Surgically induced necrotizing scleritis (SINS): Is it a standalone condition or a variant of necrotizing scleritis?

PURPOSE: To report the clinical pattern of surgically induced necrotizing scleritis (SINS) in a tertiary eye care center in Southern India. METHODS: Retrospective analysis of all SINS cases visiting the uveitis clinic of a tertiary eye institute between January 2009 and April 2019. RESULTS: In total, 15 patients with a median age of 65 (IQR:52-70) years were included in the study. Male (53%) predominance was noted, and SINS was unilateral (100%) in all cases. Most (87%) of the patients developed SINS after a single surgical procedure, with a median onset period of 251 (IQR:127-1095) days. None of these patients had any evidence of systemic association. Ocular hypertension (n = 3, 20%), and cataract (n = 5, 33%) were the most common complications. When compared with a cohort of patients with idiopathic necrotizing scleritis, the index study did not find any statistically significant difference between SINS and idiopathic scleritis. CONCLUSION: SINS is idiopathic necrotizing scleritis rather than an independent entity of scleritis.

Bilateral Scleritis with Heterogeneous Etiologies: A Diagnostic Dilemma.

PURPOSE: To report a patient with bilateral scleritis who was initially treated for infectious scleritis, and subsequently diagnosed as granulomatosis with polyangiitis (GPA). METHOD: Retrospective chart review. RESULT: A 48-year-old female with a known history of diabetes presented with pain, redness, and blurring of vision in the right eye. She was diagnosed as scleritis with retinal detachment and underwent vitrectomy, with silicone oil tamponade and intravitreal injections of antibiotics, and antifungal agents. She presented with active scleritis with additional multiple pus points. Several scleral biopsies failed to yield any microorganism and finally, the painful blind eye was enucleated. The enucleated specimen grew gram-positive bacteria which were identified as Staphylococcus arlettae by polymerase chain reaction (PCR)-based sequencing of the 16S rRNA gene. Nine months after the onset of symptoms in the right eye, the patient developed necrotizing scleritis in the left eye. Laboratory investigation revealed a positive cytoplasmic- anti-neutrophil cytoplasmic autoantibody, which was previously negative during the right eye involvement. She was diagnosed with GPA by a rheumatologist and treated accordingly. CONCLUSION: Retinal detachment may be associated with scleritis, and ANCA testing may not detect GPA in its early stages. Once infection has been excluded, clinicians should not be afraid to use high-dose immunosuppression instead of surgery to treat retinal detachment associated with scleritis.

Biochemical Analysis of Aqueous Humor in an Elderly Patient with Multiple Myeloma Presenting First as Bilateral Chronic Uveitis.

PURPOSE: To study the biochemical analysis of aqueous humor in a patient with multiple myeloma presenting first as chronic uveitis. METHODS: Observational case report. RESULTS: A 63-year-old healthy woman presented with blurred vision in both eyes for 9 months. Slit-lamp examination showed bilateral conjunctival congestion, corneal oedema, and anterior uveitis. Fundus exam revealed normal optic disc with fine retinal folds in the macula. Serum protein electrophoretogram showed a monoclonal M protein band in the gamma globulin region. The bone marrow biopsy revealed hypercellular marrow with trilineage haematopoiesis and the bone marrow aspirate showed clonal plasma cells >10%, confirming the diagnosis of multiple myeloma. Aqueous fluid showed a differential band in electrophoretic profile of aqueous humor protein that on mass spectrometry analysis was strongly suggestive of immunoglobulin band. CONCLUSION: The biochemical analysis of aqueous humor is another diagnostic test to monitor M protein in patients with multiple myeloma.

Sympathetic Ophthalmia - An Overview.

INTRODUCTION: Sympathetic ophthalmia (SO) is rare, bilateral granulomatous panuveitis that typically occurs following penetrating or perforating ocular trauma or surgery. This review aims to provide an update on the etiopathogenesis, clinical presentations, diagnosis and treatment of SO. METHODS: Reports cited in MEDLINE database, that analyzed SO in at least 5 patients, published prior to December 1st, 2021 were included. RESULTS: Initially, SO was associated with penetrating ocular trauma, however, various studies reported an increased incidence of SO after surgical procedures including vitreoretinal surgeries. Multimodal imaging including fluorescein and indocyanine green angiography, optical coherence tomography (OCT) and OCT angiography have added further insights into the understanding of SO. While pulse dose corticosteroids & immunosuppressive drugs are still the treatment of choice, TNF-α blockers & other biologic drugs represent new promising agents. CONCLUSION: There is a growing pool of evidence in understanding the pathogenesis of SO. Novel treatment options have provided better prognosis for this potentially blinding condition.

Bilateral Ocular Rosai-Dorfman Disease Presenting as Diffuse Anterior Scleritis with Multiple Enlarging Epibulbar Masses.

PURPOSE: To report a unique case of extranodal Rosai-Dorfman disease (RDD) presenting as bilateral scleritis, which progressed to multifocal epibulbar masses. METHODS: Retrospective chart review. RESULTS: A 35-year-old Asian man presented with progressive pain and redness in both eyes for 4 months. Examination revealed bilateral diffuse scleral congestion and areas of scleral thinning. The cornea and anterior chamber of each eye were unremarkable. Despite oral corticosteroid and immunosuppressive treatment, scleritis progressed to multifocal epibulbar masses. Tissue biopsy specimens revealed foamy macrophages with histiocytes staining positive for CD68 and S100, confirming a diagnosis of RDD. Systemic evaluation was unremarkable for lymphadenopathy or extranodal involvement. The patient was started on systemic chemotherapy and at last follow up after 1 year, all epibulbar lesions were completely resolved. CONCLUSIONS: Rosai-Dorfman disease can present as bilateral scleritis initially and develop epibulbar masses without any systemic involvement. Biopsy with immunohistochemical analysis can aid in the diagnosis.

Clinical Profile of Scleritis Presenting for the First Time in the Elderly.

PURPOSE: To analyze clinical characteristics of scleritis in elderly patients and also compare the data with relatively younger patients with scleritis (<60 years). METHOD: Retrospective analysis of medical records of patients with scleritis who visited a tertiary eye care centre between 2008 and 2018. RESULT: Scleritis in ≥60 years accounted for 3% of the total scleritis cases. The mean age of the patients was 67 ± 6 years, and a female (66%) predominance was noted. Of the 44 elderly patients with scleritis, 48% were 66-70 years of age and 20% were above 70 years of age. Diffuse scleritis was the most common subtypes followed by necrotizing scleritis (35%). Overall, 32% of elderly patients with scleritis had underlying disease and the most common systemic association was granulomatous with polyangiitis. When compared with a subset of patients (<60 years of age), the elderly group showed higher rate of recurrences and complications. CONCLUSION: Scleritis in elderly patients is relatively rare but can have a higher recurrence rate with a increased complications.

Uveal effusion syndrome following COVID-19 vaccination.

Purpose: To report a rare case of uveal effusion syndrome following COVID-19 vaccination. Observation: A 71-year-old Asian man presented to his ophthalmologist with blurred vision and noticing distorted lines in his left eye two weeks after the first dose of COVID-19 vaccination. Examination revealed choroidal detachment and he was advised systemic corticosteroids. The symptoms were ignored and the second vaccine dose was taken. After five months, he presented to our clinic with persistent visual complaints. He also had a history of COVID-19 infection three months prior to vaccination. Ocular examination revealed a quiet anterior chamber with annular choroidal detachment consistent with the diagnosis of Type 3 uveal effusion syndrome. B-scan ultrasonography revealed increased choroidal thickness with detachment. Optical coherence tomography showed subretinal fluid with retinal pigment epithelium and choroidal folds. Ultrasound biomicroscopy revealed all around supraciliary effusion in the left eye. The patient was treated with oral prednisolone and mycophenolate mofetil which resulted in complete resolution of uveal effusion and improvement in visual acuity. Conclusions and importance: Uveal effusion syndrome is a rare ocular disease, however it may manifest following COVID-19 vaccination. Our case highlights the importance of a complete ophthalmic examination in patients with ocular symptoms after vaccination.

Wide Field Optical Coherence Tomography and Optical Coherence Tomography Angiography in Uveitis.

BACKGROUND: We review the current literature on the use of wide-field optical coherence tomography (OCT) and wide-field optical coherence tomography angiography (OCTA) in different uveitic phenotypes as well as various sequelae of uveitis and discuss the limitations of this evolving technology. MAIN BODY: Current consensus guidelines on nomenclature in wide-field OCT and OCTA are described. The specific utility of wide-field OCT and OCTA in assessment of the retina and choroid using different en-face and cross-sectional slabs in various inflammatory diseases is reviewed. Furthermore, we discuss widefield OCT and OCTA in assessment of retinal ischemia and its limitations in assessing retinal vascular leakage. CONCLUSION: Wide-field OCT and OCTA deliver more sensitive measures of inflammation. With continued advancement in both hardware technology and software processing, these modalities will allow for more accurate assessment of uveitis, better understanding of disease mechanisms, and precise monitoring of treatment response.

Tofacitinib in Scleritis: A Case Series.

PURPOSE: To report the use of tofacitinib in ten patients with scleritis where the traditional immunomodulation was not successful or could not be used. METHOD: A retrospective chart review. RESULT: Tofacitinib was successful in the treatment of scleritis in patients either recalcitrant to or intolerant to conventional therapy in 9 out of 10 cases reported here. Two patients had developed reactivation of herpetic infection after 1 month of starting tofacitinib. The duration from diagnosis of scleritis to the institution of tofacitinib therapy varied from 1 month to 60 months. Duration of follow-up varies from 2 months to 11 months. CONCLUSION: Tofacitinib can be used as an important future option for managing recurrent and recalcitrant cases of scleritis.

COVID-19 Vaccination and Uveitis: Epidemiology, Clinical Features and Visual Prognosis.

PURPOSE: To study the epidemiological and clinical features of uveitis post-COVID-19 vaccination. PATIENTS AND METHODS: Retrospective chart review of patients presenting with uveitis after COVID-19 vaccination in tertiary uveitis services. RESULTS: In total, 25 patients, 76% females, mean age 43.2 years, were included. Uveitis occurred after Pfizer, Moderna, Astra-Zeneca and Covaxin vaccination. Anterior uveitis was the most frequent type of uveitis (56%). History of uveitis was found in 19 cases (76%), among whom 90.9% of the tested patients developed anti-Sars-CoV-2 spike antibodies after vaccination. In a mean follow-up of 5 months, one-line decrease in visual acuity was found in 12% of patients. During post-vaccine uveitis, 15.8% of cases needed an increase in their systemic therapy. According to Naranjo score, new-onset uveitis had a higher probability of being associated with vaccination (p < .01). CONCLUSION: COVID-19 vaccination can cause uveitis but has no significant impact on the visual prognosis after resolution.

Ocular Gnathostomiasis Presenting as Branch Retinal Artery Occlusion.

PURPOSE: To report a case of ocular Gnathostomiasis presenting as branch retinal artery occlusion. METHOD: Observational case report. RESULT: A 22-year-old Asian woman presented to her ophthalmologist with redness, tearing, and decreased vision in her left eye. Examination revealed anterior uveitis and branch retinal artery occlusion associated with both intra-retinal and vitreous hemorrhage. The patient was treated with topical corticosteroids and cycloplegics. After 3 weeks, she presented in our emergency, with further decrease in vision and worsening pain in the left eye. Slit lamp examination revealed a brown colored live worm on the posterior corneal surface, anterior uveitis, multiple iris holes, and vitreous cells. Indirect ophthalmoscopy showed focal retinal hemorrhages, subretinal tracts, and vitreous hemorrhage. Surgical removal of the worm from anterior chamber was done immediately. CONCLUSION: Branched retinal artery occlusion with intraretinal and vitreous hemorrhage, panuveitis, and multiple iris holes may suggest the presence of an intraocular parasite.

Voriconazole Induced Ocular Surface Dysplasia - Report of Two Cases.

PURPOSE: To report ocular surface dysplasia induced by voriconazole treatment in two patients with recalcitrant fungal keratitis. METHODS: Observational study. RESULTS: Case 1 - A 49 year old female who was a known case of fungal keratitis and treated with prolonged topical voriconazole therapy, underwent penetrating keratoplasty and the histopathological examination of corneal specimen showed multiple keratin pearls with dyskeratotic cells suggestive of squamous cell carcinoma.Case 2 - A 78-year-old man who was diagnosed as fungal keratitis in his left eye and treated with topical voriconazole 1% and itraconazole 1% for 6 months underwent therapeutic penetrating keratoplasty. Histopathology of the host corneal tissue showed squamous cells with irregular thickening with dyskeratotic cells and squamous eddies suggestive of voriconazole induced dysplasia. CONCLUSION: Prolonged topical voriconazole treatment in fungal keratitis can induce ocular surface dysplasia. Early diagnosis and treatment of the dysplastic changes can result in complete remission and prevent recurrence.

Diagnostic Value of Optical Coherence Tomography in the Early Diagnosis of Macular Complications in Chronic Vogt-Koyanagi-Harada Disease.

PURPOSE: To describe the role of optical coherence tomography (OCT) in detecting subclinical choroidal inflammation and early diagnosis of macular complications in chronic Vogt-Koyanagi-Harada (VKH) disease with sunset glow fundus. METHODS: Retrospective observational study. Clinical features, OCT images, treatment, and visual outcome were analyzed. RESULTS: Fourteen patients (9 females and 5 males) were included in the study. Mean age was 39 years (range 7-67 years). Mean duration of disease was 5.25 years (range 1-15 years). Anterior uveitis was seen in 14 eyes (52%). Fundus examination showed sunset glow fundus in all patients with no obvious macular pathology. OCT showed macular edema in 16 eyes (59%), choroidal neovascular membrane in 8 eyes (30%), and macular hole in 3 eyes (11%). CONCLUSION: OCT should be regularly used to detect subclinical inflammation and early macular complications in chronic VKH disease where sunset glow fundus may delay the clinical diagnosis thus causing permanent damage.

Ocular Co-infection with Mycobacterium Tuberculosis and Toxoplasma Gondii in an Immunocompetent Patient - A Case Report.

PURPOSE: To report a case of ocular co-infection with Mycobacterium tuberculosis and Toxoplasma gondii in an immunocompetent woman. METHOD: Retrospective observational case report. RESULT: A 61-year-old woman presented with decreased vision and floaters in the right eye of 1-month duration. Ocular examination revealed panuveitis with a large yellowish-white retinochoroiditis lesion adjacent to a chorioretinal scar. Investigations showed positive Mantoux test, QuantiFERON TB test, and HRCT chest suggestive of active pulmonary tuberculosis. Serology revealed raised IgG anti T. gondii antibody. Vitreous aspirate was positive for M. tuberculosis and T. gondii genome by polymerase chain reaction and showed high IgG and IgM T. gondii antibodies. She was treated with anti toxoplasmic and antitubercular therapy along with oral corticosteroid and therapeutic vitrectomy. CONCLUSION: Ocular tuberculosis and toxoplasmosis can not only mimic each other but also present as co-infection in rare cases.