Objective To evaluate the clinical features and follow-up effect of treatment for retinal capillary hemangioma (RCH) of myopic disc.Methods Retrospective case series studies. A total of 14 patients (15 eyes) diagnosed with juxtapapillary RCH (JRCH) at Peking Union Medical College Hospital and followed up for more than 12 months from January 2007 to December 2019 were included. Among these patients, there were 7 males (7 eyes) and 7 females (8 eyes) with the average age of 29.8 ±12.7 years. There were 6 isolated JRCH cases, all of which were monocular patients, including 1 case with a single peripheral RCH; 8 cases with von Hippel-Lindau disease, including 5 cases of binocular RCH. Out of 15 eyes, 6 eyes were merged with peripheral RCH. Three eyes were simply observed, and 12 eyes were treated with photodynamic therapy (PDT) alone or PDT combined with other treatments. The average follow-up time was 55.2 months. Its clinical characteristics and long-term treatment effects were analyzed.Results At the first visit, among the 15 eyes, 3 eyes had visual acuity＜0.1, 5 eyes were 0.1-0.4, and 7 eyes were＞0.5. The endogenous and fixed exogenous JRCH were 12 (80%, 12/15) and 3 (20%, 3/15) eyes, respectively. The size of the tumor was 1/4 to 4 optic disc diameters (DD); the combined macular edema, epiretinal membrane, and macular hole were 11, 5, and 1 eyes, respectively. There were 3 eyes with tumor diameter less than 1 DD. The visual acuity at the first diagnosis was 0.6-1.0, and no treatment was given. The follow-up time was 12 months in 2 eyes and 120 months in 1 eye. At the last follow-up, his vision remained stable and the tumor did not enlarge. Six eyes with tumor diameter ranging from 1.0 to 2.5 DD, visual acuity was 0.06 to 0.6 at first diagnosis, accompanied by retinal exudation. 5 eyes were treated with PDT alone, and 1 eye was treated with PDT combined with anti-vascular endothelial growth factor drugs. The follow-up time was 12 to 84 months. At the last follow-up, the visual acuity improved or stabilized in 5 eyes and decreased in 1 eye. There were 6 eyes with peripheral RCH, including 4 eyes with retinal detachment, 2 eyes with epiretinal membrane, and 1 eye with vitreous hemorrhage. The visual acuity at the first visit was 0.02-0.6. All patients were treated with PDT combined with transpupillary thermotherapy, extrascleral drainage, and vitrectomy. The follow-up time was 12 to 132 months. At the last follow-up, all eyesight decreased.Conclusions Visual decrease cause by JRCH often occurs at young adults. Bilateral JRCH are more common in patients with von Hippel-Lindau's disease. The tumor can cause retinal exudation, macular edema, epiretinal membrane and macular hole, resulting in gradual vision loss. PDT and/or combined anti-vascular endothelial growth factor drugs for medium sized JRCH with retinal exudation can maintain long-term vision stability. Patients with large JRCH or severe complications such as retinal detachment tend to have poor prognosis.

Citation： Chen Huan, Du Hong, Li Donghui, Yuan Mingzhen, Chen Youxin. Clinical features and long-term follow-ups of juxtapapillary retinal capillary hemangioma. Chinese Journal of Ocular Fundus Diseases, 2021, 37(5): 365-370. doi: 10.3760/cma.j.cn511434-20201009-00481 Copy