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J Neurol Surg Rep 2014; 75(01): e1-e4
DOI: 10.1055/s-0033-1348953
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Chiari I Malformation Associated with Atlanto-Occipital Assimilation Presenting as Orthopnea and Cough Syncope: A Case Report and Review of Literature

Erwin Zeta Mangubat
1   Department of Neurosurgery, Rush Professional Office Building, Chicago, Illinois, United States
,
Tom Wilson
1   Department of Neurosurgery, Rush Professional Office Building, Chicago, Illinois, United States
,
Brian A. Mitchell
1   Department of Neurosurgery, Rush Professional Office Building, Chicago, Illinois, United States
,
Richard W. Byrne
1   Department of Neurosurgery, Rush Professional Office Building, Chicago, Illinois, United States
› Author Affiliations
Further Information

Publication History

20 April 2013

13 April 2013

Publication Date:
12 December 2013 (online)

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Abstract

Although it is not uncommon for patients with Chiari I malformations to present with respiratory complaints, cough syncope is a rare presenting symptom. We report an adult patient who harbored both a Chiari I malformation and atlanto-occipital assimilation who complained of cough syncope, orthopnea, and central sleep apnea. The patient underwent decompressive craniectomy of the posterior fossa and cervical level 2 laminectomy. However, due to a possible initial underappreciation of the profound narrowing of the foramen magnum as a result of these concomitant pathologies, the patient may have had continued impaired cerebrospinal fluid flow, leading to a symptomatic pseudomeningocele and requiring a more extensive decompression that included a cervical level 3 laminectomy as well as a temporary lumbar drain. On 2-year follow-up, he has remained asymptomatic.

Keywords

Chiari I malformation - atlanto-occipital assimilation - cough syncope - orthopnea - central sleep apnea
 

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