The Management of Childhood Intracranial Tumours and the Role of the Ophthalmologist

Abstract

Objective: This study looked at a single paediatric neuro-oncology centre's experience of childhood intracranial tumours seen in the ophthalmology clinic over an approximately five-year period. This was used to analyse the role of the ophthalmologist in their long term follow up. Methods: A database was compiled of all children discussed at the neuro-oncology multidisciplinary team (MDT) meeting between January 2012 and April 2017. All children who had an intracranial tumour determined by histology or suspected on neuro-imaging, who had also been seen in the ophthalmology clinic, were included. A retrospective case review was performed to create a record for each child. Results: The database contained 129 children of which 82 (64%) were boys and 47 (36%) were girls. Of these 89 (69%) had a histological diagnosis and 40 (31%) had a tumour suspected on neuroimaging. The most common tumour locations were the posterior fossa (n = 54, 42%), diencephalon (n = 20, 16%) and the visual pathways (n = 17, 13%). Papilloedema at first presentation was only found in 39 (30%) children. The most common other neuro-ophthalmic manifestations were non-paralytic strabismus (n=33), sixth nerve palsy (n=19) and seventh nerve palsy (n=12). Non-paralytic strabismus was a presenting symptom in only one case. There were 13 ophthalmic surgical procedures required for these children, the most common being strabismus surgery. Conclusion: We report the types and locations of paediatric intracranial tumours seen in the ophthalmology clinic as well as their neuro-ophthalmic manifestations. Only 30% presented with papilloedema and approximately 10% required an ophthalmic surgical procedure.

Neuroophthalmic Manifestations of Intracranial Tumours in Children

“…Astrocytoma was the most frequent tumour seen in our study followed by medulloblastoma. This is consistent with most studies in the literature [ 2 , 6 , 15 , 26 – 28 ].…”

“…Ophthalmoparesis was seen in 15 (42%) patients in our study. Palsies of III, IV, and VI nerve were more common in children with infratentorial tumours than in children with supratentorial tumours which again correlated with the data available in the literature [ 15 ]. Acquired third nerve and fourth nerve palsies were seen in 3 cases including 2 cases of pontine tumours and one case of a sellar tumour with suprasellar and parasellar extension which were all due to direct compression on the nerves in all 3 cases.…”

Ophthalmological Findings in Youths With a Newly Diagnosed Brain Tumor

“…These findings are in line with previous studies, although the exact prevalence numbers of the specific ophthalmological diagnoses slightly differ. In particular, the percentage of papilledema was higher (74%) 34 and lower (11%-44%) 12,13,19,[35][36][37] in previous studies, whereas the percentage of visual field defects was comparable (27%) 13 or higher (50%-58%) 12,34 in previous studies, and the percentage of decreased VA (50%-54%) 13,20 and strabismus (45%-60%) 12,13,19,20 was higher in previous studies. An explanation for these differences in prevalence numbers may be referral and selection bias in previous retrospective studies, as also suggested by some of the authors 12,13,19,20 ; these biases are feasible given the incomplete ophthalmological evaluation in a substantial proportion of the included children in those studies.…”

“…Because of the use of a standardized ophthalmological screening protocol and the unselected inclusion of youths with all types of brain tumors, the association of the brain tumor with the visual function at diagnosis expand on results of previous studies. 12,13,19,20,34 The most prevalent ophthalmological abnormalities in youths at brain tumor diagnosis were papilledema (52.4%), gaze deficits (33.5%), visual field defects (28.1%), nystagmus (24.8%), strabismus (19.9%), and decreased VA (8.6%). These findings are in line with previous studies, although the exact prevalence numbers of the specific ophthalmological diagnoses slightly differ.…”

“…[14][15][16][17][18] Other studies have included children with all types of brain tumors but were primarily retrospective in nature, making them more prone to selection bias. 12,13,19,20 Prospective studies including larger numbers of patients with all types of brain tumors that investigate the visual function with standardized ophthalmological evaluation are lacking. Thus, we conducted a prospective, nationwide study of a cohort of consecutive youths with a newly diagnosed brain tumor in the Netherlands to assess the prevalence and types of abnormal ophthalmological findings.…”