Bilateral Occlusive Retinal Vasculitis in a Patient with Dermatomyositis.

A 48-year-old woman with a history of dermatomyositis (DMS) presented with 2 weeks of worsening myalgias, weakness, and diffuse edema following cessation of her systemic immunosuppression and subsequently developed severe bilateral vision loss consistent with bilateral frosted branch angiitis. Multimodal imaging was performed, and the patient was successfully treated with pulse-dose steroids and intravenous immunoglobulin, as well as intravitreal aflibercept. Ophthalmic involvement of DMS is typically limited to episcleritis, conjunctivitis, and uveitis. We present an uncommon case of bilateral occlusive retinal vasculitis with frosted branch angiitis in a patient with DMS. The significant improvement anatomically and in visual acuity in our patient suggests a role of combined anti-vascular endothelial growth factor and systemic immunosuppression in the management of DMS -related frosted branch angiitis. We suggest that retinal vasculitis should be considered in patients with known DMS and acute vision loss, with prompt referral for ophthalmologic evaluation.

Ocular, Auricular, and Oral Manifestations of Inflammatory Bowel Disease.

Inflammatory bowel disease (IBD) is primarily a disease of the gastrointestinal tract, though it can often affect other organ systems. These extraintestinal manifestations occur in a quarter to one-third of patients with Crohn's disease and ulcerative colitis. While musculoskeletal and dermatologic manifestations are the most common, it is also important to be cognizant of head, eye, ear, nose, and throat (HEENT) manifestations and educate IBD patients about them. Here we review the ocular manifestations in conjunction with the lesser-known but increasingly recognized ENT manifestations. Considering the lack of randomized controlled trials in treating HEENT manifestations of IBD, this review is primarily based on case reports, case series, and expert opinion with a particular focus on the newer literature supporting use of anti-TNF agents.

Scleral necrosis in a patient with congenital erythropoietic porphyria.

PURPOSE: To report on a patient with congenital erythropoietic porphyria who presented with scleral necrosis. METHOD: Case report. RESULTS: A 34-year-old man with a long history of congenital erythropoietic porphyria was referred to us for evaluation of necrotizing scleritis of the right eye. The patient presented with a 3-month duration of eye pain and redness, which initially responded to oral and topical corticosteroids. However, upon corticosteroid taper, the symptoms quickly recurred. The patient was initially tried on oral azathioprine therapy, which failed to induce resolution of the symptoms. Full serological investigation did not suggest that the necrotic process was secondary to an immunologically driven process, and we proceeded with scleral biopsy and patch graft. The biopsy disclosed atrophic changes of the sclera with calcium-like plaques, without evidence of inflammation. Aggressive strategy of sun avoidance was implemented and immunosuppressive therapy discontinued. One year after the surgery, the patient remains asymptomatic without recurrence of ocular disease. CONCLUSIONS: This case highlights the usefulness of tissue biopsy in porphyria when one is uncertain as to whether a necrotizing process is driven by inflammation or phototoxic damage. When the underlying mechanism is clearly identified, the appropriate therapy can then be instituted to prevent further damage.

Intravitreous bevacizumab injection: an experimental study in New Zealand white rabbits.

OBJECTIVES: To determine the effects of intraocular pressure (IOP) and needle diameter on the amount of reflux after intravitreous bevacizumab injection. METHODS: Prospective randomized interventional study. Twelve New Zealand white rabbits weighing approximately 2.5 to 3.5 kg each were randomized 1:1 to group 1 or group 2. Bevacizumab stained with trypan blue was used for intravitreous injection. To lower the IOP, eyes in group 2 underwent anterior chamber paracentesis before intravitreous injection. Two eyes in each group were injected using 27-, 30-, or 32-gauge needles. If a subconjunctival bleb formed after intravitreous injection, its diameter was measured using a caliper. RESULTS: The median IOP in group 1 was 17.5 mm Hg. Eyes injected using 27-gauge and 30-gauge needles showed stained subconjunctival blebs with median sizes of 3 mm and 1.7 mm, respectively; eyes injected using 32-gauge needles showed no subconjunctival bleb formation. The median IOP in group 2 was 10.3 mm Hg. Eyes injected using 27-gauge needles showed stained subconjunctival blebs with a median size of 0.7 mm, and eyes injected using 30-gauge and 32-gauge needles showed no subconjunctival bleb formation. CONCLUSION: Decreasing the IOP before intravitreous injection and using a smaller-gauge needle reduce the risk of drug reflux after intravitreous bevacizumab injection. CLINICAL RELEVANCE: Intravitreous injection is an increasingly common route of drug delivery to treat ocular diseases. Techniques that maximize bioavailability are examined in this study.