Intraocular melanoma, diabetes, and Turner's syndrome: presentation with proptosis

A patient with Turner's syndrome and untreated diabetes mellitus presented with a blind, painful, glaucomatous eye and progressive unilateral proptosis. Although a computerised tomographic scan failed to show evidence of a retroocular extension of the presumed choroidal melanoma, the clinical features were so suggestive of extraocular extension that an orbital exenteration was considered. Examination of the enucleated eye for histological conformation of the presence of malignant melanoma showed no extraocular extension, and histology of the retroocular connective tissue revealed only haemorrhage and fibroblastic activity. There are 3 other reported cases of malignant melanoma presenting as proptosis without extraocular extension, so that extreme caution is required when exenteration is deemed necessary on clinical grounds but where computerised scanning fails to reveal direct tumour spread.