Visual function tests including the role of optical coherence tomography in neurofibromatosis 1
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ANNUAL ISSUE PAPER
Visual function tests including the role of optical coherence tomography in neurofibromatosis 1 Daphna Mezad-Koursh 1,2
&
Anat Bachar Zipori 1,2 & Dinah Zur 1,2 & Lior Degabli 1,2 & Meital Ben-Dov 1,2 & Ainat Klein 1,2
Received: 1 May 2020 / Accepted: 25 May 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Optic pathway glioma (OPG) is a common and significant complication of neurofibromatosis 1 (NF-1) that might lead to vision loss. The main reason to treat OPG is to preserve vision. Tumor location along the visual pathway largely dictates the presenting signs and symptoms. Clinical ophthalmic evaluation is focused on optic nerve functions including evaluation of pupils’ reaction to light, visual acuity, color vision, and visual field, as well as optic nerve appearance. An important relatively new ancillary test is optic coherence tomography (OCT) that measures the volume of retinal nerve fiber layer around the optic nerve and the ganglion cell layer-inner plexiform layer (GCL-IPL) of the macula, both proved to be strongly associated with losing vision in OPG. Accurate evaluation of vision functions plays a critical role in the decision of treatment. In this review, we describe the ophthalmological assessment including new biomarkers in clinical use. We also outline prognostic factors and current recommendations for surveillance and indications for treatment. Keywords Optic pathway glioma . Visual function . Optical coherence tomography (OCT)
Neurofibromatosis 1 (NF-1) is an autosomal dominant disorder. Patients with NF-1 tend to develop multiple benign and malignant tumors of the central and peripheral nervous systems. The eye and ocular adnexa are frequently involved in NF1. Lisch nodules, plexiform neurofibromas, and optic pathway gliomas (OPGs) are part of the diagnostic criteria of NF-1, and hence, many suspected patients are referred to an ophthalmologist as part of the initial assessment to reach a diagnosis. OPG is one of the most common and significant complications of NF-1 with an estimated prevalence of 15–20% [12, 44, 45, 71]. Though OPG is a low-grade neoplasm, it may lead to a range of vision and systemic complications; from reduced visual acuity and visual field loss, proptosis, strabismus, and Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00381-020-04706-3) contains supplementary material, which is available to authorized users. * Daphna Mezad-Koursh [email protected] 1
Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
2
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
nystagmus to headaches, seizures, and precocious puberty [37, 44]. Only 30–50% of the patients will have visual signs and symptoms [27, 42, 44] that will require treatment [30]. OPG can also be sporadic. Symptomatic sporadic optic gliomas are usually more aggressive than NF-1 optic gliomas and cause more severe impairment to vision [68]. While OPG in NF-1 patients have a potential to c
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