Neuroendocrine malignancies-tumors characterized by the production of dense-core secretory granules-are most often encountered in the lungs and can also be found in extrapulmonary sites. Our patient had a primary neuroendocrine tumor of the antrum with an elusive cell of origin that secondarily invaded the inferior orbit. In the sinuses, neuroendocrine tumors may be confused with infectious sinusitis or squamous cell carcinoma. There are no known pathognomonic clinical or radiographic signs to distinguish these tumors from other conditions. Diagnosis depends on a biopsy with histopathologic and immunohistochemical analysis to identify biomarkers such as synaptophysin, chromogranin, CD56 and neuron specific enolase. Our patient's tumor defied precise immunohistochemical characterization because of its primitive character and erratic biomarker expression. The diagnosis oscillated between a neuroendocrine carcinoma and an ectopic esthesioneuroblastoma grade IV-hence the use of the more generic nosologic category of neuroendocrine neoplasm without specifying a neuronal or epithelial origin. Data to guide management are limited, particularly in the ophthalmic literature, and derive from experience with tumors of the sinonasal compartments. In the present case of a sino-orbital high grade neuroendocrine neoplasm, regional lymph node metastases developed shortly after presentation. The tumor has responded well to chemotherapy and radiation, but recurrence is often encountered within 2 years in this class of neoplasms.

Mucormycosis is a rare often fatal opportunistic fungal infection. It is typically described in patients with diabetes in ketoacidotic status and is rare in renal transplant recipients. Calciphylaxis is a rare and highly morbid disease of vascular calcification affecting patients with end-stage renal disease (ESRD). The first case of a renal transplant recipient who was inflicted with both rhinoorbitocerebral mucormycosis and calciphylaxis is reported. A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness. He had undergone renal transplant for ESRD 7 months earlier with resultant immunosuppressive therapy. MRI and nasal biopsy confirmed rhinoorbitocerebral mucormycosis. Immunosuppressive therapy was stopped and antifungal therapy begun. He had orbital exenteration for progressive rhinoorbitocerebral mucormycosis. Two months later, the patient reported new-onset intermittent bitemporal headache and bilateral swollen, tender temporal arteries. Temporal artery biopsy revealed features consistent with calciphylaxis. Clinical presentation, treatment course, and follow up are discussed.

The authors describe a 20-year-old man who sustained multiple facial fractures in a high-speed motor vehicle crash, including a bone fragment from a skull base fracture that penetrated the orbital soft tissues superomedially. Serial CT scans documented spontaneous resorption over a 6-month period. While it is known that autologous bone grafts used in craniofacial reconstruction exhibit variable amounts of bone resorption, the complete resorption of an intraorbital fracture fragment has not been documented in the literature. His clinical care and the report of his case were undertaken in a fashion in accordance with the principles of the Health Insurance Portability and Accountability Act regulations.

Lipogranulomas of the periocular tissues with fulminant fibrotic and lymphohistiocytic responses were initially described in cases of exogenous paraffin or petrolatum jelly injections ("paraffinomas"). A 49-year-old Cambodian woman slowly developed bilateral pseudoptosis with intact levator function and redundant, taut upper eyelid skin. At surgery, vesiculations or "bubbles" in the preaponeurotic fat were encountered and were demonstrated histopathologically to be empty locules surrounded by a thin collagenous lamina. Outside these extracellular spaces were CD68/CD163-positive mononucleated and univacuolated histiocytes simulating damaged fat cells or neoplastic lipoblasts in hematoxylin and eosin sections. Giant cells and chronic sclerosing inflammation were absent. The patient denied any previous injections. The bland character of the lipogranulomas in comparison with that of other injectable agents, the absence of any residual particles associated with other cosmetic fillers, and the distinctive histiocytic response of lipoblast-like cells that were sufficiently characteristic to compel the diagnosis of surreptitious silicone injections. Other conditions were excluded based on comparative clinicopathologic criteria.

We determined whether binocular central scotomas above or below the preferred retinal locus affect detection of hazards (pedestrians) approaching from the side. Seven participants with central field loss (CFL), and seven age-and sex-matched controls with normal vision (NV), each completed two sessions of 5 test drives (each approximately 10 minutes long) in a driving simulator. Participants pressed the horn when detecting pedestrians that appeared at one of four eccentricities (-14°, -4°, left, 4°, or 14°, right, relative to car heading). Pedestrians walked or ran towards the travel lane on a collision course with the participant's vehicle, thus remaining in the same area of the visual field, assuming participant's steady forward gaze down the travel lane. Detection rates were nearly 100% for all participants. CFL participant reaction times were longer (median 2.27s, 95% CI 2.13 to 2.47) than NVs (median 1.17s, 95%CI 1.10 to 2.13; difference p<0.01), and CFL participants would have been unable to stop for 21% of pedestrians, compared with 3% for NV, p<0.001. Although the scotomas were not expected to obscure pedestrian hazards, gaze tracking revealed that scotomas did sometimes interfere with detection; late reactions usually occurred when pedestrians were entirely or partially obscured by the scotoma (time obscured correlated with reaction times, r = 0.57, p<0.001). We previously showed that scotomas lateral to the preferred retinal locus delay reaction times to a greater extent; however, taken together, the results of our studies suggest that any binocular CFL might negatively impact timely hazard detection while driving and should be a consideration when evaluating vision for driving.

PURPOSE: The safety of bioptic telescopes for driving remains controversial. The ring scotoma, an area to the telescope eye due to the telescope magnification, has been the main cause of concern. This study evaluates whether bioptic users can use the fellow eye to detect in hazards driving videos that fall in the ring scotoma area. METHODS: Twelve visually impaired bioptic users watched a series of driving hazard perception training videos and responded as soon as they detected a hazard while reading aloud letters presented on the screen. The letters were placed such that when reading them through the telescope the hazard fell in the ring scotoma area. Four conditions were tested: no bioptic and no reading, reading without bioptic, reading with a bioptic that did not occlude the fellow eye (non-occluding bioptic), and reading with a bioptic that partially-occluded the fellow eye. Eight normally sighted subjects performed the same task with the partially occluding bioptic detecting lateral hazards (blocked by the device scotoma) and vertical hazards (outside the scotoma) to further determine the cause-and-effect relationship between hazard detection and the fellow eye. RESULTS: There were significant differences in performance between conditions: 83% of hazards were detected with no reading task, dropping to 67% in the reading task with no bioptic, to 50% while reading with the non-occluding bioptic, and 34% while reading with the partially occluding bioptic. For normally sighted, detection of vertical hazards (53%) was significantly higher than lateral hazards (38%) with the partially occluding bioptic. CONCLUSIONS: Detection of driving hazards is impaired by the addition of a secondary reading like task. Detection is further impaired when reading through a monocular telescope. The effect of the partially-occluding bioptic supports the role of the non-occluded fellow eye in compensating for the ring scotoma.

PURPOSE OF REVIEW: IgG4-related disease (IgG4-RD) is a systemic process that can cause significant orbital disease. It can affect both sexes and all ages, with irreversible consequences if left untreated. Diagnosis is currently based upon a combination of clinical and imaging evidence of tissue swelling or mass, serum evidence of elevated IgG4 levels and histopathologic evidence of inappropriate IgG4 presence. The cause of IgG4-RD is as of yet unclear; this lack of understanding and the dearth of prospective studies have limited our ability to manage patients effectively. In this review, we discuss the most recent published evidence regarding best-practice management of IgG4-related orbital disease. RECENT FINDINGS: Recent literature remains retrospective, and has focused on the use of corticosteroid therapy as a first-line treatment. Rituximab infusions have also received significant attention, among other second-line agents. Radiation therapy has been reported to be effective. Long-term monitoring for relapse, involvement of other organ systems and potential neoplastic transformation is required. SUMMARY: The management of orbital IgG4-RD will gain from more targeted therapy in the future as the underlying cause is better understood. In the meantime, randomized, controlled trials of varying treatment regimens would be of benefit.

PURPOSE: To describe the patterns of the melanocytic populations of 3 cases of lacrimal sac benign melanosis and 1 of atypical primary-acquired sac melanosis with a melanomatous nodule secondary to spread of atypical conjunctival primary-acquired melanosis to the sac. METHODS: Clinical records, photographs, and paraffin sections stained with hematoxylin and eosin and the Fontana reaction were critically reviewed. Additional sections were immunoreacted for melanoma antigen recognized by T cells and microphthalmia-associated transcription factor. Five nonpigmented pterygia and 4 nonpigmented lacrimal sacs served as controls. RESULTS: Three patients with obstructive dacryocystitis and benign melanosis were African-Americans whose sacs disclosed the presence of nonclustering, melanoma antigen recognized by T cells, and microphthalmia-associated transcription factor-positive intraepithelial dendritic melanocytes at all levels of the epithelium. The transferred melanin granules were concentrated in the adlumenal apical region of the epithelial cells. No fusiform melanocytes were found in the lamina propria. The fourth patient, a Caucasian, had atypical conjunctival and sac primary-acquired melanosis and conjunctival and sac melanomas. The intraepithelial sac melanocytes in this case were strikingly atypical and profusely distributed in a back to back fashion at all levels of a thickened epithelial layer focally approximating the appearance of a melanoma in situ. Five nonpigmented pterygia and 4 nonpigmented lacrimal sacs served as controls. Each displayed nonnesting dendritic melanocytes of various densities without back to back contact. CONCLUSION: Low densities of intraepithelial melanocytes were discovered in all controls and therefore represent a normal subpopulation within the conjunctival and lacrimal sacs. Due to the pseudostratification of the sac epithelium, melanocytes can move to higher levels without implying atypia. Benign melanosis is produced by small diffusely distributed individual intraepithelial melanocytes, whereas primary-acquired melanosis with atypia exhibits back to back, dense proliferations of large atypical melanocytes.