Abstract Rationale Trisomy 13 and 18 are rare aneuploidies traditionally associated with severe congenital cardiac, pulmonary, neurologic and other anomalies and high mortality in the first year of life. Patients and families have often been offered comfort measures. However, recent studies have suggested more favorable survival outcomes with a more pro-active approach, including cardiac surgery and, in cases of tracheobronchomalacia, chronic mechanical ventilation. The objective of this study was to evaluate the length of time on a ventilator and length of time with a tracheostomy compared to other children with chronic respiratory failure. Our hypothesis was that the length of time on a ventilator in Trisomy 13 and 18 would be longer compared to other children in chronic respiratory failure given other significant comorbidities. Methods This was a retrospective review of the electronic medical records of all children followed in the University of Michigan Pediatric Chronic Ventilation program. Length of time on ventilator and length of time with a tracheostomy was compared among three groups: patients with trisomy 13 or 18 (Group 1), patients with trisomy 21 (Group 2) and patients with non-genetic causes of chronic respiratory failure (Group 3). Single factor ANOVA was used to compare these 3 groups. Results Two patients with trisomy 13 and eighteen patients with Trisomy 18 were managed with chronic invasive mechanical ventilation, and eight were liberated from the ventilator during the study period. The cause for respiratory failure was tracheobronchomalacia in the majority of patients. In Group 2, 13 of 22 patients were liberated from the ventilator, and in Group 3, 108 of 177 were liberated from the ventilator. Median age in months at ventilator liberation was 28 in Group 1, 24.5 in Group 2 and 25 in Group 3 (p = 0.68). Average duration of mechanical ventilation in months was 20.4±14.6 for Group 1, 23.7±14.2 for Group 2 and 24.9±15.6 for Group 3 (p = 0.72 ). At the time of data acquisition, tracheal decannulation had occurred in only 1 patient in Group1 (5%), 5 patients in Group 2 (23%) and 59 (33%) in group 3. Conclusion In this small study, age of chronic mechanical ventilation and length of chronic mechanical ventilation was similar between patients with trisomy 13 or 18, trisomy 21 and patients with non-genetic causes of respiratory failure. However, there appeared to be a trend towards delayed tracheal decannulation among those with trisomy 13 and 18. This abstract is funded by: None
Kumar et al. (Fri,) studied this question.