色素失禁症患者的眼底表现及治疗

Fundus manifestations and treatment of pigment incontinence

摘要:

目的：探讨色素失禁症(IP)患者的眼底荧光素血管造影特征及其辅助治疗的意义。

方法：回顾性研究，收集2019-03/08确诊的IP患儿10例18眼的临床资料纳入研究，均为女性患儿，就诊年龄2周～42月龄，详细记录患儿的病史及家族史，均行眼底彩色照相及眼底荧光素血管造影检查。

结果：纳入患儿眼底检查结果提示：1期病变1例1眼，FFA提示视网膜散在异常血管，晚期无渗漏，定期随访。2期病变4例6眼，FFA提示视网膜可见异常血管吻合，无明显新生血管生成，均行视网膜光凝。3期病变7例9眼，FFA提示视网膜新生血管荧光渗漏、视网膜前出血性荧光遮蔽，均行玻璃体腔注药(雷珠单抗注射液2mg：0.2mL，注射量每眼0.25mg/0.025mL)联合视网膜光凝； 其中2例为单眼发病，对侧眼正常。4期病变2例2眼，4a期病变1眼，FFA显示视盘、黄斑向颞侧牵拉连至周边部视网膜、局部浅脱离，行玻璃体切割手术； 4b期病变1眼，眼前节照相提示晶状体后白色机化膜，眼部B超提示视网膜脱离，行玻璃体切割手术。

结论：初步证实了眼底荧光素血管造影可辅助IP相关性视网膜病变的分期及治疗，对其治疗随访有指导意义，但对于各期病变的治疗目前尚无统一标准，有待更多临床资料。

Abstract:

AIM: To investigate the characteristics of fundus fluorescein angiography in patients with pigment incontinence and the significance of its adjuvant therapy.

METHODS: A retrospective study, we collected the clinical data of 10 patients(18 eyes)diagnosed retinopathy of pigment incontinence from March 2019 to August 2019. The patients were all female children, and they were 2 weeks-42 months old. The children's medical and family histories were recorded in detail, and fundus color photography and fundus fluorescein angiography were performed.

RESULTS: The results of fundus examination of 18 eyes of 10 children showed that 1 eye with stage 1, FFA showed that the retina was scattered with abnormal blood vessels, and there was no leakage in the late stage, and we will return to this regularly. 6 eyes with stage 2, FFA showed abnormal vascular anastomosis in the retina, and no obvious neovascularization, and all underwent laser photocoagulation. 9 eyes with stage 3, FFA showed fluorescence leakage of retinal neovascularization and fluorescence obscuration of preretinal hemorrhage, all of which underwent anti-VEGF vitreous injection combined with laser photocoagulation; 2 cases had monocular disease and the opposite eye was normal. 2 eyes with stage 4 and 1 eye with stage 4a, FFA showed that the optic disc and macula were pulled to the peripheral retina and partial retinal detachment, and vitrectomy was performed; 1 eye with stage 4b, the anterior segment was photographed, and a white mechanical film was behind the lens, B ultrasonography of the eye showed retinal detachment, and vitrectomy was performed.

CONCLUSION: This article preliminarily confirmed that fundus fluorescein angiography can assist in the staging and treatment of IP retinopathy, and had guiding significance for its follow-up treatment. However, there was no unified standard for the treatment of various stages of disease, and more clinical data were needed.