We identified clinical characteristics and risk factors of choroidal neovascularization (CNV) in eyes with prior episode of central serous chorioretinopathy (CSC). This retrospective case-control study included those initially diagnosed with CSC and developed CNV secondarily (Group 1, n = 16), those diagnosed with CNV in eyes of previous putative CSC (Group 2, n = 14), and those initially diagnosed with CSC, and did not develop CNV secondarily, as a control group for Group 1 (Group 3, n = 250). Clinical characteristics including treatment outcomes were assessed. Demographics and multimodal imaging at the time of CSC diagnosis of secondary CNV were compared between the groups to identify risk factors. Duration from diagnosis of CSC to development of CNV in Group 1 was 40.2 ± 42.0 months. Classic CNV was noted in 23 (76.7%) eyes. After treatment with intravitreal antiangiogenics with average of 4.9 times, visual acuity improved in Group 1 and Group 2 ( p = 0.002). Multivariate analysis revealed that systemic hypertension, pigmentary changes, and double layer sign were associated with development of CNV secondary to CSC ( p < 0.05). Hypertension, pigmentary changes, and double layer sign were independent risk factors for CNV secondary to CSC. The CNV’s responded well to treatment, resulting in improved vision.
Stargardt-like macular dystrophy 4 (STGD4) is a rare macular dystrophy characterized by bull's eye atrophy of the macula and the underlying retinal pigment epithelium. Patients with STGD4 show decreased central vision, which often progresses to severe vision loss. The PROM1 gene encodes prominin-1, which is a 5-transmembrane glycoprotein also known as CD133 and is involved in photoreceptor disk morphogenesis. PROM1 mutations have been identified as genetic causes for STGD4 and other retinal degenerations such as retinitis pigmentosa. We report a case of STGD4 with a PROM1 p.R373C mutation in a Korean patient. Ophthalmic examinations of a 38-yr old man complaining of decreased visual acuity revealed bilateral atrophic macular lesions consistent with STGD4. Targeted exome sequencing of known inherited retinal degeneration genes revealed a heterozygous missense mutation c.1117C>T (p.R373C) of PROM1, which was confirmed by Sanger sequencing. To the best of our knowledge, this is the first case of a PROM1 mutation causing STGD4 in Koreans.
Purpose: To report a rare case of traction retinal detachment and retinal ischemia in inactive Takayasu's arteritis at ophthalmologic clinic. Case summary: A 23-year-old woman presented with a floater, photophobia, and visual loss in her right eye one week prior to visit. She had no other systemic disease, such as diabetes mellitus or hypertension, or previous ophthalmic abnormalities except for a tumor in the adrenal gland. We found bilateral retinal ischemia and traction retinal detachment in the right eye on fundus examination without iris neovascularization. Pars plana vitrectomy, traction removal, endolaser treatment, and intravitreal bevacizumab injection were performed. Steroid eye drops and steroid systemic administration relieved the inflammation. On carotid doppler sonography, we found severe stenosis and thickness of the inner layer in both carotid arteries. We diagnosed the patient with an inactive phase of Takayasu's arteritis, which was conclusively correlated with the clinical features. Vascular anastomosis surgery along with follow-up was proposed by both the cardiology and vascular surgery departments. Conclusions: When a young patient presents with traction retinal detachment and retinal ischemia, Takayasu's arteritis should be considered for differential diagnosis and a systemic work-up should be performed as soon as possible.
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