Actual Outcome in Infants with Congenital Diaphragmatic Hernia: The Role of a Standardized Postnatal Treatment Protocol

Abstract

Background: Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly with a high rate of mortality and morbidity. Objective: Our aim was to determine a possible effect of standardized treatment on outcome in infants with CDH. Methods: All prenatally diagnosed patients with unilateral CDH born alive between January 2006 and December 2009 at the Erasmus MC or the University Hospital Mannheim were eligible for inclusion. Patients who underwent a fetal tracheal occlusion were excluded. From November 1, 2007, all CDH patients were treated according to a standardized treatment protocol. Patients were divided into two chronological groups according to their date of birth: without standardized treatment (group 1, Jan 2006–Oct 2007) and with standardized treatment (group 2, Nov 2007–Dec 2009). Outcome measures were mortality by day 28, bronchopulmonary dysplasia (BPD), defined as oxygen dependency at day 28, and need for extracorporeal membrane oxygenation (ECMO) therapy. Uni- and multivariate analyses were performed. Results: 167 patients were included. By day 28, 18% of the infants had died. Of the patients who were alive at day 28, 49% had BPD. An ECMO procedure was performed in 31% of the patients. Overall mortality for all included patients was 22%. In group 1, overall mortality was 33% and in group 2 overall mortality was 12% (p = 0.004). A standardized treatment protocol was independently associated with a reduced risk for mortality by day 28 (OR 0.28, 95% CI 0.11–0.68). Higher observed-to-expected lung-to-head ratios were independently associated with a lower risk for mortality by day 28 (OR 0.97, 95% CI 0.95–0.99), BPD (OR 0.97, 95% CI 0.94–0.98) and need for ECMO (OR 0.98, 95% CI 0.96–0.99). An intrathoracic position of the liver was independently associated with an increased risk for BPD (OR 3.12, 95% CI 1.41–6.90) and need for ECMO therapy (OR 3.25, 95% CI 1.54–6.88). Conclusion: Survival rates in patients with CDH increased significantly after the implementation of a standardized treatment protocol.

Diagnosis-related deterioration of lung function after extracorporeal membrane oxygenation

“…Not all patients were tested at all three time points, which created an unfavourable but unavoidable heterogeneity in the age distribution. Our ventilation strategies and ECMO treatment protocols have indeed been adjusted over the past decade, resulting in better survival in CDH patients [26]. Recent protocols based on meta-analysis of retrospective studies provide guidelines for the use of ECMO in CDH patients [27].…”

“…The severity of lung hypoplasia and PPHN in CDH patients covers a wide range. ECMO-treated CDH patients are regarded as the most severe cases [20,26]. Their improved survival might be counterbalanced by increased morbidity.…”

Manejo pré-natal da hérnia diafragmática congênita: presente, passado e futuro

“…No entanto, o real beneficio do ECMO ainda não está completamente comprovado na HDC 19 . Mais recentemente, em alguns centros, a mortalidade vem caindo para 20 a 30% com a instituição de protocolos de tratamento padronizados 20,21 .…”

Congenital Diaphragmatic Hernia Survivors: Outcomes in Infancy, Childhood and Adolescence

“…Given the small numbers of CDH cases occurring at individual centres, cooperation and collaboration between centres and internationally is essential to develop such protocols, and to enhance further research in this field via randomized controlled trials and investigation into long-term outcomes among survivors (van den Hout et al, 2011;Wright et al, 2011).…”