MULTIMODAL IMAGING OF CRB1 RETINITIS PIGMENTOSA WITH A PERIPHERAL RETINAL TUMOR
| Autor: | Srinivas R. Sadda, Alexander Juhn, Amani A. Fawzi, Tieu Vy Nguyen, David Sarraf, Federico Corvi, Giulia Corradetti |
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| Rok vydání: | 2020 |
| Předmět: |
Adult
medicine.medical_specialty Retinal Neoplasms Visual Acuity Nerve Tissue Proteins Fundus (eye) Multimodal Imaging Lesion chemistry.chemical_compound Vascularity Ophthalmology Retinitis pigmentosa medicine Humans Fluorescein Angiography Eye Proteins Retina Retinal pigment epithelium business.industry Retinal Degeneration Membrane Proteins Retinal General Medicine medicine.disease Peripheral Retinal Degeneration medicine.anatomical_structure chemistry Female sense organs medicine.symptom business Retinitis Pigmentosa Tomography Optical Coherence |
| Zdroj: | Retinal casesbrief reports. 16(4) |
| ISSN: | 1937-1578 |
| Popis: | Purpose To report the multimodal imaging findings of a patient with CRB1-associated retinitis pigmentosa (RP) characterized by preservation of para-arteriolar retinal pigment epithelium and a peripheral retinal tumor. Methods Case report. Patient A 27 year-old woman was referred to our center due to progressive decreased vision in both eyes with a diagnosis of CRB1-associated RP. Fundus examination was remarkable for attenuated retinal vessels and bone spicule migration that was bilateral and symmetric. In addition, an elevated yellow-white mass with dilated retinal vessels was noted in the superotemporal mid-periphery of the retina in the left eye without any associated exudation. Results Diffuse retinal pigment epithelium mottling was present but spared the area along the retinal arterioles. Swept-source optical coherence tomography (OCT) showed diffuse outer retinal atrophy. OCT angiography (OCTA) of the peripheral lesion illustrated extensive vascularity and a possible retinal feeder vessel communicating with the tumor at its inferior margin. The phenotype of the lesion showed overlap with a vasoproliferative tumor or an astrocytic hamartoma. Over a period of 5 years of follow-up, the peripheral tumor was unchanged. No significant progression of the peripheral retinal degeneration was evidenced by autofluorescent imaging over this time period although the central acuity continued to decrease. Discussion CRB1-associated RP may be characterized by preservation of para-arteriolar retinal pigment epithelium and slow progression, and may also feature a benign peripheral retinal tumor. |
| Databáze: | OpenAIRE |
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