Papilledema as the Initial Manifestation of Styloidogenic Jugular Venous Compression Syndrome

Abstract Styloidogenic jugular venous compression syndrome (SJVCS) is a rare variant of Eagle Syndrome caused by compression of the internal jugular vein (IJV) by an elongated styloid process. We describe a 47-year-old woman in whom papilledema was incidentally detected during a routine optometric examination. Initial neuroimaging did not adequately image the jugular veins in the neck but demonstrated unremarkable intracranial appearances. The patient remained asymptomatic with a normal lumbar puncture opening pressure. Over 3 years, the papilledema gradually worsened in the absence of symptoms of raised intracranial pressure, until she later presented with progressive headaches, tinnitus, and blurred vision. Repeat imaging and catheter venography revealed severe bilateral IJV stenosis consistent with SJVCS. Given right-sided venous dominance, the patient underwent a right styloidectomy, resulting in the resolution of symptoms and papilledema. This case suggests two novel insights into SJVCS: (1) papilledema may represent an early manifestation before intracranial hypertension symptoms become apparent, and (2) the clinical manifestations may progress over time.