[Medium and long-term respiratory outcome in patients operated from congenital diaphragmatic hernia: From a series of 56 patients].

Introduction: Recent advances in prenatal diagnosis, high frequency oscillatory ventilation and pulmonary arterial hypertension (PAH) therapies have progressively improved the survival of patients with congenital diaphragmatic hernia (CDH). However, CDH is associated with high morbidity that affects about half of surviving children. The main sequelae observed are respiratory (asthma, persistent PAH [PPAH], bronchopulmonary dysplasia, respiratory infections) and digestive (gastroesophageal reflux disease [GERD]).
Method: This retrospective study focuses on the medium and long-term respiratory evolution of a cohort of 56 children with CDH and operated at Strasbourg University Hospital between 1999 and 2017.
Results: The mean age at assessment was 6,6 years (minimum: 5 months; maximum: 19 years). Seventeen patients (30 %) had asthmatic manifestations. Pulmonary Function Tests (PFT) showed obstructive patterns in 5/11 patients (2 with post-bronchodilator reversibility), and restrictive impairment in 5/11 patients, 3 of whom had thoracic deformity and/or scoliosis. Thirteen patients (23 %) had bronchopulmonary dysplasia. Few patients had recurrent respiratory infections (3.6 %) and chronic respiratory insufficiency (5.3 %). Thirty-nine patients (70 %) presented with an initial PAH, two of whom progressed to PPAH. Sixteen patients (29 %) had thoracic deformity and/or scoliosis and 48 patients (86 %) had GERD, 10 of whom had fundoplication.
Conclusion: The long-term respiratory outcome of CDH operated patients is characterized by a moderate prevalence of respiratory symptoms and alterations in PFT that are important to consider in their medical follow-up during adolescence and adulthood. PAH is common in the neonatal period but rarely seems to persist.
(Copyright © 2018. Published by Elsevier Masson SAS.)