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PEDIATRICS Vol. 101 No. 4 April 1998, pp. 704-705

EXPERIENCE AND REASON:
Congenital Myotonic Dystrophy Requiring Prolonged Endotracheal and Noninvasive Assisted Ventilation: Not a Uniformly Fatal Condition

Received Apr 28, 1997; accepted Jun 30, 1997.

Charles Keller

Department of Pediatrics Baylor College of Medicine Houston, TX 77030

Ann Reynolds

Meyer Center for Developmental Pediatrics Department of Pediatrics Baylor College of Medicine Houston, TX 77030

Brendan Lee

Department of Molecular and Human Genetics Department of Pediatrics Baylor College of Medicine Houston, TX 77030

Joseph Garcia-Prats

Section of Neonatology Department of Pediatrics Baylor College of Medicine Houston, TX 77030

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.

Key words: congenital myotonic dystrophy, prolonged mechanical ventilation, nasal continuous positive airway pressure, development.




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C. Campbell, R. Sherlock, P. Jacob, and M. Blayney
Congenital Myotonic Dystrophy: Assisted Ventilation Duration and Outcome
Pediatrics, April 1, 2004; 113(4): 811 - 816.
[Abstract] [Full Text] [PDF]