Pars Plana Vitrectomy and Epiretinal Membrane Peeling for Vitreoretinal Traction Associated with Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHRRPE)

Abstract

Combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) are rare, benign tumours that typically appear slightly elevated and are characterised by varying amounts of pigmentation, vascular tortuosity, and epiretinal membrane formation that can be related to macular distortion. The importance of changes at the vitreoretinal interface in this condition as a risk for visual loss was early recognised but controversy exists concerning the benefits of surgical removal of the tractional component. We describe 2 cases of CHRRPE who demonstrated significant visual acuity improvement after pars plana vitrectomy and epiretinal membrane peeling. A 12-year-old girl and a 14-year-old boy presented with progressive unilateral visual loss related with a CHRRPE. In both instances the hamartoma was associated with a thickened posterior hyaloid and epiretinal membrane inducing considerable vitreomacular traction as shown by optical coherence tomography (OCT). Best-corrected pre-operative visual acuity was 0.2 in both cases. Vitrectomy and epiretinal membrane peeling were performed in both cases. Relief of vitreomacular traction and macular distortion was achieved and documented on OCT. Visual acuity at the latest follow up was 0.5 and 0.8, respectively. Surgical dissection can be effective in the treatment of vitreomacular traction associated with CHRRPE with a good functional and anatomical outcome.