Management of cerebrospinal fluid rhinorrhea as a rare complication of antiphospholipid syndrome

Abstract

Antiphospholipid syndrome is a complex autoimmune condition associated with the formation of recurrent thrombosis in any vascular bed throughout the body. Jugular vein thrombosis is very rare with only a 0.9% occurrence and is not typically associated with cerebrospinal rhinorrhea as a result of raised intracranial pressure. A 54-year-old patient presented with a 9-month history of cerebrospinal fluid (CSF) rhinorrhea and headache on a background of antiphospholipid syndrome. Investigations showed a superior vena cava (SVC) and right internal jugular vein (IJV) obstruction with moderately elevated intracranial venous pressures. Her magnetic resonance imaging (MRI) brain was consistent with a CSF leak. The patient underwent successful endovascular stenting of her obstructed SVC and right IJV followed by surgical repair of a herniating meningocele in the posterior left ethmoid air cells. CSF rhinorrhea is uncommon and never previously reported associated with SVC thrombosis induced by antiphospholipid syndrome. A combination of endovascular techniques and surgical repair is recommended for this challenging presentation.