Idiopathic Intracranial Hypertension and Associated Optic Neuropathy in Pediatric Patients

Abstract

While idiopathic intracranial hypertension (IIH) is more commonly recognized as a disorder of adults, it affects children of all ages and can have distinctive characteristics when presenting in the prepubertal age group. It is characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. The diagnosis is usually confirmed by a high opening pressure of cerebrospinal fluid (CSF) with exclusion of secondary causes of intracranial hypertension. Objectives: The purpose of this review is to summarize relevant articles on the diagnostic tools used in evaluation and management of pediatric IIH. A summary of demographics, clinical presentation, diagnosis, neuroimaging, as well as existing evidence of treatment strategies is presented. Method: We conducted a bibliographic search in PubMed using the terms idiopathic intracranial hypertension (IIH), Pseudotumor Cerebri syndrome (PTCS). The review of the literature revealed a lack of published consistent data on the diagnosis criteria and management of idiopathic intracranial hypertension in children. Conclusion:This article provides a review of IIH in children and revised diagnostic criteria based on recent evidence and published opinion. Relative to the adult population, the demographic features and clinical presentation of IIH as well as the diagnosis and treatment guidelines for children are quite different. This review highlights the importance of early recognition and management of IIH to prevent permanent visual loss. Key words: idiopathic intracranial hypertension, pseudotumor cerebri syndrome Idiopathic intracranial hypertension (IIH), also referred to as Pseudotumor Cerebri, is characterized by elevated intracranial pressure without discernable etiology, with normal cerebrospinal fluid content, and normal contrast-enhanced computed tomography (CT) or magnetic resonance irradiation (1,2,3).

Optical coherence tomography assessment of structural changes in the optic nerve head and peripapillary retina in idiopathic intracranial hypertension

“…The development of tools for the evaluation of uncooperative patients with IIH is particularly relevant as many patients may not be able to comply with the detailed clinical evaluation used in adults for the treatment and management of this disease. 9 In our study OCT parameters proved to be a reliable tool in the diagnosis of papilledema. However, the significance of our results is limited, due to the small number of study eyes, although our results are statistically significant.…”

“…Application of diagnostic principles, such as the modified Dandy criteria and the 2013 criteria proposed by Friedman et al, assist in making the positive diagnosis in most cases 5,6 . However, an aspect of IIH that constitutes a crucial consideration in establishing the diagnosis is the reliable detection of papilledema, as papilledema associated with IIH may result in irreversible, progressive visual loss [7][8][9] .…”