Ischemic fundus lesions are a frequent complication after bone marrow transplantation. They were only observed in patients treated with total body irradiation and cyclosporin A. This combination of therapy appears to have an additive effect on the development of ocular and possibly generalized microvascular lesions.
The clinical history and the pathohistological findings of both eyes of a homosexual man with AIDS and cytomegalovirus (CMV) infection are reported. A CMV panuveitis with cytomegalic transformation of vascular endothelium was present in the posterior and anterior uvea as well as a typical CMV retinitis. In addition, a CMV infection of smooth muscle cells in the iris and ciliary body as well as of endothelial cells of the cornea and the aqueous drainage system were found for the first time.
SUMMARY Three patients with acute lymphatic leukaemia developed visual impairment due to occlusion of small retinal vessels with multiple cotton wool spots after treatment which included whole body and skull irradiation followed by bone marrow transplantation and cyclosporin A. Withdrawal of cyclosporin A and treatment with corticosteroids was followed by recovery of visual acuity. This retinopathy and the retinal changes seen in the immunodeficiency syndrome are thought to be closely related. The possible role of cyclosporin A is discussed, though cotton wool spots and retinal haemorrhages have never been described in renal transplant patients during treatment with this drug. Withdrawal of cyclosporin A, which is highly effective in preventing graftversus-host disease, can be fatal. Irradiation of the skull prior to bone marrow transplantation and intrathecal administration of methotrexate may be the most important factors causing the retinal ischaemic signs described here. The inclusion of an ophthalmologist in the team monitoring. transplant patients would lead to increased documentation and a better understanding of this disease.We have recently seen three patients who developed severe visual loss two to three months after bone marrow transplantation. The ophthalmoscopic substrate of this visual loss was a disseminated multifocal ischaemic retinopathy with extensive cotton wool patches and retinal haemorrhages. All three patients had received irradiation to the head in addition to whole body irradiation and had been treated with cyclosporin A to prevent graft-versus-host disease.The heavy maintenance immunosuppression which patients with bone marrow grafts receive suggests a possible connection between this retinopathy and that observed in the 'immunodeficiency syndrome.' However, to the best of our knowledge multifocal ischaemia of the retina with multiple cotton wool spots has not yet been reported as a complication of bone marrow transplantation.
The eyes of a patient with canthaxanthin retinopathy were obtained at autopsy and examined by light and electron microscopy. Various tissues of one eye were also studied by physicochemical methods. Morphologically, there were red, birefringent, lipid-soluble crystals in the inner layers of the entire retina. They were particularly large and numerous perifoveally, where they were also clinically visible, but they also occurred frequently in a ring-shaped form peripherally and, less frequently, equatorially. The crystals were located in a spongy degeneration of the inner neuropil, where atrophy of the inner parts of the Müller cells was noticed. The compound isolated from the retina was identical with synthetic canthaxanthin according to mass and proton-resonance spectroscopy. Quantitatively, the retina contained up to 42 micrograms canthaxanthin per gram of tissue besides a minor amount of other carotenoids. Of the other tissues of the eye, only the ciliary body contained measurable concentrations of canthaxanthin. From the great number and size of the crystals, on the one hand, and the relatively small amount of isolated canthaxanthin on the other, it was concluded that the crystals presumably represent a canthaxanthin-lipoprotein complex rather than pure canthaxanthin alone. Examination showed that clinically, only the central portion of the canthaxanthin thesaurismosis, where crystals are packed most densely, can be seen.
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