Long-term outcome of a Chinese cohort idiopathic retinitis, vasculitis, aneurysms, and neuroretinits (IRVAN) patients.

PURPOSE: To evaluate the clinical characteristics and treatment outcomes of idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) in a cohort of Chinese patients. MATERIALS: The clinical history, imaging evaluation, treatment and outcomes of 42 eyes in 21 patients diagnosed with IRVAN in a 15-year period were reviewed. RESULTS: Most patients were females (90%) ranged from 15 to 58 years old. The initial decimal best corrected visual acuity (BCVA) of the patients ranged from light perception (LP) to 1.5 (0.55 ± 0.38). Eighteen eyes were in stage 2; 21 eyes in stage 3; and 1 in stage 5 at the initial visits according to the present staging system. Two eyes had vitreoretinal fibrovascular proliferation (FVP) and tractional retinal detachment (RD) at the initial visit. Intra-retinal microvascular abnormality (IRMA) was found in 7 eyes. Thirty-four eyes received retinal photocoagulation, 27 of which were pan-retinal photocoagulation (PRP). Total of 8 PPV were performed for VH, vitreoretinal FVP and RD, and macular epimembrane. Aneurysms on the head of optic nerve and artery bifurcations disappeared in 8 eyes and decreased in number in 2 eyes 1 year after photocoagulation. However, the BCVA of the patients did not have significant difference from that at the initial visits (P = 0.534). Seven eyes suffered severe visual impairment (BCVA ≤ 0.1) due to vitreoretinal FVP and tractional RD (3), exudative maculopathy (2), paracentral acute middle maculopathy (PAMM)(1), and neovascular glaucoma (NVG) (1). CONCLUSIONS: We found that IRVAN have a predilection to female gender. Vitreoretinal FVP and tractional RD and exudative maculopathy are major causes of severe visual impairment in IRVAN patients. We propose to revise the present staging system to include vitreoretinal FVP and RD in the staging of IRVAN patients.

Surgical Removal of Intraocular Parasite in a Patient With Diffuse Unilateral Subacute Neuroretinitis.

Diffuse unilateral subacute neuroretinitis (DUSN) is a rare ocular infectious disease caused by migrating larvae of a nematode. DUSN is often a diagnostic challenge as it is difficult to identify the pathogenic nematode on funduscopic examination because it moves frequently and often resides within the subretinal space. Herein, the authors present a patient with unilateral, chronic visual loss who was noted to have a live, motile worm within the vitreous cavity. Initial attempts to treat with laser photocoagulation were unsuccessful. In the accompanying video, the authors report the first case of a live intraocular parasite successfully removed with pars plana vitrectomy.

Role of PASCAL and optical coherence tomography angiograpgy in the treatment of diffuse unilateral subacute neuroretinitis caused by large live motile worm.

A 46-year-old male presented with best corrected visual acuity (BCVA) of 20/125 in his right eye. Fundus showed disc edema, multiple yellow outer retinal crops, macular edema and a live motile worm in the subretinal space. Diagnosis of diffuse unilateral subacute neuroretinitis (DUSN) was made and pattern scanning laser photocoagulation (PSLP) was performed along with administration of oral albendazole, diethylcarbamazine and corticosteroids. Complete disappearance of the worm was observed at 2 weeks. At last follow up, final BCVA improved to 20/30. Herein, we report role of PSLP in the management of DUSN.

The fluorescein angiographic photodiagnosis of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: Outcome of combined therapy.

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroanurysms, neuroretinitis and ischemia in peripheral vessels, which are difficult to diagnose and cause visual loss if delayed. It consists of 5 stages and causes irreversible vision loss with severe complications after stage 2. In this report, photodiagnosis and combined treatment are defined in a Turkish patient with IRVAN syndrome during 6 months of follow-up. Fundus fluorescein angiography (FFA) showed that bilateral aneurysms in retinal vessels as well as aneurysms and ischemic regions were observed in the inferotemporal retina of the left eye. Argon laser photocoagulation was performed to ischemic regions at the left eye. 2 months later best corrected visual acuities were 20/20 and counting fingers from 1 m in the right and left eyes, respectively. Fundus and OCT images showed that an increase in exudations was observed at the left eye and intravitreal injection of dexametasone implant was considered. Three months after initial presentation, best corrected visual acuities were 20/20 and counting fingers from 2 m in right and left eyes, consequetively. In the last fundus and OCT images, the exudations decreased and disappeared on left eye. In this patient, we could not detect an increase in vision due to damage of photoreceptor cells because of subretinal exudation. In the shed-light of this case, the combination therapy seems to improved the anatomical and functional outcomes in IRVAN syndrome however close follow-up and frequent examinations should be prioritized.

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): case series of three patients with multimodal imaging.

PURPOSE: The purpose of this study was to report the clinical presentation, disease progression, treatment and complications of IRVAN. METHOD: Case series PATIENTS: Six eyes from three patients were included. RESULTS: All eyes were treated with pan-retinal photocoagulation (PRP). One eye received Ozurdex (dexamethasome implant) for persistent macular exudates and oedema. One eye received Avastin injections for retinal neovascularization. Oral steroids were given to all patients at some point during the disease process. One patient had additional immunosuppression with mycophenolate mofetil. Despite aggressive PRP, the visual outcomes varied widely. One patient maintained 6/6 vision bilaterally at 84 months follow-up. The second patient had progressive visual loss secondary to macular exudates and oedema, from 6/9 right eye, 6/6 left eye to 6/18 right eye, 6/60 left eye within 12 months despite Ozurdex injection. The third patient's vision at presentation was 6/5 right eye, and 6/4 left eye. Despite further interventions including Avastin and mycophenolate mofetil, he continued to have progressive neovascularization and recurrent vitreous haemorrhage. At 72 months, his vision had deteriorated to 6/60 right eye, 6/18 left eye. CONCLUSION: The progression of IRVAN can vary greatly, in spite of aggressive treatment with PRP, oral and intravitreal steroids, immunosuppressant medication and anti-VEGF agents. The variation in disease progression occurs both within the same individual as well as between individuals. An individualised approach to therapy is advocated.

A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

PURPOSE: We aimed to analyze the clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Furthermore, we aimed to correlate the number and location of retinal aneurysms with the size of retinal non-perfusion area and neovascularization. METHODS: Six patients with IRVAN syndrome (1 male and 5 females, age 5-38 years) were enrolled in this study. Fundus fluorescein angiography (FFA) was used to determine the total number of retinal aneurysms, number of aneurysms within the first branch of the retinal artery, minimum distance between the non-perfusion margin and the optic disc, and the number of retinal aneurysms in each quadrant, as well as the type of neovascularization. RESULTS: The size of the non-perfusion area was positively correlated with the total number of retinal aneurysms, the number of aneurysms within the first branch of the retinal artery, and the number of retinal aneurysms in each quadrant (P < 0.05). During the 5-year follow-up, one patient exhibited a dynamic change in the number and location of retinal aneurysms. CONCLUSIONS: In IRVAN syndrome, the number and location of retinal aneurysms correlate with the size of retinal non-perfusion area and type of neovascularization.

Long-term outcomes in a series of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome.

OBJECTIVE: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare entity with a potentially poor visual prognosis. Our objective is to review the clinical presentation and long-term outcomes of patients with IRVAN syndrome. DESIGN: This is a retrospective case series. METHODS: We reviewed the charts of all the patients diagnosed with IRVAN syndrome at our tertiary care centre from 2002 to 2015. RESULTS: We included the long-term clinical outcomes of 7 eyes (5 patients) diagnosed with IRVAN syndrome. After a mean follow-up of 84.9 months, best-corrected visual acuity was 20/40 or better in the majority of eyes (70%). Four (57.1%) patients had systemic conditions, namely, multiple sclerosis, ischemic stroke, and positive antiphospholipid titres. All eyes were treated with laser photocoagulation. Four (40%) eyes received adjunctive intravitreal bevacizumab injections. CONCLUSION: IRVAN is an important diagnosis for clinicians to recognize. When treated in a timely manner, long-term visual outcomes can be favourable.

Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Presenting With Branch Retinal Artery Occlusion.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) is a rare syndrome affecting the retinal and optic disc vasculature. Diffuse retinal ischemia, macular edema, and neovascularization may lead to bilateral vision loss. The authors report a case of a 36-year-old woman presenting with branch retinal artery occlusion (BRAO) in her right eye who was subsequently diagnosed with IRVAN syndrome. She was treated with panretinal photocoagulation for peripheral retinal ischemia and pars plana vitrectomy for vitreous hemorrhage. She later developed a BRAO in her left eye. This case demonstrates that BRAO may be a presenting feature of IRVAN syndrome. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:948-951.].

Internal limiting membrane: The innermost retinal barrier.

Recently, peeling of internal limiting membrane (ILM) has become one of the most common and effective surgical procedures for macular disorders. The authors discuss the adverse effects of such procedures and explore the possible functions of the membrane. We also suggest a barrier function of this membrane in addition to its possible other physiological roles. Thus, apart from the well-known inner and outer retinal barriers, ILM might be the third and innermost retinal barrier. The possible evidences supporting this hypothesis are presented.

Diffuse Unilateral Subacute Neuroretinitis From Raccoon Exposure Resistant to Laser Photocoagulation.

Diffuse unilateral subacute neuroretinitis (DUSN) is an ocular disease caused by a subretinal nematode worm. The authors present a unique case of a 9-year-old girl with DUSN due to presumed Baylisascaris procyonis, given the size of the worm and previous raccoon exposure. The worm was located in the inner retina and treated with laser photocoagulation and albendazole. At the 1-week follow-up, the worm was still mobile despite being inactive immediately after the initial laser treatment and required a more prolonged laser session. This case serves to highlight the importance of close follow-up in patients with DUSN to ensure the worm is inactivated. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:686-690.].

Bilateral stellate neuroretinitis revealing a pheochromocytoma.

Neuroretinitis (NR) is an inflammatory disorder characterized by optic disc edema and subsequent formation of a macular star. We present a case of a 33 year old woman patient admitted for a progressive bilateral visual loss since two weeks. Fundus examination showed bilateral stellate neuroretinitis. Physical examination revealed a malignant hypertension of 210/150 mmHg. Magnetic resonance imaging identified a left suprarenal mass, whereas urinary catecholamine level was abnormally high which supported a diagnosis of pheochromocytoma. The patient underwent a laparoscopic left suprarenal adrenalectomy after successful control of blood pressure. histopathologic examination confirmed the diagnosis of pheochromocytoma. Visual acuity was restored and the retinal alterations disappeared 7 months after surgery.

Tratamiento precoz de vasculitis idiopática, aneurismas y retinitis (IRVAN). A propósito de un caso / Early treatment of idiopathic vasculitis, aneurysms and neuroretinitis (IRVAN). A case report

CASO CLÍNICO: Se presenta el caso de una mujer de 55 años con vasculitis retiniana, aneurismas arteriales múltiples en el polo posterior, exudación macular e hipoperfusión periférica bilateral (IRVAN) tratada precozmente con panfotocoagulación periférica. Tras 3 años de seguimiento la agudeza visual permanece estable y no hay progresión de la isquemia. DISCUSIÓN: El síndrome de IRVAN con neovascularización es difícil de controlar y el riesgo de progresión a glaucoma neovascular, a pesar del tratamiento, es alto. La panfotocoagulación en estadios iniciales es eficaz para controlar la isquemia y debe realizarse lo antes posible

CASE REPORT: A 55 year old woman presented with retinal vasculitis, multiple aneurysms, macular exudation and widespread retinal nonperfusion and was diagnosed with IRVAN. She was treated with panretinal laser photocoagulation. After 3 years of follow up visual acuity remains stable and there are no complications due to ischaemic sequelae. DISCUSSION: IRVAN syndrome with neovascularisation can progress rapidly despite laser treatment. Panretinal laser photocoagulation has to be considered in the early stages as it is effective in stopping the progression of ischaemia

[Early treatment of idiopathic vasculitis, aneurysms and neuroretinitis (IRVAN). A case report]. / Tratamiento precoz de vasculitis idiopática, aneurismas y retinitis (IRVAN). A propósito de un caso.

CASE REPORT: A 55 year old woman presented with retinal vasculitis, multiple aneurysms, macular exudation and widespread retinal nonperfusion and was diagnosed with IRVAN. She was treated with panretinal laser photocoagulation. After 3 years of follow up visual acuity remains stable and there are no complications due to ischaemic sequelae. DISCUSSION: IRVAN syndrome with neovascularisation can progress rapidly despite laser treatment. Panretinal laser photocoagulation has to be considered in the early stages as it is effective in stopping the progression of ischaemia.