Kadir Darya, Lilja Helene Engstrand
Department of Women's and Children's Health, Section of Pediatric Surgery, Uppsala University, 751 85, Uppsala, Sweden.
Department of Pediatric Surgery, University Children's Hospital, Uppsala, Sweden.
Pediatr Surg Int. 2017 Mar;33(3):317-323. doi: 10.1007/s00383-016-4032-9. Epub 2016 Dec 16.
The management of congenital diaphragmatic hernia (CDH) is a major challenge. The mortality is dependent on associated malformations, the severity of pulmonary hypoplasia, pulmonary hypertension and iatrogenic lung injury associated with aggressive mechanical ventilation. The aims of the study were to investigate the mortality over time in a single paediatric surgical centre, to compare the results with recent reports and to define the risk factors for mortality.
The medical records of infants with CDH from two time periods: 1995-2005 and 2006-2016 were reviewed. Cox regression was used for statistical analysis.
The study included 113 infants. The mortality rate was significantly decreased in the later time period, compared to the earlier, 4.4 and 17.9%, respectively. At the early time period five patients (7.5%) were treated with ECMO and in the later time period ECMO was used in three patients (6.5%). The mortality in ECMO-treated patients was 50% in both time periods. Prenatal diagnosis, intrathoracic liver, low Apgar score and low birth weight were defined as independent risk factors for mortality.
Despite no significant differences in the incidence of independent risk factors and the use of ECMO between the two time periods, mortality decreased over time. The mortality was lower than previously reported. The results indicate that there are many important factors involved in a successful outcome after CDH repair. Large multicentre studies are necessary to define those critical factors and to determine optimal treatment strategies.
先天性膈疝（CDH）的治疗是一项重大挑战。死亡率取决于相关畸形、肺发育不全的严重程度、肺动脉高压以及与积极机械通气相关的医源性肺损伤。本研究的目的是调查单一儿科手术中心随时间推移的死亡率，将结果与近期报告进行比较，并确定死亡的危险因素。
回顾了1995 - 2005年和2006 - 2016年两个时间段内患有CDH的婴儿的病历。采用Cox回归进行统计分析。
该研究纳入了113名婴儿。与早期相比，后期的死亡率显著降低，分别为4.4%和17.9%。在早期，5名患者（7.5%）接受了体外膜肺氧合（ECMO）治疗，在后期，3名患者（6.5%）使用了ECMO。两个时间段内接受ECMO治疗的患者死亡率均为50%。产前诊断、胸腔内肝脏、低阿氏评分和低出生体重被确定为死亡的独立危险因素。
尽管两个时间段内独立危险因素的发生率和ECMO的使用没有显著差异，但死亡率随时间下降。死亡率低于先前报告。结果表明，CDH修复术后成功预后涉及许多重要因素。需要进行大型多中心研究来确定这些关键因素并确定最佳治疗策略。
