Évolution respiratoire à moyen et long terme des enfants opérés d’une hernie diaphragmatique congénitale : à partir d’une série de 56 patients

Abstract

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]). This retrospective study focuses on the medium and long-term respiratory evolution of a cohort of 56children with CDH and operated at Strasbourg University Hospital between 1999and 2017. The mean age at assessment was 6,6years (minimum: 5months; maximum: 19years). Seventeen patients (30%) had asthmatic manifestations. Pulmonary Function Tests (PFT) showed obstructive patterns in 5/11patients (2with post-bronchodilator reversibility), and restrictive impairment in 5/11patients, 3of 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 48patients (86%) had GERD, 10of whom had fundoplication. 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.