Extranodal Rosai-Dorfman Disease of the Orbit: Clinical Features of 8 Cases.

PURPOSE: To report on the presentation, radiography, histology, and treatment of 8 cases of extranodal Rosai-Dorfman disease involving the orbit. METHODS: Multicenter retrospective case series. RESULTS: Five males and 3 females had a median age of 10 years (range 2-78 years). Presenting signs and symptoms included proptosis, periorbital pain, palpable mass, blepharoptosis, decreased vision, diplopia, impaired extraocular motility, and afferent pupillary defect. Four patients had bilateral orbital disease, while 4 had unilateral disease. Six cases were extraconal, 1 was intraconal, and 1 was both intra- and extra-conal. Four cases had only extranodal disease without lymphadenopathy (3 of which had localized orbital disease). Diagnosis was confirmed by exam, orbital, and/or systemic radiography, and biopsy in all cases. Treatment strategies included excision or debulking, systemic corticosteroids, chemotherapy, radiotherapy, observation or a combination thereof. At last follow up, 4 patients were disease free, while 4 had residual improved disease. CONCLUSIONS: Rosai-Dorfman disease of the orbit is a rare clinical entity. Purely extranodal disease is rare, with isolated orbital disease being exceedingly rare. This study is unique in that 4 of 8 patients had strictly isolated extranodal disease of the orbit. A large majority of the cases had disease in the extraconal space, contrasting with previous reports. In addition, lacrimal gland disease, particularly bilateral involvement, was prominent in the current study. Although there is no consensus on treatment, surgical excision should be attempted if plausible in symptomatic patients especially if the orbit represents a localized site of disease.

ENDOGENOUS ASPERGILLUS ENDOPHTHALMITIS IN AN IMMUNOCOMPETENT PATIENT WITH A REMOTE HISTORY OF PULMONARY TUBERCULOSIS.

PURPOSE: To report a case of Aspergillus fumigatus endogenous endophthalmitis in an immunocompetent patient initially diagnosed as acute retinal necrosis. METHODS: Case report. PATIENT: A 67-year-old woman with a remote history of treated pulmonary tuberculosis and no ocular history presented to an outside retina specialist with a sudden onset of floaters and blurred vision in one eye. Examination and fluorescein angiography at the time revealed findings suspicious for acute retinal necrosis, and the patient was started on oral valganciclovir and an intravitreal injection of ganciclovir. Despite treatment, the patient's vision and pain worsened. After evaluation at the University of Southern California Roski Eye Institute, she was diagnosed with a likely fungal endogenous endophthalmitis based on ultrasound findings and underwent emergent vitrectomy. A chest x-ray demonstrated partial collapse of the right upper lobe with hilar enlargement. RESULTS: Aspergillus fumigatus was cultured from vitreous, blood, and bronchoalveolar lavage samples, suggesting that the patient's infection had a pulmonary origin, most likely from the right upper lobe that had healed from previous tuberculosis infection. DISCUSSION: To the best of our knowledge, this is the first reported case of Aspergillus endogenous endophthalmitis in an immunocompetent patient secondary to pulmonary changes that occurred from previously treated tuberculosis.

Ocular Adverse Effects of Intravitreal Bevacizumab Are Potentiated by Intermittent Hypoxia in a Rat Model of Oxygen-Induced Retinopathy.

Intravitreal bevacizumab (Avastin) use in preterm infants with retinopathy of prematurity is associated with severe neurological disabilities, suggesting vascular leakage. We examined the hypothesis that intermittent hypoxia (IH) potentiates intravitreal Avastin leakage. Neonatal rats at birth were exposed to IH from birth (P0)-P14. At P14, the time of eye opening in rats, a single dose of Avastin (0.125 mg) was injected intravitreally into the left eye. Animals were placed in room air (RA) until P23 or P45 for recovery (IHR). Hyperoxia-exposed and RA littermates served as oxygen controls, and equivalent volume saline served as the placebo controls. At P23 and P45 ocular angiogenesis, retinal pathology and ocular and systemic biomarkers of angiogenesis were examined. Retinal flatmounts showed poor peripheral vascularization in Avastin-treated and fellow eyes at P23, with numerous punctate hemorrhages and dilated, tortuous vessels with anastomoses at P45 in the rats exposed to IH. These adverse effects were associated with robust increases in systemic VEGF and in both treated and untreated fellow eyes. Histological analysis showed severe damage in the inner plexiform and inner nuclear layers. Exposure of IH/IHR-induced injured retinal microvasculature to anti-VEGF substances can result in vascular leakage and adverse effects in the developing neonate.

Cerebral venous sinus thrombosis with ophthalmic manifestations in 18-year-olds on oral contraceptives.

Cerebral venous sinus thrombosis (CVST) is a rare and potentially life-threatening cause of stroke. In the past few decades, the incidence and patient demographics have changed because of many factors, including the widespread use of oral contraceptives, improved detection of prothrombotic conditions, and advancement of imaging technology. The presentation of CVST is varied and can include ocular signs and symptoms. We present 2 cases of oral contraceptive-induced CVST in 18-year-old women, whose main presenting findings were ophthalmologic.

Risk factors for steroid response among cataract patients.

PURPOSE: To evaluate the age and ocular axial length (AL) of topical steroid responders after uneventful cataract surgery. SETTING: Private practice, Los Altos, California, USA. DESIGN: Case-control study. METHODS: This retrospective chart review comprised consecutive patients from a single-surgeon practice who had cataract surgery during a 2-year period. All patients routinely received topical prednisolone acetate 1.0% postoperatively. In addition to AL and patient age, the intraocular pressure (IOP) was recorded preoperatively, 1 day postoperatively, and at least 1 additional time in the first postoperative month while the patient was taking a topical corticosteroid agent. A steroid response was defined as an IOP increase greater than 25% while on topical prednisolone (minimum 28 mm Hg) followed by a decrease of more than 25% after topical prednisolone was discontinued. The age and AL were compared between steroid responders with nonresponders. RESULTS: Of the 1642 patients, 39 were diagnosed as steroid responders. Younger age and a longer AL were associated with a higher risk for steroid response, particularly in patients younger than 65 years with an AL of at least 29.0 mm. These patients had a 39-fold increased risk for an IOP higher than 28 mm Hg and a 35-fold increased risk for an IOP higher than 35 mm Hg than patients older than 65 years with a normal AL. CONCLUSION: Younger patients with high myopia had a higher risk for a postoperative steroid response after uneventful cataract surgery and may require more frequent IOP monitoring or alternative topical antiinflammatory medications. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.