Orbital inflammatory disorders: new knowledge, future challenges.

PURPOSE OF REVIEW: This review aims to bring together recent advances in basic, translational and clinical research on the pathogenesis and treatment of orbital inflammatory conditions. RECENT FINDINGS: Basic science studies provide mechanistic insights into why the orbit is targeted for inflammation by autoimmune inflammatory disorders. Using Graves' disease as a test case reveals that endocrine pathways, such as the TSH and IGF1 receptor pathways play important roles in stimulating orbital inflammation. Furthermore, orbital tissues contain high concentrations of retinoids - byproducts of the visual pathway that diffuse across the sclera and can activate de novo transcription of inflammatory cytokines. Such cytokine expression places the orbit in a hyper-inflammatory 'resting' state, prone to respond to any additional systemic or local pro-inflammatory signals. The HIF2A--LOX pathway appears important for orbital tissue fibrosis. Lastly, bench-to-bedside studies of the IGF1R pathway have led to an FDA-approved drug, teprotumumab that represents a novel treatment approach for Graves' orbitopathy. Unfortunately, high drug costs and misplaced insurance company 'step-therapy' policies may block patients from receiving therapy that can protect vision and improve quality of life. SUMMARY: Improved understanding of orbital inflammatory conditions has led to a new drug and promises additional breakthroughs. Translational research is successful, but requires time, resources, and patience.

CD56 negative extranodal NK/T-cell lymphoma of the orbit mimicking orbital cellulitis.

INTRODUCTION: To report a case of Extranodal NK/T-cell lymphoma of the orbit mimicking orbital cellulitis. CASE DESCRIPTION: A 52-year-old healthy male presented to our institution after 3 months of treatment for sinusitis with antibiotics and steroids. The patient was transferred due to the presence of an "orbital abscess" on CT with orbital signs that not responding to antibiotics. Clinical examination was significant for decreased vision in the affected orbit of 20/50, a trace RAPD OS, elevated IOP of 30 OS, proptosis and grossly decreased motility with diplopia, periorbital edema and chemosis. Dilated funded exam was unremarkable. CT imaging demonstrated a left sided pan-sinusitis, a medial "orbital process" with proptosis and erosion of the cribiform plate. The patient was taken for an emergent orbital exploration for histopathologic diagnosis. Intraorbital and sinus biopsy was consistent with extranodal NK/T-cell lymphoma, with extension into the skull base and left orbital space. The patient was started on radiation therapy followed by chemotherapy. COMMENTS: The authors demonstrate how the acute presentation of an aggressive extranodal NK/T-cell lymphoma can present in a similar fashion as orbital cellulitis. Additionally, the case highlights that a unilateral pansinusitis with involvement of the skull base and orbit is likely due an aggressive malignant process in an immune competent patient.

Cytokine expression in pediatric subperiosteal orbital abscesses.

BACKGROUND: Cytokines have been shown to play a key role in infectious and inflammatory processes. The purpose of the study was to characterize the pattern of cytokine expression in subperiosteal orbital abscesses associated with pediatric orbital cellulitis. METHODS: All pediatric patients over a 5-month period who had orbital cellulitis and a subperiosteal abscess with an adjacent sinusitis requiring surgical drainage of the orbital abscess were given the opportunity to enroll in the study. A protein array membrane and a chemiluminescent detection system were used to identify the presence of 45 cytokines in the subperiosteal abscess fluid. RESULTS: Four abscesses were analyzed with the protein array membrane. Of the 45 cytokines studied for this report, interleukin (IL)-1alpha, IL-1beta, IL-1 receptor antagonist (ra), IL-6, tumor necrosis factor (TNF)-alpha, and TNF-beta were detected in all specimens. Additionally, IL-16, epidermal growth factor related protein, and soluble TNF receptor II were detected in 3 of the 4 specimens. INTERPRETATION: Pediatric orbital cellulitis with subperiosteal abscess is an inflammatory condition with a distinct pattern of cytokine expression. The detection of IL-1, IL-1 ra, IL-6, and TNF suggests that in the future these cytokines may play a role in monitoring disease activity or as potential targets for immunotherapy.