Congenital myotonic dystrophy requiring prolonged endotracheal and noninvasive assisted ventilation: Not a uniformly fatal condition

C. Keller

Research output: Contribution to journalArticle

20 Scopus citations


In this report we present two infants with congenital myotonic dystrophy (CMD) who were successfully weaned from prolonged ventilatory support using nasal continuous positive airway pressure (N-CPAP). The first infant received 127 days of endotracheal mechanical ventilation as part of 141 days of total ventilatory support, including N-CPAP; the second infant received 27 days of endotracheal mechanical ventilation as part of 84 days of total ventilatory support. Noninvasive N-CPAP facilitated weaning these two infants from ventilatory support, thereby minimizing the morbidity associated with prolonged intubation. The developmental outcomes of our two infants were comparable to infants not requiring prolonged endotracheal mechanical ventilation. We suggest that this noninvasive modality of ventilatory support may be advantageous in the management and beneficial to the outcome of infants with CMD who are respirator-dependent for >30 days.

Original languageEnglish (US)
Pages (from-to)704-706
Number of pages3
Issue number4 I
StatePublished - May 5 1998



  • Congenital myotonic dystrophy
  • Development
  • Nasal continuous positive airway pressure
  • Prolonged mechanical ventilation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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