Spatial agreement between Goldmann visual field defects and fundus autofluorescence in patients with birdshot chorioretinopathy.

BACKGROUND: The purpose of this paper is to study the spatial agreement between visual field defects and ultra-wide field (UWF) fundus autofluorescence (FAF) in patients with birdshot chorioretinopathy (BSCR). The study is a retrospective, cross-sectional analysis of a university uveitis practice. Eight (8) eyes of five (5) patients with BSCR were included. Inclusion criteria were ability to fixate reliably. Goldmann visual fields (GVF) and UWF FAF were obtained, digitalized, and standardized. Analysis was performed by measuring areas of overlap of hypo-autofluorescent FAF lesions and GVF scotomas within the central 60°. Overlap was calculated as a percentage of the total area of FAF and GVF, respectively. Average areas were also calculated. RESULTS: The mean age of the subjects was 51 ± 12.28 years (range 38-69 years). 14 ± 23 % of the total lesion area identified as hypo-autofluorescent on FAF overlapped with scotoma. 28 ± 41 % of the GVF scotomas overlapped with hypo-autofluorescent FAF lesions. Average area of FAF hypo-autofluorescence was much larger (15.19 disc areas) than GVF (3.45 disc areas). CONCLUSIONS: There appear to be larger total areas of hypo-autofluorescence on FAF than scotoma evidenced by GVF and only a small amount of overlap. The finding suggests that GVF is relatively insensitive to anatomic loss, which can be detected using FAF. Further studies are required to assess whether this finding holds true for automated white-on-white perimetry. In addition, more selective psychophysical stimuli may have higher sensitivity in detecting early functional loss that accompanies anatomic damage.

Emixustat and Lampalizumab: Potential Therapeutic Options for Geographic Atrophy.

Two novel classes of medications are currently under extensive investigation for the treatment of dry age-related macular degeneration (AMD). Emixustat, an orally administered visual cycle inhibitor, and lampalizumab, an intravitreally administered monoclonal body directed against complement factor D, have shown promise in phase 2 clinical trials in the treatment of nonneovascular (dry) AMD. Lampalizumab is currently being evaluated in a large, multicenter, phase 3 clinical trial for dry AMD - geographic atrophy.

Combined systemic and ocular chemotherapy for anterior segment metastasis of systemic mantle cell lymphoma.

BACKGROUND: Mantle cell lymphoma (MCL) is an aggressive subtype of non-Hodgkin's lymphoma that rarely metastasizes to the iris and the anterior segment. Blastic/pleomorphic morphology is thought to have an adverse effect on prognosis in MCL. MCL is resistant to conventional chemotherapeutic regimens with a tendency for multiple relapses. Management of anterior segment metastasis of systemic MCL has not been described in literature. FINDINGS: A 58-year-old male presented with an aggressive, relapsing, metastatic, systemic blastic variant of MCL with ocular involvement. At the time of initial presentation, large tumor cells were visible in the anterior chamber (AC) along with hypopyon and fibrin. The AC cells stained positively for CD20. The iris was thickened and coated with lymphoma cells. Iris neovascularization was present. Given extensive systemic and ocular involvement, the patient was given combination chemotherapy with systemic ibrutinib and intravitreal injections of methotrexate and rituximab. The disease response was monitored using multimodal imaging, including anterior segment optical coherence tomography and ultrasound biomicroscopy. Following combination of systemic and intraocular chemotherapy, there was a marked decrease in the ocular tumor load and the systemic disease. CONCLUSIONS: Combination therapy with intravitreal injections of chemotherapeutic agents targeting monoclonal B-cell population and novel systemic agents may help to achieve remission in anterior segment metastasis of aggressive subtypes of NHL such as blastic variant of MCL. Multimodal imaging may assist in the management of these cases.

Adaptive Optics Imaging of Retinal Photoreceptors Overlying Lesions in White Dot Syndrome and its Functional Correlation.

PURPOSE: To quantify retinal photoreceptor density using adaptive optics (AO) imaging and correlate it with retinal tomography, fundus autofluorescence, and retinal sensitivity overlying lesions in various white dot syndromes (WDS). DESIGN: Prospective cross-sectional study. METHODS: setting: Stanley M. Truhlsen Eye Institute, University of Nebraska Medical Center, Omaha, Nebraska, USA. STUDY POPULATION: Thirty-five lesions of WDS from 12 patients (19 eyes; mean age: 54.4 ± 15.8 years; 9 female) were analyzed. INTERVENTION: Macular lesions (≤3 regions of interest/eye), at 2 fixed eccentric loci, were imaged using AO, spectral-domain optical coherence tomography, and fundus autofluorescence. In this study, lesions were defined as active if there was presence of hyperautofluorescence within the lesions. Photoreceptor density was calculated after manual correction and adjustment for axial length. Retinal sensitivity was assessed using microperimetry and correlated with photoreceptor density using Spearman rank correlation test. OUTCOME MEASURES: Mean retinal sensitivity and photoreceptor density at the WDS lesions. RESULTS: Mean photoreceptor density was 7331 ± 4628 cones/mm(2) overlying 16 active lesions and 6546 ± 3775 cones/mm(2) overlying 19 inactive lesions (P = .896). Mean retinal sensitivity (9.37 ± 5.34 dB) showed modest correlation with photoreceptor density (ρ = 0.42, P = .03). Retinal sensitivity over lesions with intact inner segment-outer segment (IS-OS) junction was 13.35 ± 3.75 dB and 6.33 ± 4.31 dB over lesions with disrupted IS-OS junction (P = .005). CONCLUSIONS: AO imaging may allow high-resolution analysis of photoreceptor loss among lesions in WDS. Such microstructural changes may correlate with functional loss.

Frontal sinus osteoma presenting with orbital emphysema.

Osteoma is the most common neoplasm of the paranasal sinuses usually involving the frontal sinus. Osteomas are benign, but can cause serious intracranial or orbital complications. The authors report a frontal sinus osteoma with orbital emphysema in a 16-year-old boy with a history of right-eye swelling after nose blowing. CT showed a right frontal sinus bony mass. Intraoperatively, strands of mucosa extending from the frontal sinus around the periphery of the mass in the right orbit were seen. The mass was excised and the orbital roof repaired. Histopathology was consistent with osteoma. The osteoma facilitated entrance of sinus air in the orbit, resulting in recurrent orbital emphysema with nose blowing. There was no recurrence of orbital emphysema after resection.