Fluocinolone Implant for Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome: A Case Report

Abstract

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare and progressive disorder that predominantly affects both the eyes of young female individuals and can threaten visual function. Peripheral ischemia and macular exudation are common findings in patients. The treatment options include panretinal photocoagulation (PRP), systemic immunosuppression, and intravitreal antiangiogenic and corticosteroid therapy. Fluocinolone acetonide intravitreal implant is approved for the treatment of nonanterior noninfectious uveitis and diabetic macular edema (ME), with an estimated therapeutic duration of 3 years. We describe a case of IRVAN syndrome in a child with ME who had been previously treated with PRP, antiangiogenic therapy, and several dexamethasone intravitreal implants and received a fluocinolone acetonide intravitreal implant in her right eye. The patient showed stabilization of the visual acuity and a marked reduction of the macular thickness 1 month after the treatment. At 12-month follow-up, the patient required perifoveal focal photocoagulation due to a rebound of the ME. After 2 years of follow-up, visual acuity remains stable and macular retinal thickening under control. Local long-standing steroid therapy has proved to be quite efficient in controlling the progression of the disease in our patient.

IRVAN syndrome: A case report and a literature review

“…The epidemiology of the syndrome assured that the majority of patients were young females [ 1 , 5 ]. Our literature review included publications about cases of 10 females and 6 males, and the average age at the time of diagnosis was 28.8 years (range, 9–60 years) [ [2] , [3] , [4] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] ]. Whereas, Samuel et al study determined the average age of diagnosis at 31.5 years (range, 9–60 years) in their study which involved 22 patients.…”

“…According to a study, there was no association between IRVAN and any systemic disorder [ 6 ]. The majority of reviewed cases had a clear medical history and negative serum tests [ [2] , [3] , [4] , [10] , [11] , [12] , [13] , [14] , [15] , 17 ], in comparison to positive human leukocyte antigen B51 (HLA-B51) and antinuclear antibodies (ANA) at 1/50 titer in one case [ 9 ]. AM Abu El-Asrar et al reported a case of association between Allergic fungal sinusitis (AFS) and IRVAN syndrome.…”

“…Three studies started their medical protocol with steroids [ 3 , 13 , 17 ]. Whereas there are five studies that performed a combined treatment of PRP and oral steroids as the first followed protocol [ 3 , 4 , 9 , 12 , 16 ], three of them involved other procedures in their protocol such as mycophenolate mophetil, intravitreal Bevacizumab, and Ranibizumab injections [ 2 , 12 , 16 ]. A study mentioned performing pars plana vitrectomy to treat their patient resulted in partly absorbed exudate and no observed neovascularization [ 13 ].…”

“…The latest established article mentioned a first-time performed treatment which included Fluocinolone acetonide intravitreal implant. The authors assured that it was effective and they strongly recommended local long-standing steroids to control the progression of the retinal disorder [ 9 ]. PRP was very effective and stopped the progression of retinal ischemia and exudation sequelae in our case.…”

“…On following-up, some cases which first treated with PRP needed additional PRP courses [ 3 , 13 ] or intravitreal Dexamethasone implant [ 15 ]. Whereas, one case was worsened with the combined treatment (steroids, PRP) and got better with intravitreal Fluocinolone acetonide injections [ 9 ]. Moreover, serial intravitreal aflibercept injections and pars plana vitrectomy were a sufficient curement each separately [ 13 , 14 ].…”