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

To evaluate visual outcome of eyes with combined hamartoma of the retina and retinal pigment epithelium (RPE). Noncomparative case series. Seventy-nine eyes of 77 patients. Retrospective chart review. Visual outcome. 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 >or=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. Combined hamartoma of the retina and RPE can cause profound visual acuity loss, particularly with macular tumors. The authors have no proprietary or commercial interest in any materials discussed in this article.