# Ophthalmic Manifestation in Neurofibromatosisosis Type 2  
[PERSON]  
1 Department of Sense Organs, Sapienza University of Rome, Policlinico Umberto I, Viale del Policlinico 155, 00161 Rome, Italy; [EMAIL_ADDRESS] (M.A. ); luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luca.luc.luca.luc.luca.luc.luca.luc.luca.luc.luca.luc.luc.luca.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc..luc.luc.luc..luc.luc.luc.luc.luc..luc.luc.luc..luc.luc..luc.luc.luc..luc..luc.luc..luc.luc..luc..luc..luc..luc..luc..luc..luc..luc...luc...  
[PERSON]  
1 Department of Sense Organs, Sapienza University of Rome, Policlinico Umberto I, Viale del Policlinico 155, 00161 Rome, Italy; [EMAIL_ADDRESS] (M.A. ); luca.luca.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc..luc.luc.luc.luc.luc..luc..luc..  
[PERSON]  
1 Department of Sense Organs, Sapienza University of Rome, Policlinico Umberto I, Viale del Policlinico 155, 00161 Rome, Italy; [EMAIL_ADDRESS] (M.A. ); luca.luca.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc.luc..luc.luc.luc.luc.luc..luc.luc.luc.luc.luc.luc..luc.luc.luc.luc..luc.luc.luc.luc.luc..luc.luc.luc..luc..luc.luc.luc..luc.luc..luc.luc.luc..luc..luc.luc.luc.luc..luc.luc..luc.luc.luc..luc.luc.luc.luc.luc..luc.luc..luc.luc  
intracranial and intraspinal. Moreover, low-grade central nervous system (CNS) malignancies, such as ependymomas and gliomas, and skin tumors can occur [5]. The Manchester criteria, introduced in 1992, are the best-known diagnostic criteria for NF2 [6]. According to these criteria, a patient with suspicion of NF2 must meet one of the following clinical features:  
1. Bilateral vestibular schwannomas;
2. First-degree relative with NF2 and unilateral vestibular schwannomas;
3. First-degree relative with NF2 or unilateral vestibular schwannomas and two among meningioma, cataract, glioma, neurofibroma, schwannoma, cerebral calcification;
4. Multiple meningiomas and two among unilateral vestibular schwannomas, cataract, glioma, neurofibroma, schwannoma, and cerebral calcification. These criteria have been revised many times over the years. Recently, updates have been made, to distinguish between major and minor criteria and consider genetic analysis [7]. Specifically, the diagnostic criteria for NF2 have been recently expanded from the original Manchester criteria to include LZTR1 mutation testing in individuals with unilateral VS and other schwannomas. Moreover, two further changes were introduced, including NF2 mutational testing and the insertion of an age limitation of 70 for the development of bilateral VS if no other NF2 features are present. NF2 phenotype severity is difficult to predict, and clinical manifestations appear variable among patients. [PERSON] et al. introduced a genetic severity score to predict the clinical expression of NF2. This score allows the connection of different genetic characteristics to a specific clinical disease presentation. This system consents to the individuation of tissue mosaicism form, classic and severe disease [8]. Interestingly, the genetic severity subtype correlated to NF2-related eye disease. Specifically, mutations associated with severe systemic disease resulted in greater visual morbidity at an earlier age. To date, ocular pathologic findings of NF2 have been reported in a few retrospective series. A wide spectrum of ocular manifestations has been recognized to be specifically linked to NF2. Different clinical manifestations can occur in different age groups. Congenital cataracts, optic disc anomalies, and retinal hamartomas are responsible for the failure of binocular vision development in childhood.