Surgical Outcomes of Epiretinal Membrane Removal Due to Combined Hamartoma of the Retina and RPE

Abstract

BACKGROUND AND OBJECTIVE: To evaluate the surgical outcomes of epiretinal membrane (ERM) associated with combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) after vitrectomy and membrane peel. PATIENTS AND METHODS: A retrospective review of 15 patients who underwent pars plana vitrectomy with membrane peeling. No plasmin enzyme 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 latest 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 improved VA and retinal structure. [ Ophthalmic Surg Lasers Imaging Retina. 2020;51:546–554.]

Combined Hamartoma of the Retina and Retinal Pigment Epithelium at Pediatric Age

“…Surgical data are usually restricted to smaller series and case reports. The largest series by Sun et al 8 included 15 patients treated with PPV. They reported visual improvement in 14 eyes (93%).…”

“…Several case series with small sample sizes reported favorable outcomes after pars plana vitrectomy (PPV) of CHRRPE. 8,11–14…”

“…Several case series with small sample sizes reported favorable outcomes after pars plana vitrectomy (PPV) of CHRRPE. 8,[11][12][13][14] In this article, we conducted a multicenter international collaborative retrospective study of outcomes in pediatric patients with CHRRPE based on management choice, tumor location and characteristics, and age at diagnosis.…”

“…[5][6][7] Although the generally accepted indications for surgical treatment of CHRRPE include the presence of ERM causing macular distortion and development of tractional retinal detachment, there is no consensus on which cases should be treated surgically or conservatively. Some clinicians adopted early vitrectomy and membrane peeling for removal of ERM in patients with CHRRPE, 8 whereas others followed patients without surgical intervention unless marked macular distortion, tractional retinal detachment, or vitreous hemorrhage developed. 9 There are few studies with large sample sizes that reported longitudinal data from patients with CHRRPE, and most presented conservative followup highlighting gradual decreases in visual acuity.…”

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.…”