Metastatic Ewing cell sarcoma of the sinus and osteoid osteoma of the orbit.

PURPOSE: To familiarize ophthalmologists with the management of a "solitary hot lesion" on bone scan as part of a metastatic examination. METHODS: Case report of a 27-year-old man with primary Ewing sarcoma of the fibula. Bone scan as part of a metastatic evaluation showed increased uptake in the left orbit. A metastatic lesion could not be ruled out radiologically. RESULTS: A biopsy was performed, and the lesion was found to be osteoid osteoma; therefore, the patient received only local radiation therapy to the fibula. Eight months later, the patient developed metastatic Ewing cell sarcoma of the sphenoid sinus and subsequently died. CONCLUSIONS: Because of the high sensitivity but low specificity of bone scan, secondary lesions on bone scan are not necessarily metastases. A biopsy specimen is often required for pathologic diagnosis.

Acanthamoeba keratitis: is water exposure a true risk factor?

PURPOSE: Water exposure has been commonly cited in the literature as a significant risk factor for the development of Acanthamoeba keratitis. The purpose of this study was to test the hypothesis that the incidence of tap and contaminated water exposure in Acanthamoeba keratitis patients is no higher than in the normal contact lens population. METHODS: We retrospectively reviewed all patients all patients diagnosed with Acanthamoeba keratitis at Massachusetts Eye & Ear Infirmary from 1990-1994, and compared this group to a control population of healthy contact lens wearers assessed by a self-report questionnaire. RESULTS: A history of water exposure was reported by 64% (seven patients) diagnosed with Acanthamoeba keratitis. In comparison, 100% of the control population of healthy contact lens wearers (100 patients) reported comparable water exposure, and 87% of controls reported multiple sources of water exposure. CONCLUSIONS: The finding that the incidence of water exposure is actually lower in patients with Acanthamoeba keratitis than in the general contact lens population suggests that water exposure may be less important, and other factors more important in the development of this disease than previously assumed.

Norrie disease. Diagnosis of a simplex case by DNA analysis.

Norrie disease is a rare, X-linked recessive disorder characterized by congenital blindness due to malformed retinas. We describe a simplex patient who had leukokoria and whose clinical diagnosis was confirmed only after molecular genetics analysis. DNA analysis was also used to determine the carrier status of relatives of the proband.

Cogan's syndrome: ophthalmic, audiovestibular, and systemic manifestations and therapy.

Cogan's syndrome is an idiopathic inflammatory disease which may present as interstitial keratitis, inflammation of other ocular structures, Meniere's-like attacks, or systemic vasculitis. Although the ocular manifestations respond to topical steroids and are rarely serious, permanent deafness may result if systemic steroid therapy is not promptly instituted for audiovestibular dysfunction, while major morbidity and even death may occur if systemic sequelae such as vasculitis and aortic insufficiency are not recognized. For these reasons, ophthalmologists must suspect Cogan's syndrome in any patient presenting with ocular inflammation who develops hearing loss, vertigo, ataxia, tinnitus, systemic vasculitis, or aortic insufficiency.

Acanthamoeba keratitis. Contact lens and noncontact lens characteristics.

PURPOSE: To identify potential differences in time to diagnosis and final visual outcome between contact lens and noncontact lens users with Acanthamoeba keratitis. BACKGROUND: Prior studies have shown early diagnosis and therapy to be related to outcome but have not analyzed differences among patients with and without contact lenses. METHODS: A retrospective analysis of 11 consecutive cases (involving 13 eyes) of Acanthamoeba keratitis diagnosed at one institution over a 3-year period. RESULTS: Mean time to diagnosis was significantly longer in noncontact lens users versus those who wore contact lenses (mean, 5.8 versus 3.3 weeks). Fifty percent of patients who did not wear contact lenses had a poor outcome (visual acuity worse than 20/40 or penetrating keratoplasty) versus 14% of patients who wore contact lenses. CONCLUSION: Diagnoses were made later in patients without contact lenses, and these patients had a worse visual outcome than those with Acanthamoeba keratitis who wore contact lenses. All patients with unresponsive microbial keratitis, even those without contact lens use, should be evaluated for Acanthamoeba.

Neurilemmoma of the anterior ethmoidal nerve encroaching upon the nasolacrimal duct.

The clinical, light microscopic, ultrastructural, and immunohistologic findings of a neurilemmoma encroaching upon the nasolacrimal duct are presented. This is the first reported case of this rare tumour of the nasolacrimal duct where the diagnosis has been confirmed by electron microscopy and immunohistochemical techniques.

Correlations among field dependence-independence, sex, sex-role stereotype, and age of preschoolers.

Preschoolers (27 boys, mean age = 4.7 yr.; 24 girls, mean age = 4.6 yr.) were assessed for field dependence-independence (Preschool Embedded Figures Test), sex-role stereotyping (Sex-role Learning Inventory), and receptive verbal intelligence (Peabody Picture Vocabulary Test--Revised). Relative to the girls, the boys were significantly less field-independent and significantly more sex-role stereotyped. After age and Peabody IQs were partialled out by multiple regression, same-sex typing in boys and cross-sex typing in girls were significant predictors of field independence. The regression analysis also suggested that, by 5.3 yr. of age, the boys as a group surpassed the girls on field independence. Limitations of the present research and educational implications of the over-all findings are discussed.