Chou Wei-Yi, Lee Fenq-Lih
Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan.
Taiwan J Ophthalmol. 2020 May 27;11(2):197-199. doi: 10.4103/tjo.tjo_15_20. eCollection 2021 Apr-Jun.
Around 50% of patients with tuberous sclerosis have the manifestation of retinal astrocytomas. Symptomatic retinal astrocytomas are very rare, with no consensus on optimal treatment. A 7-year-old patient with tuberous sclerosis presented with progressive blurred vision in the right eye for more than half a year. On examination, best-corrected visual acuity was 20/30 in the right eye and 20/20 in the left eye. Dilated fundus examination of the right eye showed a well-circumscribed, elevated, opaque lesion, with surrounding lipid exudate, and retinal detachment involving the fovea. Spectral-domain optical coherence tomography of the right eye revealed an intraretinal lesion with adjacent subretinal fluid. A diagnosis of retinal astrocytoma with exudative retinal detachment was made. The patient was treated with verteporfin photodynamic therapy (PDT) on the lesion in the right eye. After 1 month, complete resolution of subretinal fluid was observed. At 21-month follow-up, the right eye vision was stable at 20/20, without ocular or systemic adverse events. In conclusion, PDT had a good safety profile in a cooperative pediatric patient, and was able to induce regression of astrocytoma as well as resolution of exudation with excellent visual outcome.
约50%的结节性硬化症患者有视网膜星形细胞瘤表现。有症状的视网膜星形细胞瘤非常罕见,对于最佳治疗尚无共识。一名7岁结节性硬化症患者右眼渐进性视力模糊半年余。检查发现,右眼最佳矫正视力为20/30,左眼为20/20。右眼散瞳眼底检查显示一个边界清晰、隆起、不透明的病变,伴有周边脂质渗出,以及累及黄斑的视网膜脱离。右眼的光谱域光学相干断层扫描显示视网膜内病变伴有相邻的视网膜下液。诊断为视网膜星形细胞瘤伴渗出性视网膜脱离。该患者右眼病变接受了维替泊芬光动力疗法(PDT)治疗。1个月后,观察到视网膜下液完全消退。在21个月的随访中,右眼视力稳定在20/20,无眼部或全身不良事件。总之,PDT在配合治疗的儿科患者中安全性良好,能够使星形细胞瘤消退以及渗出物吸收,并获得极佳的视力结果。