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Respiratory outcomes in children with congenital myotonic dystrophy

Jaclyn Omura1, Maida Chen2, Miriam Haviland3

  • 1Pediatric Rehabilitation Medicine, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.

Journal of Pediatric Rehabilitation Medicine
|November 22, 2021
PubMed

Insights

Newborns with congenital myotonic dystrophy (CDM) show shorter invasive mechanical ventilation (IMV) durations and earlier transitions to non-invasive partial pressure ventilation (NIPPV). Further research is needed to link respiratory support needs with mortality rates in these infants.

Area of Science:

  • Neonatology
  • Pediatric Pulmonology
  • Neuromuscular Disorders

Background:

  • Congenital myotonic dystrophy (CDM) presents with hypotonia and acute respiratory distress at birth.
  • Prolonged intubation (>4 weeks) in neonates with CDM is associated with increased mortality.
  • Understanding respiratory support patterns is crucial for managing CDM infants.

Purpose of the Study:

  • To describe the utilization and duration of respiratory support in newborns diagnosed with CDM.
  • To investigate the relationship between respiratory support methods and mortality in this population.

Main Methods:

  • Retrospective chart review at a tertiary pediatric hospital.
  • Inclusion criteria: confirmed diagnosis of CDM.
  • Outcome measures: mortality, duration of invasive mechanical ventilation (IMV), and non-invasive partial pressure ventilation (NIPPV).

Main Results:

  • 18 subjects met inclusion criteria.
  • 83% experienced respiratory distress at birth.
  • 50% required intubation, with NIPPV initiated as early as day one and extubation to NIPPV by day 17.

Conclusions:

  • This cohort demonstrated shorter IMV durations and earlier transitions to NIPPV.
  • Suggests a potential shift towards earlier NIPPV use in recent practice.
  • Further data are required to establish a correlation between NIPPV/IMV needs and mortality rates.
Abstract

Keywords:
Congenital myotonic dystrophyintubationrespiratory distress

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