Surgery for Combined Hamartoma of the Retina and Retinal Pigment Epithelium

Abstract

There is no consensus on whether and when surgical treatment is indicated for combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). We aim to discuss the benefits of surgical intervention and techniques that may improve the outcome. A 24-year-old man experienced progressive visual loss for 6 months in his left eye due to CHRRPE. At presentation, visual acuity was 1.3 LogMAR and fundoscopy revealed extensive tractional pre- and epiretinal membranes, subretinal exudation, and a vasoproliferative tumor in the inferior periphery. A complete vitrectomy was performed, while paying special attention to vitreous shaving at the vitreous base and removal of vitreoschisis-induced vitreous cortex remnants (VCR) from the retinal surface posterior to the vitreous base. Tractional membranes and internal limiting membrane were peeled, and the vasoproliferative tumor was excised. Silicone oil tamponade was removed 11 weeks after surgery. No intra- or postoperative complications occurred. Visual acuity improved to 0.8 LogMAR and remained stable for 48-month follow-up. Vitreoretinal surgery can prevent complications that occur with CHRRPE. In addition, visual function may improve even if the initial visual acuity is low. Timely and complete vitrectomy with extensive membranectomy and detection and removal of VCR is recommended to avoid complications in challenging CHRRPE.

Description and surgical management of epiretinal membrane due to combined hamartoma of the retina and retinal pigment epithelium

“…A total of 13 small case reports (43 patients) have shown that surgery results shows good visual outcomes (success rate >70%). 17 , 18 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 It was found that 39 of the 43 patients (90.7%) had postoperatively improved in VA. Because of rarity of the disease the largest cohort study was published by Sun et al. 30 in 2020 with only 15 patients.…”

“…Because of the publication of these small case studies, including improved fluidics control, small-gauge instruments, better internal light sources, and the development of new dyes, that surgical outcomes can be improved and complications reduced. 31 …”