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Successful use of BiPAP in infants with congenital myotonic dystrophy
1Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, LKS Faculty of Medicine, University of Hong Kong, Hong Kong.
Insights
Two infants with severe congenital myotonic dystrophy (CDM) experienced respiratory failure at birth. They were successfully treated with bi-level positive airway pressure (BiPAP), a novel approach for this condition.
Area of Science:
- Pediatrics
- Genetics
- Respiratory Medicine
Background:
- Congenital myotonic dystrophy (CDM) is a severe genetic disorder.
- Infants with CDM often present with significant respiratory insufficiency at birth.
Observation:
- Two cases of severe CDM infants with respiratory insufficiency were reported.
- These infants were managed using bi-level positive airway pressure (BiPAP) via a nasal mask.
Findings:
- BiPAP was successfully used to manage respiratory insufficiency in infants with severe CDM.
- This represents the first reported use of BiPAP in infants with CDM.
Implications:
- BiPAP may be a more effective respiratory support method than continuous positive airway pressure for severe CDM.
- This finding could improve outcomes for infants with this severe genetic condition.
Abstract:
Reported herein are two cases of severe phenotype of congenital myotonic dystrophy (CDM) with presentation of respiratory insufficiency at birth. The infants were successfully managed with bi-level positive airway pressure (BiPAP) via nasal mask. The use of BiPAP in infants with CDM has not been reported before. The rationale for using BiPAP is discussed. BiPAP may be more effective than continuous positive airway pressure in managing respiratory insufficiency, especially in infants with the more severe phenotype of CDM.
