Long term evolution of combined hamartoma of the retina and retinal pigment epithelium

Abstract

Combined hamartoma of the retina and of the retinal pigment epithelium are extremely rare benign tumors distinguished by retinal pigment epithelium (RPE) and glial tissue proliferation, provoking severe peripapillary and retinal distortion. There is no established management for combined hamartoma of the retina and of the retinal pigment epithelium. A 46-year-old male and a 35-year-old female, diagnosed as having a combined hamartoma of the retina and retinal pigment epithelium were observed for four years. One of them was treated with pars plana vitrectomy due to large associated epiretinal membrane (ERM), and the other patient declined surgery. Initially, the evolution was satisfactory in the patient who underwent vitreoretinal surgery, with anatomic improvement and visual acuity stabilization. However, four years later, he complained of visual loss, presenting severe macular distortion, and, consequently, decrease in visual acuity. The patient who preferred a conservative management, showed no funduscopic changes during the follow-up. Vitreoretinal surgery for combined hamartoma of the retina and retinal pigment epithelium may improve retinal architecture and visual acuity, but in the long-term functional recovery is frequently not possible, leading to a decrease in VA. We can conclude that the evolution of this pathology can be similar in both cases treated with vitreoretinal surgery and in cases without surgery. Therefore, conservative management may be adopted. A larger number of cases are required to be studied to confirm our clinical observations.

Keywords

• Combined Hamartomas of Retina
• Retinal Pigment Epithelium, vitrectomy.