Froncie A. Gutman

A Twin Study of Age-related Macular Degeneration

PURPOSE To determine the importance of genetic factors in age-related macular degeneration by using a twin study to compare the concordance of age-related macular degeneration in monozygotic and dizygotic twin pairs. METHODS We prospectively examined 134 consecutive twin pairs and two triplet sets for age-related macular degeneration. The zygosity was determined by genetic laboratory tests. RESULTS The concordance of age-related macular degeneration was 100% (25 of 25) in monozygotic and 42% (five of 12) in dizygotic twin pairs. The other twins or triplets had no macular changes of age-related macular degeneration. CONCLUSIONS The statistically significant higher concordance of age-related macular degeneration in monozygotic than in dizygotic twin pairs and the clinical heterogeneity of age-related macular degeneration strongly suggest the importance of genetic and nongenetic factors, respectively, in age-related macular degeneration.

Multiple Recurrent Serosanguineous Retinal Pigment Epithelial Detachments in Black Women

Three middle-aged black women suffered recurrent, multiple, bilateral, asymmetric, serosanguineous retinal pigment epithelial detachments. These involved the posterior fundus with resolution and recurrences producing subretinal hemorrhages, vitreous hemorrhages, retinal pigment epithelial pigmentary mottling, and chorioretinal scars. Fluorescein angiography showed evidence of choroidal neovascular membranes, choroidal serosanguineous leaks, or both. Ocular inflammation was not evident. Systemic laboratory tests were noncontributory. The clinical pattern was not characteristic of any other entity producing serosanguineous retinal pigment epithelial detachments. The origin remains to be defined.

The natural course of central retinal vein occlusion.

Thirty-five patients with central retinal vein occlusion had an extensive medical and laboratory evaluation to identify possible etiologic factors. We describe the natural course of the two forms of retinal venous occlusive disease, hemorrhagic retinopathy and venous stasis retinopathy, by observing a group of 25 untreated patients from one to eight years. Systemic vascular disease was most commonly associated with retinal venous occlusive disease. The visual prognosis is favorable in venous stasis retinopathy and extremely poor in hemorrhagic retinopathy. However, two of ten patients with venous stasis retinopathy developed hemorrhagic retinopathy with poor visual outcome.

Comparison of Pneumatic Retinopexy with Alternative Surgical Techniques

To determine the efficacy of pneumatic retinopexy, the authors conducted a retrospective study of 56 cases of uncomplicated retinal detachments (RDs) treated with this technique (follow-up, 6-18 months). These were compared with 28 similar cases treated with a Lincoff balloon and 78 similar cases treated with a scleral buckle. The long-term success rates for these three groups were 71, 64, and 96%, with the incidence of new breaks being 20, 18, and 1.3% respectively. In aphakic or pseudophakic patients with an absent or ruptured posterior capsule, the success rate for pneumatic retinopexy was only 43%. In phakic and aphakic patients with an intact posterior capsule, the success rate for pneumatic retinopexy improved to 81%. These data suggest that this technique is less effective in aphakic or pseudophakic patients with an absent or ruptured posterior capsule and that careful follow-up to detect new breaks is necessary in all cases. A prospective randomized study is needed to verify these data.

Evaluation of a Patient with Central Retinal Vein Occlusion

Patients suffering a central retinal vein occlusion should be evaluated for conditions that promote and/or contribute to the formation of a thrombosis in the central retinal vein. These conditions include alterations of blood flow, altered viscosity of the blood, abnormalities of coagulation, and abnormalities of vessel walls. Patients with either a partial or complete central retinal vein occlusion have similarities in the age of onset, associated systemic findings, and laboratory data. At the time of onset, 90% of patients are older than 50 years. Seventy-four percent of patients will have symptoms or signs of associated systemic vascular disease (ie, hypertension, arteriosclerotic heart disease, and diabetes mellitus.) The most common laboratory findings include elevated blood lipids, abnormalities of glucose tolerance testing, and abnormalities of protein chemistries. Evaluation should include both a complete ocular and medical examination with appropriate laboratory tests and special studies. Assessment of central retinal venous pressure may be helpful in identifying those patients who have a severe, complete thrombosis and are at significant risk to develop anterior segment neovascularization.

Posterior segment manifestations of inflammatory bowel disease.

Thirteen patients with inflammatory bowel disease and posterior segment disease were subject to a retrospective review. Eight patients had Crohns disease and five had ulcerative colitis. In six patients, the inflammatory bowel disease was active when ocular inflammation occurred. Patients had one or more posterior segment findings that included serous retinal detachment (8), choroidal infiltrates (6), retrobulbar neuritis (1), papillitis (1), retinal pigment epithelium disturbance (1), and choroidal folds (1). Posterior segment disease responded to systemic and periocular corticosteroids in 9 of 13 cases. Four patients whose disease relapsed after corticosteroid therapy was suspended responded to bowel resection. Ophthalmologists should be aware of the wide spectrum of posterior segment abnormalities associated with inflammatory bowel disease that may require and respond to anti-inflammatory agents.

Macular edema secondary to occulusion of the retinal veins

Occlusion of the central and branch retinal veins causes macular edema by provoking increased venous pressure which is transmitted to the perifoveal capillaries. Damage to the perifoveal capillary endothelium results in leakage with macular edema. The severity of the retinal findings (i.e., macular hemorrhages, macular edema, etc.) is determined by the location, completeness, duration, and evolution of the obstruction (i.e., recanalization of a thrombus). Spontaneous remission of macular edema secondary to either a partial central or branch occlusion carries a relative good visual prognosis. Chronic macular edema secondary to occlusion of either central or branch veins is associated with a relatively poor visual prognosis. In selected cases, paramacular photocoagulation can reverse chronic macular edema and result in visual improvement.

PARTIAL OCCLUSION OF THE CENTRAL RETINAL VEIN

Twenty-three untreated patients (14 men and nine women ranging in age from 31 to 74 years) with partial occlusion of the central retinal vein were followed up prospectively for a minimum of 12 months. Seventeen had systemic vascular disease. At the conclusion of the study, 12 patients had visual acuities of 20/40 or better and eight had visual acuities of 20/100 or worse. Eleven patients had complete resolution of the retinopathy and had final visual acuities of 20/30 or better. Five eyes progressed to complete occlusion with final visual acuities of 20/300 or less. Seven eyes had chronic macular edema and visual acuities ranging from 20/40 to 20/200. One eye with background diabetic retinopathy developed optic disk neovascularization. None of the patients developed neovascular glaucoma.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy After Acute Group A Streptococcal Infection

PURPOSE We studied a case of acute posterior multifocal placoid pigment epitheliopathy in a 40-year-old man who had had an acute febrile illness. METHODS The medical record was reviewed for clinical manifestations, course of disease, and laboratory findings, including results of fluorescein and indocyanine green angiography. RESULTS The patient had the typical clinical course of acute posterior multifocal placoid pigment epitheliopathy with spontaneous resolution of posterior pole lesions and improvement in visual acuity from 20/60 to 20/20. The laboratory evaluation was remarkable for a rise in the anti-DNAse B antibody titer between initial and convalescent-phase serum samples, providing evidence of recent group A streptococcal infection. CONCLUSION Although acute posterior multifocal placoid pigment epitheliopathy is often attributed to a postviral condition, this syndrome may also develop after an acute group A streptococcal infection.

SPONTANEOUS REGRESSION OF PARAFOVEAL EXUDATES AND SEROUS RETINAL DETACHMENT IN A PATIENT WITH TUBEROUS SCLEROSIS AND RETINAL ASTROCYTOMAS

The authors describe an unusual case of a serous detachment of the fovea and decreased vision with parafoveal exudates from a retinal astrocytoma with subsequent spontaneous resolution and return of central vision in a patient with tuberous sclerosis.