Title

CASE REPORT OF A CONGENITAL SIMPLE HAMARTOMA OF THE RETINAL PIGMENT EPITHELIUM

Introduction

Report a case of a Congenital Simple Hamartoma of the retinal pigment epithelium (CSHRPE) and discuss the topic.

Methods

Analysis of medical records from Hospital Oftalmológico Santa Beatriz (HOSB). A 51-year-old female was referred for a routine ophthalmic examination to our institution. She had no known comorbidities and her Visual Acuity with best correction was 20/20 J1 in both eyes. Intraocular pressure was 19 mmHg (OD) and 18 mmHg (OS) and the Biomicroscopy examination was normal in both eyes. Fundus examination in OD presented a circumscribed and pigmented lesion in the macular region, without any other remarkable findings. OS fundus examination was normal. Fluorescein Angiography ( Fig 1) showed a nonfluorescent lesion during all phases and the Optical Coherence Tomography (OCT) (FIG 2) presented an abruptly elevated hyper-reflective mass with deep optical shadowing. Ecography was realized and reveled no alterations.

Results

The patient was followed up by observation since the lesion was compatible with a CSHRPE.

Discussion

Clinical examination of a CSHRPE reveals a well-defined, round, hyper-pigmented lesion that is present on the retinal surface (1). The clinical appearance of this rare benign tumor is suggestive and the OCT appearance is pathognomonic of the diagnosis (2). Differential diagnosis include RPE congenital hypertrophy, RPE adenocarcinoma adenoma, RPE hyperplasia, intraretinal foreign body, and choroidal nevus (melanocytoma) or choroidal melanoma. It is necessary to rule out these pathologies due to the potential damage some of them can cause to the patient. Since there are some case reports of vitreomacular traction and macular edema, regular ophthalmic examinations of the patient are recomended.

Keywords

HAMARTOMA, CONGENITAL ABNORMALITIES, CASE REPORT

Area

CLINICAL CASE

Authors

THIAGO SIQUEIRA ROCHA, TULIO SANTOS SIQUEIRA PECANHA, RAPHAEL DE ALMEIDA PENTAGNA, ARMANDO MAGALHÃES NETO