Sydorak Roman M, Harrison Michael R
Department of Surgery, Fetal Treatment Center, University of California-San Francisco, 513 Parnassus Avenue, HSW-1601, San Francisco, CA 94143-0570, USA.
Clin Perinatol. 2003 Sep;30(3):465-79. doi: 10.1016/s0095-5108(03)00057-5.
Despite intensive clinical and experimental efforts, mortality from CDH remains high. More than two decades of research in multiple centers has led to a better understanding of the pathophysiology, prognosis, and treatment options for fetuses that have CDH. It now appears that fetuses that have prenatally diagnosed CDH can be stratified into high- and low-risk groups based upon sonographic parameters. Fetuses that do not have liver herniation into the chest that have a favorable LHR have an excellent chance of survival with postnatal therapy. Prenatal diagnosis allows the time and place of delivery to be planned in advance so these infants can be treated in a tertiary care nursery that has maximal medical and surgical therapy. Fetuses that have liver herniation into the chest and an unfavorable LHR have a grim prognosis. These fetuses might benefit from in utero intervention. There is no role for open fetal repair of the diaphragmatic detect; however, fetoscopic temporary tracheal occlusion might improve lung growth and development and might decrease morbidity and mortality in these infants. The FETENDO strategy appears to work, and for the first time it offers hope to the fetus that has high-risk CDH, but its efficacy must be proven in a proper randomized, controlled trial.
尽管进行了深入的临床和实验研究，但先天性膈疝（CDH）的死亡率仍然很高。多个中心二十多年的研究使人们对患有CDH的胎儿的病理生理学、预后和治疗选择有了更好的理解。现在看来，产前诊断为CDH的胎儿可以根据超声参数分为高风险和低风险组。胸部无肝脏疝出且肺头比（LHR）良好的胎儿经产后治疗有极好的存活机会。产前诊断可以提前规划分娩时间和地点，以便这些婴儿能够在具备最大程度医疗和手术治疗条件的三级护理新生儿重症监护室接受治疗。胸部有肝脏疝出且LHR不利的胎儿预后不佳。这些胎儿可能会从宫内干预中获益。开放性胎儿膈疝修补术并无作用；然而，胎儿镜下临时气管阻塞可能会改善肺的生长发育，并可能降低这些婴儿的发病率和死亡率。胎儿内镜下气管阻塞治疗先天性膈疝策略（FETENDO）似乎有效，它首次为患有高风险CDH的胎儿带来了希望，但其疗效必须在适当的随机对照试验中得到证实。
