Supplementary Material for: Surgery for Combined Hamartoma of the Retina and Retinal Pigment Epithelium

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.

目前针对视网膜与视网膜色素上皮联合错构瘤（combined hamartoma of the retina and retinal pigment epithelium, CHRRPE）的手术治疗指征及实施时机，学界尚未达成统一共识。本研究旨在探讨该疾病手术干预的获益，以及可改善预后的手术技术。一名24岁男性因罹患CHRRPE，左眼出现6个月的进行性视力下降。就诊时，患者视力为1.3 LogMAR，眼底镜检查可见广泛的牵拉性视网膜前膜、视网膜下渗出，以及视网膜下方周边部的血管增殖性肿瘤。术者为患者实施全玻璃体切割术，术中特别注意对玻璃体基底部进行玻璃体切削，并清除玻璃体基底部后方视网膜表面由玻璃体劈裂诱导产生的玻璃体皮质残留（vitreoschisis-induced vitreous cortex remnants, VCR）。随后剥离牵拉性膜与内界膜，并切除血管增殖性肿瘤。术后11周，取出眼内硅油填充剂。术中及术后均未出现并发症。术后随访48个月期间，患者视力提升至0.8 LogMAR且保持稳定。玻璃体视网膜手术可预防CHRRPE相关并发症；即便初始视力低下，患者的视功能仍可得到改善。建议对复杂CHRRPE患者实施及时且完整的玻璃体切割联合广泛膜剥离，并检出并清除VCR，以规避相关并发症。