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

OBJECT: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Preoperative vincristine for an inoperable choroid plexus papilloma: a case discussion and review of the literature.

The authors report the case of a 14-month-old boy with a large right intraventricular choroid plexus papilloma (CPP) for which the first attempt at resection resulted in life-threatening intraoperative hemorrhage. The tumor was unsuitable for embolization, and neoadjuvant ifosfamide, carboplatin, etoposide (ICE) chemotherapy had no effect on tumor size. However, chemotherapy with vincristine, although not impacting on CT perfusion parameters, resulted in a significant decrease in tumor size, enabling complete resection with manageable blood loss. The mechanism underlying the effect of vincristine in this case is uncertain, but it is a treatment strategy that warrants further evaluation for the treatment of CPPs that are not amenable to embolization.