Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation.
Bottom Line: 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.
Affiliation: Tondela-Viseu Hospital Center, Viseu, Portugal.
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.
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Mentions: A 44-year-old female patient diagnosed with CM-I in 2008 (Figure 1A) underwent decompressive surgery of the posterior cranial fossa.She underwent suboccipital craniectomy, laminectomy of C1 and C2, and duraplasty. Herpostoperative complications included cerebrospinal fluid fistula, which requiredreoperation and application of biological glue, and surgical wound infection, which wastreated with debridement. Twelve months after surgery, the patient presented withworsening of neurological symptoms, including occipital headaches, dizziness, gaitimbalance, left facial paresis, and dysphagia for liquids. Magnetic resonance imagingshowed bony malformation of the craniovertebral junction associated with a syringomyeliccavity involving C2 and C3 (Figure 1B). Additionalsurgical intervention was ruled out because of the associated risk of respiratorydepression. Three years after surgery, the patient was referred to our sleep laboratoryfor suspected sleep apnea. She reported nonrestorative sleep and morning headaches, butshe had no daytime sleepiness (Epworth score of 4) or symptoms suggestive of restlesslegs syndrome or narcolepsy. Additional information obtained from the patient's familyconfirmed the presence of snoring and witnessed apneas. The patient had a history ofhypothyroidism and received levothyroxine regularly. She was a nonsmoker and did notdrink alcohol.