Congenital diaphragmatic hernia, etiology and management, a 10-year analysis of a single center
Congenital diaphragmatic hernia, etiology and management, a 10-year analysis of a single center
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DOI：
10.1007/s00404-007-0407-4
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发表时间：
2008-01-01
影响因子：
2.6
通讯作者：
von Kaisenberg, Constantin S.
中科院分区：
文献类型：
--
作者：
Beck, Cornelia;Alkasi, Oezkan;von Kaisenberg, Constantin S.
目的分析基尔大学1995年至2004年10年间先天性膈疝（CDH）的诊断，以制定改进产前诊断的策略，以便能够考虑对选定病例进行内镜治疗，并评估目前的产后治疗策略。方法数据来源于胎儿医学超声科、出生登记处、死亡登记处、新生儿重症监护病房、儿科外科和遗传数据库。对染色体、遗传综合征、分离性CDH和相关异常的数据进行亚选择，评估肺头比和肺体积。分别分析诊断时的妊娠情况、CDH类型、围产期处理和产后结局。结果29例CDH患儿中，10/29例（34%）父母要求终止妊娠，其中2例已在妊娠期间死亡，12/19例（63%）存活，从新生儿重症监护病房出院，7/19例（37%）新生儿在医院死亡，其中5/7例（71%）在基尔分娩。产前诊断分别为16/29（55%）、1/16（6%）、7/16（43%）和8/16（50%）。10/29（34%）的诊断是在产后进行的，3/29（10%）的诊断是在终止妊娠后尸检进行的。当肝脏在腹部时，9/10（90%）的患儿存活，而当肝脏在胸腔时，只有3/8（43%）存活。24周时肺头比为0.81，因肺发育不全而死亡。结论CDH总生存率在50%左右，如果诊断在妊娠中期早期，可考虑产前内镜治疗，可采用肺头比、相关缺陷、染色体状况等选择标准。
Objective To analyze congenital diaphragmatic hernia (CDH) during a 10-year period at the University of Kiel, from 1995 through 2004, in order to develop a strategy to improve prenatal diagnosis, to be able to consider endoscopical treatment for selected cases and to assess the current postnatal treatment strategies.Methods Data were obtained from the fetal medicine ultrasound department, from the birth registry, from the postmortem registry, from the neonatal intensive care unit, from pediatric surgery and from the genetic database. Data were subselected for chromosomes, genetic syndromes, for isolated CDH and for associated anomalies, the lung to head ratio and lung volumes were assessed. Data were analyzed respectively for gestation at diagnosis, the type of CDH, the perinatal management and the postnatal outcome.Results There were 29 cases of CDH, in 10/29 (34%) the parents requested termination of pregnancy of which two had already died during pregnancy, 12/19 (63%) survived, which was defined as discharge from the neonatal intensive care unit, seven newborns 7/19 (37%) died in the hospital, 5 of these 5/7 (71%) were delivered in Kiel. A prenatal diagnosis was performed in 16/29 (55%), 1/16 (6%), 7/16 (43%) and 8/16 (50%) in the 1st, 2nd and 3rd trimester, respectively; in 10/29 (34%) diagnosis was performed postpartum, in 3/29 (10%) the diagnosis was performed at autopsy following termination of pregnancy. When the liver was in the abdomen, 9/10 (90%) of the children survived, compared to only 3/8 (43%) when the liver was located in the thorax. A lung to head ratio of 0.81 at 24 weeks resulted in death due to pulmonary hypoplasia.Conclusions The overall survival in CDH is around 50%, antenatal endoscopical therapy may only be considered, if the diagnosis is performed in the early second trimester, and selection criteria such as the lung to head ratio, associated defects and the chromosomal status can be applied.