Langham M R, Kays D W, Ledbetter D J, Frentzen B, Sanford L L, Richards D S
Department of Surgery, University of Florida, College of Medicine, Gainesville, USA.
Clin Perinatol. 1996 Dec;23(4):671-88.
Congenital diaphragmatic hernia is a relatively common birth defect. It affects about 1114 babies a year in the United States. Reported survival averages 60% but may be significantly lower. We do not understand the etiology of CDH. Its association with other anomalies and several distinct patterns of presentation suggest that more than one cause may exist. There is a high degree of variability in both treatment and outcomes, but no data exist to allow a rigorous comparison of the efficacy of various treatment strategies. Stratification of patients into more homogeneous groups will be a necessary prerequisite for the design of meaningful comparative trials. The incidence of the lesion prevents any single institution from accruing sufficient patients to conduct such a trial. An ad hoc multicenter study group (the Congenital Diaphragmatic Hernia Study Group) has been formed for this purpose. This organization has begun collecting data with an initial goal of developing a stratification scheme. Prospective data collection should allow verification of several of the estimates made in this article. Current data make it clear that CDH represents a major cause of perinatal morbidity and mortality.
先天性膈疝是一种相对常见的出生缺陷。在美国，每年约有1114名婴儿受其影响。据报道，其平均存活率为60%，但可能会显著更低。我们尚不了解先天性膈疝的病因。它与其他异常情况以及几种不同的表现模式表明，可能存在不止一种病因。在治疗和预后方面存在高度变异性，但尚无数据可用于严格比较各种治疗策略的疗效。将患者分层为更同质化的组将是设计有意义的对照试验的必要前提。该病变的发病率使得任何单一机构都无法积累足够数量的患者来开展此类试验。为此已成立了一个特设多中心研究小组（先天性膈疝研究小组）。该组织已开始收集数据，初步目标是制定一种分层方案。前瞻性数据收集应能验证本文中的几项估计。目前的数据清楚地表明，先天性膈疝是围产期发病和死亡的一个主要原因。
