Single Tertiary Centre Experience of Management of Pre-Pubertal Children Presenting With Papilledema and Normal Pressure

Introduction:

Pre-pubertal children with Idiopathic Intracranial Hypertension(IIH) have atypical presentations like being asymptomatic, having normal CSF-pressures. We reviewed management of pre-pubertal children with papilloedema and normal intracranial-pressure(ICP) on lumbar puncture(LP) recordings over 20-30minutes.

Methods:

Retrospective case-note review of 98 pre-pubertal children referred into East-of-England IIH service over past 5years was done. Pre-pubertal is defined as <11years based on average pubertal-age. CSF pressures of >20mmHg in children is considered typical in IIH and </=15 mmHg considered normal.

Results:

Of 98 children,12 had headache and papilloedema with normal ICP. 6 were girls and median-age at presentation was 8.5years. 8 had normal BMI, 4 were obese. MRI+MRV showed radiological-signs of IIH in 4, Chiari-malformation in 1. None had visual-impairment requiring Ventriculo-periteonal-shunt.

Mean ICP was 10.92mmHg(median-11.82mmHg). Acetazolamide was started in 1 due to papilloedema and CSF infusion-studies consistent with IIH, requiring ongoing follow-up for papilloedema.

Rest 11children were closely-monitored and pseudo-papilloedema confirmed in 3, papilloedema spontaneously-resolved in 4 with mean follow-up duration of 20.5months. 4 children have papilloedema on last follow-up with mean duration of follow-up being 32.7months.

Conclusion:

This is first detailed-review of prepubertal children with ‘normal’ICP recording. The gold-standard of ICP recordings with ICP bolt will be next step if papilloedema is persisting or refractory to medication. This may be due to short-periods of recording and possible CSF-leaks resulting in normal-low pressures. Opening-pressure may vary at different times. Hence, imperative to consider ICP-monitoring if initial pressure is normal. As in our case-series, children with severe-papilloedema, treatment should be offered to prevent visual-loss.