Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation

Abstract

The Chiari malformation type I (CM-I) has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery.

A case of hypercapnic respiratory failure

“…In patients with CM1 and SRBD, the treatment of choice is decompressive brain surgery to relieve the underlying cause [ 9 ]. However, in most cases, the SRBD is diagnosed prior to the CM and ventilation therapy is initiated before surgery [ 17 – 19 ].…”

“…Several studies have documented definite improvement of sleep apnoea after PFD, objectified with a PSG [ 12 , 13 , 17 , 18 ]. However, cases with residual or recurrent sleep apnoea, especially CSA, are reported [ 12 , 19 , 20 ].…”

Chiari I malformation: Case report

“…Its treatment is complex and is defined according to the malformations presented (BRITO et al, 2019). The most commonly used surgical option is decompression of the posterior cranial fossa (ALZATE et al, 2001;VALE et al, 2014;BRITO et al, 2019).…”

Malformação de Chiari: Relato de caso

“…O tipo I é o mais comum e é caracterizado pelo deslocamento caudal das tonsilas cerebelares e da parte medial e inferior do lobo posterior do cerebelo pelo canal vertebral, acima de 3 a 5mm a partir do forame magno (Oliveira et al, 2011;Brito et al, 2019). Raramente é visto abaixo de C2 e geralmente é diagnosticada na vida adulta (Vale et al, 2014). A mielomeningocele é ausente e a fossa posterior possui dimensões reduzidas (Magalhães et al, 2019); o tipo II é uma anormalidade do sistema nervoso central, está associado a uma mielodisplasia que é a mielomeningocele e hidrocefalia.…”