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


CONCLUSIONS
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.
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Section of Sleep Neurology, Mayo Clinic , Rochester, MN , USA ; Department of Neurology, Mayo Clinic , Rochester, MN , USA ; Department of Medicine, Mayo Clinic , Rochester, MN , USA ; Sleep Clinical Research Unit Laboratory, Mayo Clinic Hospital, Mayo Clinic , Rochester, MN , USA.
Erik K
EK
St Louis
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Department of Medicine, Mayo Clinic , Rochester, MN , USA.
Praveen
P
Jinnur
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Department of Neurology, Mayo Clinic , Rochester, MN , USA ; Department of Medicine, Mayo Clinic , Rochester, MN , USA ; Sleep Clinical Research Unit Laboratory, Mayo Clinic Hospital, Mayo Clinic , Rochester, MN , USA.
Stuart J
SJ
McCarter
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Department of Neurology, Mayo Clinic , Rochester, MN , USA ; Department of Medicine, Mayo Clinic , Rochester, MN , USA ; Sleep Clinical Research Unit Laboratory, Mayo Clinic Hospital, Mayo Clinic , Rochester, MN , USA.
Ethan J
EJ
Duwell
Department of Neurology, Mayo Clinic , Rochester, MN , USA.
Eduardo E
EE
Benarroch
Department of Radiology, Mayo Clinic , Rochester, MN , USA.
Kejal
K
Kantarci
Department of Neurosurgery, Mayo Clinic , Rochester, MN , USA.
Mark A
MA
Pichelmann
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Section of Sleep Neurology, Mayo Clinic , Rochester, MN , USA ; Department of Neurology, Mayo Clinic , Rochester, MN , USA.
Michael H
MH
Silber
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Section of Sleep Neurology, Mayo Clinic , Rochester, MN , USA ; Department of Neurology, Mayo Clinic , Rochester, MN , USA.
Bradley F
BF
Boeve
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Department of Medicine, Mayo Clinic , Rochester, MN , USA.
Eric J
EJ
Olson
Mayo Center for Sleep Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic , Rochester, MN , USA ; Department of Medicine, Mayo Clinic , Rochester, MN , USA.
Timothy I
TI
Morgenthaler
Department of Medicine, Mayo Clinic , Rochester, MN , USA ; Sleep Clinical Research Unit Laboratory, Mayo Clinic Hospital, Mayo Clinic , Rochester, MN , USA.
Virend K
VK
Somers
Chiari malformation; adaptive servoventilation; central sleep apnea; pregnancy; presentation