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.

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.

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.

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.

Primary intraocular central nervous system lymphoma masquerading as diffuse retinal vasculitis.

A 49-year-old woman had blurred vision and floaters of 4 days duration in the right eye. Ocular examination revealed granulomatous panuveitis, vitritis and diffuse retinal vasculitis. Following a strongly positive tuberculin skin test, she received antitubercular therapy with oral steroids and immunosuppressants. A year later, despite therapy, vitritis and vasculitis persisted. Additionally, yellowish white lesions appeared beneath the retinal pigment epithelium. Fluorescein angiography revealed a leopard skin appearance. Following a negative vitreous biopsy, she was subjected to a chorioretinal biopsy which revealed non-Hodgkin's lymphoma. MRI was normal. The ocular lesions resolved following intravitreal methotrexate injections. MRI of the brain was repeated every 3 months to rule out central nervous system (CNS) involvement. About 2.5 years after initial presentation, she complained of ataxia, hypersomnia and speech difficulty. MRI of the brain now showed lesions in the thalamocapsular region and the corpus callosum splenium suggestive of CNS lymphoma. She underwent a whole brain radiation with symptomatic improvement followed by chemotherapy.

Assessment of gelatinase and tumor necrosis factor-α level in the vitreous and serum of patients with Eales disease: role of inflammation-mediated angiogenesis in the pathogenesis of Eales disease.

BACKGROUND: Eales disease (ED) is an idiopathic, inflammatory, venoocclusive disorder of peripheral retina resulting in retinal angiogenesis and vitreous hemorrhage. The objective of the present study is to investigate the expression and activation of gelatinase associated with the retinal neovascularization in ED and the relation between the levels of gelatinase and the cytokine tumor necrosis factor-α, known to upregulate matrix metalloproteinase (MMP) expression on various cells. METHODS: Vitreous and serum samples from 19 patients with ED who underwent retinal surgery were estimated for levels of MMP-2, MMP-9, tissue inhibitor of metalloproteinase-1, tissue inhibitor of metalloproteinase-2, and tumor necrosis factor-α by enzyme-linked immunosorbent assay method. Matrix metalloproteinase-2 and MMP-9 activities in serum and vitreous samples were evaluated by gelatin zymography method. Vitreous samples from 16 patients with macular hole undergoing vitrectomy were used as controls. RESULTS: Among the 2 gelatinase examined in vitreous and serum samples, only level and activity of MMP-9 were significantly higher in serum (P = 0.0001) and vitreous (P = 0.0002) samples of patients with ED than those of control subjects. Simultaneously, a positive correlation was found between intraocular tumor necrosis factor-α and MMP-9 concentration (Spearman correlation coefficient, r = 0.7040, P = 0.0023) in patients with ED. CONCLUSION: Increase in MMP-9 activity and its concentration in serum and vitreous of patients with ED compared with that of control subjects and correlation between intraocular levels of MMP-9 and tumor necrosis factor-α in patients with ED seem to provide a plausible explanation for inflammation-mediated angiogenesis during the development of this condition.

Pars plana vitrectomy in the management of paediatric uveitis: the Massachusetts Eye Research and Surgery Institution experience.

PURPOSE: To assess the effectiveness and safety of pars plana vitrectomy (PPV) in the management of chronic paediatric uveitis. METHODS: We reviewed records of patients 16 years old or younger who underwent PPV due to persistent uveitis. Data including inflammatory status, ocular findings, visual acuity, dosage and duration of various medical therapies, surgical techniques and complications were collected. RESULTS: Twenty-eight eyes of 20 patients were included in the study. The diagnoses of uveitis included pars planitis in 15 eyes (54%), idiopathic panuveitis in 8 eyes (29%), and juvenile idiopathic arthritis-associated iridocyclitis in five eyes (18%). Six eyes presented with associated retinal vasculitis. The mean age at the time of PPV was 11.2 years. The mean follow-up after surgery was 13.5 months. All 28 eyes had active uveitis with or without medical therapy at the time of PPV. At last follow-up, uveitis control was achieved with or without adjuvant medical therapy in 27 eyes (96%). These included five of the six eyes with persistent retinal vasculitis. Two eyes that had 20-G PPV developed intra-operative retinal tears. Four eyes with pre-operative clear lenses developed cataract within the first 6 months after PPV. CONCLUSIONS: PPV is effective and safe in the management of chronic paediatric uveitis and its complications. It was able to reduce the amount of systemic medications required to control inflammation in this study. Patients with uveitis complicated by retinal vasculitis, however, are more likely to require long-term medical therapy to achieve inflammatory control.

Intravitreal bevacizumab to facilitate vitrectomy in idiopathic ischaemic retinal vasculitis.

To describe the use of intravitreal bevacizumab in idiopathic retinal vasculitis prior to vitrectomy and delamination. A young healthy male presented with idiopathic retinal vasculitis. His condition was refractory to panretinal photocoagulation and systemic steroids. The progression of his condition necessitated vitrectomy and delamination. Intravitreal bevacizumab was used to facilitate the surgical procedure, with effective results. Intravitreal bevacizumab can be an adjunct to vitrectomy in idiopathic retinal vasculitis. However, caution must be taken due to its ability to promote fibrosis and to produce further tractional and/or rhegmatogenous retinal detachment. Further controlled studies are required to understand its benefits in managing complicated idiopathic retinal vasculitis.

[Eales' disease--10 years experience with a rare disease]. / Morbus Eales--10 Jahre Erfahrung mit einem seltenen Krankheitsbild.

BACKGROUND: Eales' disease is an uncommon vasoproliferative retinal disease affecting otherwise healthy young men. We report on our treatment results in a large patients group with long-term follow-up. PATIENTS AND METHODS: The treatment results in 50 eyes (35 patients) with Eales' disease, that were consecutively treated from May 1995 to August 2005, were analysed retrospectively. Recorded data included age, sex, race, association of systemic disease, medications, laboratory evaluation and the surgical treatment. Mean follow-up was 5.8 years (minimum: 3.2, maximum: 8.6 years). RESULTS: Systemic and laboratory evaluations detected a factor V Leiden mutation (4x), vestibuloauditory problems (1x) and migraine (1x). 10 eyes with peripheral non-perfusion, teleangiectasia and mild neovascularisations were treated by scatter laser photocoagulation alone. 18 eyes with advanced neovascularisations/vitreous haemorrhages received cryocoagulation too. 14 eyes with persistent or recurrent bleedings despite coagulation therapy and/or development of tractional retinal detachment were treated by vitrectomy. 5 of them received a silicone oil endotamponade. All eyes could be stabilised without further bleedings. Visual acuity increased in 25 eyes. Only 5 eyes showed a visual loss. The visual results (rate of improvements and the stages of visual acuity as well) were the best in those eyes that received vitrectomy. CONCLUSIONS: These results suggest that early retinal laser and cryocoagulation and - if necessary - vitrectomy in due time may led to a stabilised retina without further bleedings and visual improvement too. Coagulopathy could play a role in the pathogenesis of Eales' disease.

Incontinentia pigmenti: a rare cause of retinal vasculitis in children.

BACKGROUND: Incontinentia pigmenti is a genetic disease with cutaneous, dental, neurologic and ophthalmologic manifestations. Ocular changes are very varied. Retinal vasculitis is uncommon, but can be observed in this disease. AIM: To report a case of a patient with retinal vasculitis associated to incontinentia pigmenti. REPORT: A seven year old girl was followed for cutaneous lesions with the diagnosis of incontinentia pigmenti confirmed by a skin biopsy. She had delayed eruption of her teeth some of which were conical. She had no neurologic nor developmental abnormalities. Ophthalmological examination and fluorescein angiography was performed and showed retinal neovascularization in the left eye, secondary to retinal ischemia and retinal sequelae vasculitis without inflammation or complications in the right eye. Laser photocoagulation was indicated to the left and a strict surveillance to the right eye. After one month of the beginning of treatment, we noticed an involution of the retinal neovascularisation. CONCLUSION: Early diagnosis and management of retinal changes in incontinentia pigmenti is essential because they may be serious leading to blindness.

Therapeutic effects of laser photocoagulation and/or vitrectomy in Eales' disease.

PURPOSE: To determine visual outcomes and regression of retinal neovascularization following laser photocoagulation and/or vitrectomy in eyes with Eales' disease. METHODS: In a retrospective noncomparative study, the authors reviewed the existing data of 67 eyes of 54 patients with a diagnosis of Eales' disease who had undergone laser photocoagulation and/or vitrectomy based on their clinical presentations. Main outcome measures were visual acuity changes and regression of retinal neovascularization of the eyes following treatment. RESULTS: Both laser therapy and vitrectomy improved visual acuity and induced regression of retinal neovascularization. Forty-three eyes had undergone laser therapy; their rate of visual acuity 320/30 improved from 53% before treatment to 60% after treatment. Twenty-four eyes had undergone vitrectomy; rate of visual acuity 320/30 improved from 13% before surgery to 38% after surgery. In eyes that had undergone laser therapy, additional laser therapy controlled recurrent neovascularization in 47% of the eyes, but ultimately, 12% of them required vitrectomy. In the primary vitrectomized group, additional required treatment was repeat vitrectomy in 21%, and/or laser therapy in 29% of the eyes. CONCLUSIONS: Although laser photocoagulation should be the first line of treatment in Eales' disease, it cannot always induce regression of retinal neovascularization. In such cases vitrectomy may further enhance therapeutic success.