A family with oculoauriculovertebral dysplasia is reported in which there are nine affected members spanning three consecutive generations. It is concluded that despite the known genetic heterogeneity in this disorder, there is an autosomal dominant form. Thus, genetic counseling can only be given after proper completion of all the necessary clinical and family studies.

SUMMARY This report describes a 41-year-old man with an intraocular tumour misinterpreted clinically as choroidal melanoma. The fluorescein angiographic features were not fully characteristic of uveal malignancy, and indeed histopathology revealed the diagnosis of adenocarcinoma of the retinal pigment epithelium. It is suggested that, in cases with the fundus and angiographic findings described here, the rare possibility of adenocarcinoma of retinal pigment epithelium should be kept in mind. Of particular interest were the changing pathological findings in the various parts of the tumour, which paralleled the fluorescein angiographic pattern.Adenocarcinoma of the retinal pigment epithelium is a very rare neoplasm which is notoriously difficult to diagnose clinically and has always prompted enucleation because of its resemblance to choroidal melanoma. '-1 This report describes the case of a male patient who underwent enucleation for a retinal mass that looked like a melanoma and seemed to grow during a follow-up period of one year. The angiographic characteristics were not fully suggestive of melanoma, and indeed the histopathological studies revealed the tumour to be an adenocarcinoma of the retinal pigment epithelium. In such a rare condition, where reports on individual patients constitute the main source of knowledge of the tumour characteristics, it seems worthwhile to publish our case, both for what it illustrates and for the areas of ignorance it exposes. Case reportA 41-year-old male was admitted for investigation following the diagnosis of an intraocular tumour in his left eye. The initial symptoms had appeared six months before admission, when he complained of metamorphopsia and disturbances of visual acuity in his left eye.Ocular examination revealed an entirely normal right eye with 20/20 visual acuity. In his left eye the vision was 20/40, the intraocular pressure was normal, and no anterior segment abnormalities were present, Corrcspondcncc to Victor Godcl, MD,

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A transconjunctival cryotechnique for the management of retrobulbar tumours is described. Fourteen retrobulbar tumours, evaluated by CT scan, were treated by this simple and minimally invasive operation.Patient's recovery is prompt and uneventful.