17 Fundus Manifestations of the Oculoneurocutaneous Syndromes (Phakomatoses) |
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There is no feeder artery and usually no yellow exudation, but white fibroglial tissue, possibly due to prior hemorrhage, is characteristically present on the surface of the tumor. The main complication of retinal cavernous hemangioma is vitreous hemorrhage. Severe fibrogliosis and dragging of the retina can occur. During fluorescein angiography, the vascular channels comprising the lesion remain hypofluorescent until the late venous phase when fluorescein begins to slowly enter the vascular spaces and produces the characteristic fluorescein-blood interface.
Controversies and Perspectives
This chapter has described the fundus manifestations of a diverse group of syndromes called the oculoneurocutaneous syndromes. There is some controversy regarding the terminology of these entities, and newer terminology may develop as their genetics are better understood. The fundus findings are fairly consistent in these entities, and the ophthalmic clinician should be able to recognize them and be aware of their ocular complications and system associations.
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Retinal and Choroidal |
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Manifestations of Systemic Lupus |
Erythematosus (SLE)
J. Fernando Arévalo, Careen Yen Lowder,
and Reinaldo A. Garcia
Abstract
Systemic lupus erythematosus (SLE) is a chronic, immunological disorder that may affect multiple organ systems. Keratoconjunctivitis sicca (KCS) is the most common ocular manifestation, but visual morbidity is usually due to retinal and neuro-ophthalmic manifestations of the disease. Ocular manifestations of lupus are a reflection of systemic disease. The presence of ocular manifestations should alert the clinician to the likely presence of disease activity elsewhere. Therefore, all patients with ocular lupus should be carefully evaluated for systemic involvement to detect potentially treatable and preventable complications of the disease. In addition, the ophthalmologist should include SLE in the differential diagnosis of many retinal vascular and neuro-ophthalmic disorders. The ophthalmologist may play an important role in the care of patients with SLE since ocular inflammatory lesions may precede potentially serious extraocular disease.
Keywords
Chronic immunological disorder • Lupus choroidopathy • Ocular manifestations • Systemic lupus erythematosus (SLE)
J.F. Arévalo, M.D., F.A.C.S.( ) |
R.A. Garcia, M.D. |
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Chief of Vitreoretinal Division, The King Khaled Eye |
Retina and Vitreous Department, Clínica Oftalmológica El |
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Specialist Hospital, Riyadh, Kingdom of Saudi Arabia |
Viñedo, Av. Andres Eloy Blanco con calle 139. El Viñedo, |
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Professor of Ophthalmology, Wilmer Eye Institute, |
Valencia, Carabobo 2001, Venezuela |
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e-mail: reinaldogarcia2003@hotmail.com |
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The Johns Hopkins University, Baltimore, MD, USA |
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e-mail: arevalojf@jhmi.edu |
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C.Y. Lowder, M.D., Ph.D. |
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Cleveland Clinic, Cole Eye Institute, 9500 Euclid Avenue |
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I-32, Cleveland, OH 44195, USA |
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e-mail: lowderc@ccf.org |
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J.F. Arévalo (ed.), Retinal and Choroidal Manifestations of Selected Systemic Diseases, |
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DOI 10.1007/978-1-4614-3646-1_18, © Springer Science+Business Media New York 2013 |
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