This updated review of pediatric idiopathic intracranial hypertension focuses on epidemiology, clinical presentations, diagnostic criteria, evaluation, clinical course, and treatment. General guidelines for the clinical management of idiopathic intracranial hypertension are discussed. A new algorithm outlines an efficient management strategy for the initial diagnostic evaluation of children with signs or symptoms of intracranial hypertension. This algorithm provides a systematic approach to initial evaluation and management, and identifies important decision-making factors. The risk of permanent visual loss with idiopathic intracranial hypertension necessitates a prompt, thorough collaborative approach in the management of patients. Although idiopathic intracranial hypertension has been recognized for over a century, the need remains for prospectively collected data to promote a better understanding of the etiology, risk factors, evaluative methods, and effective treatments for children with this syndrome.
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