Combined Hamartoma of the Retina and Retinal Pigment Epithelium in 77 Consecutive Patients Visual Outcome Based on Macular versus Extramacular Tumor Location

Purpose: To evaluate visual outcome of eyes with combined hamartoma of the retina and retinal pigment epithelium (RPE). Design: Noncomparative case series. Participants: Seventy-nine eyes of 77 patients. Methods: Retrospective chart review. Main Outcome Measures: Visual outcome. Results: The presenting symptoms were decreased vision (n = 32; 40%), strabismus (n = 22; 28%), both (n = 3; 4%), irritation (n = 4; 5%), and none (n = 18; 23%). The tumors had the following characteristics: a mean diameter of 7.6 mm, a mean thickness of 1.9 mm, round (n = 52; 66%) or curvilinear (n = 27; 34%) configuration, and other features including intralesional corkscrew vessels (n = 51; 65%), feeding straight vessels (n = 50; 63%), retinal traction (n = 64; 81 %), fibrosis/gliosis (n = 36; 46%), and exudation (n = 10; 13%). Referring diagnosis was unknown (n = 40; 51 %) or incorrect (n = 19; 24%) as retinoblastoma (n = 4), astrocytoma (n = 1), toxocariasis (n = 2), choroidal nevus (n = 5), melanoma (n = 6), and hemangioma (n = 1). The mean initial visual acuity by logarithm of the minimum angle of resolution (Snellen) for macular (n = 29) versus extramacular (n = 28) tumors was 1.2 (20/320) versus 0.61 (20/80) and at 4 years was 1.72 (20/800) versus 0.79 (20/125). Visual acuity loss of >= 3 Snellen lines was 60% versus 13%. By univariate analysis, the most important factors predictive of poor visual acuity included macular location and clock hour meridian of the tumor. Conclusions: Combined hamartoma of the retina and RPE can cause profound visual acuity loss, particularly with macular tumors. Financial Disclosures: The authors have no proprietary or commercial interest in any materials discussed in this article. Ophthalmology 2008;115:2246-2252 (C) 2008 by the American Academy of Ophthalmology.