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Abstract：
Objective: To present a case of secondary type 2 choroidal neovascularization (CNV) and exudative maculopathy in a patient with Grönblad-Strandberg syndrome. Methods: A 37-year-old male was admitted with bilateral progressive painless visual acuity loss and metamorphopsias. A thorough ophthalmologic and clinical examination was performed. Results: Best-corrected visual acuity (BCVA) on presentation was 20/ 200 OD (Oculus Dexter) and 20/ 60 OS (Oculus Sinister). Fundus examination revealed angioid streaks and subretinal hemorrhages on OU (Oculus Uterque), macular fibrosis on OD and \"peau d\'orange\" pigmentary mottling on OS. Leakage areas on fundus fluorescein angiography (FFA) revealed active CNV on OU, which was confirmed by Optical Coherence Tomography (OCT). The presence of typical \"plucked chicken\" skin lesions in the latero-cervical area and their biopsy confirmed the diagnosis of Pseudoxanthoma elasticum (PXE). Consequently, the diagnosis of Grönblad-Strandberg syndrome was established. Conclusions: Every new diagnosis of angioid streaks entails not only a thorough ophthalmologic evaluation for secondary sight-threatening complications, but also a multidisciplinary evaluation due to the possibility of severe underlying systemic disease. Abbreviations: BM = Bruch\'s membrane, RPE = Retinal Pigmented Epithelium, PXE = Pseudoxanthoma Elasticum, ABCC6 = ATP binding cassette subtype C number 6, CNV = Choroidal Neovascularization, BCVA = Best-Corrected Visual Acuity, OD = Oculus Dexter, OS = Oculus Sinister, OU = Oculus Uterque, FFA = Fundus Fluorescein Angiography, OCT = Optical Coherence Tomography, IPO = Intraocular Pressure, ECG = Electrocardiogram, anti-VEGF = anti-vascular endothelial growth factor.
摘要：
目的：介绍Grönblad-Strandberg综合征患者继发2型脉络膜新生血管（CNV）和渗出性黄斑病变的病例。方法：一名37岁的男性因双侧进行性无痛性视力丧失和变形而入院。进行了彻底的眼科和临床检查。结果：最佳矫正视力（BCVA）为20/200OD（OculusDexter）和20/60OS（OculusSinister）。眼底检查显示OU（OculusUterque）上有血管样条纹和视网膜下出血，OD上的黄斑纤维化和OS上的“橙色”色素斑点。荧光素眼底血管造影（FFA）上的渗漏区域显示OU上有活性的CNV，光学相干断层扫描（OCT）证实了这一点。宫颈后区典型的“拔毛鸡”皮肤病变及其活检证实了弹性假性黄瘤（PXE）的诊断。因此,Grönblad-Strandberg综合征的诊断得以确立.结论：每一次血管样条纹的新诊断不仅需要对继发性威胁视力的并发症进行彻底的眼科评估，而且由于严重的潜在全身性疾病的可能性，还进行了多学科评估。缩写：BM=布鲁赫膜，RPE=视网膜色素上皮，PXE=弹性假性黄瘤,ABCC6=ATP结合盒C亚型6，CNV=脉络膜新生血管，BCVA=最佳矫正视力，OD=OculusDexter,OS=OculusSinister,OU=OculusUterque,FFA=眼底荧光素血管造影,OCT=光学相干断层扫描，IPO=眼内压，ECG=心电图,抗VEGF=抗血管内皮生长因子。
