López Fontanet José, Ayala Rodríguez Sofía C, Oliver Armando L
Ophthalmology, University of Puerto Rico School of Medicine, Medical Sciences Campus, San Juan, USA.
Cureus. 2023 Apr 26;15(4):e38154. doi: 10.7759/cureus.38154. eCollection 2023 Apr.
We report a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome in a patient whose cystoid macular edema (CME) was successfully treated with aflibercept and pan-retinal photocoagulation (PRP). A 56-year-old male was sent to our uveitis service for further evaluation after a fluorescein angiogram revealed symmetric retinal ischemia for 360 degrees in both eyes. A fundus examination revealed an aneurysm, neuroretinitis, and occlusive vasculitis, all consistent with a diagnosis of IRVAN syndrome. An optical coherence tomography examination revealed CME of the left eye. A chest X-ray revealed minimally prominent interstitial markings. The patient had a positive QuantiFERON-TB Gold test and was treated for tuberculosis with a one-year course of isoniazid and pyrimethamine. A further workup for other infectious and autoimmune etiologies was negative. The initial treatment consisted of bilateral PRP of the areas of peripheral ischemia, treatment for which was provided in a fragmented fashion over the course of seven months. Soon after the diagnosis, he received treatment with two intravitreal injections of aflibercept (2 mg/0.5 mL), one month apart, to the left eye. Subsequently, four months following the presentation, he developed CME in the right eye, which was treated with a single intravitreal injection of aflibercept (2 mg/0.5 mL). At his last follow-up visit, four years after the initial presentation, the patient remained asymptomatic with 20/20 visual acuity in both eyes and no evidence of CME recurrence. Our case suggests that aflibercept may serve as an adjuvant to the standard treatment with PRP, especially in cases that present with associated macular edema.
我们报告了一例特发性视网膜血管炎、动脉瘤和神经视网膜病变(IRVAN)综合征患者,其黄斑囊样水肿(CME)通过阿柏西普和全视网膜光凝(PRP)成功治疗。一名56岁男性在荧光素血管造影显示双眼360度对称性视网膜缺血后,被送至我们的葡萄膜炎专科进行进一步评估。眼底检查发现一个动脉瘤、神经视网膜病变和闭塞性血管炎,均符合IRVAN综合征的诊断。光学相干断层扫描检查显示左眼有CME。胸部X线显示间质纹理轻度增粗。该患者结核菌素皮肤试验阳性,接受了为期一年的异烟肼和乙胺嘧啶抗结核治疗。对其他感染性和自身免疫性病因的进一步检查均为阴性。初始治疗包括对周边缺血区域进行双眼PRP,在七个月的时间里分阶段进行。诊断后不久,他接受了两次左眼玻璃体内注射阿柏西普(2 mg/0.5 mL)治疗,间隔一个月。随后,就诊四个月后,他右眼出现CME,接受了一次玻璃体内注射阿柏西普(2 mg/0.5 mL)治疗。在初次就诊四年后的最后一次随访中,患者无症状,双眼视力均为20/20,且无CME复发迹象。我们的病例表明,阿柏西普可作为PRP标准治疗的辅助药物,尤其是在伴有黄斑水肿的病例中。