Chiari I malformation associated with atlanto-occipital assimilation presenting as orthopnea and cough syncope.
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 had both a Chiari I malformation and atlanto-occipital assimilation, and complained of cough syncope, orthopnea, and central sleep apnea. The patient underwent decompressive craniectomy of the posterior fossa and a cervical level 2 laminectomy. However, due to an initial under-appreciation of the profound narrowing of the foramen magnum as a result of these concomitant pathologies, the patient had continued impaired cerebrospinal fluid flow, leading to a symptomatic pseudomeningocele and required a more extensive decompression that included a cervical level 3 laminectomy as well as a temporary lumbar drain. On 2 year follow-up, he remained asymptomatic.
Rush University Medical Centre, Rush Professional Office Building, 1725 W. Harrison Street, Suite 1115, Chicago, IL 60612, USA. Electronic address: emangu1@gmail.com.
Erwin Zeta
EZ
Mangubat
Rush University Medical Centre, Rush Professional Office Building, 1725 W. Harrison Street, Suite 1115, Chicago, IL 60612, USA.
Tom
T
Wilson
Rush University Medical Centre, Rush Professional Office Building, 1725 W. Harrison Street, Suite 1115, Chicago, IL 60612, USA.
Brian A
BA
Mitchell
Rush University Medical Centre, Rush Professional Office Building, 1725 W. Harrison Street, Suite 1115, Chicago, IL 60612, USA.
Richard W
RW
Byrne
D001139:Arnold-Chiari Malformation / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D001921:Brain / Q000473:pathology; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging; D003371:Cough; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012120:Respiration Disorders / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D013575:Syncope / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D016896:Treatment Outcome
Atlanto–occipital assimilation; Central sleep apnea; Chiari I malformation; Cough syncope; Orthopnea