Profile of Patients with Craniovertebral Junction Anomalies with Posterior Circulation Strokes

Abstract

Craniovertebral junction (CVJ) anomalies are rare and treatable risk factors for posterior circulation strokes (PCSs). Most of the literature comprises case reports and from varied specialities. Chart review of patients with PCS with CVJ anomalies admitted in a single tertiary stroke care unit. We describe their clinical profile, investigative findings, and therapeutic outcomes. We saw 7 patients (6 males, mean age 20.1 ± 12.0 years), all previously undiagnosed during the 6-year period. Three patients had a combination of atlantoaxial dislocation (AAD), basilar invagination (BI) and Klippel-Feil anomalies, 2 patients had os odontoideum with AAD, and 1 patient each had isolated AAD and BI. Clinically, they presented with first or recurrent episodes of stroke with neck pain. Examination revealed noticeable skeletal markers and neurologic deficits involving the arterial territory. Routine stroke risk factors were absent. X-ray of CVJ was abnormal and diagnostic in all the patients. Other imaging modalities had their own contributions for identifying associated defects, demonstrating pathophysiology, and management of these patients. Acute care with anticoagulation and supportive measures helped in recovery of all of them. Posterior fusion was done in 4 patients, and 2 patients are awaiting the surgery. During follow-up all patients have improved and none had recurrences. In case of a young patient with PCS (first/recurrent) in the absence of routine risk factors for stroke, careful physical examination and imaging with basic X-ray may help in the diagnosis CVJ abnormalities, which may be effectively managed by a multidisciplinary team to prevent recurrences.