Corticosteroid therapy for optic disc neovascularization secondary to chronic uveitis.

PURPOSE: To report successful corticosteroid treatment of optic disc neovascularization associated with uveitis. METHODS: Retrospective review of medical records. RESULTS: Nine patients were identified with chronic uveitis and optic disc neovascularization without clinical or angiographic evidence of retinal ischemia. Ages ranged from 14 to 37 years (median age, 27). All patients were treated with either oral and/or subtenon's corticosteroids. Partial regression of the neovascularization was observed in all patients within 2 to 6 weeks (median, 5 weeks) after initiating treatment. Eight of nine patients had complete resolution of disc neovascularization at a median of 3 months (range, 2 to 42 months) after initiation of treatment and a median follow-up of 24 months (range, 7 to 144 months). Recurrence of disc neovascularization occurred in two patients, but it regressed again after further corticosteroid therapy. CONCLUSIONS: Optic disc neovascularization may occur in patients with chronic uveitis in the absence of retinal ischemia. This neovascularization can be successfully treated with corticosteroids.

Bilateral exudative retinal detachment complicating systemic corticosteroid therapy in the presence of renal failure.

PURPOSE: We describe an elderly patient with chronic renal failure who developed bilateral exudative retinal detachments after initiation of systemic corticosteroid therapy, followed by prompt resolution of the retinal detachments after the corticosteroids were discontinued. METHODS: The medical record was examined and reviewed to document the clinical course, treatment, and therapeutic response. RESULTS: Two weeks after receiving systemic corticosteroids for deteriorating renal failure, the patient had painless bilateral visual loss. Rapid tapering of the corticosteroids concurrent with hemodialysis and subsequent improvement of renal failure resulted in resolution of the exudative retinal detachments. The right eye developed a 360-degree retinal pigment epithelial tear in the macula. CONCLUSIONS: Exudative retinal detachments may complicate systemic corticosteroid therapy.

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.

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.

Retinal changes associated with neurofibromatosis 2.

INTRODUCTION: Neurofibromatosis (NF) is now known to be more than one disease. NF2, formerly classified as central neurofibromatosis, is characterized by bilateral vestibular schwannomas, previously termed "acoustic neuromas", and is much less common than NF1. Lens opacities at an early age have been described in approximately 85% of NF2 patients. PURPOSE: To determine the frequency of retinal abnormalities in NF2 patients. METHODS: We prospectively examined 15 consecutive patients who met the diagnostic criteria of NF2. RESULTS: We observed an epiretinal membrane in the macular or paramacular area in 12 of 15 patients, and a combined pigment epithelial and retinal hamartoma in the macula of one patient who also had an epiretinal membrane in the macula of the other eye. Additionally, 11 patients had central posterior cortical, subcapsular, or peripheral cortical lens opacities. CONCLUSIONS: Children or young patients with epiretinal membranes, combined pigment epithelial retinal hamartoma, and lens opacities that are not the result of other ocular disorders should have a neurologic evaluation and a careful family history for NF2.

Systemic diseases associated with intermediate uveitis.

BACKGROUND: Intermediate uveitis is characterized by vitreal inflammation with associated inflammation of the vitreous base and peripheral anterior retina and choroid. It may be found as an isolated and idiopathic condition or in association with systemic disorders such as multiple sclerosis and sarcoidosis. OBJECTIVE: To identify the clinical features of intermediate uveitis and assess its association with systemic diseases. METHODS: Retrospective study of 83 patients presenting with intermediate uveitis between 1970 and 1991. RESULTS: Evidence of systemic disorders was found in 26 of 83 patients (31.3%). Of these 26 patients, 10 had presumed sarcoidosis, 6 had multiple sclerosis, 2 had isolated optic neuritis, 2 had inflammatory bowel disease, 4 had isolated thyroid abnormalities, and 2 had histories suggestive of Epstein-Barr virus infection. Associated ocular findings included cystoid macular edema, peripheral retinal perivascular sheathing, cataracts, posterior vitreous detachment, fine keratic precipitates, preretinal macular fibrosis, retinal tears, retinal detachment, and optic disc edema. CONCLUSIONS: Patients with intermediate uveitis may have associated systemic diseases and should have careful follow-up with regular systemic evaluation.

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 Crohn's 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.

Symptomatic retinoschisis-detachment involving the macula.

We treated three patients (four eyes) in whom posteriorly situated retinoschisis-detachments became symptomatic because of elevation of a limited area of full-thickness retina at the macula adjacent to these lesions. Laser photocoagulation alone was successful in achieving long-term macular reattachment in one eye but failed in both eyes of a bilaterally affected patient. In this patient, retinal cryopexy, external drainage of subretinal and retinoschisis cavity fluid, and intravitreal air injection attained long-term macular reattachment and retinoschisis cavity collapse in both eyes. In the third patient, cryopexy, drainage of subretinal and retinoschisis cavity fluid, and scleral buckling failed to reattach the retina. Subsequent laser photocoagulation induced reabsorption of subretinal fluid but without retinoschisis cavity collapse. Alternative management strategies for these unusual cases include retinal cryopexy alone and vitrectomy techniques.

Acute multifocal inner retinitis.

Two patients developed acute changes in vision two to four weeks after a febrile illness. On ophthalmic examination, each patient had bilateral vitreitis without anterior segment inflammation and multiple, bilateral, round, yellow-white inner retinal lesions that were located in the posterior pole and midperiphery. Laboratory tests did not contribute to a diagnosis. Symptomatic visual loss was caused by neuroretinitis and serous retinal detachment in one patient and by an occluded branch retinal artery in the other. The multifocal retinal lesions resolved gradually without treatment over several months with minimal or no residual retinal changes. Acute multifocal inner retinal lesions may be associated with a preceding nonspecific viral illness and may cause a sudden change in vision if associated with neuroretinitis, serous retinal detachment, or retinal vessel occlusion. We have termed this constellation of ophthalmic findings acute multifocal inner retinitis.

The management of retinal detachment complicating degenerative retinoschisis.

We repaired six retinal detachments complicating degenerative retinoschisis by using simultaneous external subretinal fluid drainage and intraocular gas injection without a scleral buckle or vitrectomy. The outer wall breaks were 30 to 135 degrees in size, and in three cases, extended close to the arcade vessels. We achieved retinal reattachment and collapse of the schisis cavity at surgery in all six cases. In one case, the retina redetached postoperatively, but it was repaired with a scleral buckle and gas injection. This technique simplified the management of retinal detachments complicating degenerative retinoschisis, particularly those with large or posterior outer-layer breaks.

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.

Treatment of retinal detachments with multiple breaks by pneumatic retinopexy.

Pneumatic retinopexy is a recent innovation in the treatment of uncomplicated retinal detachments due to a superior retinal break extending for 30 degrees or less. We describe four patients with retinal detachments involving multiple breaks who were successfully treated by a modification of this technique. One case involved a calculation of the size of the gas bubble required to achieve tamponade of both breaks simultaneously. The gas was then administered in two sequential injections, as the volume was too large to be given in a single dose. In the remaining three cases, the gas bubble was rotated from the position of tamponade for one break into a position where tamponade of the second break or groups of breaks was achieved.

Acute bilateral posterior angiopathy with influenza A viral infection.

We studied an unusual case of an acute bilateral posterior angiopathy with a secondary maculopathy and papillitis that resulted in a severe reduction of visual acuity which resolved spontaneously within several days. The clinical appearance and subsequent viral titers suggested influenza A as a specific cause.

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.

Macular edema secondary to occlusion 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.

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.

Ultrasonographic mapping of vitreoretinal abnormalities.

We conducted a prospective study of 50 consecutive eyes to determine the accuracy of ultrasonographic mapping of vitreoretinal abnormalities. The 50 eyes, all of which had opaque media, underwent A- and B-scan ultrasonography before undergoing vitrectomy. The location and differentiation of any abnormalities were mapped on a fundus drawing sheet. At the time of vitrectomy, the surgeon independently examined the fundus and recorded his findings on a second drawing sheet. When we compared the two sets of drawings, we found that ultrasonography correctly predicted the position of the retina in 45 of 50 eyes and the presence or absence of macular detachment in 48 of 50 eyes. These two findings are particularly important to the surgeon. Ultrasonography also correctly predicted the presence or absence of posterior vitreous detachment in all 50 eyes, correctly described the exact extents of the retinal detachments in 44 of 50 eyes, and located points of vitreoretinal adhesion in 39 of 50 eyes.

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.