Managing Sleep-Disorders Breathing in Chiari Malformation Type I: A Case Report

Keywords

case reports - Chiari malformation type I - sleep disordered breathing

Introduction: Chiari malformation is a congenital condition involving the downward displacement of the cerebellar structures through the foramen magnum, leading to various neurological symptoms. Type I is the most common and is often associated with sleep-disordered breathing (SDB), including both obstructive and central apneas.

Objective: To report a case of an adolescent with Chiari Type I, focusing on his clinical course marked by SDB, treatment, and outcomes.

Methods: We conducted a descriptive, observational case report study by accessing and reviewing medical records with informed consent, ensuring confidentiality. Data were collected on clinical presentation, diagnostic tests, therapeutic interventions, and patient outcomes, supplemented by a literature review for context.

Results: A 17-year-old male with Chiari Type I, diagnosed at age 13, was admitted on January 2, 2024, with worsening neurological symptoms, including imbalance, vertigo, and severe somnolence. His medical history included chronic bronchitis and epilepsy. Initial evaluation revealed severe respiratory acidosis and significant cerebellar tonsillar herniation on imaging. He required intubation and mechanical ventilation due to prolonged apneas. On January 12, 2024, he underwent decompressive craniectomy and tonsillar coagulation. Postoperatively, he experienced persistent apneas, necessitating a tracheostomy and nightly bilevel positive airway pressure (BiPAP) use. A February 19, 2024, polysomnography (PSG) confirmed severe central sleep apnea (apnea-hypopnea index, AHI 40.5 events/h). Further evaluations showed ongoing severe obstructive sleep apnea (AHI 61.2 events/h) with reduced sleep efficiency, necessitating continued specialized respiratory care. Chiari Type I malformation can significantly impact respiratory function, with up to 70% of patients experiencing SDB. Neurosurgical intervention, while often necessary, may not fully resolve SDB, as seen in this case. Persistent post-surgery symptoms may result from incomplete decompression or irreversible brainstem dysfunction. BiPAP therapy can be crucial in managing residual SDB.

Conclusion: Our case report highlights that managing Chiari Type I with severe SDB requires a multidisciplinary approach involving neurosurgeons, pulmonologists, and sleep specialists. Persistent respiratory symptoms following surgery underscore the need for ongoing, individualized care.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
16 September 2025