S.D. King1, K. Burgi3, A. Filbrun2, J. Church1 1University Of Michigan, Pediatric Surgery, Ann Arbor, MI, USA 2University Of Michigan, Pediatric Pulmonology, Ann Arbor, MI, USA 3University Of Michigan, School Of Medicine, Ann Arbor, MI, USA
Introduction:
Congenital diaphragmatic hernia (CDH) is a known but not fully understood pathology, particularly as it relates to pulmonary disease. There is limited data reviewing the incidence of tracheostomy and the long-term outcomes of chronic ventilator dependence in these patients. This study aims to describe the CDH-chronically ventilated patient population, their characteristics and long-term outcomes at our institution.
Methods:
Retrospective chart review was performed on all patients at the University of Michigan who were treated for CDH and underwent tracheostomy during their initial admission, from January 1, 2004, through June 30, 2024. Patients were excluded if they had either CDH repair or tracheostomy creation during a subsequent admission. Demographic and clinical data of 7 patients was collected.
Results:
Three hundred and eight newborn CDH patients were treated at the University of Michigan over the study period, but only 7(2%) were discharged with tracheostomy and chronic ventilation. There was a bimodal distribution, 4(57%) in 2006-2007 and 3(43%) from 2018-2023. Five (71%) of the 7 patients were male and 2(29%) were born preterm. Two (29%) patients had chromosomal anomalies and 5(71%) had cardiac anomalies. Six (86%) had left-sided CDH defects. Four (57%) patients underwent extracorporeal life support, all prior to CDH repair. One (14%) patient had a Type A defect and there were two patients of each other defect type—B, C and D. The Type A defect and one Type B defect were repaired primarily but the other 5(71%) patients had patch repairs. The Type B defect that was repaired primarily was the only laparoscopic repair in the series. Of the 6(86%) patients who required medical management of pulmonary hypertension, 2(33%) were weaned from medications prior to discharge. All the patients had treatment for viral or bacterial pneumonia during admission. All the patients were diagnosed with variable degrees of tracheobronchomalacia. In the patients with bronchomalacia, the worse side was often the same side as the diaphragmatic defect; however, defect size did not appear to have an association with type or severity of malacia. At the time of review, 2(29%) patients were deceased—both living only 9 months—but 3(43%) had been successfully decannulated.
Conclusion:
The incidence and long-term outcomes of CDH patients with tracheostomy and chronic ventilation are not well understood. We found that the incidence is rare, but all cases were associated with pneumonia and malacia. The bimodal distribution of tracheostomy in our series suggests a movement away from chronic ventilation until the last decade; reinforcing the idea that as more CDH patients are living to discharge, the incidence of associated tracheostomy is increasing.