Undifferentiated sarcoma of the orbit with angiomyxoid features.

A 7-month-old female infant developed a grade 3 un-differentiated sarcoma with angiomyxoid features of the right orbit. The tumor expanded rapidly 2 months after an initial sub-total resection and extended posteriorly toward the right cavernous sinus. After treatment with ifosphamide, doxorubicin, and proton beam radiation, there was no discernible residual tumor 20 months after starting chemotherapy.

Sensitivity of fluid detection in patients with neovascular amd using spectral domain optical coherence tomography high-definition line scans.

PURPOSE: To determine the sensitivity of the Cirrus high-definition (HD) 5-line raster scans for detecting retinal fluid in neovascular age-related macular degeneration when using the spectral domain optical coherence tomography macular cubes as a gold standard. METHODS: Patients were retrospectively identified from their initial follow-up visit after being newly diagnosed with neovascular age-related macular degeneration in at least one eye. Patients were imaged with Cirrus spectral domain optical coherence tomography using the 512 × 128 macular cube scan and HD 5-line raster scan settings. Patients with other diseases that cause subretinal or intraretinal fluid, or who had an epiretinal membrane causing macular traction were excluded from the analysis. We recorded the presence or absence of subretinal or intraretinal fluid in the macular cube and on the HD 5-line raster scans. RESULTS: Seventy-nine patients met the study requirements. Of the 63 patients who had fluid present on the macular cube, 1 did not seem to have fluid on the HD 5-line raster scans. Taking the macular cube as a gold standard, the sensitivity of the HD 5-line raster scans for detecting retinal fluid in this cohort was 98.4%. CONCLUSION: The Cirrus HD 5-line raster scans have a high sensitivity for detecting fluid in the macular cube in patients with neovascular age-related macular degeneration.

Retinal vascular tortuosity in DiGeorge syndrome complicated by solar retinopathy.

PURPOSE: To report a case of vascular tortuosity associated with DiGeorge syndrome that was complicated by solar retinopathy. METHODS: Case report and literature review. RESULTS: A 56-year-old woman with DiGeorge syndrome with secondary schizophrenia and developmental delay presented with decreased vision that was worse in her left eye. Ocular examination revealed bilateral retinal vascular tortuosity involving both the arteries and veins. Both eyes had an abnormal foveal light reflex with a central yellowish hue, which was more pronounced in the left eye. Optical coherence tomography showed disruption of the photoreceptor inner segment-outer segment junction and retinal pigment epithelium centrally, which was also more prominent in the left eye. CONCLUSION: DiGeorge syndrome is associated with retinal vascular tortuosity in a large percentage of patients. The patient's decreased vision is likely caused by solar retinopathy (prolonged sun gazing as a result of the secondary schizophrenia and developmental delay).

Intravitreous ranibizumab for persistent macular edema in retinal vein occlusion unresponsive to bevacizumab.

PURPOSE: To report successful treatment of persistent retinal edema associated with retinal vascular occlusion with ranibizumab after initial failure with bevacizumab intravitreal injections. METHODS: A retrospective chart review of all patients undergoing treatment with intravitreal bevacizumab for retinal vascular occlusion was undertaken. Clinical features of patients who were switched to monthly ranibizumab because of persistent and/or worsening retinal edema despite multiple (six or more) monthly intravitreal bevacizumab injections were selected for review. The type of retinal vascular occlusion, age of the patient, visual acuity at each visit, number of intravitreal bevacizumab injections, optical coherence tomography findings at each visit, fluorescein angiography, and number of intravitreal ranibizumab injections with optical coherence tomography findings at each subsequent visit were recorded. RESULTS: Six patients (five women and one man) were identified. Age ranged from 56 years to 74 years. Five had central retinal vein occlusion and one a branch retinal vein occlusion. All were treated with at least 6 sequential intravitreal bevacizumab injections (range, 6-13) before treatment with ranibizumab. All demonstrated an immediate and marked reduction in retinal edema that had not occurred with any of the previous treatments. The average central foveal thickness on optical coherence tomography was 592 µm before ranibizumab injection and 346 µm after the injection. Visual acuity improved in four of the six patients and was stable in the remaining two patients. CONCLUSION: Eyes of patients with persistent macular edema secondary to retinal vein occlusion despite bevacizumab may experience prompt improvement after initiation of intravitreal ranibizumab injections.

Congenital narrow-angle glaucoma and iris nevi in a neonate with epidermal nevus syndrome.

Epidermal nevus syndrome (ENS) is a neurocutaneous disorder characterized by epidermal hamartomas and abnormalities of the brain, eye, and other systems. We report the occurrence of congenital angle closure glaucoma in a patient with epidermal nevus syndrome. Intraoperative use of ultrasound biomicroscopy was essential in making the diagnosis.