Duess Johannes W, Zani-Ruttenstock Elke M, Garriboli Massimo, Puri Prem, Pierro Agostino, Hoellwarth Michael E
Department of Pediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria,
Pediatr Surg Int. 2015 May;31(5):465-71. doi: 10.1007/s00383-015-3695-y. Epub 2015 Mar 24.
Right-sided congenital diaphragmatic hernia (R-CDH) occurs in 14 to 25% of all CDH cases. The current literature comparing the outcome of R-CDH vs left CDH (L-CDH) is inconsistent, with some studies reporting higher and others lower mortality in R-CDH compared to L-CDH. The aim of our multicentre study was to analyse characteristics and outcome of R-CDH.
We retrospectively reviewed the medical records of 178 consecutive infants with CDH who underwent surgical repair of CDH at three European tertiary pediatric surgical centres from three different countries between 2000 and 2009. The analysis focused on demographic data, morbidity and mortality in R-CDH compared with L-CDH.
Out of a total of 178 children, 32 (18.0%) right-sided and 146 (82.0 %) left-sided cases of CDH were identified. Prenatal diagnosis was made in 8 R-CDH vs 67 L-CDH (25.0 vs 45.9%, p = 0.030). Median gestational age in R-CDH was 39 weeks (range 29-42 weeks) and 39 weeks in L-CDH (range 28-43 weeks, p = 0.943). Median birth weight in R-CDH was 3233 g (range 905-4480 g) and in L-CDH was 3060 g (range 1065-5240 g, p = 0.184). Major associated anomalies were present in 19 R-CDH vs 46 L-CDH (59.4 vs 31.5%, p = 0.003). Extracorporeal membrane oxygenation (ECMO) was required in 3 R-CDH vs 19 L-CDH (9.4 vs 13.0%, p = 0.571). A diaphragmatic patch was used in 13 R-CDH and 59 L-CDH (40.6 vs 40.4%, p = 0.982). Fundoplication for GERD was required in 1 R-CDH and 19 L-CDH (3.1 vs 13.0 %, p = 0.109). No significant differences were observed in recurrence rate (9.4 vs 8.9%, p = 0.933). Postoperative mortality rate was significantly higher in R-CDH compared to L-CDH (21.9 vs 8.2%, p = 0.023). In R-CDH, prenatal diagnosis and patch repair correlated with mortality by univariate regression (p = 0.005 and p = 0.019).
This multicentre study shows that prenatal diagnosis and patch repair were associated with an increased mortality rate in R-CDH. However, the morbidity following repair of R-CDH was not significantly different from that in L-CDH in survivors.
右侧先天性膈疝（R-CDH）占所有先天性膈疝（CDH）病例的14%至25%。目前比较R-CDH与左侧先天性膈疝（L-CDH）预后的文献结果并不一致，一些研究报告R-CDH的死亡率高于L-CDH，而另一些研究则相反。我们多中心研究的目的是分析R-CDH的特征和预后。
我们回顾性分析了2000年至2009年间在三个欧洲三级儿科手术中心接受CDH手术修复的178例连续婴儿的病历。分析重点在于R-CDH与L-CDH的人口统计学数据、发病率和死亡率。
在总共178名儿童中，确诊为CDH的右侧病例32例（18.0%），左侧病例146例（82.0%）。8例R-CDH和67例L-CDH进行了产前诊断（25.0%对45.9%，p = 0.030）。R-CDH的中位孕周为39周（范围29 - 42周），L-CDH为39周（范围28 - 43周，p = 0.943）。R-CDH的中位出生体重为3233克（范围905 - 4480克），L-CDH为3060克（范围1065 - 5240克，p = 0.184）。19例R-CDH和46例L-CDH存在主要相关畸形（59.4%对31.5%，p = 0.003）。3例R-CDH和19例L-CDH需要体外膜肺氧合（ECMO）（9.4%对13.0%，p = 0.571）。13例R-CDH和59例L-CDH使用了膈肌补片（40.6%对40.4%，p = 0.982）。1例R-CDH和19例L-CDH因胃食管反流病需要进行胃底折叠术（3.1%对13.0%，p = 0.109）。复发率无显著差异（9.4%对8.9%，p = 0.933）。R-CDH的术后死亡率显著高于L-CDH（21.9%对8.2%，p = 0.023）。在R-CDH中，单因素回归分析显示产前诊断和补片修复与死亡率相关（p = 0.005和p = 0.019）。
这项多中心研究表明，产前诊断和补片修复与R-CDH死亡率增加相关。然而，R-CDH修复后的发病率与存活的L-CDH患者无显著差异。
