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Purpose: To describe and evaluate the cause of a clinical entity characterized by bilateral intraocular inflammation, multiple arterial ectasias including beading, macroaneurysms, comma-like ectasias and kinking, with vasculitis, staining of the optic disk and multiple peripheral round punched-out hypopigmented chorioretinal scars in elderly patients. The formation and the course of the arterial ectasias is analyzed., Methods: Seven patients with this syndrome were evaluated by clinical examination, fluorescein angiography, and systemic investigations., Results: Three of the seven patients had a biopsy characteristic of sarcoidosis, two others showed positive bronchoalveolar lavage, as well as other analyses and tests suggesting sarcoidosis, and two showed other findings suggestive of sarcoidosis. The patients were all over 60 years of age and had arterial hypertension. In two patients, an arterial ectasia developed at the site of previous focal inflammation. The macroaneurysms either remained unchanged, became comma-like ectasias, arterial kinks, or completely vanished., Conclusion: Elderly patients with multiple arterial ectasias, uveitis, disk staining, and peripheral chorioretinitis should be thoroughly investigated for sarcoidosis. We suggest that sarcoidosis may cause some degree of arteritis, which may weaken the arterial wall, with resulting ectasia. Arterial hypertension may play a role in the formation of the ectasias by increasing the pressure on the arterial wall weakened by inflammation.

Oral corticosteroids remain the main therapeutical choice in patients with uveitis not responding to topical treatment, however their chronic use can be very toxic, especially for bones (osteoporosis or growth retardation). Immunosuppresive agents are used as corticosteroid sparing agents and/or as agents able to control refractory uveitis in sight threatening uveitis. Cyclosporin A is very efficacious but is nephrotoxic, in particular in old people. Methotrexate is well tolerated in young people, is not carcinogenic but not very active. Azathioprine is well tolerated but it's carcinogenic effect and its delayed action are helpful in chronic uveitis of old women. Cyclophosphamide and intravenous steroids are helpful for emergencies. Chlorambucil is toxic and carcinogenic but might lead to an increased rate of remission and might be useful as a short treatment. In any case, a careful and regular follow-up in collaboration with a competent internist is mandatory.

Increased iris vessel permeability observed in diabetics has also been reported to occur in diabetic animals and galactose-fed rats. The potential role of aldose reductase in the induction of iris vessel changes has been investigated in rats fed a 50% galactose diet with/without the aldose reductase inhibitors AL 1576, sorbinil or ponalrestat for 7 to 18 months. Compared to normal control rats, long-term galactose-fed rats display a breakdown of the blood-aqueous barrier due to iris vessel changes that include focal straightening, dilation, constriction, increased permeability, ischemia and new vessel proliferation. The onset and progression of these iridal vessel changes were prevented by the aldose reductase inhibitors AL 1576 and sorbinil, and reduced by Ponalrestat. Computerized analyses of lumen areas of iris vessels indicated an 18-fold decrease in the vascular area near the pupillary boarder in untreated galactose-fed rats compared with age-matched controls and galactose-fed rats treated with aldose reductase inhibitors. These observations linking iris vessel changes with galactose-feeding, coupled with the fact that aldose reductase inhibitors also prevent these changes, strongly suggest a link between the sorbitol pathway and the appearance and progression of iris vessel changes., (Copyright 1999 Academic Press.)

Blood-retinal barrier (BRB) and blood-aqueous barrier (BAB) permeability were assessed by various methods to clarify conflicting reports on whether blood-retinal barrier permeability changes occur in diabetic and galactose-fed rats and to assess the potential role of aldose reductase in this process. Different molecular weight probes were utilized in rats fed for 7-11 months either a normal diet or a diet containing 50% galactose with/without the aldose reductase inhibitor AI1576 or Ponalrestat. BRB and BAB were assessed through radiolabelled sucrose permeability studies in eyes where the anterior segment was frozen during dissection compared to eyes where the anterior segments were not frozen and by quantitative autoradiography. In addition, histological studies using Evans Blue dye, microperoxidase and horseradish peroxidase were conducted. In untreated galactose-fed rats a 4-fold increase in the mean permeability surface area product (PA) to sucrose at the BRB was observed when the aqueous humor was not frozen during retinal dissection. However, no increase in the mean PA to sucrose was observed when the aqueous humor of similar eyes was frozen during dissection. Similarly, no retinal vessel permeability increase was observed by either quantitative autoradiography of [3H]-sucrose after 15 minutes of circulation or histological studies with microperoxidase, horseradish peroxidase or Evans Blue dye. Examination of the BAB revealed an increase in the permeability of iris vessels but not the ciliary body in galactose-fed rats which was reduced by treatment with the aldose reductase inhibitor AI1576. These data indicate that while BRB permeability is not increased in galactose-fed rats, BRB permeability measurements with isotopes are subject to possible contamination from the aqueous humor in untreated galactose-fed rats, which can result in false observations of increased BRB permeability.

A 77 year old man presented with a decreased vision, quite anterior segments, vitreous haze (++) (cells and flare) OD > OS, snow banks and snow balls at the inferior pars plana, suggesting an intermediate uveitis. Two subTenon injections of steroids were performed on the right eye, with improvement of his right visual acuity from 5 to 10/10. Cells and snow balls disappeared but the vitreous became more fluffy and more organized. Investigations did not demonstrate any systemic disease. The vitreous haze progressed bilaterally and failed to respond to systemic steroids. A year later, visual acuity was limited to hand movements (OD) and remained at 9/10 (OS). The vitreous haze + (OD) and ++ (OS) was extremely fluffy and moderately organized without inflammatory cells. Vitrectomy was performed in both eyes. Non inflammatory fibro-cellular membranes in a dense collagenous network with amorphous precipitates were observed in electron microscopy. One year later, he developed a systemic B-cell lymphoma. This unusual case illustrates the possible evolution of intermediate uveitis in a secondary PVR-like progressive non inflammatory vitreous haze with a particular increase of the amount of collagen fibrils and amorphous precipitates responsible for the fluffy aspect of the vitreous failing to respond to steroids.

We report a case of atypical bilateral endogenous mycotic endophthalmitis in an infant. A diagnostic vitrectomy was done on the left eye. Electron microscope examination of the vitreous demonstrated fungal mycelia. Intravenous treatment with amphotericin B seemed effective for treating the infection of the right eye, but a vitrectomy was necessary to prevent deprivation amblyopia and retinal traction, secondary to persistence of a dense vitreous membrane.

Cyclosporine (cyclosporine A) at 7 to 16 mg/kg/day was administered for eight to 18 months to four patients with Behçet's disease with bilateral panuveitis resistant to the combination of prednisone, colchicine, and chlorambucil. Visual acuity was maintained or improved in all eyes. Color vision also improved significantly. A dramatic and significant decrease of the chronic vitreous haze occurred within the first two weeks of therapy and persisted until the end of the study. Retinal inflammation, including hemorrhages, exudates, edema, and fluorangiographic abnormalities, decreased progressively in all eyes. The severity of the anterior and posterior acute attacks also decreased significantly. This study suggests that cyclosporine is beneficial for the treatment of refractory and severe Behçet's disease, but its side effects require careful follow-up.

Sequential fluorescein angiography, using fluorescein-labeled dextran molecules of several different sizes, was carried out in monkeys with intraocular inflammation induced with interphotoreceptor retinoid-binding protein. The vascular leakage seen with the dextrans was compared with that seen with standard fluorescein sodium angiography. The angiograms demonstrated that different-sized leaks appear in the retinal vessels in adjacent areas during the course of the inflammation. Most retinal vessels leaked only fluorescein sodium and no dextran of any size, suggesting that it is the unbound fluorescein that leaks out of these vessels and not fluorescein bound to plasma albumin. It was not possible to tell by clinical examination which areas would leak the larger-molecular weight tracers. Ultrastructural studies of the veins leaking the dextrans revealed areas of abnormal endothelial tight junctions, whereas the tight junctions were normal in areas where leakage occurred with fluorescein alone.

Nerve water content and the permeability-surface area product (PA) to [3H]-or [14C]sucrose at the blood-nerve barrier were determined in unanesthetized control rats fed a normal diet and in rats fed galactose with or without an aldose reductase inhibitor (Statil or AL 1576) or a thromboxane synthetase inhibitor (CGS 12970). Nerve water content was determined by taking the difference between dry and wet weights of whole tibial nerves. PA was determined by an intravenous bolus injection of radiotracer with multiple-time-point graphic and quantitative autoradiographic methods. The mean nerve water content in galactosemic rats was 15% higher than in control rats after 7-11 mo on the diet. Statil and AL 1576 prevented nerve edema, but CGS 12970 was only partially effective in preventing an increase in nerve water content in galactose-fed rats. In galactosemic rats, the mean PA to sucrose at the blood-nerve barrier, calculated from nerve dry weight, was twofold higher than in control rats. Treatment with Statil, AL 1576, or CGS 12970 prevented increased PA. Our results suggest that nerve edema and increased blood-nerve barrier PA are secondary to polyol production and can be prevented by inhibiting aldose reductase.