Congenital diaphragmatic hernia presenting with symptoms within the first day of life; outcomes from a non-ECMO centre in Denmark

Ulla Lei Larsen,Søren Jepsen,Niels Qvist,Palle Toft,Thomas Strøm

doi:10.1186/s12887-020-02072-2

Abstract

BackgroundBetween 1998 and 2015, we report on the survival of congenital diaphragmatic hernia (CDH)-infants presenting with symptoms within the first 24 h of life, treated at Odense University Hospital (OUH), a tertiary referral non-extracorporeal membrane oxygenation (ECMO) hospital for paediatric surgery.MethodsWe performed a retrospective cohort study of prospectively identified CDH-infants at our centre. Data from medical records and critical information systems were obtained. Baseline data included mode of delivery and infant condition. Outcome data included 24-h, 28-day, and 1 year mortality rates and management data included intensive care treatment, length of stay in the intensive care unit, time of discharge from hospital, and surgical intervention. Descriptive analyses were performed for all variables. Survivors and non-survivors were compared for baseline and treatment data.ResultsNinety-five infants were identified (44% female). Of these, 77% were left-sided hernias, 52% were diagnosed prenatally, and 6.4% had concurrent malformations. The 28-day mortality rate was 21.1%, and the 1 year mortality rate was 22.1%. Of the 21 non-survivors, nine died within the first 24 h, and 10 were sufficiently stabilised to undergo surgery. A statistically significant difference was observed between survivors and non-survivors regarding APGAR score at 1 and 5 min., prenatal diagnosis, body length at birth, and delivery at OUH.ConclusionsOur outcome results were comparable to published data from other centres, including centres using ECMO.

Highlights

Between 1998 and 2015, we report on the survival of congenital diaphragmatic hernia (CDH)-infants presenting with symptoms within the first 24 h of life, treated at Odense University Hospital (OUH), a tertiary referral non-extracorporeal membrane oxygenation (ECMO) hospital for paediatric surgery
Late-presenting CDH has an overall good outcome [4], when compared with infants presenting with symptoms in the neonatal period, which often require stabilising intensive care therapy
Twenty-five infants presented with symptoms after 24 h of life and these cases were excluded from the study

Summary

Introduction

Between 1998 and 2015, we report on the survival of congenital diaphragmatic hernia (CDH)-infants presenting with symptoms within the first 24 h of life, treated at Odense University Hospital (OUH), a tertiary referral non-extracorporeal membrane oxygenation (ECMO) hospital for paediatric surgery. Congenital diaphragmatic hernia (CDH) is a rare, but serious congenital malformation. The reported overall mortality is between 40 and 48% depending on the CDH-population, and an incidence of 0.08–0.38/1000 live born infants is described [1, 2]. The majority of CDH-cases are left-sided, but right-sided and, in rare cases, bilateral hernias may occur [3]. Late-presenting CDH has an overall good outcome [4], when compared with infants presenting with symptoms in the neonatal period, which often require stabilising intensive care therapy. Further deterioration increases right ventricular strain and eventually, circulatory failure may occur

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