Affiliations: [a] Pediatric Rehabilitation Medicine, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA, USA
| [b] Pediatric Pulmonology, Seattle Children’s Hospital, Seattle, WA, USA
| [c]
Seattle Children’s Research Institute, Seattle, WA, USA
| [d] Pediatric Rehabilitation Medicine, Children’s Hospital Colorado, Denver, CO, USA
Correspondence:
[*]
Corresponding author: Jaclyn Omura, Pediatric Rehabilitation Medicine, Seattle Children’s Hospital, University of Washington School of Medicine, 4800 Sandpoint way NE, M/S OB.8.410, Seattle, WA 98105, USA. E-mail: Jaclyn.omura@seattlechildrens.org.
Abstract: PURPOSE:Congenital myotonic dystrophy (CDM) results in hypotonia and acute respiratory distress at birth. Previous studies show that prolonged periods of intubation (>4 weeks) correlate with increased mortality rates. The objective is to describe the use and duration of respiratory support in newborns with CDM and how these relate to mortality. METHODS:A retrospective chart review was performed at a tertiary pediatric hospital among children with confirmed diagnosis of CDM. The main outcome measures were: mortality, duration of invasive mechanical ventilation (IMV) and non-invasive partial pressure ventilation (NIPPV), along with long-term use of respiratory support and equipment. RESULTS:A total of 18 subjects met inclusion criteria, 83%.f which had documented respiratory distress at birth, 39%.equired NIPPV, and 50%.equired intubation in the neonatal period. The earliest NIPPV was initiated at day one of life, and the latest extubation to NIPPV was at 17 days of life. CONCLUSION:This cohort required IMV for shorter periods with earlier transitions to NIPPV which suggests a possible change in practice and earlier transition to NIPPV recently. Further data are needed to determine if there is a possible correlation between the need for NIPPV/IMV and mortality rates.
