Stenosis Before Thrombosis: Intracranial Hypertension from Jugular Foramen Stenosis Secondary to Renal Osteodystrophy.

Background: Venous outflow obstructions are rare anatomic findings that can appear with symptoms of elevated intracranial pressure, including headache and vision loss, and can be mistaken for more common diagnoses, such as idiopathic intracranial hypertension (IIH) or cerebral venous sinus thrombosis (CVST). Although venous outflow obstructions have been reported in rare bone dysplasias and congenital abnormalities, to date they have not been reported in renal osteodystrophy (ROD), a relatively common disorder seen in patients with chronic kidney disease.

Case Description: In this case, the authors describe a patient with marked intracranial hypertension from jugular foramen stenosis secondary to ROD. After diagnosis by CT and magnetic resonance venography, catheter venography confirmed an osseus band around the left jugular bulb, and a 40-mm Hg pressure gradient across the stenotic foramen. The patient subsequently underwent ventriculoperitoneal shunting and optic nerve sheath fenestration with symptom improvement. The postoperative course was significant for development of CVST, necessitating treatment.

Conclusions: This report reviews the presentation, pathology, and neurosurgical treatment of patients with ROD and venous outflow obstructions, and explores the differential diagnoses of outflow obstructions, IIH, and CVST. To our knowledge, this is the first report of intracranial hypertension from jugular foramen stenosis secondary to renal osteodystrophy.