Tracheostomy and Chronic Ventilator Dependence After Congenital Diaphragmatic Hernia Repair: Long-Term Outcomes

There are limited data regarding long-term outcomes in patients with congenital diaphragmatic hernia (CDH) who require tracheostomy with chronic ventilator dependence. The aim of this study is to describe the characteristics of these patients and their associated long-term outcomes in our institutional cohort.

Retrospective chart review was performed on all patients with CDH at our institution who underwent tracheostomy during their initial admission, from January 1, 2004 through October 31, 2024. Patients were excluded if they had tracheostomy creation during a subsequent admission. Demographic and clinical data were collected.

Out of 308 newborn CDH patients treated at our institution over the study period, 8 (2.6%) were discharged with tracheostomy and mechanical ventilation. Two (25%) were born preterm, 2 (25%) had chromosomal anomalies, and 5 (62.5%) had cardiac anomalies. Four (50%) patients underwent extracorporeal life support, all prior to CDH repair. Seven (87.5%) patients required medical therapy for pulmonary hypertension during hospitalization, with 4 (50%) discharged on antipulmonary hypertensive medication. All patients were treated for viral or bacterial pneumonia during their index admission and all patients were diagnosed with variable degrees of tracheobronchomalacia. At the time of review, 2 (25%) patients were deceased-both living a total of 9 mo-while 3 (37.5%) had been successfully decannulated and 3 (37.5%) remain on ventilator support.

Requiring tracheostomy with home mechanical ventilation is rare in CDH patients. In this small series, we noted an association of home ventilation with both pneumonia and tracheobronchomalacia.