Chiari 1 Malformation Presenting as Central Sleep Apnea during Pregnancy: A Case Report, Treatment Considerations, and Review of the Literature

Abstract

Purpose: Chiari malformation (CM) type-1 frequently causes obstructive or central sleep-disordered breathing (SDB) in both adults and children, although SDB is relatively rare as a presenting manifestation in the absence of other neurological symptoms. The definitive treatment of symptomatic CM is surgical decompression. We report a case that is, to our knowledge, a novel manifestation of central sleep apnea (CSA) due to CM type-1 with severe exacerbation and initial clinical presentation during pregnancy.Methods: Case report from tertiary care comprehensive sleep medicine center with literature review of SDB manifestations associated with CM type-1. PubMed search was conducted between January 1982 and October 2013.Results: We report a 25-year-old woman with severe CSA initially presenting during her first pregnancy that eventually proved to be caused by CM type-1. The patient was successfully treated preoperatively by adaptive servoventilation (ASV), with effective resolution of SDB following surgical decompression, and without recurrence in a subsequent pregnancy. Our literature review found that 58% of CM patients with SDB had OSA alone, 28% had CSA alone, 8 (10%) had mixed OSA/CSA, and 6 (8%) had hypoventilation. Of CM patients presenting with SDB, 50% had OSA, 42% had CSA, 8% had mixed OSA/CSA, and 10.4% had hypoventilation. We speculate that CSA may develop in CM patients in whom brainstem compression results in excessive central chemoreflex sensitivity with consequent hypocapnic CSA.Conclusion: Chiari malformation type-1 may present with a diversity of SDB manifestations, and timely recognition and surgical referral are necessary to prevent further neurological deficits. ASV therapy can effectively manage CSA caused by CM type-1, which may initially present during pregnancy.

Coronavirus disease 2019–associated persistent cough and Chiari malformation type I resulting in acute respiratory failure: illustrative case

“…9,34 Although a large percentage of patients with CM-I are asymptomatic, a sudden unexpected deterioration and/or death has been reported in CM-I, most often after surgery, infection, or other significant stressors such as pregnancy. 30,32,35 The pathology of CM-I leading to tonsillar herniation, CMI-associated syringomyelia, and subsequent symptoms is not completely understood. However, studies have suggested that a combination of both extradural and intradural pathology contributes to its pathogenesis, often showing signs of posterior fossa crowding, brainstem compression, an enlarged fourth ventricle, or mechanical obstructions of CSF outflow.…”

Determinants of Chiari I progression in pregnancy

“…Both epidural and spinal anesthesia case studies have been reported in literature [7,9,21]. Nonetheless, regional anesthesia techniques also must be preceded with a caution, as an accidental dural puncture may have severe consequences [5]. Dural punctures lead to unavoidable loss of CSF which may cause tonsillar herniation through the foramen magnum and further leads to an increase in intracranial pressure [7,9].…”

“…CM-I affects people of all races, although with an increased prevalence in women than men. The symptom complex of CM-I is very broad range which could include post-tussive suboccipital headaches, vertigo, dizziness, vertebrobasilar insufficiency, blurring of vision, nystagmus, tinnitus, poor coordination, numbness & tingling, swallowing difficulty, central sleep apnea, tremors, nausea, and/or mental fog [2,5,14,15].…”

Managing Sleep Disorders during Pregnancy

“…Treatment during pregnancy is with an adaptive servoventilaton device, and postpartum such patients might need surgical decompression of the base of the skull. 15…”