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OCULAR-AND-DERMATOLOGICAL-MANIFESTATIONS-OF-NEUROFIBROMATOSIS

Fatimah Fawzi Alfaleh , Mohammed Sharaf Saad Alshahrani , Saad Abdulla Saad AlGhamdi , Ali Hassan J Alzahrani , Hussain Mosaed Saif Alqhtani , Mohammed Mousa Alghamdi , Fahd B. Altherwi , Rafeef Abdulaziz Alsaleem , Norah Saad Jubran Alkahtani , Faten Saad Jubran Alkahtani , Wejdan Qublan Almuqati

Introduction: Optic nerve sheath meningiomas (ONSMs) are tumors of the meninges surrounding the optic nerve, they are usually benign neoplasms. It is usually presented with vision loss, optic atrophy, and optociliary shunt vessels on fundoscopy, in patients between 30-39 years old. Although the tumors of optic nerve are considered rare, ONSMs account for nearly 30% of optic nerve intrinsic tumors. ONSMs can be associated with type II neurofibromatosis. Usually, ONSMs are not associated with any mortalities and with very little nonvisual morbidities. On the other hand, they are often associated with visual loss in the affected eye. Treatment of ONSMs also have a high risk of loss of vision, so, the goal of the management of ONSMs patients is to balance the risk of progression leading to visual loss with the risk that accompany the treatment Aim of work: In this review, we will discuss Ocular-and-dermatological-manifestations-of-neurofibromatosis. Methodology: We did a systematic search for Ocular-and-dermatological-manifestations-of-neurofibromatosis using PubMed search engine (http://www.ncbi.nlm.nih.gov/) and Google Scholar search engine (https://scholar.google.com). All relevant studies were retrieved and discussed. We only included full articles. Conclusions: ONSMs are considered difficult to diagnose and manage, due to the nature of this lesions, treatmentinduced morbidity and the effect of the different lines of treatment on the vision in the affected eye. Given the variable natural history of these lesions, there will always be uncertainty around when and how to initiate treatment. Nevertheless, with earlier detection with improved imaging technology, more accurate radiotherapy delivery, and improved case selection for surgical management, it is hoped that outcomes will continue to improve for these patients. Key words: Ocular, dermatological manifestations, neurofibromatosis

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