Atopic Dermatitis Associated Retinal Detachment and Retinal Vasculitis: A Case Report.

Atopic dermatitis is usually associated with various ocular complications. We report a 21-year-old Chinese male who presented to our ophthalmology clinic with bilateral retinal detachment and cataracts. The patient had a clear medical history of atopic dermatitis, which had been diagnosed eight years earlier and had been treated with loratadine and pimecrolimus. Cataract surgery was performed for both eyes, combined with scleral buckling for the right eye and pars plana vitrectomy for the left eye. During postoperative follow-up, fundus fluorescein angiography showed retinal vasculitis in both eyes and macular edema in the left eye, which coincided with an exacerbation of atopic dermatitis. Macular edema improved after four months of regular dupilumab treatment in the dermatology department. The ocular condition remained stable three years postoperatively.

Toxic posterior segment syndrome with retinal vasculitis likely caused by intraocular cotton fiber after vitreoretinal surgery - a case report.

BACKGROUND: Intraocular inflammation is common after anterior or posterior segment surgery. They typically manifest either as non-infectious inflammation of the anterior or posterior segment, known as toxic anterior or posterior segment syndrome (TPSS), or as sterile or infective endophthalmitis. In this report, we describe a rare case of TPSS following vitreoretinal surgery, presenting as hemorrhagic retinal vasculitis. CASE PRESENTATION: A 58-year-old male diagnosed with a left eye acute rhegmatogenous retinal detachment underwent an uneventful primary pars plana vitrectomy with silicone oil endotamponade on the same day of presentation. At presentation, there were no signs of intraocular inflammation, and his visual acuity in the affected eye was 20/200. RESULTS: The retina was well-attached with silicone oil in place on the first post-operative day. Along the inferior retinal periphery, a hemorrhagic occlusive vasculitis was observed. Clinical examination revealed retained intraocular cotton fiber along the inferotemporal quadrant over the retinal surface. In addition to the standard post-operative medications, a course of systemic steroids (40 mg per day of Prednisolone tablets) was started. At the end of the first post-operative week, clinical signs of hemorrhagic retinal vasculitis were beginning to resolve, and by the end of the fourth post-operative week, they had completely resolved. CONCLUSION: This report describes an unusual diagnosis of TPSS after vitreoretinal surgery, most likely due to the presence of an intraocular cotton fiber. This excessive inflammation of the posterior segment usually responds to a course of topical and systemic steroids.

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.

Vitrectomy Due to Vitreous Hemorrhage and Tractional Retinal Detachment Secondary to Eales' Disease

Objectives: To investigate visual and anatomical outcomes of vitreoretinal surgeries in patients with Eales' disease. Materials and Methods: In this retrospective study, 22 eyes of 21 patients with vitreous hemorrhage (VH) or tractional retinal detachment (TRD) secondary to Eales' disease who underwent vitreoretinal surgery between January 1997 and December 2015 and had at least 1 year of follow-up were included. Results: The mean best corrected visual acuity (BCVA) was significantly higher at final visit (0.6±0.9 logMAR) than the preoperative values (1.8±1.1 logMAR) (p<0.001). After surgery, BCVA was stable in 4 eyes (18.2%), increased in 16 eyes (72.7%), and decreased in 2 eyes (9.1%). Although the mean BCVA was better in the VH group (0.3±0.34 logMAR) than the TRD group (0.9±1.1 logMAR), the difference was not statistically significant (p=0.1). Multivariable linear regression analyses revealed that final BCVA was negatively associated with preoperative or postoperative proliferative vitreoretinopathy grade C (PVR-C), preoperative retinal detachment involving the macula, postoperative neovascular glaucoma, and long preoperative duration of disease, and positively associated with preoperative BCVA. Final BCVA was not associated with preoperative retinal and disc neovascularization, rubeosis iridis, total posterior hyaloid detachment, preoperative retinal laser photocoagulation, indication of surgery, diameter of sclerotomy (20 or 23 gauge), preoperative lens status, preoperative or postoperative epimacular membrane, peroperative iatrogenic retinal breaks, postoperative hypotony, cystoid macular edema, and new or recurrent retinal detachment. The primary anatomic success rate was 81.8% and the final anatomic success rate was 90.9%. Conclusion: In Eales' disease, good visual results can be obtained with vitreoretinal surgery if the detachment area does not involve the macula and PVR-C does not develop pre- or postoperatively.

Clinical spectrum, genetic associations and management outcomes of Coats-like exudative retinal vasculopathy in autosomal recessive retinitis pigmentosa.

Background: Coats-like retinal vasculopathy in retinitis pigmentosa (RP) is rare. This study describes its clinical spectrum, management outcomes and genetic associations in patients with autosomal recessive RP (arRP).Materials and methods: Retrospective review of ophthalmic, multimodal imaging, genetic findings and treatment outcomes of arRP patients who developed Coats-like features. Identification of patients included searching a retinal dystrophy registry of 798 patients.Results: Ten eyes of six patients with arRP (4 males, 2 females, mean age 33 years) demonstrated Coats-like features, namely inferotemporal peripheral retinal telangiectasis combined with unilateral inferotemporal vasoproliferative tumor (VPT) in 4 eyes. Exudative retinal detachment (ERD) developed in five eyes of which four had VPT. Ablation of the vasculopathy using retinal laser photocoagulation and/or cryotherapy in eight eyes, allowed ERD and/or lipid exudation to decrease in seven eyes despite incomplete vasculopathy regression. Additional intravitreal triamcinolone acetonide injection in one eye failed to regress the ERD and associated VPT. Observation in one eye caused increased exudation. Six mutations, including three novel mutations, were found in CRB1, CNGB1, RPGR, and TULP1.Conclusions: Coats-like features in arRP range from retinal telangiectasis to VPTs with extensive ERD and occur predominantly in the inferotemporal retinal periphery. In addition to their classic association with CRB1 mutations, other genes are implicated. To the best of our knowledge, this is the first report describing CNGB1 mutations in Coats-like RP. Awareness of the vasculopathy spectrum is important, and timely ablation of the vasculopathy with long-term monitoring is recommended to prevent additional visual loss in RP patients.

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.

Frosted branch angiitis with penetrating ocular trauma and retained intraocular foreign body.

A 54-year-old male sustained ocular trauma with a projectile. Examination of the right eye revealed an intraocular foreign body (IOFB) adjacent to the optic nerve head, vitritis, vitreous hemorrhage, and translucent perivascular sheathing of the retinal vessels in all quadrants suggesting frosted branch angiitis (FBA). The patient underwent vitrectomy with removal of the IOFB and silicone oil tamponade under steroid cover. With continued use of systemic and topical steroids after surgery, complete resolution of FBA and improvement in vision were noted in a week. Prompt resolution of FBA after IOFB removal points toward a strong association between the presence of IOFB and FBA.

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.].

A rare presentation of exudative macroaneurysms in unilateral Eales' disease.

BACKGROUND: A case of exudative macroaneurysms in unilateral Eales' disease is reported. 'Eales disease' is an idiopathic bilateral occlusive periphlebitis with neovascularisation and mostly presents with vitreous hemorrhage. CASE: A 30 year old male presented with diminution of vision in left eye (6/36) for 1 month. Slit Lamp examination of both eyes revealed normal anterior segment. Fundus examination of left eye with + 90 D lens revealed reddish lesions at the macula with surrounding circinate exudation. On peripheral examination hemorrhages were seen along with vasculitis in the superotemporal quadrant.The right eye fundus was normal.All laboratory investigations were found within normal limits. CONCLUSION: Eales' disease is mostly a bilateral condition but this case is rare as there is uniocular involvement with exudative macroaneurysms.Laser therapy was instituted which was effective in management of this condition with restoration of normal visual acuity.There was no relapse on follow up for 2 years.

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.

Surgical outcomes of minimally invasive vitrectomy surgery in Eales' disease.

INTRODUCTION: The results of surgical outcomes of 20 gauge pars plana vitrectomy in Eales' disease are available in the scientific literature. However, all these studies have been done using the 20 gauge vitrectomy systems and most studies have been conducted in a retrospective manner. OBJECTIVE: To evaluate the outcomes and safety of 23 gauge vitrectomy in complications of Eales' disease. STUDY DESIGN: Consecutive interventional case series. PARTICIPANTS: Seventy-six eyes of 72 nonconsecutive patients undergoing 23-gauge vitrectomy for complications of Eales' disease were enrolled. The participants were followed up for a minimum of one year. INTERVENTION: The participants underwent a complete demographic, medical and ophthalmic evaluation. A 23-gauge vitrectomy was performed. Endotamponade was used when necessary. Perioperative and postoperative events were recorded. Primary outcome measures were visual acuity and complications arising due to surgery. RESULTS: Indication for surgery was non-clearing vitreous hemorrhage in 89.4% (68/76) and secondary retinal detachment in 10.6% (8). Visual acuity improved from Log Mar 1.80 ± 0.19 units preoperatively to Log Mar 0.47±0.59. Best-corrected visual acuity equivalent to Snellen 6/9 was achieved in 77. 6% of eyes. . Surgical failure was seen in 6.5% cases. Four cases were lost due to progression to neovascular glaucoma and 1 case was lost to severe residual retinal detachment. Iatrogenic portside retinal breaks occurred in 3.9% (3), post-vitrectomy retinal detachment 2.6% ( 2), hypotony 1.3% (1) and cataract in 38.1% (28) cases. CONCLUSION: 23-gauge sutureless vitrectomy in patients with Eales' disease is a safe and effective technique with acceptable level of risk and complications.

Mesenchymal stem cell therapy unable to rescue the vision from advanced Behcet's disease retinal vasculitis: report of three patients.

OBJECTIVE: Retinal vasculitis (RV) is the most aggressive lesion of ocular manifestations of Behcet's disease, seen in 32.1% of patients. Although visual acuity (VA) improves with early and aggressive treatment, in the long run it is seen in only 48% of patients. Mesenchymal stem cell (MSC) transplantation (MSCT) can theoretically reverse the RV process. PATIENTS AND METHODS: Three patients with advanced RV and very low VA were selected. Eyes selected for MSCT were legally blind (no useful vision) with severe retinal damage due to vasculitis, resistant to combinations of monthly pulse-cyclophosphamide (1000 mg) + azathioprine 2-3 mg/kg/day + prednisolone 0.5 mg/kg/day. After patient signed written consent, 30 mL of bone marrow were taken and cultured for MSC growth. After having enough MSCs in culture (4-5 weeks) and taking into consideration all safety measures, cells were injected in one eye of each patient (approximately 1.8 million MSCs). VA was measured. Disease Activity Index (DAI) was calculated for anterior uveitis (AU), posterior uveitis (PU) and RV. RESULTS: Visual acuity was light perception (LP) for two patients and finger count (FC) for the third. Follow-up at 1, 6 and 12 months were respectively LP/LP/FC at 0.5 m, no-light perception (NLP)/LP/LP, NLP/LP/NLP. DISCUSSION: Results showed a total failure of the procedure, essentially due to the late and advanced state of vasculitis. However, the autoimmune/inflammatory reaction was greatly controlled by the procedure. CONCLUSION: Earlier cases have to be selected for further trials.