Vitreous opacities in primary amyloid disease. A clinical, histochemical, and ultrastructural report

Three patients who initially presented to the ophthalmologist with a history of gradual deterioration of visual acuity showed, on biomicroscopic examination, a linear meshwork of opacities in the vitreous attached to the posterior surface of the lens in the form of pseudopodia lentis. Amyloidosis of the vitreous associated with systemic amyloid disease was diagnosed because of the characteristic nature of the opacities. This was confirmed initially in two of the cases by light and electron microscopical examination of a biopsy of the clinically normal conjunctiva, subsequently by systemic investigations, and finally, by direct histochemical and elecron microscopical examination of the vitreous which became available after vitrectomy in all three cases. It was not possible to examine other members of the families. The relevant literature on vitreous amyloidosis is reviewed in relation to our own findings and the importance of a diagnostic conjunctival biopsy is emphasized. This paper is the first British report on primary vitreous amyloidosis. The pathogenesis of vitreous amyloid opacities is discussed in the light of current concepts of amyloidosis. Since the vitreous opacities due to amyloid deposits appear to be one of the manifestations of systemic amyloid disease, surgical removal of the opacities by vitrectomy may not produce longlasting results. Moveover, in contemplating surgical treatment the ophthalmologist should bear in mind the poor prognosis of systemic amyloidosis.