Department of Ophthalmology and Visual Sciences, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Department of Ophthalmology, Maisonneuve-Rosemont Hospital Research Centre, University of Montreal, Montreal, Canada.
Medicine (Baltimore). 2021 Oct 22;100(42):e27578. doi: 10.1097/MD.0000000000027578.
Bilateral diffuse uveal melanocytic proliferation (BDUMP) is an extremely rare retinal exudative disease with physical disorders and no established treatment standard. We describe treatment courses in 3 cases of BDUMP.
Three male patients complained active vision loss. One male patient in his 70s (patient 1) was treated with prednisolone, mesalazine, and ciclosporin for hypoplastic anemia and ulcerous colitis. One male patient in his 60s (patient 2) was on prednisolone therapy for adult Still disease. Another male patient in his 70s (patient 3) was on prednisolone therapy for polymyalgia rheumatica, giant cell arteritis, and pancreatic body tumor.
Retinal specialists diagnosed these patients with BDUMP based on characteristic fundus findings of multiple red patches and retinal exudate.
Two patients (patients 1 and 2) with poor response to anti-vascular endothelial growth factor (VEGF) monotherapy and/or triamcinolone acetonide sub-Tenon injection were treated with combined anti-VEGF therapy and photodynamic therapy. One patient (patient 3) was treated with 3 rounds of monthly anti-VEGF monotherapy.
Retinal exudates were resolved in all patients. No recurrence of retinal exudates was observed for at least 10 months, 2 years, or 4 months after the therapy in patients 1, 2, and 3, respectively. However, best-corrected visual acuity of the right eye was low (20/200) compared with that of the left eye (20/22) in patient 2 despite exudate resolution, due to permanent outer retinal damage secondary to long-term retinal exudate.
Combined anti-VEGF therapy and photodynamic therapy may be a feasible therapeutic option for treatment-resistant exudate in patients with BDUMP. Early diagnosis of BDUMP and prompt administration of combination therapy are crucial.
双侧弥漫性葡萄膜黑素细胞增生（BDUMP）是一种极为罕见的视网膜渗出性疾病，存在身体机能紊乱但尚无既定的治疗标准。我们描述了 3 例 BDUMP 的治疗经过。
3 名男性患者主诉视力进行性下降。1 名 70 多岁男性（患者 1）因再生障碍性贫血和溃疡性结肠炎接受泼尼松龙、美沙拉嗪和环孢素治疗。1 名 60 多岁男性（患者 2）因成人Still 病接受泼尼松龙治疗。另 1 名 70 多岁男性（患者 3）因巨细胞动脉炎、多发性肌痛、胰腺体肿瘤接受泼尼松龙治疗。
眼底专家根据多发性红色斑块和视网膜渗出的特征性眼底发现诊断这些患者为 BDUMP。
2 名对血管内皮生长因子（VEGF）单药治疗和/或曲安奈德前Tenon 注射反应不佳的患者（患者 1 和 2）接受了抗 VEGF 联合治疗和光动力疗法。1 名患者（患者 3）接受了 3 轮每月抗 VEGF 单药治疗。
所有患者的视网膜渗出均消退。治疗后至少 10、2 或 4 个月，患者 1、2 和 3 的视网膜渗出分别未见复发。然而，尽管渗出消退，患者 2 的右眼最佳矫正视力（20/200）仍低于左眼（20/22），这是由于长期视网膜渗出导致的永久性外层视网膜损伤所致。
对于 BDUMP 患者的治疗抵抗性渗出，联合抗 VEGF 治疗和光动力疗法可能是一种可行的治疗选择。早期诊断 BDUMP 并及时进行联合治疗至关重要。
