The hidden mortality of congenital diaphragmatic hernia: a 20-year review

The hidden mortality of congenital diaphragmatic hernia: a 20-year review
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DOI:
10.1016/j.jpedsurg.2008.10.076
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发表时间:
2009-02-01
影响因子:
2.4
通讯作者:
Sabharwal, A. J.
Sabharwal, A. J.
中科院分区:
医学3区
文献类型:
--
作者:
Brownlee, E. M.;Howatson, A. G.;Sabharwal, A. J.

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目的:与先天性膈疝(CDH)相关的真实死亡率是隐藏的,因为生存分析不包括患有CDH的胎儿。对20年来诊断为CDH的所有尸检(pm)进行回顾性审查,以突出这种隐藏的死亡率,并测量相关异常的性质和数量。方法:对1996年1月至2005年12月的死后病例资料进行回顾性分析。收集了活产、死产、治疗性流产和自然流产的数据。结果:在研究期间,pm的年数量有所下降。四个五年间隔的中位数分别为609(570-657)、528(489-565)、515(413-537)和373(357-388)。共鉴定出130例PMs,其中包括CDH的诊断;左侧97例(75%),右侧22例(17%),双侧11例(8%)。69例活产,46例治疗性流产,10例死产,5例宫内死亡;活产流产组和治疗流产组右侧/双侧流产占22%,后两组右侧/双侧流产占53%。在130例中,82例(63%)有主要相关异常,其中50%至少有1例进一步的主要异常。最常见的异常类型是心脏(30例)、胃肠道/腹壁缺陷(28例)和神经管缺陷(25例)。结论:CDH的真实发生率明显高于新生儿外科实践中的发生率。本区域pmms数量的下降将加剧对真实发病率的低估。在子宫内死亡的患者中,右侧/双侧疝的发生率较高,且不止一种主要异常。(C) 2009爱思唯尔公司版权所有。
Aims: The true mortality associated with congenital diaphragmatic hernia (CDH) is hidden because survival analyses do not include fetuses with CDH. A retrospective review of all postmortems (PMs) with a diagnosis of CDH over a 20-year period was carried out to highlight this hidden mortality and also measure the nature and number of associated anomalies.Methods: Postmortem case record details were reviewed for the period January 1996 to December 2005. Data were collected on live birth, stillbirth, therapeutic abortion, and spontaneous abortion.Results: There was a decline in the annual number of PMs during the period Of the Study. The median for the four 5-year intervals being 609 (570-657), 528 (489-565), 515 (413-537), and 373 (357-388). A total of 130 PMs were identified, which included a diagnosis of CDH; 97 (75%) were left sided, 22 (17%) were right sided, and 11 (8%) were bilateral. There were 69 live births, 46 therapeutic abortions, 10 stillbirths, and 5 intrauterine deaths; 22% were right sided/bilateral in the live and therapeutic abortion groups, whereas 53% were right sided/bilateral in the latter 2 groups. Of 130, 82 (63%) had major associated anomalies, and 50% of these had at least I further major anomaly. The commonest categories of anomalies were cardiac (30), gastrointestinal/abdominal wall defect (28), and neural tube defects (25).Conclusions: The true incidence of CDH is considerably higher than that seen in neonatal surgical practice. The decline in number of PMs in our region will exacerbate the underestimation of the true incidence. There is a higher incidence of right-sided/bilateral hernias and more than one major anomaly in those Who die in utero. (C) 2009 Elsevier Inc. All rights reserved.