OSLI Retina

October 2020

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546 Ophthalmic Surgery, Lasers & Imaging Retina | Healio.com/OSLIRetina ■ C L I N I C A L S C I E N C E ■ Surgical Outcomes of Epiretinal Membrane Removal Due to Combined Hamartoma of the Retina and RPE Lucy S. Sun, MD; Sherief Raouf, MD; David Rhee, MD; Philip J. Ferrone, MD BACKGROUND AND OBJECTIVE: To evaluate the sur- gical outcomes of epiretinal membrane (ERM) asso- ciated with combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) after vitrec- tomy and membrane peel. PATIENTS AND METHODS: A retrospective review of 15 patients who underwent pars plana vitrec- tomy with membrane peeling. No plasmin en- zyme was used. RESULTS: The mean age at surgery was 10 years old, with an average follow-up of 5.7 years. The average preoperative visual acuity (VA) was 20/514. The average postoperative VA was 20/138 (P = .0251) at 1-year follow-up and 20/89 (P = .0025) on the lat- est exam on final follow-up. VA was improved in 14 patients (93%) and deteriorated in one patient (7%). All 15 patients (100%) had improvement of retinal/macular anatomy postoperatively. CONCLUSION: In the treatment of patients with ERM due to CHRRPE, vitrectomy with membrane peeling without plasmin injection can result in im- proved VA and retinal structure. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:546-554.] INTRODUCTION Combined hamartoma of the retina and the reti- nal pigment epithelium (CHRRPE) is a benign pro- liferation of the pigment epithelium, neurosensory retina, retinal vasculature, and adjacent vitreous. 1,2 It is usually solitary, unilateral, and can be located on or adjacent to the optic disc, at the macula, or in the peripheral retina. 1 The clinical appearance of the le- sion falls on a spectrum depending on the predomi- nating tissue subtype: melanocytic, vascular, or glial. 3 Common characteristics include vascular tortuosity (93%), variable pigmentation (87%), elevation (80%), and epiretinal membrane (ERM) formation (78%). 3 Visual impairment may occur from direct involve- ment of the tumor on the optic nerve head, papil- lomacular bundle, or fovea. Or in cases where the lesion is located elsewhere, visual loss can be due to foveal distortion from ERM formation leading to tractional retinal elevation via tumor or ERM contrac- tion. Diagnosis is typically made in early childhood speculating the congenital nature of CHRRPE, but visual acuity (VA) may continue to deteriorate over time secondary to progressive distortion of the retinal architecture. 3,4 These hamartomas are relatively uncommon with an unknown prevalence. Literature on the benefit of surgery in the management of ERMs in combined hamartoma has historically been controversial and is currently limited. The purpose of this study is to describe the surgical outcomes in CHRRPE patients with dense ERMs that were visually significant and progressive who underwent vitrectomy with mem- From the Department of Ophthalmology, Northwell Health, Great Neck, New York (LSS, SR, DR, PJF); and Long Island Vitreoretinal Consultants, Great Neck, New York (DR, PJF). Originally submitted June 10, 2020. Revision received August 12, 2020. Accepted for publication September 3, 2020. The authors report no relevant financial disclosures. Address correspondence to Philip J. Ferrone, MD, Long Island Vitreoretinal Consultants, 600 Northern Boulevard, Suite 216, Great Neck, NY 11021; email: pjferrone@gmail.com. doi: 10.3928/23258160-20201005-02

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