The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy.

OBJECTIVE: To expand the clinical spectrum of idiopathic polypoidal choroidal vasculopathy based on historical cases and newly recognized observations. METHODS: A review of the previously reported 45 cases was carried out. An additional 20 cases were retrospectively reviewed to examine the clinical nature and course of idiopathic polypoidal choroidal vasculopathy. RESULTS: New observations on the clinical spectrum of idiopathic polypoidal choroidal vasculopathy were noted for demographic features, the nature and course of the vascular lesion, the possible association with intraocular inflammation, and the indocyanine green angiographic characteristics. CONCLUSIONS: Idiopathic polypoidal choroidal vasculopathy seems to be a distinct clinical entity that has a predilection for individuals of pigmented races. The disorder should be differentiated from typical choroidal neovascularization and other known choroidal degenerative, inflammatory, and ischemic disorders because of differences in clinical course and treatment.

The expanding clinical spectrum of unilateral acute idiopathic maculopathy.

OBJECTIVE: To report on new features of unilateral acute idiopathic maculopathy (UAIM). PATIENTS: We have evaluated an additional 17 patients with UAIM since 1991. This is a report of new features of the maculopathy noted in seven patients from this new series. RESULTS: New clinical findings in UAIM included eccentric macular lesions, subretinal exudation, papillitis, and bilaterality. The occurrence of UAIM in association with pregnancy and human immunodeficiency virus was also observed. CONCLUSIONS: The description of these newly reported features broadens our understanding of the nature of UAIM. With recognition of the expanded clinical spectrum of this disorder, a more confident approach to diagnosis and management may be achieved.

Microrips of the retinal pigment epithelium.

We describe six patients with exudative age-related macular degeneration who had retinal pigment epithelial detachments with associated overlying neurosensory detachments. During fluorescein angiography, each patient demonstrated a solitary, intense, central serouslike leak at the edge of the retinal pigment epithelial detachment with passage of fluorescein into the subretinal space. In patients in whom the location of associated choroidal neovascularization was evident, the leakage site was remote to the area of neovascularization. Our observations suggest that these leaks result from small retinal epithelial rips, which we termed "microrips," that differ from conventionally described retinal pigment epithelial rips in clinical course and response to laser treatment. We hypothesize that the mechanisms and forces that generate these microrips are different from those producing conventionally described retinal pigment epithelial rips.

Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration.

OBJECTIVE: To determine the nature and frequency of polypoidal choroidal vasculopathy (PCV) in a series of patients suspected of having neovascularized age-related macular degeneration (AMD). METHODS: A prospective analysis of 167 consecutive, newly diagnosed patients aged 55 years or older with presumed neovascularized AMD was performed. All patients were examined with fundus biomicroscopy as well as fluorescein and indocyanine green angiography. RESULTS: Choroidal neovascularization secondary to AMD was diagnosed in 154 (92.2%) of 167 patients; 13 (7.8%) patients had PCV. The patients affected by PCV were younger than those with AMD (P = .01). Peripapillary choroidal neovascularization was seen in 3 (1.9%) of 154 patients with AMD and 3 (23.1%) of 13 patients with PCV (P = .006). Significant drusen were present in 63 (70%) of 90 fellow eyes with unilateral AMD compared with only 1 (16.7%) of 6 eyes with PCV (P = .02). Only 5 patients with AMD (3.2%) were nonwhite compared with 3 patients with PCV (23.1%) (P = .02). CONCLUSIONS: A measurable number of elderly patients with findings suggestive of neovascularized AMD and serosanguineous macular manifestations will instead have PCV. Polypoidal choroidal vasculopathy can occur in any sex or race, but is more commonly seen in the peripapillary area, without associated drusen, and in nonwhite patients. It is important to differentiate AMD from PCV because there are significant differences in the demographic risk profile, natural course, visual prognosis, and management of these patients.

Combined hamartoma of the retina and retinal pigment epithelium associated with juvenile nasopharyngeal angiofibroma.

PURPOSE: To report a patient who had concomitant combined hamartoma of the retinal pigment epithelium and retina and juvenile nasopharyngeal angiofibroma. METHOD: Case report. RESULTS: An 18-year-old man presented with a juvenile nasopharyngeal angiofibroma and a combined hamartoma of the retina and retinal pigment epithelium in the ipsilateral eye. The diagnosis of juvenile nasopharyngeal angiofibroma was confirmed by histopathology, and the diagnosis of combined hamartoma was established by typical ophthalmoscopic and fluorescein angiographic findings. CONCLUSION: Juvenile nasopharyngeal angiofibroma may be associated with combined hamartoma of the retina and retinal pigment epithelium.

Estrogen and visual hallucinations in a patient with Charles Bonnet syndrome.

PURPOSE: To describe the occurrence of visual hallucinations in a patient with Charles Bonnet syndrome associated with estrogen intake. METHOD: Case report. RESULTS: An 84-year-old woman with poor visual acuity secondary to bilateral, nonexudative, age-related macular degeneration had nonthreatening visual hallucinations 2 weeks after starting oral estrogen for osteoporosis. The estrogen was stopped, and the hallucinations subsided. The patient was given estrogen twice more and each time the hallucinations recurred. CONCLUSION: We report a case of Charles Bonnet syndrome associated with estrogen intake in an 84-year-old woman. Estrogen may have promoted release phenomena and triggered the hallucinatory episodes in our patient.

Epstein-Barr virus antibodies in multifocal choroiditis and panuveitis.

Although it has been reported that patients with multifocal choroiditis and panuveitis have serologic evidence of a chronic or persistent Epstein-Barr virus infection, our patients did not seem to have other stigmata of Epstein-Barr virus infection. To reappraise the serologic evidence of chronic Epstein-Barr virus infection, the Epstein-Barr antibody levels in 11 patients with multifocal choroiditis and panuveitis and 11 sex- and age-matched control patients were measured. Neither the antiviral capsid antigen IgG (P = .15) nor the antinuclear antigen (P = .2) antibody titers of the patients with multifocal choroiditis and panuveitis were significantly different than those of the control patients. Neither the patients with multifocal choroiditis and panuveitis nor the control patients had increased antiviral capsid antigen IgM titers. One patient with multifocal choroiditis and panuveitis and three control patients had positive anti-early antigen antibody titers (P = .59). The results of this study do not support the hypothesis that patients with multifocal choroiditis and panuveitis have serologic evidence of chronic or persistent Epstein-Barr virus infection as a characteristic finding.

Frosted branch angiitis associated with cytomegalovirus retinitis.

We examined three patients with acquired immunodeficiency syndrome who had frosted branch angiitis associated with small patches of cytomegalovirus retinitis. Each patient had a low CD4-helper T-lymphocyte count and a T-lymphocyte helper-suppressor ratio of less than 0.1. Treatment with intravenous anticytomegalovirus antibiotics caused the vascular sheathing to resolve within two weeks in all three patients, but each patient continued to have a smoldering retinitis. Retinal biopsy in one of the patients demonstrated virions whose morphologic characteristics were consistent with cytomegalovirus on electron microscopy and the identity of which was confirmed by immunohistochemistry. Although frosted branch angiitis in otherwise healthy patients responds to corticosteroids, similar treatment with corticosteroids for frosted branch angiitis associated with cytomegalovirus retinitis in patients with AIDS does not seem to be indicated. Before corticosteroid treatment is started for a patient with the clinical signs and symptoms of frosted branch angiitis, careful medical examination of the patient is necessary.

Central serous chorioretinopathy after epidural corticosteroid injection.

PURPOSE: To report three patients who developed central serous chorioretinopathy after epidural corticosteroid injection for treatment of back pain. DESIGN: Interventional case series. METHODS: Three men, aged 73, 52, and 73 years, presented with bilateral central serous chorioretinopathy after corticosteroid injection in the epidural space for treatment of back pain. In all three cases, we did not initially elicit the history of corticosteroid use. RESULTS: Two of the three patients, aged 52 and 73 years, had diffuse retinal pigment epitheliopathy and one, aged 73 years, had classic central serous chorioretinopathy. Two patients had a spontaneous resolution of the subretinal fluid in both eyes. One patient had laser photocoagulation in both eyes but continued to have diffuse leakage in one eye. CONCLUSIONS: A careful history to determine corticosteroid use, including possible intrajoint and epidural injection, should be performed in older people with serous detachment of the macula, particularly when bilateral.

Optical coherence tomography of bleb-like subretinal lesions after retinal reattachment surgery.

PURPOSE: To report the optical coherence tomographic characteristics of persistent bleb-like subretinal lesions after retinal reattachment surgery, including scleral buckling and cryopexy. METHODS: Case reports. RESULTS: In two eyes of two patients yellow to yellowish-orange bleb-like lesions were found in the posterior pole after retinal reattachment surgery. The common aspects of the surgery were scleral buckling procedures and cryopexy. There were subretinal precipitates in some of the lesions. The lesions showed no associated leakage during fluorescein or indocyanine green angiography. Optical coherence tomography demonstrated that the bleb-like lesions were tiny retinal detachments. CONCLUSION: Optical coherence tomography examination of bleb-like subretinal lesions showed that they were actually retinal detachments. Our patients and previously reported patients developed these lesions after scleral buckling and cryopexy. These lesions may represent persistent retinal detachment because of the presence of protein in the subretinal fluid.

Ocular findings in cystic fibrosis.

We examined 32 patients with cystic fibrosis, paying special attention to optic nerve performance and pupillary function. Decreased visual acuity occurred in nine of 64 eyes. Three of 17 patients (18%) who used chloramphenicol had bilaterally delayed P100 waves of the visual-evoked response of greater than 3 standard deviations. This was not found in patients who did not use chloramphenicol. Contrast sensitivity in patients with cystic fibrosis was decreased at every spatial frequency when compared to healthy controls. This decrease was noted in patients who did and did not use chloramphenicol, suggesting that chloramphenicol is not the only cause of decreased contrast sensitivity in cystic fibrosis. With pharmacologic pupil testing we determined that patients with cystic fibrosis display a preganglionic oculosympathetic paresis that corresponded to the disease severity, as measured by the Shwachman score.

Varicella-zoster virus retinitis in patients with the acquired immunodeficiency syndrome.

We examined five patients infected with the human immunodeficiency virus who developed a rapidly progressive necrotizing retinitis characterized by early patchy choroidal and deep retinal lesions and late diffuse thickening of the retina. In all but one case, the retinitis began in the posterior pole with little or no clinical evidence of vasculitis. All five patients had relentless progression of disease and were left with atrophic and necrotic retinae, pale optic-nerve heads, and narrowed vasculature. None of the patients developed aqueous or vitreal inflammation or retinal detachment. Clinical and laboratory evidence suggested that varicella-zoster virus was the causal agent in all five cases. First, the onset of retinitis in four cases either succeeded or was coincident with an eruption of dermatomal zoster. Second, varicella-zoster virus was cultured from the two chorioretinal specimens and varicella-zoster virus antigen was detected in the vitreal aspirate from one case. Third, by means of immunocytochemistry, varicella-zoster virus antigen was found in the outer retinae of both enucleation specimens. Fourth, viral capsids with the size and shape of herpesviridae were found in the outer retinae of both enucleation specimens. The clinical features observed in this study are distinct from those described for the acute retinal necrosis syndrome and appear to constitute a new and highly characteristic pattern of varicella-zoster virus-induced disease.

Systemic findings associated with central serous chorioretinopathy.

PURPOSE: To determine systemic factors associated with central serous chorioretinopathy. METHODS: In a retrospective study, 230 consecutive patients with central serous chorioretinopathy examined in a referral setting were compared with a historical gender-matched and age-matched control group of 230 patients with ocular findings who were examined in the same referral setting. RESULTS: The median age of the patients was 49.8 years, and of the control subjects, 50.0 years. The male-female ratio for both groups was 2.7:1. Patients with central serous chorioretinopathy were more likely to use psychopharmacologic medications (odds ratio = 2.6; 95% confidence interval = 1.30 to 5.19; P = .0049) and corticosteroids (odds ratio = 3.17; 95% confidence interval = 1.30 to 7.70; P = .0067) and were more likely to have hypertension (odds ratio = 2.25; 95% confidence interval = 1.39 to 3.63; P = .0008) than were the control subjects. CONCLUSIONS: This study identified psychopharmacologic medication use, corticosteroid use, and hypertension as factors associated with central serous chorioretinopathy. These findings reinforce the concept that stress and adaptations to stress play a role in this disorder. The findings of possible associations between central serous chorioretinopathy and both hypertension and corticosteroid usage suggest that these modifiable factors may influence morbidity of central serous chorioretinopathy.

Conjunctival biopsy in the diagnosis of sarcoidosis.

We prospectively studied 47 sarcoidosis suspects and compared conjunctival and transbronchial lung biopsies in these patients. Thirty-four patients had positive findings on biopsy by either method. The transbronchial biopsy was positive in 31 patients, and the conjunctival was positive in 19. The transbronchial lung biopsy was more likely to be positive in black patients (p = 0.009) and in patients with pulmonary infiltrates on chest x ray (p = 0.0044). In comparison, the conjunctival biopsy was more likely to be positive in patients with conjunctival follicles (p = 0.036), ocular abnormalities consistent with sarcoidosis (p = 0.02), and in patients with pulmonary infiltrates on chest x ray (p = 0.029). Iritis was present in 12 patients, enlarged lacrimal glands in three, and vitritis in five. We conclude that the conjunctival biopsy is an effective means of diagnosing sarcoidosis and that every sarcoidosis patient should have an ophthalmic examination.

Choroidal vasculitis in acute posterior multifocal placoid pigment epitheliopathy.

A 24-year-old Caucasian female presented with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and associated infiltration round some of the larger choroidal blood vessels. This infiltration dissipated as the patient's clinical condition improved and did not induce any permanent alteration of the overlying retinal pigment epithelium. We suggest that the infiltration round the choroidal vessels was due to a choroidal vasculitis. The finding of choroidal inflammation in this case lends support to the hypothesis that choroidal vasculitis is an underlying pathological process in APMPPE.

The contralateral reduction of intraocular pressure by timolol.

Administration of timolol to one eye is associated with a decrease in intraocular pressure in both eyes. To further investigate this effect the contralateral decrease in intraocular pressure was measured in a three arm crossover study using a 0.1 mg dose of timolol given topically to an eye, a 0.1 mg dose of timolol given lingually, and a placebo given topically. Two hours after topical timolol administration the mean intraocular pressure reduction in the fellow eye was 3.1 mm Hg compared with baseline (p = 0.0007). Two hours after lingual timolol administration the mean intraocular pressure reduction was 3.9 mm Hg compared with baseline (p = 0.0004). Two hours after topical administration of placebo the mean intraocular pressure reduction in the fellow eye was only 0.33 mm Hg (p = 0.6). These findings suggest the contralateral reduction in intraocular pressure from timolol is caused by systemic absorption. The significant intraocular pressure reduction obtained from lingual timolol raises the possibility that this route of drug administration may be useful in selected patients who cannot use eye drops.

Lack of the HLA-DR2 specificity in multifocal choroiditis and panuveitis.

The prevalence of the HLA-B7 and HLA-DR2 specificities in 17 unrelated patients with multifocal choroiditis and panuveitis, 11 with and six without subretinal neovascularisation, was evaluated and compared with those of two different groups. The first group was 17 patients with subretinal neovascularisation associated with presumed ocular histoplasmosis syndrome, and the second was a group of 105 eye patients with no retinal disease. HLA-DR2 was not found in any patient with multifocal choroiditis and panuveitis, but it was found in 13 patients with presumed ocular histoplasmosis syndrome (p = 6.72 x 10(-5), comparison of the groups with subretinal neovascularisation). The lack of HLA-DR2 was also significant in comparison with the control group of eye patients (p = 0.041). This study suggests that patients with multifocal choroiditis and panuveitis and presumed ocular histoplasmosis syndrome have differing genetic predispositions, though the fundus pictures in these entities have many similarities.

Rapidly expanding exophthalmos: an unusual presentation of small cell lung cancer.

A 61-year-old male presented with a rapidly progressive exophthalmos from small cell lung cancer metastatic to the right orbit. His vision in that eye was 20/200, and his intraocular pressure was 36 mmHg. The orbital metastasis responded dramatically to chemotherapy. One week after starting the chemotherapy the patient did not have exophthalmos, his vision was 20/20, and three weeks later the intraocular pressure was 12 mmHg.

Subretinal exudative deposits in central serous chorioretinopathy.

The presence of subretinal exudation in a patient with neurosensory detachment of the macula frequently suggests the diagnosis of choroidal neovascularisation. A retrospective chart review of newly diagnosed cases of central serous chorioretinopathy revealed 11 patients, seven men and four non-pregnant women, who had plaques of subretinal exudate, which presumably were fibrin. Each of these patients had a solitary plaque that ranged in size from 300 to 1500 microns in diameter. These patients had no signs or a clinical course suggestive of choroidal neovascularisation. In each case the subretinal plaque was overlying an exuberant leak in the retinal pigment epithelium. The exudate was generally present at the initial examination, and usually showed dissolution before or coincident with the resolution of the neurosensory detachment. After resolution of the central serous chorioretinopathy, patients were left with subtle alterations in the retinal pigment epithelium in the areas of the subretinal plaque. These findings are important for two reasons. Firstly, the presence of subretinal exudation does not necessarily rule out the diagnosis of central serous chorioretinopathy. Secondly, pathophysiological theories of central serous chorioretinopathy must explain how the plaques are deposited behind the retina.