Cirafici Paola, Musolino Maria, Saccheggiani Michela, Traverso Carlo Enrico, Nicolò Massimo
Di.N.O.G.Mi, Clinica Oculistica, Università di Genova, Ospedale Policlinico San Martino IRCCS, Genova, Italy.
Fondazione per la Macula Onlus, Genova, Italy.
Case Rep Ophthalmol. 2021 Apr 6;12(1):92-97. doi: 10.1159/000509850. eCollection 2021 Jan-Apr.
This is a report of 3 patients diagnosed with idiopathic macular telangiectasia type 1 (MacTel 1) at Medical Retina Center of the University of Eye Clinic of Genova and then prospectically followed for a mean time of 26 months between 2016 and 2019. It is the first report of patients affected by MacTel 1 treated with dexamethasone (DEX) implant as a first choice of treatment. Aim of our study is to better characterize the disease using a multimodal wide-field imaging and to determine efficacy of DEX implant on MacTel 1 in terms of central macular thickness (CMT) and best-corrected visual acuity (BCVA). MacTel 1 is a rare unilateral disease, characterized by telangiectatic retinal capillaries, cystoid macular edema, and lipid deposition occurring temporal to the fovea. Patients underwent a comprehensive ophthalmic examination, BCVA, swept-source optical coherence tomography (SS-OCT), SS-OCT angiography (SS-OCTA), ultra-widefield (UWF) color, and fluorescein angiography (FA) fundus photograph. All the patients presented monolateral reduced BCVA and macular edema with increased CMT evaluated by SS-OCT. With SS-OCTA, we showed that the telangiectasia-associated vascular changes originate in the deep retinal vascular plexus and as a consequence macular edema and exudation develop causing vision loss. Furthermore, UWF imaging helped us to highlight vascular changes typical of Coats Disease at the far retinal periphery. All the patients were treated with DEX intravitreal implant, showing a decrease in CMT and a stabilization of visual acuity. Due to the recurrent nature of macular edema, patients underwent a mean of 4 DEX implants during the follow-up period. In order to address the clinical features of this uncommon disease avoiding diagnostic errors, it might be important to use a multimodal imaging approach. The anatomical and functional beneficial effects of DEX implant were well evident although transient.
这是一篇关于3例在热那亚大学眼科诊所医学视网膜中心被诊断为1型特发性黄斑毛细血管扩张症（MacTel 1）的患者的报告，这些患者于2016年至2019年期间接受了前瞻性随访，平均随访时间为26个月。这是首次关于将地塞米松（DEX）植入作为首选治疗方法治疗MacTel 1患者的报告。我们研究的目的是使用多模态广域成像更好地描述该疾病，并确定DEX植入物对MacTel 1在中心黄斑厚度（CMT）和最佳矫正视力（BCVA）方面的疗效。MacTel 1是一种罕见的单侧疾病，其特征为视网膜毛细血管扩张、黄斑囊样水肿以及在黄斑中心凹颞侧出现脂质沉积。患者接受了全面的眼科检查、BCVA、扫频光学相干断层扫描（SS - OCT）、SS - OCT血管造影（SS - OCTA）、超广角（UWF）彩色和荧光素血管造影（FA）眼底照相。所有患者均表现为单眼BCVA下降以及黄斑水肿，通过SS - OCT评估CMT增加。通过SS - OCTA，我们显示与毛细血管扩张相关的血管变化起源于视网膜深层血管丛，进而导致黄斑水肿和渗出，引起视力丧失。此外，UWF成像帮助我们在视网膜远周边突出了典型Coats病的血管变化。所有患者均接受了DEX玻璃体内植入治疗，CMT降低且视力稳定。由于黄斑水肿具有复发性，患者在随访期间平均接受了4次DEX植入。为了避免诊断错误，采用多模态成像方法来处理这种罕见疾病的临床特征可能很重要。尽管DEX植入的解剖和功能有益效果是短暂的，但很明显。
