The authors describe the ophthalmological findings and clinical course in two patients with EEC syndrome and one patient with anhydrotic ectodermal dysplasia. A retinal complication was seen in a 43-year-old patient which seems to be associated with the EEC syndrome and has not yet been described. A review of the literature indicates the frequency of ocular findings. So far, primary ocular changes in anhydrotic ectodermal dysplasia have never been observed. The older the patient, the more severe the secondary complications affecting the lids and cornea can be; the eye may even be lost.
A total of 455 enucleated globes were examined for a pseudoexfoliation syndrome. The typical alterations could be diagnosed most clearly from the ciliary processes. The diagnosis is frequently missed in first examinations of routine sections. Among 323 nonselected eyes with secondary glaucoma, which had to be enucleated following painful amaurosis, pseudoexfoliation syndrome was found in 3.4%. On the other hand, in 132 eyes with intraocular tumors it was found only once. Our examinations provide indications that pseudoexfoliation syndrome possibly causes an unusually high incidence of deleterious secondary changes which render enucleation necessary.
Examination of specially stained sections (e.g. with mucicarmine) should be routinely performed for those conjuctival neoplasms that contain a squamous component. Aggressive surgical management, such as early enucleation including normal appearing tissue next to the globe, should be considered for treatment of primary mucoepidermoid carcinoma to avoid later exenteration or metastasis.
Between 1983 and 1986, six eyes with diffuse malignant melanomas of the anterior uvea were enucleated at the Eye Hospital of the University of Erlangen-Nürnberg. The characteristic clinical findings were a monolateral open-angle glaucoma in the same eye and a diffuse spreading of malignant melanocytes on the inner walls of the anterior chamber. These cells can be differentiated biomicroscopically (40 x magnification, Zeiss slit-lamp) from melanin granula and benign melanocytes. Findings in two patients show that individual cells can be biocytologically classified in this way. Malignant cells are characterized by large, prominent nuclei and long cytoplasmic processes. Biocytological evaluation of individual melanocytic cells can be helpful in the differential diagnosis of melanocytic processes of the anterior uvea.
Phthisical eyes of 2 patients revealed clinically unsuspected, partially necrotic and partially vital malignant melanomas of the uvea and extensive intraocular ossification. Both eyes had had recurrent intraocular inflammatory episodes for years before they were enucleated because of pain. Intraocular ossification does not rule out the presence of clinically unsuspected malignant melanoma of the uvea.
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