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Isolated and combined hamartomas of the RPE
Author(s) -
SCHALENBOURG A
Publication year - 2013
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2013.3242.x
Subject(s) - enucleation , posterior pole , medicine , metamorphopsia , hamartoma , retinal detachment , retina , asymptomatic , ophthalmology , retinal , anatomy , pathology , surgery , biology , neuroscience
RPE hamartomas are rare, presumed congenital proliferations of the RPE (isolated) or both the RPE and neuroretina (combined), without malignant potential. Isolated hamartomas are well delineated, small, pigmented and asymptomatic lesions, most often in the macular region and not requiring any treatment. Combined hamartomas are unilateral malformations consisting of a densification of the RPE and vascularized gliosis of the overlying neuroretina. Its location at the posterior pole is correlated with varying degrees of visual impairment, for which epiretinal membrane peeling remains controversial. Treatment is occasionally required in case of complications such as neovascular membrane, vitreous haemorrhage, retinal detachment or macular hole. Being benign, it is crucial that RPE hamartomas are differentiated from other pigmented lesions, and most importantly choroidal melanoma, to avoid unnecessary radiotherapy or even enucleation in the –usually young‐ patients that present them.