Novel Manifestation of Retinal Hemangioblastomas Detected by OCT Angiography in von Hippel-Lindau Disease
Novel Manifestation of Retinal Hemangioblastomas Detected by OCT Angiography in von Hippel-Lindau Disease
复制标题
OCT 血管造影检测 von Hippel-Lindau 病视网膜血管母细胞瘤的新表现
DOI：
10.1016/j.ophtha.2023.02.008
复制
发表时间：
2023
期刊：
影响因子：
13.7
通讯作者：
Tsujikawa Akitaka
中科院分区：
文献类型：
--
作者：
Takahashi Ayako;Muraoka Yuki;Koyasu Sho;Arakawa Yoshiki;Nakamura Eijiro;Tsujikawa Akitaka
目的阐明使用OCT血管造影术（OCTA）的von Hippel-Lindau（VHL）病患者中非典型视网膜血管增生的临床特征。设计前瞻性观察性研究2019年1月至2022年3月期间在京都大学医院就诊的57例VHL病患者。视网膜血管母细胞瘤分为结节型和扁平型两种表型。结节性RH定义为典型的RH，即球形、边界清楚的肿瘤，常伴有扩张的供血动脉和引流小静脉。平坦的RH缺乏突出的红色或彩色的质量，有变化和模糊的边界，并没有伴随着供血和引流vessel.Main结果MeasuresThe患病率，分布，和描述的非典型平坦RHS.ResultsAmong 57例连续VHL疾病，37例（64.9%）显示RH在至少1只眼睛。23例患者（62.2%）出现双侧RH。37例（58眼）RHs中，54眼为典型结节状RHs。结节状视网膜脱离主要见于周边视网膜，偶见于视乳头周围，部分病例呈渗出性改变。7眼（12.1%）出现平坦型RH。4只眼仅表现为平坦型RH，3只眼在同一只眼同时表现为两种类型。大多数平坦型RH在眼底检查时表现为视网膜内的视网膜血管瘤或微弱的平坦异常视网膜血管，常位于黄斑区或视乳头周围。在所有平坦RH眼中，OCTA显示病灶内血流丰富。OCT显示，平坦的RHS主要见于视网膜神经纤维层和神经节细胞层之间，偶尔见于内核层内。在平均随访20.4 ± 15.0个月期间，没有平坦的RH伴随渗出性变化，牵引性视网膜脱离，或在size.ConclusionsPatients与VHL疾病的进展可以证明2种不同类型的RH：典型的结节型和非典型的平坦型。OCT血管造影可用于改善非典型平坦RH的检测，这在临床上可能难以检测。财务披露专有或商业披露可在参考文献后找到。
PurposeTo elucidate the clinical characteristics of atypical retinal vascular proliferation in patients with von Hippel-Lindau (VHL) disease using OCT angiography (OCTA).DesignProspective, observational study.ParticipantsFifty-seven consecutive patients with a diagnosis of VHL disease who visited Kyoto University Hospital between January 2019 and March 2022.MethodsRetinal hemangioblastomas (RHs) were assessed using multimodal imaging including OCTA. Retinal hemangioblastomas were classified into 2 phenotypes: nodular and flat. Nodular RHs were defined as typical RHs that were globular, well-circumscribed tumors, often accompanied with dilated feeder arterioles and draining venules. Flat RHs lacked a protruded red or colored mass, had variable and indistinct borders, and were not accompanied with feeder and draining vessels.Main Outcome MeasuresThe prevalence, distribution, and description of atypical flat RHs.ResultsAmong 57 consecutive patients with VHL disease, 37 patients (64.9%) showed RHs in at least 1 eye. Bilateral RHs were seen in 23 patients (62.2%). Among 58 eyes of 37 patients with RHs, typical nodular RHs were detected in 54 eyes. Nodular RHs were seen mainly in the peripheral retina and occasionally in the peripapillary region, and they showed exudative changes in some cases. Flat RHs were detected in 7 eyes (12.1%). Four eyes showed only flat RHs, and 3 eyes showed both types in the same eye. Most flat RHs appeared as retinal hemorrhages or faint flat abnormal retinal vessels in the inner retina on the fundus examination, often within the macula area or peripapillary. In all eyes with flat RHs, OCTA showed abundant blood flow in the lesions. OCT revealed that flat RHs were seen mainly between the retinal nerve fiber layer and the ganglion cell layer, and occasionally within the inner nuclear layer. During a mean follow-up period of 20.4 ± 15.0 months, no flat RHs accompanied exudative change, tractional retinal detachment, or progression in size.ConclusionsPatients with VHL disease can demonstrate 2 distinct types of RHs: the classic nodular type and an atypical flat type. OCT angiography can be useful in improving the detection of atypical flat RHs, which can be difficult to detect clinically.Financial Disclosure(s)Proprietary or commercial disclosure may be found after the references.