Sleep Disordered Breathing and Its Predictors in Pediatric Muscular Dystrophies.

Background/

Objectives

To evaluate the prevalence, age at diagnosis, non-invasive ventilation pressures used in management, and clinical predictors for sleep disordered breathing (SDB) in pediatric patients with muscular dystrophies (MDs).

Methods

A retrospective analysis of 195 polysomnography (PSG) studies conducted over 20 years for 98 children with different MDs was performed. Diagnosis of SDB was established if a child met the diagnostic criteria for one or more of the following conditions: obstructive sleep apnea (OSA), central apnea, nocturnal hypoxemia, or nocturnal hypoventilation. Outcomes were assessed and compared between MDs. Positive and negative predictive values (PPV, NPV), sensitivity, and specificity for detecting SDB were calculated for certain clinical parameters.

Results

SDB was diagnosed in 73.6% of children with MDs, including OSA in 67%, followed by nocturnal hypoxemia (15.3%), nocturnal hypoventilation (7.7%), and central apnea (6.6%). The age at diagnosis and BiPAP pressures used varied between MDs. Patients with Congenital MD had the lowest mean age and required higher pressures (p < 0.05). PPV was high for maximum inspiratory or expiratory pressures (MIP, MEP) < 40% or <60%, forced vital capacity < 50% or <80%, total lung capacity < 60%, left ventricular ejection fraction < 50%, non-ambulation, and body mass index ≥ 95% for the presence of SDB. However, NPV, sensitivity, and specificity varied.

Conclusions

SDB is common in pediatric patients with MDs, with OSA being the most prevalent disorder. The age at diagnosis and required BiPAP pressures for management differ among MD groups. Certain clinical measures may help identify some patients with the disease given the high PPV.