Idiopathic intracranial hypertension in Omani children

Abstract

Idiopathic intracranial hypertension (IIH) is a relatively rare disease in children. It is defined as raised intracranial pressure in the absence of clinical, laboratory or radiological evidence of an intracranial space occupying lesion. We aimed to evaluate the clinical and therapeutic features of IIH in a presenting group of Omani pediatric population. We conducted a retrospective chart review of patients below 18 years of age diagnosed with IIH between 1992 and 2006 according to Dandy's Modified Criteria at Sultan Qaboos University Hospital, Oman. Statistical analyses were conducted using McNemar's test. Nineteen patients were included in the study, 11 (58%) were males and 8 (42%) were females, with a ratio of 1.4:1. The median age at presentation was 8.5 years. The average body mass index of the patients was 18.1 with only 2 (11%) children being obese, both males and pubertal. All the patients except 1 (5%) were symptomatic at presentation. The commonest symptom was headache found in 16 (84%) patients. This was followed by visual symptoms in half of them in the form of diplopia (16%) or transient visual obscuration (37%). All patients had bilateral papilledema, 7 (37%) had visual field defects, 4 (21%) had an enlarged blind spot and 3 (17%) had restricted visual field. Secondary causes of intracranial hypertension were not identified but the associated conditions were sickle cell disease, Behcet disease, neurofibromatosis type 1, varicella infection, and brucellosis. Seventeen (89%) patients showed good response to treatment with acetazolamide, steroids or both, with resolution of symptoms and signs ( p< 0.001). Two (11%) had deterioration in their vision despite aggressive medical treatment and were referred for surgical intervention. The epidemiologic features in the studied pediatric population were similar to that of other studies. The prompt recognition, evaluation, and treatment of IIH are needed to alleviate symptoms and to preserve visual function. There was a favorable response to medical treatment in this study cohort.