Congenital hypertrophy of retinal pigment epithelium presenting as incidental finding in a middle aged woman
Journal Title: IP International Journal of Ocular Oncology and Oculoplasty - Year 2018, Vol 4, Issue 2
Abstract
Introduction Retinal Pigment epithelial hypertrophy are uncommon benign epithelial malformation characterised by typical fundus findings which is almost always congenital in nature RPE hypertrophy occurs in three varieties unifocal unilateral lesion multifocal unilateral lesion and atypical multifocal bilateral lesion While the atypical multifocal bilateral lesions are associated with familial adenomatous polyposis syndrome requires systemic evaluation the other two varieties have benign coarse doesnot require any interventionCase Report A 42yr Female noticed diminished vision in RE following trivial trauma with fingernail 14 days back it was not associated with pain redness or any other symptoms On evaluation BCVA was 618p N6 RE 66 N6 LE rest anterior segment evaluation was normal RE fundus showed focal 1 DD sized welldemarcated hyperpigmented lesion just temporal to fovea with blocked fluorescence in all phase of FFA OCT Bscan showed slightly elevated retinal lesion with optical shadowing Diagnosis of Retinal pigment epithelial hamartoma RPEH was made Lesion was documented followedDiscussion and Conclusion RPE hypertrophy are presumed congenital stationary retinal malformation with characteristic ophthalmic fluorescerin angiographic OCT features These lesions present as asympotomatic incidental findings in childhood or adults with good visual prognosis if located at extrafoveal sites Gass et al shields et al had described various types of RPE malformations their characteristic features in FFA OCTIn present case we have reported a rare case of RPEH also demonstrated the role of ancillary tests for diagnosis followupKeywords Congenital retinal pigment epithelial hypertrophy RPE hypertrophy Congenital retinal malformation
Authors and Affiliations
Harsh Vardhan Singh, Iva Rani Kalita, Shubhra Das
Keywords
Related Articles
- EP ID EP513424
- DOI 10.18231/2581-5016.2018.0027
- Views 105
- Downloads 0
Chemofailure and chemoresistance in retinoblastoma.
Retinoblastoma (RB) is the most common malignant intraocular tumor in children constituting 4% of all pediatric cancers. (1) RB1 gene mutation affects the developing retina resulting in this malignant tumor which is most...
Heterogeneity of xeroderma pigmentosa: A case series
Xeroderma Pigmentosa (XP) results in hypersensitivity to ultraviolet radiation. We intend to report different clinical presentations of XP presenting to a tertiary eye care centre. 3 cases of different age groups i.e. 3,...
Lacrimal sac diverticulum associated with Rhinosporidiosis –Atypical presentation
Lacrimal sac diverticulum can be congenital, inflammatory or traumatic in origin, in our case inflammation secondary to Rhinosporidiosis was the cause for development of diverticulum. Rhinosporidiosis is a chronic granul...
Orbital Lymphoma: Clinical Profile, at a tertiary care hospital, Bangladesh
Purpose: To explore the baseline clinical profile of orbital lymphoma, at a tertiary care in Bangladesh. Materials and Method: As part of routine delivery services, we evaluated data from January 2010 to June, 2017 of th...
A case report: Bardet-Biedle syndrome
Bardet-Biedle syndrome is an autosomal recessive disorder named after George Bardet & Arthur Biedle. Bardet-Biedle syndrome is characterized by rods-cones dystrophy, truncal obesity, postaxial polyductyly, cognitive impa...