Simple hamartoma of the retinal pigment epithelium with macular edema. 2015

Austin Bach, and Aaron S Gold, and Victor M Villegas, and Andrea C Wildner, and Azeema Latiff, and Fiona J Ehlies, and Timothy G Murray
*DO, MPH †OD ‡MD §CRA ∥BSN, MBA **BSc, CDOS ††MD, MBA Larkin Community Hospital, South Miami, Florida (AB); and Murray Ocular Oncology and Retina, Miami, Florida (ASG, VMV, ACW, AL, FJE, TGM).

OBJECTIVE Presumed congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare intraocular finding that is described as a focal, nodular, jet black lesion. These lesions frequently occur at or near the macula and have no known association with changes in the surrounding neurosensory retina, retinal pigment epithelium, or choroid, nor have they been related with exudation or hemorrhage. Until now, there have been no cases reported of CSHRPE with associated vascular activity. METHODS A 14-year-old Hispanic adolescent girl with a presumed CSHRPE presented with adjacent macular edema. The patient was treated with an off-label intravitreal bevacizumab injection to decrease the edema. Two months later, the patient presented with improved visual acuity and decreased macular edema. CONCLUSIONS This is the first documented case of macular edema secondary to vascular activity being associated with these rare lesions. When CSHRPE is found to have secondary vascular activity and associated macula edema, treatment of intravitreal bevacizumab may lead to improved visual and anatomical outcomes.

UI MeSH Term Description Entries
D008269 Macular Edema Fluid accumulation in the outer layer of the MACULA LUTEA that results from intraocular or systemic insults. It may develop in a diffuse pattern where the macula appears thickened or it may acquire the characteristic petaloid appearance referred to as cystoid macular edema. Although macular edema may be associated with various underlying conditions, it is most commonly seen following intraocular surgery, venous occlusive disease, DIABETIC RETINOPATHY, and posterior segment inflammatory disease. (From Survey of Ophthalmology 2004; 49(5) 470-90) Central Retinal Edema, Cystoid,Cystoid Macular Edema,Macular Edema, Cystoid,Cystoid Macular Dystrophy,Cystoid Macular Edema, Postoperative,Irvine-Gass Syndrome,Macular Dystrophy, Dominant Cystoid,Edema, Cystoid Macular,Edema, Macular,Irvine Gass Syndrome,Syndrome, Irvine-Gass
D012164 Retinal Diseases Diseases involving the RETINA. Disease, Retinal,Diseases, Retinal,Retinal Disease
D005260 Female Females
D005451 Fluorescein Angiography Visualization of a vascular system after intravenous injection of a fluorescein solution. The images may be photographed or televised. It is used especially in studying the retinal and uveal vasculature. Fluorescence Angiography,Fundus Fluorescence Photography,Angiography, Fluorescein,Angiography, Fluorescence,Fluorescence Photography, Fundus,Photography, Fundus Fluorescence
D006222 Hamartoma A focal malformation resembling a neoplasm, composed of an overgrowth of mature cells and tissues that normally occur in the affected area. Hamartomas
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000068258 Bevacizumab An anti-VEGF humanized murine monoclonal antibody. It inhibits VEGF RECEPTORS and helps to prevent PATHOLOGIC ANGIOGENESIS. Avastin,Bevacizumab-awwb,Mvasi,Bevacizumab awwb
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D014792 Visual Acuity Clarity or sharpness of OCULAR VISION or the ability of the eye to see fine details. Visual acuity depends on the functions of RETINA, neuronal transmission, and the interpretative ability of the brain. Normal visual acuity is expressed as 20/20 indicating that one can see at 20 feet what should normally be seen at that distance. Visual acuity can also be influenced by brightness, color, and contrast. Acuities, Visual,Acuity, Visual,Visual Acuities
D016896 Treatment Outcome Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series. Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes

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