Retrospective study observes severe obstructive sleep apnea in children with pycnodysostosis, suggesting stepwise surgery and continuous positive airway pressure improve respiratory outcomes.
Background: Pycnodysostosis is a rare skeletal disorder often associated with craniofacial anomalies that may increase the propensity for obstructive sleep apnea (OSA). This retrospective study aimed to explore the prevalence and treatment of OSA in pediatric patients with Pycnodysostosis. Objectives: To describe the clinical characteristics, polysomnographic findings, and treatment in 10 children with Pycnodysostosis. Methods: Retrospective study conducted at the Sleep Medicine Center of CHU Hassan II, Fès, Morocco of 10 children with Pycnodysostosis diagnosed between 2010 and 2024 and based on clinical and/or genetic criteria who underwent overnight polysomnography. Results: All patients suffered from moderate to severe OSA (apnea-hypopnea index: 5.9-43.8 events/h). Treatment included adenoidectomy, tonsillectomy, and CPAP, the latter leading to near normalization of respiratory patterns and improvement in sleep quality. Conclusion: OSA in Pycnodysostosis imposes a significant impact on quality of life which responds favorably to stepwise interventions aimed at eliminating or mitigating OSA severity. This study highlights the importance of early diagnosis and appropriate treatment to prevent long-term complications, especially involving maxillofacial growth.
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Bouhamdi et al. (2025) studied this question.
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