Abstract

Abstract Introduction Cerebral Cavernous malformations (CCMs) consist of a collection of capillaries in the central nervous system (CNS) that are enlarged and irregular in structure. Patients with CCMs are at increased risk of hemorrhage into the brain or spinal cord, resulting in seizures, focal neurologic deficits, hydrocephalus, and death. Given the importance of the brainstem and central chemoreceptors in regulating respiratory function, rupture of CCMs can also lead to dysregulation of breathing. We present a series of 3 pediatric patients who have a diagnosis of central sleep apnea (CSA) due to rupture of CCMs managed with home ventilators with significant differences in clinical presentation from mild CSA to night time respiratory support for severe CSA and chronic respiratory failure requiring continuous mechanical ventilation. Report of Cases: Subject 1 is a 13 yo M with a history of ruptured posterior fossa arteriovenous malformation (AVM) complicated by post-hemorrhagic hydrocephalus, right hemiparesis and severe central apnea requiring nighttime mechanical ventilation via tracheostomy. Subject 2 is a 19 yo M with a history of mid-pontine cavernoma with multiple episodes of hemorrhage failing surgical resection resulting in right hemiparesis, severe central apnea and hypoventilation requiring continuous mechanical ventilator support via tracheostomy. Subject 3 is a 6 yo F with a history of thoracic cavernous hemangioma resulting in spinal cord injury and mild central sleep apnea requiring mask ventilation via home ventilator during sleep. All of these patients experienced loss of respiratory drive as a result of complications from brainstem or spinal cord lesions with varying degrees of ventilator support requirement and clinical presentation. Conclusion Due to the propensity for CNS bleeds among patients with CCM they are at increased risk of respiratory compromise. Home ventilator support can be used effectively to treat central apneas and chronic respiratory failure but this is a moving paradigm as subsequent bleeds worsen respiratory compromise. Support (If Any) Support (if any):

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