Parent reported long-term quality of life outcomes in children after congenital diaphragmatic hernia repair

Abstract

Purpose: The aim of this study was to determine long-term outcomes for congenital diaphragmatic hernia (CDH) patients including quality of life (QoL), symptom burden, reoperation rates, and health status.

Methods: A chart review and phone QoL survey were performed for patients who underwent CDH repair between 2007 and 2014 at a tertiary free-standing children's hospital. Comprehensive outcomes were collected including subsequent operations and health status. Associations with QoL were tested using Wilcoxon Rank-Sum tests and Pearson correlation coefficients.

Results: Of 102 CDH patients identified, 46 (45.1%) patient guardians agreed to participate with mean patient age of 5.8 (SD, 2.2) years at time of follow-up. Median PedsQL^TM and PedsQL^TM Gastrointestinal scores were 91.8 (IQR, 84.8-95.8) and 95.8 (IQR, 93.0-98.2), out of 100. Thoracoscopic repair was associated with higher PedsQL^TM scores while defects with an intrathoracic stomach were associated with increased gas and bloating. No difference in QoL was found when comparing defect side, patch vs primary repair, prenatal diagnosis, extracorporeal membrane oxygenation, or recurrence. Older age weakly correlated with worse school functioning and heartburn.

Conclusion: Children with CDH have reassuring QoL scores. Given the correlation between older age and poor school function, longer follow-up of patients with CDH may be warranted.

Level of evidence: III (Retrospective comparative study).

Keywords: Congenital diaphragmatic hernia; Long term outcomes; Quality of life.