Idiopathic intracranial hypertension: adult versus pediatric patients

Abstract

Idiopathic intracranial hypertension (IIH), also known as benign intracranial hypertension or pseudotumor cerebri, is characterized by increased intracranial pressure secondary to unknown causes. IIH affects both adults and children but predominately young obese females of child-bearing age. IIH gives rise to headache, visual disturbances, and symptoms of raised intracranial pressure. The pathophysiology of IIH remains elusive; postulations include obesity-related hormonal changes, obstruction of the central venous sinus, and hereditary causes. Diagnosing IIH is based on modified Dandy criteria. Diagnosis in children is challenging as symptoms and presentations vary; atypical and delayed presentations are not uncommon. Treatment for IIH includes oral acetazolamide or lumbar puncture; surgical treatment with shunts and optic nerve sheath fenestration is reserved for complicated and recurring cases. Treatment options for children are limited and associated with considerable risks and side effects. Vision loss is debilitating and occurs in 10% of IIH patients. A thorough and collaborative approach in managing IIH is needed. This study reviews the epidemiology, clinical presentation, diagnosis, and management of IIH in adults and pediatric patients.