Purpose: To describe an 8-year-old girl with diagnosis of Sturge–Weber syndrome (SWS) presenting with glaucoma, abnormal iris vessels, diffuse choroidal hemangioma, and small white dot-shaped “microdrusen-like” alterations of the retina in the right eye. Patient and methods: Complete ophthalmological examination was performed with slit-lamp anterior segment assessment and fundus ophthalmoscopy. Near infrared reflectance (NIR) and enhanced depth imaging (EDI) spectral domain optical coherence tomography (SD-OCT) were carried out. Results: Microdrusen-like retinal alterations observed with fundus ophthalmoscopy appeared as multiple hyperreflective dots surrounded by a hyporeflective ring on NIR. EDI SD-OCT showed increased choroidal thickness exceeding 1000 μm. B-scan cross-sectional examination on the hyperreflective dots revealed focal alterations of the retinal pigment epithelial (RPE)–photoreceptor layer. Conclusions: The increase of the choroidal thickness due to the diffuse choroidal hemangioma caused alterations of the RPE–photoreceptor layer similar to reticular pseudodrusen or pachychoroid pigment epitheliopathy.
Retinal pigment epithelium–photoreceptor layer alterations in a patient with Sturge–Weber syndrome with diffuse choroidal hemangioma / Abdolrahimzadeh, Solmaz; Parisi, Francesco; Mantelli, Flavio; Perdicchi, Andrea; Scuderi, Gianluca. - In: OPHTHALMIC GENETICS. - ISSN 1381-6810. - ELETTRONICO. - 38:6(2017), pp. 567-569. [10.1080/13816810.2017.1313995]
Retinal pigment epithelium–photoreceptor layer alterations in a patient with Sturge–Weber syndrome with diffuse choroidal hemangioma
Abdolrahimzadeh, Solmaz;Scuderi, Gianluca
2017
Abstract
Purpose: To describe an 8-year-old girl with diagnosis of Sturge–Weber syndrome (SWS) presenting with glaucoma, abnormal iris vessels, diffuse choroidal hemangioma, and small white dot-shaped “microdrusen-like” alterations of the retina in the right eye. Patient and methods: Complete ophthalmological examination was performed with slit-lamp anterior segment assessment and fundus ophthalmoscopy. Near infrared reflectance (NIR) and enhanced depth imaging (EDI) spectral domain optical coherence tomography (SD-OCT) were carried out. Results: Microdrusen-like retinal alterations observed with fundus ophthalmoscopy appeared as multiple hyperreflective dots surrounded by a hyporeflective ring on NIR. EDI SD-OCT showed increased choroidal thickness exceeding 1000 μm. B-scan cross-sectional examination on the hyperreflective dots revealed focal alterations of the retinal pigment epithelial (RPE)–photoreceptor layer. Conclusions: The increase of the choroidal thickness due to the diffuse choroidal hemangioma caused alterations of the RPE–photoreceptor layer similar to reticular pseudodrusen or pachychoroid pigment epitheliopathy.File | Dimensione | Formato | |
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