Morton F. Goldberg

Ocular manifestations of sickle hemoglobinopathies.

The ocular manifestations of sickle cell hemoglobinopathies, while often not severe enough to affect vision, may develop in proliferative stages, resulting in arteriolar-venular anastomoses, neovascular proliferations, vitreous hemorrhage and retinal detachment. The authors review the historical, biochemical, and geographic aspects of the disease, and provide detailed descriptions of findings in theconjunctiva, uvea and fundus, with particular emphasis on the retina. Pathogenesis, histopathologic and clinical appearance, and modes of treatment are discussed.

Early Detection of Retinal Involvement In Diabetes By Vitreous Fluorophotometry

Vitreous fluorophotometry is a new quantitative method for evaluation of the blood-retinal barrier (BRB). The application of this method to a series of diabetic patients with apparently normal fundi revealed the presence of a significant breakdown of the BRB in the early stages of retinal involvement in diabetes. This alteration of the BRB appears to be the earliest clinically detectable change to occur in the retina in diabetes. Higher vitreous fluorophotometry values, indicating a more marked breakdown of the BRB, occurred in patients who were under worse metabolic control. Higher vitreous fluorophotometry values were also associated with the development of visible retinal lesions.

The Diagnosis and Treatment of Secondary Glaucoma After Hyphema in Sickle Cell Patients

Four patients with sickle cell hemoglobinopathies (one sickle cell hemoglobin C disease (SC); three sickle cell trait (AS)) and hyphemas had a higher percentage of erythrocytes sickled in their anterior chambers than in their circulating venous blood. Intraocular pressure (IOP) was severely increased, despite relatively small amounts of intracameral blood. Systemic hypotensive agents were not always successful in reducing IOP, and in patients with sickle cell hemoglobinopathy, are probably contraindicated in high or repeated dose regimens. Moderate increase of IOP in sickle cell hemoglobinopathy patients may produced rapid deterioration of visual function, because of a greater than usual effect on vascular perfusion in the central retinal artery and optic nerve. Early anterior chamber paracentesis may be the best treatment for this type of hyphema-induced secondary glaucoma.

Choroidal infarction, anterior ischemic optic neuropathy, and central retinal artery occlusion from polyarteritis nodosa.

Purpose Ocular ischemia from polyarteritis nodosa (PAN) is rare. The authors present a case of multifocal ocular infarction from PAN. Methods and Results A 70-year-old woman developed hand and foot numbness followed by intermittent blurred vision and binocular horizontal diplopia. Two weeks later, she suddenly lost vision in the right eye from a central retinal artery occlusion and then developed a left anterior ischemic optic neuropathy and bilateral triangular choroidal abnormalities consistent with infarction. Her erythrocyte sedimentation rate and C-reactive protein were elevated. Although giant cell arteritis was suspected, a multiple mononeuropathy was demonstrated by electromyogram and nerve conduction velocity studies. Biopsy specimens from her sural nerve and biceps muscle showed a necrotizing vasculitis with fibrinoid necrosis, consistent with PAN. Conclusions Polyarteritis nodosa can produce ischemia of a variety of ocular structures, including the retina, choroid, and optic nerve. In our patient, all three structures were affected. To our knowledge, this is the first reported case of the triangular sign of Amalric in PAN.

Spontaneous Regression (Autoinfarction) of Proliferative Sickle Retinopathy

Of 45 patients with proliferative sickle retinopathy in stages III, IV, and V, nine patients (eight with hemoglobin SC disease, one with sickle cell thalassemia) showed spontaneous regression (autoinfarction) of retinal sea fans. One mechanism involved in autoinfarction of neovascular tissue is progressive, centripetal retraction of the anterior vascular arcade of the peripheral retina. In addition, vitreous traction on feeder vessels may result in sluggish blood flow and occlusion of these vessels, or may tear the sea fan completely away from its feeder vessels. In view of the many incidences of vitreous hemorrhages that occur in patients with proliferative retinopathy, however, we recommend treatment of neovascularization rather than prolonged observation.

Fundus Photocoagulation with the Argon and Krypton Lasers: A Comparative Study

Photocoagulation of the monkey fundus with an argon-krypton laser photocoagulator was performed. Lesions produced by argon and krypton wavelengths were compared at various power settings, spot sizes, and exposure durations. At low energy levels argon laser lesions appeared clinically stronger than those produced by the krypton mode. Lesions in the foveal area produced with the argon laser demonstrated more extensive microscopic damage to the inner retinal layers than did those produced with the krypton laser. Histologically, both modalities affected the outer layers of the retina. However, the krypton wavelength affected more of the choriocapillaries and the larger choroidal vessels than the argon wavelengths. Conversely, argon laser coagulation of the retinal vessels damaged the perivascular tissue in the nerve fiber layer more than the krypton laser did. Rupture of Bruchs membrane and choroidal hemorrhage occurred frequently with the krypton laser at low energy levels when a small spot size and a short coagulation time were used. Both complications were less likely to occur with argon burns at similar dosage parameters. By choosing larger spot sizes and longer coagulation times for the krypton laser, these complications were prevented.

Therapy of Scleritis with Cytotoxic Agents

Four patients with severe anterior scleritis that had not responded to conventional therapy were treated with cytotoxic immunosuppressive alkylating agents alone (one case) or in combination with prednisone (three cases). Two of the patients had Wegeners granulomatosis, one pyoderma gangrenosum, and one rheumatoid vasculitis. All patients responded well to this therapy with a decrease in inflammation and a healing of the sclera. Other systemic manifestations of the underlying diseases often responded well also.

Scatter Retinal Photocoagulation for Proliferative Sickle Cell Retinopathy

We treated sea-fan neovascularization in 19 patients (21 eyes) who had proliferative sickle cell retinopathy with localized scatter photocoagulation. We placed burns of light to moderate intensity adjacent to 45 sea fans in a scatter fashion. In general, treatment extended from 1.5 mm posterior to 1.5 mm anterior to the sea fan and one clock hour to each side of each lesion. Flat sea fans responded dramatically to treatment, with complete regression in 24 of 28 lesions. Elevated sea fans (especially large ones) responded less rapidly, with complete regression in only four of 17 lesions. Localized scatter photocoagulation is valuable in treating patients with early proliferative sickle cell retinopathy.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy Associated With A Systemic Necrotizing Vasculitis

Purpose The authors present a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with a systemic necrotizing vasculitis with mixed features of Wegener granulomatosis and polyarteritis nodosa (PAN). Methods Case report. Results A 29-year-old woman developed a severe nosebleed, followed by a low-grade fever, night sweats, and a productive cough. One month later, she began experiencing high fevers, headache, myalgia, neck stiffness, and abdominal pain as well as bilateral blurred vision from APMPPE. Systemic evaluation revealed nasal ulcerations, bilateral pleural effusions, and a bilateral maxillary and ethmoid sinusitis, consistent with Wegener granulomatosis. However, ANCA testing was negative, and a renal and mesenteric angiogram showed aneurysmal dilatations suggestive of PAN. Her ocular and systemic symptoms markedly improved with systemic corticosteroids. Conclusions The cause of APMPPE is unknown. This case of APMPPE associated with systemic necrotizing vasculitis provides support for the choroid as being primarily involved by a diffuse vasculitic process that interrupts choroidal perfusion and causes the characteristic fundus findings in APMPPE.

Choroidoretinal Vascular Anastomoses After Blunt Trauma to the Eye

Six weeks after blunt ocular trauma in a 38-year-old white man, choroidoretinal vascular anastomoses occurred at the site of choroidal and retinal rupture. Neovascularization was not observed. Anastomoses of this type have been documented in other cicatrizing and granulomatous disease processes.