Diagnosis and Therapy of Obstructive Sleep Apnea Syndrome in Children with Premature Craniosynostosis Syndromes

Introduction: Sleep related breathing disorders are a common symptom in children with craniosynostosis syndromes, as upper airways may be narrowed by midfacial hypoplasia. Patients and methods: To better characterize the sleep related apneas, 24 children with syndromal craniofacial dysplasia underwent 68 polysomnographies. 9 patients had reexaminations after therapeutic procedures. Results: 4 patients had severe obstructive sleep apnea syndrom (OSAS), 8 patients had moderate and 11 patients mild obstructive sleep apnea respectivly. Only one child had no obstructive sleep apnea. Children with Morbus Crouzon tended to have moderate to severe breathing disorders (9/14) whereas Apert patients mostly had no or light breathing disorders (6/7). Number of central apneas was increased as well. Sleep architecture was not significantly impaired. Apneas were more frequent during REM-sleep. Nasal CPAP, Bi-PAP and adenotonsillectomy improved respiratory parameters. Conclusion: Pulse oxymetry can be used as a screening method because of the good correlation of oxygen desaturation index with severity of OSAS. Frequent examinations and, if necessary, adaptation of therapy is indicated as OSAS in these children may be rapidly changing. We suggest a guideline for diagnostics and therapy.