Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, Delhi, India
Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, Delhi, India.
BMJ Case Rep. 2024 May 10;17(5):e254972. doi: 10.1136/bcr-2023-254972.
A late adolescent with tuberous sclerosis (TS) presented with reduced vision in one eye to our tertiary care university hospital 4 years ago. Fundus examination revealed multiple retinal astrocytic hamartomas (RAHs) in both eyes. His younger sibling, who also had TS, was found to have RAH on retinal screening. The swept-source optical coherence tomography (SS-OCT) findings were typical of RAH. We further noted that some of the RAH lesions showed segmental whitening of the outer walls of the arterioles, which traversed through them. The segmental whitening may suggest the enveloping of normal retinal vessels by the tumour. En-face and B-scan SS-OCT angiography of patients with TS showed vascularity within the tumour. The vessels within the tumour appeared to be in continuity with the retinal vasculature. Both siblings were reviewed annually. At the end of 4 years, there was no change in visual acuity, tumour size, number, vascularity and behaviour.
四年前,一位患有结节性硬化症(TS)的青少年患者因单眼视力下降到我们的三级教学医院就诊。眼底检查显示双眼均有多发性视网膜星形细胞瘤(RAH)。他的弟弟也患有 TS,在视网膜筛查中发现了 RAH。扫频源光学相干断层扫描(SS-OCT)的结果符合 RAH 的典型表现。我们进一步注意到,一些 RAH 病变的动脉外壁出现节段性变白,这些动脉穿过病变。节段性变白可能提示肿瘤包裹了正常的视网膜血管。TS 患者的面扫和 B 型 SS-OCT 血管造影显示肿瘤内有血管。肿瘤内的血管似乎与视网膜血管连续。这对兄弟姐妹每年复查一次。四年后,视力、肿瘤大小、数量、血管生成和行为均无变化。