Ischemic maculopathy in patients with acquired immunodeficiency syndrome.

PURPOSE: To describe the characteristics of ischemic maculopathy in patients with human immunodeficiency virus (HIV) infection, as a means of understanding this uncommon disorder more fully. METHODS: This is a multicenter, retrospective review of clinical data available for five HIV-infected patients who were given the diagnosis of ischemic maculopathy. RESULTS: All cases had been diagnosed on the basis of fluorescein angiograms obtained after patients complained of vision loss. Four of the five patients had bilateral macular disease. Visual acuity at presentation in the nine affected eyes ranged from 20/20 to count fingers. Vision loss was gradual in both eyes of one patient and was abrupt in onset in seven eyes. Each of the seven eyes with abrupt vision loss had opacification of the superficial retina and/or intraretinal hemorrhages near the fovea. Fluorescein angiography revealed enlargement of the foveal avascular zone and mild staining of the juxtafoveal vessels in affected eyes. Six eyes had active or clinically inactive cytomegalovirus retinitis at presentation, and a seventh eye developed cytomegalovirus retinitis 2 weeks later. All patients were receiving anticytomegalovirus drugs when they developed visual symptoms. Visual acuity remained stable in five eyes, became worse in two eyes, and improved in two eyes; final visual acuity ranged from 20/25 to count fingers. CONCLUSIONS: Ischemic maculopathy may cause profound and permanent vision loss in HIV-infected individuals. Fluorescein angiography should be considered in all HIV-infected patients with unexplained loss of vision. The pathogenesis of ischemic maculopathy remains unknown.

Preretinal neovascularisation associated with choroidal melanoma.

BACKGROUND: The rare occurrence of iris neovascularisation and choroidal (subretinal) neovascularisation in patients with choroidal melanoma has been reported. However, the occurrence of preretinal neovascularisation (NVE) fed from the retinal circulation in eyes with choroidal melanoma is far less frequently reported. METHODS: Three case reports of choroidal melanoma with the very rare finding of overlying NVE. RESULTS: The three patients had choroidal melanomas, localised serous retinal detachment, and NVE. Two cases showed definite retinal capillary non-perfusion, and one of these two cases demonstrated retinal telangiectasis. One patient's melanoma responded quickly to iodine-125 plaque radiotherapy; however, the retinal neovascularisation persisted and caused vitreous haemorrhage. Localised scatter photocoagulation was successful in causing the complete regression of the neovascularisation. The other two patients had their eyes enucleated (one with planned pre-enucleation external beam radiotherapy). Demonstration of preretinal vessels in one of the cases was possible in histological sections. CONCLUSION: Preretinal neovascularisation may occur as a complication of choroidal melanoma. Possible aetiologies include the release of tumour angiogenic factors, inflammation, chronic retinal detachment with secondary retinal ischaemia, retinal vascular occlusion secondary to retinal vessel invasion by the tumour, or following radiation therapy. Optimal management of the neovascularisation is not known at this time. Supplemental localised scatter photocoagulation may be of benefit in some cases.

Retinal findings in melanoma-associated retinopathy.

PURPOSE: To report unusual fundus findings in two cases of melanoma-associated retinopathy. METHODS: Observational case reports. The histories of two patients with melanoma-associated retinopathy were reviewed. Sera from both patients were examined for antibodies against retinal bipolar cells. Immunofluorescence was performed on cryostat sections of unfixed normal human retinas. Sera and IgG from both patients were tested against a known melanoma-associated retinopathy patient as well as a control subject. RESULTS: Our patients had metastatic, cutaneous melanoma and a clinical syndrome consistent with melanoma-associated retinopathy. Both patients had serum antibodies that were reactive against retinal bipolar cells. They had unusual fundus changes not previously described in association with melanoma-associated retinopathy. One patient also developed vitiligo. CONCLUSION: Patients with metastatic cutaneous melanoma and melanoma-associated retinopathy may present unusual fundus lesions that may be caused by autoimmunity which is part of the clinical picture of melanoma-associated retinopathy or metastatic melanoma.

Variations in the presentation of primary intraocular lymphoma: case reports and a review.

Primary intraocular lymphoma is a distinct subset of primary non-Hodgkin's lymphoma of the central nervous system (CNS). Diagnosis can be difficult and is often delayed, as the clinical presentation can mimic a number of other ocular conditions. This report describes four different presentations of intraocular lymphoma and focuses on its modes of clinical presentation. Primary intraocular lymphoma can present with a wide variety of manifestations frequently mimicking diffuse uveitis that is refractory to corticosteroids. Subretinal pigment epithelium tumors may be seen. However, other presentations may include multiple deep white dots in the retina secondary to tumor infiltration; retinal infiltration, causing a necrotizing retinitis; or infiltration of the retinal vasculature, causing arterial or venous obstruction. Finally, optic nerve invasion may be seen. CNS lymphoma develops in the majority of patients before, in conjunction with, or after the development of eye disease. Intraocular lymphoma often has a fatal outcome, but recognition of its modes of presentation facilitates early diagnosis and treatment that may improve prognosis.

Sympathetic ophthalmia associated with pars plana vitrectomy without antecedent penetrating trauma.

PURPOSE: To evaluate, describe, and categorize the clinical presentation, clinical course, histopathology, and response to therapy in patients without a history of penetrating ocular trauma who developed sympathetic ophthalmia following pars plana vitrectomy. METHODS: The records of patients without a history of trauma who underwent pars plana vitrectomy and developed sympathetic ophthalmia were retrospectively reviewed. Cases were analyzed with respect to clinical presentation, fluorescein angiographic findings, anatomic and visual outcomes, histopathology, and response to therapy. RESULTS: Eight eyes were identified. The median age at presentation was 55 years, with a range of 14 to 62 years. The time from vitrectomy to diagnosis of sympathetic ophthalmia ranged from 2 months to greater than 2 years, with a median of 7 months. Six of eight patients (75%) presented with anterior chamber reaction. All eight patients presented with a vitreous inflammatory response. The optic nerve was inflamed clinically or angiographically in four of eight cases (50%). Small yellow-white sub-retinal pigment epithelial deposits were present in four of eight cases (50%). Two eyes had lesions characterized as multifocal choroiditis. One eye had larger yellow placoid-like lesions. One eye presented with vitritis but no retinal lesions. Subretinal choroidal neovascularization was noted in the inciting eye of one patient. Vision improved in the sympathizing eye with immunosuppressive therapy in five of eight cases (62.5%). CONCLUSIONS: Sympathetic ophthalmia can be seen following pars plana vitrectomy in patients without penetrating injuries or a history of trauma. Indeed, it may be seen after successful vitrectomy for retinal detachment. Diverse clinical presentations are possible, and persistent or atypical uveitis following vitrectomy should alert the surgeon to the development of sympathetic ophthalmia.

Differentiation between presumed ocular histoplasmosis syndrome and multifocal choroiditis with panuveitis based on morphology of photographed fundus lesions and fluorescein angiography.

OBJECTIVE: To evaluate whether inactive cases of presumed ocular histoplasmosis syndrome (POHS) and multifocal choroiditis with panuveitis (MFC) can be differentiated from each other by their appearance on fundus photography and fluorescein angiography. METHODS: Two masked observers classified 50 patients' photographs (27 with fluorescein angiograms) as POHS, MFC, or "indeterminate." Twenty-five patients had known POHS and 25 had known MFC. Statistical analysis was performed to assess agreement and interrater reliability. RESULTS: Observer A classified 33 patients and was indeterminate on 17. Of the 33, he was correct on 26 (79% crude accuracy; kappa = 0.560; 95% confidence interval [CI], 0.286-0.834). Observer B classified 40 patients and was indeterminate on 10. Of the 40, he was correct on 33 (82% crude accuracy; kappa = 0.650; 95% CI, 0.422-0.878). Both observers ventured a diagnosis on 28 common patients. Of these, they selected the same diagnosis on 26 (93% crude agreement). When the 2 observers' diagnoses were compared and indeterminate patients were factored in, the kappa value was 0.408 (95% CI, 0.215-0.601). When the indeterminate patients are excluded, the kappa agreement increased to 0.825 (95% CI, 0.592-1). When pictures only were available, observer A and observer B kappa values against the gold standard were 0.625 (95% CI, 0.270-0.980) and 0.588 (95% CI, 0.235-0.940), respectively. The pictures-only kappa values for observer A vs observer B were 0.582 (95% CI, 0.316-0.848) with indeterminate patients factored in and 1.0 (95% CI, 1.0-1.0) when indeterminate patients were excluded. Pictures and fluorescein angiogram kappa values were 0.493 (95% CI, 0.076-0.909) for observer A and 0.706 (95% CI, 0.413-0.999) for observer B against the gold standard. For observer A vs observer B, the kappa value was 0.261 (95% CI, -0.002 to 0.524) with indeterminate patients factored in and 0.567 (95% CI, 0.032-1) excluding indeterminate patients. Sensitivity for all cases for observer A was 60% (+/-13%) for POHS and 94% (+/-6%) for MFC. For observer B, the sensitivity for all cases was 70% (+/-10%) for POHS and 95% (+/-5%) for MFC. CONCLUSIONS: Given adequate funduscopic information, the experienced observer can often accurately distinguish between POHS and MFC without the need for ancillary testing. Angiography in addition to fundus photography does not appear to increase diagnostic ability. There appears to be a higher sensitivity for MFC than for POHS.

Management of eyes with both idiopathic macular hole and choroidal neovascularization.

PURPOSE: To describe the characteristics, treatment, and outcome of five eyes with both choroidal neovascularization (CNV) and macular hole. METHODS: Medical records of five patients with both macular hole and CNV were reviewed. RESULTS: All eyes had full-thickness macular holes. Most eyes had atypical-appearing macular holes (subretinal hemorrhage, prominent subretinal fluid, or discoloration at the hole margin) at presentation or subsequently when CNV developed. Fluorescein angiography (FA) confirmed the presence of CNV in each eye. Three eyes underwent combined macular hole repair and CNV removal, and sustained closure of these macular holes was achieved. A fourth eye underwent successful argon laser photocoagulation of extrafoveal CNV, and macular hole surgery was declined. The final eye underwent two macular hole repairs before sustained closure was achieved. Final visual acuity, ranging from 20/100 to hand motions, was limited by macular pathology and/or cataract. CONCLUSIONS: Choroidal neovascularization can occur in association with a macular hole. In eyes with an atypical-appearing macular hole, FA should be obtained to detect CNV. Excision of the CNV can be done safely in conjunction with macular hole surgery. Final visual acuity may be limited by cumulative retinal and retinal pigment epithelium damage, especially in eyes with underlying macular disease.

Relentless placoid chorioretinitis: A new entity or an unusual variant of serpiginous chorioretinitis?

OBJECTIVE: To characterize an unusual clinical entity resembling acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and serpiginous choroiditis but with an atypical clinical course. PATIENTS: We describe 6 patients, aged 17 through 51 years, exhibiting this unusual entity who were seen at 6 different centers from 1984 to 1997. RESULTS: The acute retinal lesions in this series were similar to those of APMPPE or serpiginous choroiditis, both clinically and on fluorescein and indocyanine green angiography. However, the clinical course, number of lesions, and location of these lesions were atypical. These patients had evidence of numerous posterior and peripheral retinal lesions predating or occurring simultaneously with macular involvement. Older, healing pigmented lesions were often accompanied by the appearance of new active white placoid lesions. Additionally, these cases all demonstrated prolonged periods of activity resulting in the appearance of more than 50 and sometimes hundreds of lesions scattered throughout the fundus. Growth of subacute lesions and the appearance of new lesions continued for 5 to 24 months after initial examination, and relapses were common. CONCLUSIONS: This entity has clinical features similar to APMPPE and serpiginous choroiditis but has a prolonged progressive clinical course and widespread distribution of lesions. It may represent a variant of serpiginous choroiditis or may be a new entity. We call it relentless placoid chorioretinitis. Arch Ophthalmol. 2000;118:931-938

Progressive optic nerve cupping and neural rim decrease in a patient with bilateral autosomal dominant optic nerve colobomas.

PURPOSE: To document progressive optic nerve cupping and neural rim decrease in a patient with normal intraocular pressures and bilateral autosomal dominant optic nerve colobomas. METHODS: The ophthalmology records, stereoscopic fundus photographs, and visual fields of a 27-year-old woman with familial (autosomal dominant) optic nerve colobomas were reviewed. The appearance of the optic nerves was documented over a 13-year period (1985 to 1998). RESULTS: Despite repeatedly normal intraocular pressures, the patient showed progressive optic nerve cupping and neural rim decrease in both eyes. Visual field testing was available over a 5-year period (1993 to 1998) and was abnormal, but no progression was seen. CONCLUSIONS: This case of progressive cupping and neural rim decrease in a patient with autosomal dominant optic nerve coloboma in both eyes may provide insight into the optic nerve cupping associated with normal tension glaucoma. Careful follow-up of patients with optic disk colobomas or patients is indicated to detect possible optic nerve changes or field loss.

Pigmented adenoma mimicking a juxtapapillary melanoma. A 20-year follow-up.

A 52-year-old white woman was first diagnosed with a tumor of the right optic nerve in 1972. She remained asymptomatic until 1992, when she had a seizure on the left side of her body from a frontoparietal glioblastoma multiforme. Ophthalmic examination revealed enlargement of the eye tumor. This case provides clinical documentation spanning 20 years of a growing, pigmented tumor of the optic nerve head shown histopathologically to be a retinal pigment epithelial adenoma.