Internal Jugular Compression Syndrome Because of Compression Between the Styloid Process and C1 Transverse Process: Clinical Presentation, Diagnosis, and Management.

BACKGROUND AND

objectivesInternal jugular vein (IJV) compression at the skull base represents a rare cause of cerebral venous outflow obstruction and may mimic Eagle's syndrome. Unlike classic Eagle's syndrome, this condition results from venous compression between the hypertrophic C1 transverse process and the styloid process, producing symptoms such as pulsatile tinnitus, headache, and cognitive disturbances. We describe our experience highlighting the diagnostic protocol and management strategies.

methodsWe retrospectively reviewed 5 patients with symptomatic IJV compression syndrome. Demographics, clinical presentation, and treatment outcomes were analyzed. Diagnostic evaluation included computed tomography venography, duplex ultrasonography with head rotation, and cerebral angiography. Four (80%) patients underwent microsurgical decompression of the IJV through a posterolateral suboccipital approach with resection of the hypertrophic C1 transverse process and decompression of the V2-V3 vertebral artery. One patient was managed conservatively.

resultsThe mean age was 57 years (3 females, 2 males). The mean symptom duration was 7.2 years. Presenting symptoms included pulsatile tinnitus, headache, dizziness, syncope, ear pain, and cognitive complaints. Two patients (40%) had undergone previous treatment without symptom relief. Postoperatively, 3 patients (75%) experienced complete resolution of symptoms and one demonstrated partial symptom improvement with persistent tinnitus attributable to neurovascular compression of cranial nerve VIII. Imaging confirmed restoration of IJV patency in all cases. One patient had spontaneous symptom resolution because of extensive venous collaterals. One patient developed intraoperative vertebral artery dissection because of atherosclerosis which was managed by endarterectomy and reconstruction with Hemashield patch.

conclusionIJV compression syndrome caused by the hypertrophic C1 transverse process is a distinct clinical condition that should be considered in patients with refractory pulsatile tinnitus or suspected venous intracranial hypertension. Computed tomography venography of head and neck along with head rotation is critical for diagnosis. Microsurgical decompression through a posterolateral far-lateral approach provides effective symptom relief and durable restoration of venous outflow.