From Associated Vitreoretinal and Uveitis Consultants, Indianapolis, Indiana. Reprint requests: Ramana S. Moorthy, MD. Associated Vitreoretinal and Uveitis Consultants, 8704 North Meridian Street, Indianapolis. IN 46260.

OBJECTIVE:The authors evaluated the clinical, fluorescein, and indocyanine green (ICG) angiographic characteristics of the macular variant of idiopathic polypoidal choroidal vasculopathy (IPCV).DESIGN:Observational case series.PARTICIPANTS:The records, photographs, and fluorescein and ICG angiograms of eight eyes of seven patients with IPCV lesions confined to the macula were reviewed.MAIN OUTCOME MEASURES:The visual acuity, fundus examination, fluorescein and ICG angiographic characteristics, and clinical course were compared.RESULTS:All patients demonstrated polypoidal lesions arising from macular choroidal vessels on ICG angiography. One patient had bilateral lesions. These lesions appeared hyperfluorescent in the early phases of both fluorescein and ICG angiography. Late-phase leakage was seen in cases associated with subretinal fluid or exudate. None of these patients demonstrated polypoidal lesions arising from the peripapillary choroidal circulation or peripapillary choroidal neovascularization. Three eyes with polypoidal lesions that were associated with subretinal fluid and exudates were treated with photocoagulation. Five eyes were not treated. Final visual acuity ranged from 20/20 to hand motions. Severe visual loss was associated with vitreous and subretinal hemorrhage, but this resolved without permanent severe visual loss in several cases.CONCLUSIONS:In the macular variant of IPCV, ICG and fluorescein angiography demonstrate characteristic macular polypoidal lesions without evidence of peripapillary lesions. The vascular origin of these polypoidal lesions appears to be the macular choroidal circulation. This is distinguished from classic IPCV, in which lesions appear to arise from the peripapillary choroidal circulation. Visual prognosis appears to be good, with most patients retaining visual acuity of 20/80 or better. If subretinal fluid or exudates reduce visual acuity, photocoagulation should be considered.

Uveitis has been reported in association with a variety of topical, intraocular, periocular, and systemic medications. To establish causality of adverse events by drugs, in 1981, Naranjo and associates proposed seven criteria, which are related to the frequency and documentation of the event; circumstances of occurrence, recovery, and recurrence; and coexistence of other factors or medications. Rarely does a drug meet all seven criteria. The authors review reports of drug-associated uveitis, applying the seven criteria and examining possible mechanisms. Only systemically administered biphosphonates and, perhaps, topical metipranolol meet all seven criteria. Systemic sulfonamides, rifabutin, and topical glucocorticoids fulfill at least five criteria. (Surv Ophthalmol 42:557-570, 1998.

AIMS/BACKGROUND Varicella zoster virus retinitis (VZVR) in patients with AIDS, also called progressive outer retinal necrosis (PORN), is a necrotising viral retinitis which has resulted in blindness in most patients. The purposes of this study were to investigate the clinical course and visual outcome, and to determine if the choice of a systemic antiviral therapy affected the final visual outcome in patients with VZVR and AIDS. METHODS A review of the clinical records of 20 patients with VZVR from six centres was performed. Analysis of the clinical characteristics at presentation was performed. Kruskall–Wallis non-parametric one way analysis of variance (KWAOV) of the final visual acuities of patients treated with acyclovir, ganciclovir, foscarnet, or a combination of foscarnet and ganciclovir was carried out. RESULTS Median follow up was 6 months (range 1.3–26 months). On presentation, 14 of 20 patients (70%) had bilateral disease, and 75% (15 of 20 patients) had previous or concurrent extraocular manifestations of VZV infection. Median initial and final visual acuities were 20/40 and hand movements, respectively. Of 39 eyes involved, 19 eyes (49%) were no light perception at last follow up; 27 eyes (69%) developed rhegmatogenous retinal detachments. Patients treated with combination ganciclovir and foscarnet therapy or ganciclovir alone had significantly better final visual acuity than those treated with either acyclovir or foscarnet (KWAOV: p = 0.0051). CONCLUSIONS This study represents the second largest series, the longest follow up, and the first analysis of visual outcomes based on medical therapy for AIDS patients with VZVR. Aggressive medical treatment with appropriate systemic antivirals may improve long term visual outcome in patients with VZVR. Acyclovir appears to be relatively ineffective in treating this disease.

Raised intraocular pressure is a common and frequently serious complication of anterior uveitis. The milieu of inflammatory cells, the mediators they release, and the corticosteroid therapy used to treat the uveitis can participate in the pathogenesis of uveitic glaucoma. These factors alter the normal anatomic structure of the anterior chamber and angle, influencing aqueous production and outflow. These changes act to disrupt the homeostatic mechanisms of intraocular pressure control. Structural changes in the angle can be acute, such as in secondary angle closure with pupillary block glaucoma, or chronic, such as combined steroid-induced and secondary open angle glaucoma. Management of uveitic glaucoma may be difficult because of the numerous mechanisms involved in its pathogenesis. Diagnostic and therapeutic decisions are guided by careful delineation of the pathophysiology of each individual case. The goal of treatment is to minimize permanent structural alteration of aqueous outflow and to prevent damage to the optic nerve head. This article reviews the pathogenesis of uveitic glaucoma, with specific attention to etiology. Medical and surgical therapies are also discussed, with emphasis on the more recent developments in each category.

Despite numeious reports of atypical mycobacterial infections,'"3 to our knowledge there have been no reports of atypical mycobacterial skin infections following oculoplastic procedures.We present a case of atypical mycobacterial wound infection after blepharoplasty.

BACKGROUND:Primary intraocular lymphoma is an uncommon clinical entity with poor visual and systemic prognosis. Optimal management of intraocular lymphoma remains uncertain.METHODS:Three patients with intraocular lymphoma, two of whom had documented CNS involvement, were treated based on a modification of the Sloan-Kettering Cancer Center protocol for primary CNS lymphoma. All patients underwent diagnostic pars plana vitrectomy and histopathologic confirmation of primary intraocular large B-cell lymphoma. Treatment involved systemic chemotherapy with methotrexate and high-dose ARA-C, radiation therapy of the brain and orbits, and intrathecal methotrexate delivered via an Ommaya reservoir.RESULTS:Resolution of the ocular lymphoma was seen in all three patients, and resolution of the intracranial disease also was seen in the two patients with CNS involvement. All patients have remained disease free, with lymphoma in remission for at least 24 months after completion of treatment.CONCLUSION:The Sloan-Kettering protocol for the treatment of primary CNS lymphoma also appears to be effective in some cases of primary intraocular large cell lymphoma. Furthermore, the Ommaya reservoir works well for intrathecal delivery of methotrexate in patients with CNS or leptomeningeal spread.

BACKGROUND:Vogt-Koyanagi-Harada (VKH) syndrome is associated with human leukocyte antigen (HLA)-B54, -DR4, -DR beta 1*0405, -DQ4, and -DR53 in Japanese patients. Disease-associated HLA specificities may differ among races. This study examined HLA associations with VKH syndrome in Hispanic patients living in southern California, a racial subgroup at increased risk for the disease.METHODS:Human leukocyte antigen specificities were determined on 25 Hispanic patients with VKH syndrome and compared with HLA specificities of 217 healthy Hispanic control subjects. Inclusion criteria for study patients were nontraumatic panuveitis with exudative retinal detachments, with or without extraocular manifestations. Tests were performed using standard cytotoxic assays.RESULTS:HLA-DR4 was present in 14 (56%) patients with VKH syndrome and in 62(29%) control subjects (relative risk = 1.96). HLA-DR1 was present in 9 (36%) patients with VKH syndrome and in 19 (9%) control subjects (relative risk = 4.11). HLA-DR1 and DR4 share a common epitope within the DR beta 1 gene. HLA-DR1 and/or DR4 were present in 21 (84%) patients with VKH syndrome and in 76 (35%) control subjects (relative risk = 2.40).CONCLUSIONS:HLA-DR1 and -DR4 were found in a significantly disproportionate number of Hispanic patients with VKH syndrome living in southern California. HLA-DR4, although not HLA-DR1, has been previously associated with VKH syndrome in other groups. These associations suggest a common immunogenic predisposition to VKH among different racial groups, and suggest that a common epitope shared by DR1 and DR4 may be involved in the pathogenesis of the disease.

BACKGROUND:Coccidioidomycosis has reached epidemic proportions in the southwest region of the United States. Despite the greater numbers of cases, isolated anterior segment ocular coccidioidomycosis in the absence of systemic infection continues to be rare, although its incidence may be increasing.METHODS:Two patients without clinical evidence of systemic disease and one patient with previously treated pulmonary coccidioidomycosis had granulomatous iridocyclitis and iris nodules that were unresponsive to corticosteroid therapy. All three patients underwent iris biopsy, anterior chamber tap, and washout for histopathologic diagnosis of anterior segment disease, and all subsequently received systemic antifungal therapy. Two patients also received multiple intraocular injections of amphotericin B.RESULTS:Papanicolaou and hematoxylin-eosin-stained preparations of anterior chamber tap and biopsies of the iris in each of these patients showed fibrinopurulent or granulomatous inflammatory exudate with intact and disrupted Coccidioides spherules. Despite aggressive systemic and intraocular therapy, one patient required enucleation for a blind, painful eye. The other two patients continue to have limited visual acuity but with at least partial resolution of the intraocular lesions.CONCLUSIONS:Ocular coccidioidomycosis without clinical evidence of systemic involvement is rare. Isolated anterior segment disease is also uncommon; however, because of the current epidemic in the southwest region of the United States, ocular coccidioidomycosis should be considered in any patient who traveled through or lived in endemic areas and who has a granulomatous iridocyclitis associated with iris mass that is unresponsive to corticosteroid therapy.

Of 65 consecutive patients (130 eyes) with Vogt-Koyanagi-Harada syndrome, 26 (40%) developed cataracts (49 eyes, 38%). Twenty-eight cataracts were posterior subcapsular, six nuclear, 14 combined nuclear and posterior subcapsular, and one anterior cortical. Risk factors for the development of cataracts included age (P = .003), long-standing recurrent anterior segment inflammation (P < .001), and systemic corticosteroid therapy for six months or more (P < .001). After at least three months of minimal to no intraocular inflammation, 19 eyes underwent cataract extraction, of which 11 also had posterior chamber intraocular lens implantation. Median visual acuity improved significantly from 20/400 to 20/40 (P < .01) after a median follow-up time of 13 months. Route of preoperative corticosteroids, pars plana vitrectomy, and glaucoma surgery did not markedly affect visual prognosis. If preoperative intraocular inflammation is rigorously controlled, cataract surgery can be performed safely and can result in significant visual improvement in patients with Vogt-Koyanagi-Harada syndrome.

We studied two patients, a 43-year-old Hispanic man with a one-year history of acquired immunodeficiency syndrome (AIDS) and a 34-year-old Hispanic man with newly diagnosed AIDS. Both had necrotizing retinitis that progressed to panophthalmitis and orbital cellulitis. Toxoplasmosis was not diagnosed in the first patient early in the course of the disease. The second patient had a history of toxoplasmic retinochoroiditis. Despite anti-toxoplasmosis therapy, visual acuity deteriorated to no light perception in both patients. Diagnostic biopsy of the eye wall was performed on the first patient and enucleation of the globe on the second. Toxoplasmic panophthalmitis and orbital cellulitis were diagnosed in each patient by light microscopy and confirmed by electron microscopy. When patients with AIDS develop necrotizing retinitis, toxoplasmosis must be considered in the differential diagnosis, along with cytomegalovirus retinitis, progressive outer retinal necrosis, and syphilitic retinitis. Unlike cytomegalovirus retinitis, progressive outer retinal necrosis, and syphilitic retinitis, however, toxoplasmosis can cause a progressive intraocular infection, panophthalmitis, and orbital cellulitis in patients with AIDS.

Subretinal neovascular membranes cause late visual loss and are important determinants of final visual acuity in patients with Vogt-Koyanagi-Harada syndrome. The purpose of this study was to determine the prevalence and risk factors associated with the development of subretinal neovascular membranes in Vogt-Koyanagi-Harada syndrome. We reviewed the charts of 58 patients (116 eyes) with Vogt-Koyanagi-Harada syndrome. We separated patients into two groups, one with and one without subretinal membranes. Demographic characteristics, namely age, gender, and race, initial and final visual acuities, duration and method of treatment, anterior chamber and vitreous cell, presence of fundus pigmentary disturbances, and phase of inflammation, were evaluated for each group. Overall, ten eyes (9%) of seven patients developed the subretinal neovascular membranes. Demographic characteristics among the two groups of patients were not markedly different (P > .10). Visual outcome of patients with subretinal membranes was significantly poorer than was that of patients without these membranes (P < .001). Eyes with subretinal membranes had a significantly greater degree of anterior chamber and vitreous inflammation (P < .02), greater incidence of fundus pigmentary disturbances (P < .02), and a greater frequency of chronic recurrent phase of inflammation, marked by recurrence of predominantly anterior chamber, and occasionally posterior segment, inflammation (P < .02). The membranes were located in the peripapillary (four eyes), subfoveal (three eyes), and macular extrafoveal (three eyes) regions.