2019 - 4 Issue

Original article

Neurofibromatosis Type 1 and Optic Pathway Glioma

Aneta Siwá
Rudolf Autrata ORCID – Klára Vejmělková – Zdeněk Pavelka – Pavel Zitterbart

Full Text links

DOI: 10.31348/2019/4/4

Published in Czech and Slovak Ophtalmology: 2019 - 4

Published on website: 06.01.2020

Keywords: neurofibromatosis, children, optic pathway glioma, chemotherapy, treatment

Summary

Purpose: Evaluate the effectiveness of treatment of patients with optic pathway glioma.

Materials and Methods: Comparison of literature research on neurofibromatosis and optic pathway glioma with a cohort of pediatric patients treated at the Children‘s Ophthalmology Clinic of the University Hospital in Brno from January 2013 until June 2018.

Discussion: The main challenge of this and other retrospective studies is variable intervals between ophthalmologic examinations. In some pediatric patients it is also difficult to objectively assess visual functions. The main risk factors are age at the time of treatment and tumor localization. Tumor progression itself does not always correlate with worse visual acuity outcomes, and it remains to be evaluated whether some patients would be better off without treatment. As of now, there are no clinical biomarkers able to predict impending visual acuity loss.

Conclusion: The cohort outcome agrees with literature. Chemotherapy remains a treatment of choice and its most likely outcome is visual acuity stabilization. In order to properly evaluate the treatment’s effectiveness, better collaboration between medical specialists and regular standardized ophthalmology examinations are required.

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