Neurofibromatosis 2 leads to higher incidence of strabismological and neuro-ophthalmological disorders.

Purpose: Ophthalmic features of neurofibromatosis 2 (NF2) include juvenile cataract, retinal hamartomas and tumours of the cranial nerves. We hypothesize that these tumours lead to strabismological and neuro-ophthalmological symptoms, including palsies of cranial nerves III, IV and VI, nystagmus and gaze palsies.

Methods: We carried out a retrospective review of 73 patients with known genotype.

Long-term visual function and relative amblyopia in posterior persistent hyperplastic primary vitreous (PHPV).

Background: Favorable results in the management of PHPV, made possible by improved surgical techniques, are described in the literature. However, the functional results are in general in the low-vision sector (rarely better than 0.025-0.