The ophthalmic natural history of paediatric craniopharyngioma: a long-term review

Evangelos Drimtzias, Kevin Falzon, Susan Picton, Irfan Jeeva, Danielle Guy, Olwyn Nelson, Ian Simmons

We present our experience over the long-term of monitoring of visual function in children with craniopharyngioma. Our study involves an analysis of all paediatric patients with craniopharyngioma younger than 16 at the time of diagnosis and represents a series of predominantly sub-totally resected tumours. Visual data, of multiple modality, of the paediatric patients was collected. Twenty patients were surveyed. Poor prognostic indicators of the visual outcome and rate of recurrence were assessed. Severe visual loss and papilledema at the time of diagnosis were more common in children under the age of 6. In our study visual signs, tumour calcification and optic disc atrophy at presentation are predictors of poor visual outcome with the first two applying only in children younger than 6. In contrast with previous reports, preoperative visual field (VF) defects and type of surgery were not documented as prognostic indicators of poor postoperative visual acuity (VA) and VF. Contrary to previous reports calcification at diagnosis, type of surgery and preoperative VF defects were not found to be associated with tumour recurrence. Local recurrence is common. Younger age at presentation is associated with a tendency to recur. Magnetic resonance imaging (MRI) remains the recommended means of follow-up in patients with craniopharyngioma.