Long-Term Outcomes of Congenital Diaphragmatic Hernia Patients

Background/Purpose: Short-term outcomes of congenital diaphragmatic hernia (CDH) are well described. We present the long-term outcomes and co-morbidities found in patients who underwent CDH repair at our institution.

Methods: We conducted a retrospective review of all patients from 2004 – 2019 who underwent repair of CDH with survival to discharge. Means and percentages were used for analysis of the data.

Results: A total of 176 patients underwent CDH repair with survival to discharge at our institution between 2004 and 2019, 71 females (41.0%) and 104 males (59.0%). Left side defect was most common (77.8%), followed by right side (18.75%), Morgagni (2.27%) and bilateral defects (0.57%). Average age at repair was 16 days with 56.8% of repairs requiring a patch. Extracorporeal membrane oxygenation (ECMO) was required in 32 patients (18.2%) and average length of stay after birth was 42 days.

Patients were followed for an average of 61 months. Long term outcomes included recurrence of diaphragmatic hernia (16.6%), asthma (21.6%), neurodevelopmental delay (26.7%), attention deficit hyperactivity disorder (7.9%), autism (1.7%), chest wall deformity (14.2%) with pectus excavatum the most common abnormality, scoliosis (11.4%), inguinal hernia (7.4%) and undescended testis in 20% of males. Gastroesophageal reflux disease (GERD) was diagnosed in 80.1% of patients at discharge and 43.6% on long term follow-up. Sixty-one of these patients underwent esophagogastroduodenoscopy for which 22.9% were also diagnosed with eosinophilic esophagitis. Of patients diagnosed with GERD, 57.7% underwent gastric or gastrojejunostomy tube placement while 16.9% underwent fundoplication. At most recent follow-up, 30.6% of patients required medication for GERD.

Conclusion: With increasing survival in CDH patients, appropriate screening for and treatment of long-term outcomes unique to this patient population should be further investigated.