To describe a cohort of children with idiopathic intracranial hypertension (IIH)

Retrospective case record analysis of children presenting with a clinical diagnosis of IIH (2016-2019)  

Nineteen children (13boys) with IIH were identified. The median age at presentation was 36 months (range: 9months- 11years). Acute-onset squint(10/19) followed by headache(9/19) were the common presenting symptoms.Examination revealed macrocephaly(3/19), small head (<-2 SD; 2/19), papilledema (14/19), and subtle facial dysmorphism(4/19). All children had elevated CSF opening pressure. Brain MRI (evaluated in 16 children) revealed features such as optic nerve tortuosity (7/16), prominent optic nerve sheath (8/16), scleral indentation (6/16), empty sella (7/16), transverse sinus stenosis (1/16), obliteration of cistern (1/16), and features ofbenign enlargement of subarachnoid space(1/16). Six children were found to have some form of craniosynostosis on CT head but none of them had grossly abnormal skull shape.

Investigations revealed hypervitaminosis D(1/19) and vitamin B12 deficiency(1/19). None had thyroid abnormalities, adrenal insufficiency, or any prior medication intake. All children were managed with acetazolamide while 5 required therapeutic lumbar taps and 2 received topiramate as an add-on therapy. One child underwent optic nerve fenestration and one child underwent cranial vault surgery. Two children with craniosynostosis are planned for surgery.

This cohort suggests that tradionally described risk factors are not so common in pediatric IIH. Mild forms of craniosynostosis should be kept in mind during evaluation for IIH, especially in young children with subtle dysmorphism and/or small head. Hence, we suggest that CT head may be a useful investigation in young children with IIH.