Understanding the complex pathophysiology of idiopathic intracranial hypertension and the evolving role of venous sinus stenting: a comprehensive review of the literature

Abstract

Idiopathic intracranial hypertension (IIH) is a disease defined by elevated intracranial pressure without established etiology. Although there is now consensus on the definition of the disorder, its complex pathophysiology remains elusive. The most common clinical symptoms of IIH include headache and visual complaints. Many current theories regarding the etiology of IIH focus on increased secretion or decreased absorption of cerebrospinal fluid (CSF) and on cerebral venous outflow obstruction due to venous sinus stenosis. In addition, it has been postulated that obesity plays a role, given its prevalence in this population of patients. Several treatments, including optic nerve sheath fenestration, CSF diversion with ventriculoperitoneal or lumboperitoneal shunts, and more recently venous sinus stenting, have been described for medically refractory IIH. Despite the availability of these treatments, no guidelines or standard management algorithms exist for the treatment of this disorder. In this paper, the authors provide a review of the literature on IIH, its clinical presentation, pathophysiology, and evidence supporting treatment strategies, with a specific focus on the role of venous sinus stenting.

Keywords: CSF = cerebrospinal fluid; CSF diversion; ICP = intracranial pressure; IIH = idiopathic intracranial hypertension; LP = lumbar puncture; LPS = lumboperitoneal shunt; MRV = magnetic resonance venography; ONSF = optic nerve sheath fenestration; RCTs = randomized controlled trials; VPS = ventriculoperitoneal shunt; VSS = venous sinus stenting; benign intracranial hypertension; idiopathic intracranial hypertension; optic nerve sheath fenestration; pseudotumor cerebri; venous sinus stenting.