Atlas of Ophthalmology

Homonymous Retinal Ganglien Cell Layer Thinning in Neurofibromatosis (Colour Photography Posterior Segment, VF, SD-OCT, MRI)

Patient: 36 years of age, male, BCVA 0.7 at OD, 0.8 at OS. General Medical History: Histology confirmed Morbus Recklinghausen, spastic hemiplegia after partial tumor resection of pilocytic atrocytoma, shunt installation and postoperative radiation therapy. Ocular Medical History: homonymous visual field defect to the right side. Purpose: to present reduced homonymous retinal ganglien cell layer of OD and OS corresponding to homonymous visual field defect after partial tumor resection of pilocytic atrocytoma in cerebral neurofibromatosis. Methods: Colour Photography Posterior Segment, optical coherence tomography (SD-OCT), visual field, MRI. Findings: Colour Photography Posterior Segment: optic nerve atrophy in OD and OS. SD-OCT : decreased thickness of peripapillary retinal nerve fiber layer in nasal and temporal area, OD>OS. SD-OCT : homonymous thinning of of retinal ganglion cell layer in OD and OS nasal, temporal, superior, and inferior to the fovea corresponding to homonymous visual field field defect. Visual Field: homonymous field defect in OD and OS. MRI: after partial tumor resection extensive defect area with surrounding gliosis in the left temporal and occipital lobes involving the left optic radiation. Discussion: It is recognized that significant retinal ganglion cell (RGC) loss occurs in MS patients following optic neuritis. A reduction in RGC may be identified earlier due to transient edema in the acute stage of ON masking any early RNFL loss. Several recent studies (1, 2) have suggested that RGC layer thinning is a good measure of disease-related visual and general disability and brain atrophy. Ocular manifestations of Von Recklinghausen disease are frequent and can reveal the disease. Benchekroun O et al. (3) examined 11 patients with Neurofibromatosis during two years. He suggested that an ophthalmologic examination must always be performed if Von Recklinghausen is suspected. Literature: (1) Saidha S, Syc S, Ibrahim MA, et al. Primary retinal pathology in multiple sclerosis as detected by optical coherence tomography. Brain. 2011; 134: 518–533. (2) Saidha S, Al-Louzi O, Ratchford JN, et al. Optical coherence tomography reflects brain atrophy in multiple sclerosis: a four-year study. Ann Neurol. 2015; 78: 801–813. (3) Benchekroun O, Lamari H, Moutawakkil A, Laouissi N, Zaghloul K, Amraoui A. Ophthalmological manifestations of Von Recklinghausen disease. J Fr Ophtalmol. 1997;20(10):753-7.

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