Optic Disc Edema Is an Under-Recognized Feature of Birdshot Chorioretinitis.

BACKGROUND: Optic disc edema is a feature of many ophthalmic and neurologic conditions. It remains an underappreciated feature of birdshot chorioretinitis (BSCR), leading to delay in diagnosis and treatment. The purpose of our study was to identify clinical features that are concomitant with optic disc edema and suggest a diagnosis of BSCR. METHODS: Retrospective multicenter case series of 29 patients who were referred to a neuro-ophthalmologist or uveitis specialist for evaluation of disc edema and were ultimately diagnosed with BSCR. RESULTS: Fifty-four eyes of 30 patients, from the practices of 15 uveitis specialists, met the eligibility criteria. In addition to disc edema, concomitant features in all patients included vitritis, chorioretinal lesions, and retinal vasculitis. Visual recovery to 20/40 or better occurred in 26 of 29 patients. Visual acuity remained 20/100 or worse in 2 patients previously diagnosed with idiopathic intracranial hypertension, 1 patient previously diagnosed with optic neuritis, and 1 patient for whom treatment was delayed for years, leading to optic disc atrophy. CONCLUSIONS: Optic disc edema is a presenting feature in some cases of BSCR. A diagnosis of BSCR should be considered when disc edema occurs with vitritis, chorioretinal inflammation, and retinal vasculitis. Patients should be referred to a uveitis specialist for treatment.

Association of Dupilumab with Intraocular Inflammation.

PURPOSE: To report four cases of uveitis after treatment with dupilumab for atopic dermatitis. METHODS: Retrospective case series. RESULTS: Data was collected from four patients with inflammation that developed after treatment with dupilumab. The first patient was thought to have developed posterior scleritis or Harada's-type disease related to her treatment with dupilumab, with recurrence of intraocular inflammation upon restarting treatment. The second patient developed anterior and intermediate uveitis as well as cystoid macular edema in her right eye about two years after starting dupilumab treatment. The third patient developed a distinct relentless placoid chorioretinitis while on therapy with dupilumab. The fourth patient developed bilateral cystoid macular edema while on treatment with dupilumab. CONCLUSION: We report a case series of patients treated with dupilumab who developed intraocular manifestations of inflammatory disease.

Horner's Syndrome Following Varicella Vaccination.

A 52-year-old female with no significant past medical history presented with disseminated herpes zoster as well as a Horner's syndrome and retinal haemorrhages 3 weeks after receiving the varicella vaccine. The patient began treatment with valacyclovir, 1g three times per day, and gabapentin, 300mg three times a day, for pain. Over the following month, the rash and the retinal haemorrhages resolved. The ptosis and anisocoria also resolved over the following two months.

Clinical characteristics and visual outcomes in infectious scleritis: a review.

Infection is a very important but rare cause of scleritis, occurring in about 5%-10% of all patients presenting with scleral inflammation. However, due to the similarity of its presentation, infectious scleritis is often initially managed as autoimmune, potentially further worsening its outcome. The overall visual outcome in infectious scleritis is generally worse than its autoimmune counterparts, perhaps because of the delay in diagnosis or because of the aggressive nature of associated microbes. Thus, there is a definite need for insight into the diagnostic approach and treatment options for this ocular disease process. Several studies and case reports have been published in recent years that have provided useful information regarding the presenting clinical features and etiologic microbial agents in infectious scleritis. This review summarizes the important findings in the literature that may aid in differentiating infectious scleritis from other etiologies, including predisposing factors, microbe-specific characteristics, diagnostic tools, treatment modalities, and outcomes.

Macular hole overlying pigment epithelial detachment after intravitreal injection with ranibizumab.

PURPOSE: To describe a previously unrecognized complication of intravitreal injection in a patient with exudative macular degeneration and vitreomacular adhesion. METHODS: Case report of a 69-year-old woman. RESULTS: One month after intravitreal injection with ranibizumab for exudative macular degeneration, our patient developed a full-thickness macular hole with visual acuity of 20/100. After pars plana vitrectomy with membrane peeling and fluid-gas exchange, the macular hole was closed and the visual acuity improved to 20/60. CONCLUSIONS: Macular hole formation after intravitreal injection with ranibizumab is a potential complication that should be considered before treatment, especially in those patients with large subfoveal pigment epithelial detachments and vitreomacular adhesion. Although successful closure may be attained after pars plana vitrectomy, this potential morbidity should be considered when treating these patients. In addition to the already well-described tractional forces of the vitreous overlying the fovea, and the potential contractile nature of a choroidal neovascular complex in response to ranibizumab, a posterior pushing or stretching mechanism of a large pigment epithelial detachment may also contribute to macular hole formation.

Uveitis in Blau syndrome from a de novo mutation of the NOD2/CARD15 gene.

Blau syndrome (MIM 186580) is a rare autoinflammatory, familial granulomatous condition that occurs secondary to a single amino acid mutation of the NOD2/CARD15 gene on chromosome 16p12-q21. We report the case of a 2.5-year-old girl who presented for ophthalmic examination in the setting of rash and synovitis. Initially, small, evanescent, ovoid corneal subepithelial opacities unique to Blau syndrome were observed. She later developed a fulminant panuveitis that responded to immunomodulatory therapy. Subsequent genetic testing confirmed the diagnosis of Blau syndrome. Despite immunosuppression, at almost 7 years of age, she continues to have persistent panuveitis with vision of 20/20.

Scleritis and systemic disease association in a community-based referral practice.

PURPOSE: To evaluate the association between scleritis and systemic disease in a non-university, non-tertiary referral practice and to describe our experience with scleritis treatment. DESIGN: Retrospective chart review. METHODS: The medical records of patients with scleritis between 2001 and 2007 were reviewed for associated systemic disease. RESULTS: In our series of 86 patients with scleritis, 55 patients (64.0%) had isolated scleritis while 31 patients (36.0%) had associated systemic-disease. Twenty-six patients (83.9%) with systemic disease had diagnosed systemic disease at the time of initial scleritis presentation, while 5 patients (16.1%) were diagnosed following systemic work-up. Those diagnosed after systemic work-up were more likely to have systemic vasculitic disease as opposed to a rheumatic or infectious disease. Patients with and without associated systemic disease were likely to require systemic therapy at similar rates (93.5% and 92.7%, respectively). Five patients with steroid-refractory scleritis were treated with infliximab (Remicade; Centocor Inc, Horsham, Pennsylvania, USA) and all responded without evidence of adverse effect. Seven patients were treated with mycophenolate mofetil (CellCept; Roche Laboratories, Nutley, New Jersey, USA), of which three improved. CONCLUSIONS: The association between scleritis and systemic disease in a community-based referral practice may be lower than in tertiary referral or university-based centers. Although thorough systemic disease evaluation is warranted in scleritis patients, most patients with associated systemic disease will have such a diagnosis prior to the development of scleritis. The need to institute aggressive systemic therapy cannot be predicted by the presence of an associated systemic disease. Infliximab and mycophenolate mofetil are useful additions to the scleritis practitioner's armamentarium for steroid-refractory scleritis.