Title

BEST DISEASE IN A KIDNEY TRANSPLANTED PATIENT

Introduction

Best vitelliform macular dystrophy (BVMD) is one of the most common macular dystrophies and typically presents in childhood. Its characteristic presentation is a bilateral fundus changes of egg-yolk appearance at the macula in both eyes. The term vitelliform is used to describe the typical fundoscopic findings of yellowish and oval subretinian deposits. Retinal pigmented epithelium (RPE) is primarily affected. BVMD can complicate with choroidal neovascularization (CNV), resulting in substantial vision impairment. Patients undergoing kidney transplantation may suffer some damage to the eye’s tissues, such as reduction of the retinal nerve fiber layer (RNFL) thickness, retinal drusen and hypertensive retinopathy.

Methods

Medical record review

Results

A 30-years-old female patient presented with a history of kidney transplantation 5 years ago and complained about progressive bilateral visual loss. Her best corrected visual (BVC) was 20/100 in the right eye and 20/60 in the left eye. Fundoscopy of both eyes showed yellowish lesion with vitelliform aspect in the fovea . The OCT showed a hyperreflective subretinal deposit. The Fundus Autofluorescence revealed a round central area of hyperautofluorescence in the posterior pole. Fluorescein angiography demonstrated the presence of hyperfluorescence by staining in the macular region. She had unaltered arterial pressure and the laboratory workup showed normal renal function.

Discussion

Our patient did not have any of the classical retinal findings related to kidney transplantation. Her fundoscopy was the typical phenotypic manifestation of BVMD without CNV. Therefore, she needs no ophthalmologic treatment by now. Regular follow-up is vital for early detection of BVMD’s complications, especially CNV. In such cases, the visual outcome may be better when these patients are treated with anti-VEGF agents. In our case, the patient did not have CNVM ad is being clinically followed up.

Keywords

macular dystrophy, vitelliform, neovascularization,

Area

CLINICAL CASE

Authors

CAROLINA Maria Barbosa LEMOS, CAROLINA Maria Barbosa LEMOS, CAROLINA Maria Barbosa LEMOS, Paulo Henrique Horizonte, Paulo Henrique Horizonte, Paulo Henrique Horizonte, Rafael Garcia, Rafael Garcia, Rafael Garcia, Walther de Oliveira Campos Neto, Walther de Oliveira Campos Neto, Walther de Oliveira Campos Neto, Fernanda De Castro Rollo Sampaio, Fernanda De Castro Rollo Sampaio, Fernanda De Castro Rollo Sampaio, Andréia Novelli, Andréia Novelli, Andréia Novelli, Nina Rosa Konichi Da Silva, Nina Rosa Konichi Da Silva, Nina Rosa Konichi Da Silva, André Marcelo Vieira Gomes, André Marcelo Vieira Gomes, André Marcelo Vieira Gomes