally and the degree of pulmonary hypoplasia, which determines the postnatal course, can be measured to make an -individual prognosis. In severely affected patients, prenatal surgery may improve neonatal outcome by reversing pulmonary hypoplasia. This is currently implemented by percutaneous fetoscopic endoluminal tracheal occlusion (FETO) to trigger fetal lung growth. Although there are no maternal complications, preterm rupture of the membranes remains the major drawback of the procedure (20 % < 34 weeks). However, as compared to historical controls of a similar severity, survival as well as early neonatal morbidity are significantly improved by FETO. As a consequence, a multicentre randomised-controlled trial in fetuses with moderate hypoplasia on FETO compared to expectant management has been started ( www.totaltrial.eu). Primary outcome measure is survival without chronic lung disease (i. e., with-out bronchopulmonary dysplasia). A trial in severely affected -fetuses with survival as main outcome is currently under review by ethics committee. A standardised neonatal management enables optimal treatment and multicentre compatibility. It remains to be proven if fetoscopic surgery can maintain a solid position in the prenatal treatment of CDH to improve both mortality and morbidity of the affected children.

摘要

先天性膈疝(CDH)为散发性疾病,活产儿发病率为1:2500。尽管新生儿重症监护取得了进展,但在孤立病例中,CDH的死亡率仍至少为30%。本质上,膈肌的手术可纠正缺陷使得腹部器官在产前疝入胸腔。由此导致的气道和肺血管异常发育会引起新生儿呼吸功能不全和持续性肺动脉高压。该疾病可在产前诊断,且可测量决定产后病程的肺发育不全程度以进行个体预后评估。对于严重受影响的患者,产前手术可通过逆转肺发育不全来改善新生儿预后。目前通过经皮胎儿镜腔内气管阻塞术(FETO)来实现这一点,以促进胎儿肺生长。尽管没有母体并发症,但胎膜早破仍然是该手术的主要缺点(20%<34周)。然而,与历史上类似严重程度的对照组相比,FETO显著提高了生存率以及早期新生儿发病率。因此,一项关于FETO与期待治疗相比对中度发育不全胎儿进行多中心随机对照试验已经启动(www.totaltrial.eu)。主要结局指标是无慢性肺病(即无支气管肺发育不良)的生存情况。一项以生存为主要结局的严重受影响胎儿试验目前正在接受伦理委员会审查。标准化的新生儿管理可实现最佳治疗和多中心兼容性。胎儿镜手术能否在CDH的产前治疗中稳固地位以改善患病儿童的死亡率和发病率仍有待证实。

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