Styloidogenic Jugular Venous Compression Syndrome Mimicking a Subarachnoid Hemorrhage: A Case Report

Objective and Design: We report a single rare case of unilateral internal jugular venous compression in the upper cervical region presenting as a sudden headache, with meningismus and photophobia, and we review the current literature about this rare phenomenon as well as possible treatment options.

Patients/Materials and Methods: We report a case of a 26-year-old female doctor previously healthy that presented to the Emergency Department with a sudden severe headache and neck pain associated with meningismus and photophobia. She presented with no cognitive or neurological deficits.

Results: The initial CT scan and lumbar puncture were negative for subarachnoid hemorrhage. CT angiography revealed extracranial epidural venous ectasia near the left craniocervical junction surrounding the V3 segment of the left vertebral artery and a large occipital emissary vein communicating this venous variation with jugular bulb. Digital angiography ruled out arteriovenous malformations or dural arteriovenous fistula. Only after an MRI was an extrinsic compression of the left internal jugular vein between the lateral tubercle of C1 detected along with a prominent, posterior and medially located styloid process. The patient recovered well with rest and analgesics, and was discharged for conservative treatment.

Conclusion: Intracranial venous hypertension may result from extrinsic osseous compression of the jugular veins at the skull base. Previously thought as a rare phenomenon, this venous anomaly is increasingly being described, and in some cases it may be necessary to perform a surgical correction due to possible hazardous consequences.