Styloidogenic jugular venous compression syndrome: Clinical features and case series

Abstract

BACKGROUND: Styloidogenic jugular venous compression syndrome (SJVCS) is a rare cause of idiopathic intracranial hypertension (IIH). OBJECTIVE: To elucidate the pathophysiology and the hemodynamics of SJVCS. METHODS: We conducted a retrospective review of medical records, clinical images, dynamic venography, and manometry for consecutive patients with SJVCS undergoing microsurgical decompression from April 2009 to October 2017. Patients with IIH with normal venography and manometry findings served as controls. RESULTS: Data were analyzed for 10 patients with SJVCS who presented with headaches. Neck flexion exacerbated headaches in 7 patients. Eleven patients with IIH provided control data for normal intracranial venous pressure and styloid process anatomy. Patients with SJVCS had bilateral osseous compression of venous outflow. The styloid processes were significantly longer in patients with SJVCS than in those with IIH (mean [standard deviation (SD)] distance, 31.0 [10.6] vs 19.0 [14.1] mm; P < .01). The styloid process–C1 lateral tubercle distance was shorter in patients with SJVCS than in those with IIH (mean [SD] distance, 2.9 [1.0] vs 9.9 [2.8] mm; P < .01). Patients with SJVCS had significantly higher global venous pressure and a higher pressure gradient across the stenosis site than controls (mean [SD] pressure, 2.86 [2.61] vs 0.13 [1.09] cm H2O; P = .09). All 10 patients with SJVCS experienced venous pressure elevation during contralateral neck turning (mean [SD] pressure, 4.29 [2.50] cm H2O). All 10 patients with SJVCS underwent transcervical microsurgical decompression, and 9 experienced postoperative improvement or resolution of symptoms. One patient had transient postoperative dysphagia and facial drooping, and another patient reported jaw numbness. CONCLUSION: SJVCS is a novel clinical entity causing IIH. Patients should be evaluated with dynamic venography with manometry. Surgical decompression with removal of osseous overgrowth is an effective treatment in select patients.