Aim- To describe a profile of children with idiopathic intracranial hypertension (IIT)

Methods- This is a retrospective cohort study of children registered in pediatric neurology clinic in a tertiary care institute. All children with diagnosis of IIT were evaluated for risk factors, neuroimaging and treatment using a pre-defined proforma.

Results- A total of 11 children with IIT were identified. The median age was 45 months and 75% were boys. Headache, squint and vomiting were the commonest  presenting symptoms. Only two children had anemia. One child had hypervitaminosis D. None of the evaluated children had thyroid abnormalities, adrenal insufficiency, abnormalities in serum sodium levels or any prior medication intake. In Five out of seven children (with mild midfacial crowding, mild proptosis), evaluation revealed some form of craniosynostosis. One child had microcephaly. While the remaning one child had no skeletal abnormality. None of the children had grossly abnormal skull shape or any evidence of CNS infection or cerebro-sinus venous thrombosis. The median duration of follow up is 10 mnths (IQR 7-23months). All children were treated with acetazolamide. Two children needed frequent therapeutic lumbar taps, while one child needed optic nerve fenestration; and topiramate. Surgery for craniosynostosis is planned in three children while two other children are under close follow-up.

Conclusion-The described cohort suggests tradionally described risk factors are not common. Though Craniosynostosis is uncommon, mild forms escape attention and present as IIT. In idiopathic cases, CT scan of head may help in diagnosing mild forms of craniosynostosis.