Clinical manifestations of benign tectal glioma in children and adolescents

Introduction: Tectal gliomas are commonly associated with the paucity of focal neurological signs due to the expansive mass itself, and their presentation with obstructive hydrocephalus.

Objectives: To analyze retrospectively the clinical features of tectal glioma in relation to age, its delay of onset, tectal tumour volume, and MRI flowmetric study in children who underwent neuroendoscopic third ventricle fenestration (ETV).

Methods: Clinical and imaging data of children who underwent ETV were reviewed. A successful outcome was defined as resolution of symptoms and signs of raised intracranial pressure, with MRI evidence of ventriculostomy patency.

Results: Between 1998 and 2006, 16 patients (9m; 7 f) underwent ETV for the management of hydrocephalus secondary to a tectal plate glioma. The median age at the time of surgery was 8.9 (range 3–18) years. As far as clinical appearance, two separate subgroups have been identified: Group A (n=8) with acute-onset hydrocephalus, necessitating urgent neurosurgical treatment; and Group B (n=8) with chronic intracranial hydrocephalus with subtle clinical signs of chronic intracranial hypertension. In Group A the clinical manifestations were associated with vomiting, with a mean clinical interval to diagnostic imaging of 4.5 days. In Group B the clinical onset resulted in lethargy (n=7), memory loss (n=5), tremors (n=3), diplopia (n=2), tetraparesis (n=2) with a mean clinical interval to diagnostic imaging of 69 weeks. There was no difference in tumour volume in these two subgroups (mean=1.4mL). 7/8 patients with ages <8 years were included in Group A, and 8/8 patients with ages >8 years in Group B.

Conclusion: This data suggests that the manifestation of tectal glioma comprises a subacute picture of intracranial hypertension occurring in young children, and a clinical entity of chronic intracranial hypertension which is prevalent instead in children >8 years old and adolescents, with each of these groups displaying different delays of clinical onset. Tectal volume was not correlated to clinical coming-out.