Orbital malignancies in infancy and childhood in Denmark. Recommendations for diagnosis and therapy.
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Biomedical subjects
Publications and source records attributed to H Fledelius.
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A Danish nation-wide 20-year series of orbital tumours in infancy is reported, making up a total of 80 histopathologically verified cases. Secondary tumours were not included except for a few cases where the initial manifestation of the disease was an orbital mass, suggesting a primary tumour of the orbit. A scant half of the series were benign choristomas. Otherwise there was a considerable share of more serious diseases, often threatening vision and/or life; thus ten embryonal sarcomas and nine optic nerve gliomas were found. The malignancies made up a total of 17 cases. Because of the many medical specialities necessary for prompt diagnosis and efficient therapy, a centralization of the more serious orbital cases is feld mandatory. Considering the rather low overall frequency of orbital tumours, a centralization will not constitute a load numerically; an estimate for Denmark (population 5 million) is about five infantile cases a year.
A malignant melanoma of the choroid was observed in an 11-month-old fair-skinned Danish boy - one of the youngest cases ever published. There was no evidence of pre-existing melanosis of eyes or skin. The tumour showed histopathologically a typical "adult" pattern and was classified as a mixed tumour. The melanin content was marked. In some earlier reports on uveal tumours within the first year of life, pigmentation was sparse or lacking; a hypothesis has therefore previously been advanced: That melanomas are unable to produce melanin so early in life. The present case renders proof against this hypothesis.
Cataracta ossea is described in a 5-year-old Danish boy, representing a sequal to a unilateral measles-endophthalmitis 3 years earlier. Ossification was not demonstrated elsewhere in the eye. Further, cases of intraocular ossification over a 30-year period were reviewed, based on the files of the Eye Pathology Institute. The total of 155 made up about 3% of enucleated eyes from Danish material received for examination. Regarding age at the initial eye lesion, more than half appeared within the first decade of life. The most frequent underlying lesions were trauma (32%) and uveitis (25%). Among the less frequent causes, emphasis is given to three cases of malignant uveal melanoma. Ossification of the lens was not encountered in the series - except for the case which motivated the review.
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Ultrasound oculometry (A mode) was performed in 30 eyes with three different standard equipments and great effort was made to make the three sets of measurements comparable. In spite of careful calibration procedures significant inter-equipment differences were found: anterior chamber depths and lens thicknesses showed the greatest discrepancies. These findings should be kept in mind when oculometric results of different authors are compared. A standardized eye-like phantom is desirable as a common reference for all who work in ultrasonic oculometry.
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PURPOSE: To describe ophthalmic findings with emphasis on exophthalmometry and ultrasonic assessment of extraocular eye muscle diameter in a consecutive group of females with Graves' disease (GD), compared with healthy controls and patients with other thyroid diseases. We also investigated the relationship with biochemical markers of thyroid autoimmunity such as TSH receptor antibodies (TRAb) and anti-thyroid peroxidase antibodies (anti-TPO). METHODS: Seventy adult women (age 26-74 years) with various types of thyroid disease consecutively entered the study at a tertiary referral center for thyroid-associated ophthalmopathy (TAO). Twenty-three had long-standing GD with TAO. Clinically, TAO was mainly absent in 22 with newly diagnosed GD and in seven with relapse of GD. Nine with Hashimoto's thyrolditis and nine with multinodular goiter were included for comparison and 18 healthy females served as controls. A full ophthalmic status included B-scan ultrasonic assessment of the four horizontal rectus muscle thicknesses, and a clinical NOSPECS score was attempted for each. RESULTS AND CONCLUSIONS: Besides higher NOSPECS scores, the TAO subgroup had higher exophthalmometry and muscle thickness. The GD groups without significant TAO also scored higher in these ratings compared to controls. Hertel recordings, NOSPECS and muscle thicknesses were all correlated in GD but showed no correlation to thyroid antibodies (TRAb and anti-TPO). Thus, the muscle thickness did not correlate with thyroid autoimmune activity. Nevertheless, we found extraocular muscle assessment useful since a) thicker muscles were usually found in patients with GD, with or without evidence of TAO, and b) other space-occupying orbital lesions could be excluded, thereby reducing the need for the more elaborate imaging techniques (CT, MRI, etc.).