Styloidogenic Jugular Venous Compression Syndrome: Diagnosis and Treatment (P05.261)

Abstract

Objective: To present a case of Superficial Siderosis of the CNS in a patient with bilateral internal jugular vein narrowing from an external compression of elongated styloid processes. Background Superficial Siderosis of CNS is an uncommon disorder that results from hemosiderin deposition in leptomeninges, subpial layers of brain, and spinal cord from chronic subarachnoid hemorrhage (SAH). The source of SAH is not identified in half of the cases. We present a case of superficial siderosis in a patient with bilateral jugular vein narrowing causing venous congestion from an external compression of styloid processes. Design/Methods: Case report from a tertiary medical center. Results: A 59 year old female presented with slowly progressive gait ataxia, sensorineural hearing loss, and episodic headaches for 6 months. MRI of brain and spinal cord revealed a pituitary adenoma and T2 hypointensity involving the cerebellum, brainstem, and spinal cord consistent with superficial siderosis. CSF studies demonstrated elevated protein without xanthochromia. A dynamic cerebral venogram with extension and flexion views revealed severe bilateral internal jugular vein narrowing at the level of C1-C2, more severe with neck flexion, with considerable congestion of the paravertebral veins and spinal epidural venous plexus. Manometry study revealed an increased pressure gradient across the narrowing between the sigmoid sinuses and internal jugular veins, which was worse with neck flexion compared to neutral position. Computerized tomography angiography of the neck demonstrated elongated styloid processes with external compression of the internal jugular veins at the level of the narrowing. The patient was referred to surgery for styloidectomy. Conclusions: We present a case of superficial siderosis with possible etiology related to bilateral internal jugular vein narrowing leading to significant venous congestion from an external compression of elongated syloid processes. Disclosure: Dr. Gupta has nothing to disclose. Dr. Pandya has nothing to disclose. Dr. Lazzaro has nothing to disclose. Dr. Fitzsimmons has nothing to disclose.