1245 REM Associated Increase in Intracranial Pressure in a Child with Craniosynostosis

Abstract

Introduction: Clinical associations between increased intracranial pressure (ICP) during rapid eye movement (REM) sleep and upper airway resistance have been described previously but few pediatric patients have undergone ICP monitoring during sleep. This case report describes a child with Saethre-Chotzen (craniosynostosis) syndrome with headache, vomiting and snoring who had elevated ICP during REM sleep. Report of Case: A 7 year-old girl with Saethre-Chotzen syndrome presented with headaches, vomiting and loud snoring. She subsequently underwent intracranial pressure (ICP) monitoring, which demonstrated increase in ICP during sleep with pressure normalization during wakefulness. Overnight polysomnography with ICP monitoring was performed to evaluate for obstructive sleep apnea (OSA). Polysomnography demonstrated no OSA but revealed increase in ICP during REM sleep with two spikes in ICP at onset of REM. Interestingly, oxygen desaturations to the 80s as a result of multiple central events occurred during her first ICP elevation. Both ICP elevations normalized with transition to non-rapid eye movement (NREM) sleep. In healthy individuals, studies demonstrated 10-20% reduction in cerebral blood flow (CBF) during NREM sleep and 20-35% increase in CBF during REM sleep compared with wakefulness. The child underwent anterior cranial vault expansion with distraction resulting in markedly improved headache frequency. Repeat polysomnography demonstrated no OSA or desaturations and REM-related ICP elevations were lower than before surgery. Conclusion: This case highlights the interesting finding of elevated ICP secondary to increased intracranial blood flow during REM sleep. Due to worsening cephalocranial disproportion and intracranial pressure dynamics, children with craniosynostosis may develop headaches due to sleep related increases in ICP. This child may have been particularly susceptible given history of craniosynostosis and dysmorphic cranium with flattening of frontal occipital bones. Symptoms improved markedly status-post cranial vault expansion. Although OSA can increase ICP, it is important to consider cephalocranial dynamics in patients with REM-related increases in ICP particularly when they are symptomatic.