Frosted branched angiitis due to viral meningo-encephalitis and ocular toxoplasmosis; a rare case report from Nepal.

Frosted branched angiitis (FBA) is characteristic florid translucent retinal perivascular sheathing of both arterioles and venules in association with variable uveitis and vasculitis affecting the entire retina. The vascular sheathing is supposed to be an immune-mediated reaction, possibly due to immune complex deposition in vessel walls secondary to various underlying etiologies. The authors aim to report a case of FBA secondary to herpes simplex virus and Toxoplasma gondii infection causing the diagnostic dilemma. This is the first case report on FBA from Nepal. Case report: An 18-year-old young boy hospitalized with the diagnosis of acute viral meningo-encephalitis presented with the complaint of diminution of vision and floaters in both eyes for a week. Herpetic infection was confirmed with the cerebro-spinal fluid analysis and was under antiviral drugs. His presenting visual acuity was 20/80 in both eyes and ocular features were suggestive of FBA. The vitreous sample analysis revealed raised toxoplasma titre so intravitreal clindamycin was administered twice. The ocular features resolved in the subsequent follow ups with intravenous antiviral treatment and intravitreal antitoxoplasma treatment. Conclusions: FBA is a very rare clinical syndrome secondary to many immunological or pathological causes. So, possible etiologies must be ruled out for timely management and good visual prognosis.

Case report peripheral ulcerative keratitis with nodular scleritis, presents as the primary manifestation of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is an ANCA-associated autoimmune disorder that typically affects small and/or medium-sized blood vessels. Being a life-threatening disease, with timely suspicion, targeted laboratory investigations, and collaborative management from the ophthalmologist and rheumatologist led to long-term remission of the disease. Case: A 38-year-old female complaining of recurrent deep boring pain with redness in her left eye for many years, which was diagnosed as nodular scleritis with peripheral ulcerative keratitis. The patient also has recurrent bouts of epistaxis and in suspicion of GPA laboratory investigations were carried out and later diagnosed. She was started on cyclophosphamide and is currently under maintenance with rituximab. Case discussion: Ocular involvement has been shown to occur in 20-50% of the population in several studies. It causes conjunctivitis, episcleritis, scleritis, necrotizing keratitis, corneoscleral perforation, posterior uveitis, and optic neuritis. The positive C-ANCA and high PR3 autoantibody have high sensitivity and association with GPA. Cyclophosphamide has been shown to be an effective treatment in multiple studies whereas rituximab has been emerging as a new treatment modality for maintenance, which in turn helps in the remission and relapse of GPA cases. Conclusion: Scleritis and peripheral ulcerative keratitis can be the manifestation of a GPA. Careful evaluation, diagnosis, and management with a multidisciplinary medical team, early start of cyclophosphamide, and rituximab have a huge role in decreasing the disease activity and is life-saving.

The role of a multicentre data repository in ocular inflammation: The Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS).

In the current literature, clinical registry cohorts related to ocular inflammation are few and far between, and there are none involving multi-continental international data. Many existing registries comprise administrative databases, data related to specific uveitic diseases, or are designed to address a particular clinical problem. The existing data, although useful and serving their intended purposes, are segmented and may not be sufficiently robust to design prognostication tools or draw epidemiological conclusions in the field of uveitis and ocular inflammation. To solve this, we have developed the Ocular Autoimmune Systemic Inflammatory Infectious Study (OASIS) Clinical Registry. OASIS collects prospective and retrospective data on patients with all types of ocular inflammatory conditions from centers all around the world. It is a primarily web-based platform with alternative offline modes of access. A comprehensive set of clinical data ranging from demographics, past medical history, clinical presentation, working diagnosis to visual outcomes are collected over a range of time points. Additionally, clinical images such as optical coherence tomography, fundus fluorescein angiography and indocyanine green angiography studies may be uploaded. Through the capturing of diverse, well-structured, and clinically meaningful data in a simplified and consistent fashion, OASIS will deliver a comprehensive and well organized data set ripe for data analysis. The applications of the registry are numerous, and include performing epidemiological analysis, monitoring drug side effects, and studying treatment safety efficacy. Furthermore, the data compiled in OASIS will be used to develop new classification and diagnostic systems, as well as treatment and prognostication guidelines for uveitis.

Ocular toxicity among patients taking anti-tubercular treatment.

INTRODUCTION: Tuberculosis remains a major public health problem in Nepal and anti-tubercular drugs used for the treatment of pulmonary and extrapulmonary tuberculosis can be associated with ocular toxicity. This prospective study aimed to evaluate the incidence of ocular toxicity among patients receiving anti-tubercular therapy and to assess the change in visual functions and ocular imaging before and after use of anti-tubercular therapy. MATERIALS AND METHODS: A total of 89 eyes of 45 TB patients taking anti-tubercular therapy were enrolled. Detailed history and examination including best-corrected visual acuity (BCVA), colour vision (Farnsworth D-15t), contrast sensitivity (Pelli-Robson chart), Goldman visual field analysis and spectral domain optical coherence tomography for retinal nerve fibre layer (RNFL) analysis were assessed at baseline and at 6 months after starting anti-tubercular therapy. Visual evoked potential (VEP) was performed in suspected cases. RESULTS: The mean age of the patients was 29.13±14.00 years and 62.2% were males. The mean weight of the subjects was 54.37±10.36 kg, mean daily dosage of ethambutol was 17.91±1.74 mg/day/kg and mean administration duration was 2.71±1.54 months. The incidence of ocular toxicity was 2.24%. Bilateral retrobulbar optic neuropathy occurred in a 27-year female of 55 kg receiving ethambutol (20 mg/kg/day) for 6 months for Pott's spine. Her best-corrected visual acuity in both eyes was reduced to 6/36 from 6/6 and developed non-specific color vision defect, decreased contrast sensitivity, bilateral cecocentral visual field defect and mean decrease in retinal nerve fibre layer thickness compared to the baseline data. In rest cases, a statistically significant decrease in mean retinal nerve fibre layer thickness in both eyes suggested the evidence of subclinical toxicity. CONCLUSION: Though less common, ethambutol toxicity can occur in patients under anti-tubercular therapy in the form of retrobulbar optic neuritis. Decreased contrast sensitivity and thinning in the mean retinal nerve fibre layer thickness can be the indicator of subclinical toxicity.

Challenges of Pediatric Cataract Surgery in a Case of Seasonal Hyperacute Panuveitis (SHAPU).

A four-year-old female child diagnosed as a case of severe Seasonal Hyperacute Panuveitis (SHAPU) underwent lens-sparing core vitrectomy in her left eye with intravitreal antibiotic and steroid. Patient responded well to treatment and intraocular inflammation subsided. However, three months later, she developed vision impairing dense cataract which also made posterior segment assessment difficult. Lens aspiration with primary posterior capsulotomy and anterior vitrectomy with intraocular lens (IOL) implantation was performed. However, four weeks later, the patient developed occlusio pupillae with iris bombe. She did not respond to medical management so synechiolysis with surgical iridectomy was performed after which a normal depth anterior chamber was attained. Synechia and iris bombe were also relieved, and vision was regained.

Branch Retinal Vein Occlusion in a Case of Sarcoidosis.

BACKGROUND: Sarcoidosis is a chronic inflammatory disorder characterized by non-caseation granuloma. It is an inflammatory disorder of unknown etiology. Sarcoidosis has variable ocular presentations from anterior uveitis to rare venous occlusions. CASE: We present a rare case of sarcoidosis with bilateral hilar lymphadenopathy and a compatible uveitis. Positive findings of mutton fat keratic precipitates, Koeppes nodule, posterior synechiae, trabecular meshwork nodules, Candle wax dripping sign, Branch retinal vein occlusion and choroidal granuloma in one eye supported the diagnosis of sarcoidosis. Elevated serum acetylcholine esterase and bilateral hilar lymphadenopathy also confirmed the diagnosis of sarcoidosis. OBSERVATION: The patient responded well to oral steroids and laser photocoagulation of the vein occlusion area. The patient developed complications of steroid like Herpes Zooster of abdomen and avascular necrosis of femur. CONCLUSION: Branch Retinal Vein Occlusion is a rare vascular complication in ocular sarcoidosis. Systemic steroids and laser for vein occlusion is mainstay of treatment. Physician must be aware of serious complications of steroid therapy, as seen in this case. Keyword: Sarcoidosis, Branch retinal vein occlusion, Steroids.

Pediatric Eales Disease: An Indian Tertiary Eye Center Experience.

PURPOSE: To report the clinical profiles, etiologies, treatment modalities, and outcomes for Eales disease in patients younger than age 16 years in India. METHODS: Retrospective review of medical records of patients with Eales disease who had a minimum 5-year follow-up period. RESULTS: A total of 25 eyes of 13 patients were included. Of these 13 patients, 12 (94%) had bilateral Eales disease and 11 (84.6%) were men. Mean patient age was 14.1 years (range: 11 to 16 years). Diminution of vision (36%) was the most common presenting complaint, followed by both diminutions of vision and floaters (32%). Sclerosed vessels were seen in all eyes, and 21 (84%) eyes had active periphlebitis at presentation. Neovascularization elsewhere was seen in 20 (80%) eyes and neovascularization of the optic disc was seen in 1 (4%) eye. Veno-venous shunts were found in 12 (48%) eyes, and 18 (72%) eyes had vitreous hemorrhage. All eyes received photocoagulation; 84.6% of patients received oral steroids, with 7.7% of patients treated with azathioprine and 38.4% treated with anti-tubercular therapy. Vitrectomy was performed in 36% of eyes for non-clearing vitreous hemorrhage and tractional retinal detachment. Vision improved in 7 (28%) eyes, was stable in 12 (48%) eyes, and worsened in 6 (24%) eyes. Recurrence of the disease more than five times during the 5-year follow-up period occurred in 20% of patients. CONCLUSIONS: Recurrent vasculitis and vitreous hemorrhage in children should raise the suspicion of pediatric Eales disease. [J Pediatr Ophthalmol Strabismus. 2018;55(4):270-274.].

Langerhans cell histiocytosis with hemorrhagic uveitis and exudative retinal detachment.

Bilateral intraocular involvement in Langerhans cell histiocytosis (LCH) is uncommon. A 15-year-old boy presented with painless decreased vision in right and painful left red eye of 2 weeks duration. Visual acuity was 20/500 and 20/200, respectively. A fixed dilated pupil with exudative retinal detachment was present in the right eye and hemorrhagic iris nodules with hyphema and hypopyon were seen in the left eye. Intraocular pressure was 12 and 31 mmHg, respectively, in each eye. Ocular symptoms were preceded by fever with multiple skin rashes, subcutaneous nodules, and lymph node enlargement. The histopathological examination of skin and lymph node showed histiocytes positive for histiocytic S 100. He was treated with topical steroids and antiglaucoma eye drops along with intravenous vinblastine 6 mg and oral prednisolone (1 mg/kg). Hyphema and hypopyon were resolved, vision improved to 20/320 and 20/80, and intraocular pressure was under control. However, as multisystemic LCH has a poor prognosis, we were unable to save him. Hence, fatal conditions like LCH should also be considered in the differential diagnosis of any hemorrhagic uveitis.

Retinal Vasculitis in a Patient with Ankylosing Spondylitis: A rare Association.

PURPOSE: To report a case of retinal vasculitis in a patient with ankylosing spondylitis. BACKGROUND: Posterior segment involvement in HLA B-27-associated uveitis is uncommon but we report a case wherein retinal vasculitis was associated with HLA-B 27 uveitis. CASE: A 36-year-old male, a diagnosed case of ankylosing spondylitis, presented to us with severe anterior segment inflammation associated vitritis in both the eyes. He received topical, oral steroid and immunosuppressive and 3½-month after the control of his uveitis, he underwent cataract surgery in his left eye. Fundus evaluation following cataract surgery revealed sclerosed retinal vessels and wide-field fundus fluorescein angiography confirmed retinal vascular involvement in both the eyes. CONCLUSION: Retinal vasculitis, though rare can occur in patients with ankylosing spondylitis especially in conditions which present with panuveitis-like picture.

A Case of Orbital Myiasis in Recurrent Eyelid Basal Cell Carcinoma Invasive into the Orbit.

Introduction. Orbital myiasis is the infestation of the orbital tissues by fly larvae or maggots. Compromise of periorbital tissues by malignant disease, surgery, ischemia, or infection may predispose the patient to orbital myiasis. Case Report. A 73-year-old male patient with neglected recurrent basal cell carcinoma of the eyelid invasive into the orbit presented with complaints of intense itching and crawling sensation with maggots wriggling and falling from the wound of left orbit. The patient improved following manual removal of the maggots along with oral Ivermectin treatment. Recurrence of the basal cell carcinoma was confirmed by punch biopsy from the wound and extended exenteration of the orbit followed by reconstructive surgery was done. Conclusion. Orbital myiasis is a rare and preventable ocular morbidity that can complicate the malignancies resulting in widespread tissue destruction. The broad spectrum antiparasitic agent, Ivermectin, can be used as noninvasive means to treat orbital myiasis. In massive orbital myiasis and those associated with malignancies, exenteration of the orbit must be seriously considered.