Central retinal vein occlusion in young adults (papillophlebitis).

We performed a retrospective study of 103 cases of central retinal vein occlusion (CRVO) in young, nondiabetic adults that were followed for at least six months. Of these patients, 64% were men and 36% were women. While visual acuity was usually good, 33 eyes (32%) had a final visual acuity of 20/200 or worse, including 6 eyes (6%) with a final visual acuity of no light perception. Ocular complications included chronic cystoid macular edema, macular pigmentary changes (37%), sheathing of retinal vessels (22%), venous collaterals of the disc (33%), macular hole formation (1%), neovascularization of the disc (1%), retina (1%), and iris (19%), neovascular glaucoma (8%), and vitreous hemorrhage (7%).

Ophthalmologic complications in hemoglobinopathies.

The various forms of sickle cell disease share the common feature of an abnormal globin chain that, under certain conditions such as hypoxia, results in the sickling of red blood cells and obstruction of blood vessels. The ophthalmic manifestations of sickle cell disease are present in various segments of the eye, which include the conjunctiva, iris, retina, and optic nerve. Patients with SC disease and SB thalassemia tend to have more severe ophthalmic complications than their counterparts with the more severe systemic form of sickle cell disease--SS disease. It is important for patients with sickle cell disease to have periodic examinations by ophthalmologists, because the early identification of peripheral retinal disease can be adequately treated, with preservation of vision. Without treatment, peripheral retinal changes can lead to retinal detachments and vitreous hemorrhages, which account for the majority of visual impairment in patients with sickle cell disease.

Bilateral optic disc neovascularization in association with retinitis pigmentosa.

Bilateral neovascularization of the optic disc developed in a 16-year-old Hispanic girl with retinitis pigmentosa. The neovascularization regressed in both eyes over 1 year without treatment. Ocular and systemic evaluation failed to reveal a recognized cause for the neovascularization. To our knowledge this is only the third case reported of this unusual association.

Pathologic observations made by retinal biopsy.

The authors report four cases in which retinal biopsy findings yielded unexpected or previously unreported diagnoses in patients with inflammatory retinitis. The tissue diagnoses included Wegener's retinal vasculitis in an immunosuppressed patient with a clinical diagnosis of cytomegalovirus retinitis, a novel viral form in the retina of a patient with cytomegalovirus retinitis, a case of acute retinal necrosis due to cytomegalovirus infection in an immunologically normal adult, and a case of ganciclovir-resistant herpes family viral retinitis. These cases illustrate the use of retinal biopsy in obtaining tissue for diagnosis and guiding treatment in selected cases of retinitis.

Atypical presumed CMV retinitis.

Cytomegalovirus (CMV) retinitis is a common and one of the most serious ophthalmic manifestations of the acquired immunodeficiency syndrome (AIDS). Early recognition is essential in the proper management of this necrotizing and progressive infection. We discuss an unusual presentation of CMV retinitis which appeared initially only in the foveal region.

Demonstration of herpes group virus in acute retinal necrosis syndrome.

Tissue for pathologic examination was obtained from three cases of acute retinal necrosis syndrome. Virus particles belonging to the herpesvirus family were demonstrated in retinal biopsies from two patients, one of whom was immunosuppressed. Despite removal of large biopsy specimens, the retina has remained attached for 20 months postoperatively in one case and for three months in the other. In a third patient with acquired immune deficiency syndrome, the clinical course and postmortem immunopathology were suggestive of a herpes simplex virus infection, initially affecting the retina and subsequently the optic nerves, chiasm, tracts, and central nervous system. These cases illustrate that the virus associated with the acute retinal necrosis syndrome is easily demonstrable using vitrectomy and endoretinal biopsy in the acute phase of the disease, but may be difficult to demonstrate in chronically detached atrophic retinas.

A prospective study of the ophthalmologic findings in the acquired immune deficiency syndrome.

A prospective evaluation of ophthalmologic findings in 26 patients (25 men and one woman) with the acquired immune deficiency syndrome disclosed that 19 patients had significant ocular abnormalities. These included isolated retinal hemorrhages, cotton-wool spots, cytomegalovirus retinitis, acute retinal necrosis, cranial nerve palsies, and orbital Kaposi's sarcoma. Hemorrhages and cotton-wool spots appeared and disappeared spontaneously. Cytomegalovirus retinitis and acute retinal necrosis were progressive and destructive. The fundus findings did not correlate with the patient's general clinical status.

Acute retinal necrosis.

Acute retinal necrosis represents a distinct, recently recognized clinical syndrome. Four patients who presented with rapid visual loss associated with uveitis and coalescent areas of retinal necrosis, followed by development of retinal detachments were examined. This paper emphasizes the following: (1) unilateral involvement does occur, (2) the distribution of lesions can be peripheral or central, and (3) early lesions are not associated with retinal vascular abnormalities either clinically or angiographically. Signs and symptoms are suggestive of an infectious process, possibly viral.

Simultaneous bilateral retinal arterial occlusions treated by exchange transfusions.

Bilateral retinal vascular occlusions occurred simultaneously in a 25-year-old man. They were treated by exchange transfusions. After the transfusions, the patency of the previously occluded vessels was reestablished. Subsequently, the patient recovered good central vision. It is interesting to speculate how exchange transfusions, used during the past few years for treatment of life-threatening situations, may be useful for the treatment of retinal arterial occlusions in patients with sickle cell disease.

Central serous retinopathy. A generalized disease of the pigment epithelium.

Among cases of central serous retinopathy, patients have been isolated with retinal pigment epithelial (RPE) and sensory retinal detachments. An analysis of 104 cases occurring during the period from 1972 to 1977 indicates that this disorder is frequently bilateral and that it is associated with different disturbances of the epithelial cell layer, i.e. foci of leakage, transmission defects and serous detachments. Based upon the angiographic studies in 76 cases, we propose the following classification for cental serous retinopathy: I. Unilateral Retinopathy. (A) Uniocular involvement: (1) with focal leakage but no other RPE changes; (2) with focal leakage and other RPE changes; (3) with focal leakage, RPE changes and an exudative retinal detachment. (B) Binocular involvement: (1) mild; (2) moderate; (3) severe (with an exudative retinal detachment in the affected eye). II. Bilateral Retinopathy. (A) Mild; (B) moderate, (C) severe (in association with bilateral exudative retinal detachments). This classification emphasizes the fact that central serous retinopathy, whatever its etiology, represents a generalized affectation of the pigment epithelium and should be construed as a potentially serious disorder requiring thorough evaluation and follow-up care.

Optic nerve damage following argon laser photocoagulation in a human eye.

Argon laser photocoagulation of diabetic neovascular formations overlying the optic disc risks injury to the nerve fibers. Isolated reports of postoperative field defects have appeared but extensive pathologic studies of the effects on nerve fibers are lacking. In an eye with a malignant melanoma nasally, laser photocoagulation was directed at the superior temporal artery on the disc. Applications were made repeatedly until spasm was produced at two sites. The following day, the eye was enucleated. Histopathologic examination revealed loss of endothelial cells and absence of nuclei in the media of the treated artery, and coagulative necrosis of nerve fibers around the vessel. Extensive damage to the peripapillary outer segments and retinal pigment epithelium also was demonstrated.

Macroaneurysms of the retinal arteries.

Six patients with spontaneous retinal and/or vitreous hemorrhages were found, on evaluation by fluorescein angiography, to have macroaneurysms involving the major retinal arteries. All showed evidence of other vascular disease. With absorption of the hemorrhages, five patients experienced appreciable improvement in vision. The aneurysms appeared to diminish in size as a result of fibrous changes within the arterial walls. In one patient, however, a second vitreous hemorrhage occurred several months later. The possibility of repeated hemorrhage suggests that more consideration should be given to treatment of these aneurysms.

The treatment of acute horseshoe retinal tears by transconjunctival cryopexy.

During the past 6 years, 59 consecutive eyes with operculated retinal tears have been treated successfully by transconjunctival cryopexy. This type of treatment risks fewer complications than scleral buckling. Its success implies that vitreous traction upon the retina may be short lived in most eyes or that its intensity diminishes considerably after the development of a retinal tear.