Treatment of neuro-ophthalmic and orbitofacial manifestations of neurofibromatosis type 1

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Abstract

Purpose of review

Optic pathway gliomas (OPGs) and orbitofacial plexiform neurofibromas are two of the more common ophthalmic manifestations of neurofibromatosis type 1. This article reviews recent advances in the treatment of these challenging lesions.

Recent findings

Recent advances in the treatment of OPGs include chemotherapeutic, radiation-based, and surgical interventions. Chemotherapy continues to be the mainstay of treatment of these lesions, but the effects on visual outcome are variable. Fractionated radiotherapy and gamma knife treatment have been studied as an alternative to conventional radiotherapy and have demonstrated fewer vision and life-threatening side-effects. Surgical resection can be undertaken through multiple approaches depending upon the extent of the lesion. The management of the orbitofacial neurofibroma is primarily surgical, and the systematic surgical approach to these lesions is discussed.

Summary

OPGs and orbitofacial neurofibromas are clinically diverse. Although the advances in the treatment of both are promising, recent studies demonstrate the great variety in treatment approaches and suggest a need for standardized outcome metrics for research that can ultimately contribute to guidelines for treatment.

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