Central sleep apnea and associated Chiari malformation in children with syndromic craniosynostosis: treatment and outcome data from a supraregional national craniofacial center

Abstract

The association of Chiari malformation Type I (CM-I) with syndromic craniosynostosis (SC) in children is well established. Central sleep apnea (CSA) may subsequently occur. However, sleep studies performed in these patients have been focused mainly on assessing the severity of obstructive sleep apnea. Therefore, the incidence and management of CSA in these patients remains poorly defined. Authors of this study aimed to assess the efficacy of foramen magnum decompression (FMD) in resolving CSA, initially detected incidentally, in a small cohort of patients with CM-I and SC. The clinical data for 5 children who underwent FMD for CSA at Alder Hey Children's Hospital between December 2007 and December 2009 were retrospectively analyzed. Outcomes were evaluated with respect to FMDs by utilizing pre- and postdecompression sleep studies. Of the 5 patients, 2 had Crouzon syndrome and 3 had Pfeiffer syndrome. Patient age at the time of surgery ranged from 1.1 to 12.6 years (median 4.1 years). The median postoperative follow-up was 3.6 years. Sleep studies revealed that 2 children experienced a > 80% reduction in CSAs at 1.5 and 21 months after decompression. The remaining 3 children experienced a > 60% reduction in CSAs when reevaluated between 2 and 10 months after decompression. The associated central apnea index improved for all patients. Findings suggested that FMD is an effective treatment modality for improving CSA in patients with SC and associated CM-I. The use of multimodal polysomnography technology may improve the evaluation and management of these patients.