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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 legions 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

• Stereoscopic transparencies studied with magnification and projection suggest that the retinal elevation that communicates with optic pits is frequently a schisislike separation of the internal layers of retina. Thirteen of 15 eyes with optic pits and maculopathy fit the schisis pattern. Separation of the outer layers of the retina is a secondary phenomenon that starts in the macula.

• Interferon alfa is used to treat various systemic disorders and recently has been suggested as a possible treatment for choroidal neovascularization. We report 10 cases of retinal ischemia associated with the use of interferon alfa for various illnesses. The retinal findings include cotton-wool spot formation, capillary nonperfusion, arteriolar occlusion, and hemorrhage. The retinal complications may sometimes be reversible when treatment is stopped. Our findings emphasize the need to have patients who are receiving interferon alfa therapy monitored for these retinal complications, which may rarely be associated with permanent loss of vision secondary to closure of retinal capillaries.

BACKGROUND: The pathogenesis of central serous chorioretinopathy (CSC) is poorly understood. Abnormalities in the choroidal circulation have been hypothesized to be causative factors. Fluorescein angiography has not been particularly useful in identifying specific choroidal defects in CSC, largely because of inherent limitations in imaging with this technique. Recent technologic advances in digital indocyanine green videoangiography allow enhanced imaging of the choroid and other subretinal structures in comparison with fluorescein angiography. METHODS: We performed digital indocyanine green videoangiography in 29 consecutive eyes with CSC and compared our results with clinical and fluorescein angiographic findings. RESULTS: Several newly recognized subretinal abnormalities in CSC were noted with digital indocyanine green videoangiography, including (1) presumed hyperpermeability of the choroidal circulation surrounding active retinal pigment epithelial leaks, (2) additional focal and multifocal areas of presumed choroidal hyperpermeability not associated with abnormalities detectable by fluorescein angiography or clinical examination, and (3) multiple presumed "occult" serous retinal pigment epithelial detachments with a characteristic indocyanine green videoangiographic pattern. CONCLUSION: We suggest that the pathogenesis of CSC may be due to a choroidal vascular hyperpermeability with and without associated active pigment epithelial leaks and multiple presumed "occult" serous retinal pigment epithelial detachments. Based on these findings, a hypothetical model can be constructed related to the pathogenesis of CSC, beginning with choroidal abnormalities that secondarily affect the retinal pigment epithelium and neurosensory retina.

OBJECTIVE: To define the functional deficit that correlates with the inner layer separation and the outer layer detachment that have been observed in optic pit maculopathy and to determine the effect of a gas tamponade that compresses or displaces the two layers. DESIGN: The central visual field before and after a gas tamponade on the posterior pole were charted on a 1-m tangent screen and compared with the changes in the retina observed biomicroscopically and with stereophotography. SETTING: The gas operations were done at four hospitals in the New York, NY, area and one in Cleveland, Ohio. The retinal examinations, visual field testing, and stereoscopic photography for eight patients were done at The New York Hospital. The patient who lived in Cleveland was examined and photographed in Cleveland. PARTICIPANTS: Nine patients between the ages of 18 and 46 years with optic pit maculopathy. INTERVENTION: The patients were operated on by five retinal surgeons with a variety of procedures. Four patients had a vitrectomy and intraocular gas injected. Five patients had gas injected after external compression. Two patients had laser applied in conjunction with the intraocular injection. The gas tamponade was the unifying factor. RESULTS: The dense central scotoma in optic pit maculopathy relates to the outer layer detachment and displacement of it from the posterior pole yields an improvement in visual acuity. The inner layer separation persists centrally after a gas tamponade and continues to provide access for the flow of fluid from the pit. The scotoma that relates to the inner layer separation is mild and consistent with relatively good visual acuity.

• 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.

PURPOSE: To evaluate the use of digital indocyanine green videoangiography in patients with clinical and fluorescein angiographic evidence of "occult" choroidal neovascularization in age-related macular degeneration and to investigate indocyanine green videoangiography-guided laser photocoagulation as a therapeutic approach. METHODS: Three hundred forty-seven consecutive patients with exudative age-related macular degeneration and symptoms and clinical manifestations of occult choroidal neovascularization were studied with indocyanine green videoangiography. Patients were selected for laser treatment, using conventional guidelines, when indocyanine green videoangiography demonstrated a well-delineated area of hyperfluorescence, presumed to be a focal area of choroidal neovascularization. RESULTS: Seventy-nine (23%) of 347 eyes were found to have a localized and definable lesion that was potentially amenable to laser photocoagulation therapy; 44 (56%) of these 79 treated eyes had complete resolution of their exudative manifestations. Visual acuity improvement was noted in 10 (13%) of 79 eyes, and stabilization of vision achieved in 42 eyes (53%). CONCLUSION: Laser photocoagulation treatment guided by indocyanine green videoangiography was shown to produce promising anatomical and visual improvement in a small number of patients with occult choroidal neovascularization secondary to age-related macular degeneration. This pilot study warrants further research to investigate the efficacy and safety of this form of treatment.

PURPOSE: Digital indocyanine green videoangiography (ICG-V) was used to study recurrent choroidal neovascularization (CNV) in patients with the clinical and fluorescein angiographic findings indicative of ill-defined, or recurrent occult, CNV (RO-CNV). The use of ICG-V-guided laser caphotocoagulation as an alternative form of treatment was also investigated when a well-delineated area of CNV was imaged with this technique. METHODS: A consecutive series of 66 patients were studied who presented with exudative age-related macular degeneration and symptoms and clinical manifestations of recurrent CNV in which fluorescein angiography did not reveal classic, or well-defined, neovascularization. Patients were selected for laser treatment based on conventional guidelines if ICG-V imaged a well-delineated area of recurrent CNV. RESULTS: Indocyanine green videoangiography showed late staining that was consistent with recurrent CNV in 64 (97%) of these 66 patients with RO-CNV. Twenty-nine (44%) of the 66 were eligible for laser treatment, and 18 (62%) of these 29 patients experienced successful anatomic and visual results, which were defined as resolution of the exudative manifestations and improvement or stabilization (±1 line on a Snellen chart) of vision. CONCLUSIONS: This pilot study suggests that ICG-V is of value in imaging patients with RO-CNV after laser photocoagulation for CNV secondary to age-related macular degeneration. Laser treatment of RO-CNV with ICG-V guidance may be successful both anatomically and functionally in a promising number of these otherwise untreatable cases. Further studies are necessary to validate these preliminary findings.

Some degree of pigment migration invariably follows cryosurgery of the retina. Immediately upon thawing, pigment granules lysed from the pigment epithelium of the choroid may migrate through the retinal tear and into the vitreous. Pigment migration may also occur through the subretinal fluid to form a deposit behind the macula, or a pigmented line representing the farthest advance of the retinal separation. Pigment deposited in the foveal area may impair macular function. The important difference between cryopexy and diathermy applications to the choroid is the apparent absence of coagulation of the tissue proteins in the cryosurgical lesion. The complications of pigment migration are perhaps the most serious observed with the cryosurgical method.

Systemic vascular ischemic manifestations of neurofibromatosis (NF) have been described in the aortic, cerebral, and renal vasculature.1 The occlusive manifestations include stenosis of small vessels, progressive capillary ischemia, and compensatory collateral circulation. Presented herein is a report of a case of a child with NF and unilateral, diffuse, retinal, vascular ischemia. REPORT OF A CASE. A 4-year-old Hispanic boy with a history of NF type I presented with an exodeviation and amblyopia involving the right eye. The best-corrected visual acuity was 20/40 OD and 20/20 OS.Diagnosis of NF type I was made on the basis of multiple café au lait spots, plexiform neurofibroma, and a paternal history of NF. There was no history of prematurity, low birth weight, or familial history of vitreoretinal disorders.Funduscopic examination of the right eye revealed a slightly pale, oval optic disc (Figure 1). The fovea was slightly ectopic superiorly and temporally. Diffuse

In some cases of occult choroidal neovascularization (CNV) visualized with fluorescein angiography, indocyanine green videoangiography (ICGV) performed on an outpatient basis may identify distinct areas of ICG dye (Cardiogreen, Becton Dickinson, Cockeysville, Md) hyperfluorescence.1 A recent histopathologic report has confirmed the presence of CNV in an area of ICG hyperfluorescence (W. R. Green, MD, oral communication, December 1993); however, the precise clinical significance of these areas remains to be determined. Indocyanine green dye excitation and emission spectra occur in the nearinfrared range, thereby allowing improved penetration of fluorescence through intraocular pigments, fluid, or thin layers of hemorrhage.We describe herein a system that can be used to perform real-time intraoperative ICGV during subretinal surgery. This system may be used to identify CNV after removal of thick subretinal hemorrhage, to identify areas of CNV for potential excision or endolaser treatment, and to confirm the adequacy of surgical excision of CNV

EVER SINCE THE efficacy and safety of laser photocoagulation of extrafoveal and juxtafoveal choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD) were initially confirmed by clinical trials, some ophthalmologists have assumed that subfoveal CNV treatment could offer a similar benefit. In the past, the standard of care for such treatment by these clinicians was governed empirically by the size of the imaged neovascularization and the state of the vision: treatment was recommended for small membranes with poor vision but was not recommended for large membranes with good vision. In September 1991, the Macular Photocoagulation Study Group (MPS) surprised many members of the ophthalmic community with the results of its clinical trial on laser photocoagulation of subfoveal CNV secondary to AMD.1 The earlier analysis of the data supported the recommendation for laser treatment of patients with well-demarcated, or so-called classic, CNV with certain limitations based on strict definitions of

TO THE EDITOR. —Chronic leukemia can be associated with manifestations involving virtually any structure in the eye or ocular adnexa.1 We report a case of perifoveal ischemia associated with chronic granulocytic leukemia without evidence of peripheral retinal nonperfusion. REPORT OF A CASE. —A 45-year-old woman with a medical history of chronic granulocytic leukemia of ten years' duration who had been under treatment intermittently with busulfan (5 mg/d) complained of progressive loss of vision in the right eye more than the left of one week's duration. The response to chemotherapy was effective without substantial complication such as pulmonary fibrosis or skin pigmentation. The patient was essentially asymptomatic during the course of treatment except for her visual symptoms.Her ocular examination disclosed a best corrected visual acuity of 20/400 OD and 20/25 OS. Slit-lamp biomicroscopic examination showed the anterior segment to be unremarkable. The optic nerve cup/disc ratio was normal without

In 1991, it was reported that interferon alfa was an effective treatment in some cases of choroidal neovascularization secondary to age-related macular degeneration.1 We will review the scientific rationale for this treatment, and suggest that it may be too early to recommend interferon alfa for general use in these ocular diseases until its efficacy and safety are further demonstrated.See also p 1366.Interferon alfa has been used for the treatment of hairy-cell leukemia and other neoplastic diseases since 1984.2 Recent experimental studies in the laboratory and the clinic have revealed a new property of interferon, the ability to inhibit angiogenesis.3-8 Angiogenesis, the growth of new capillary blood vessels, is a fundamental process of reproduction, development, and repair.9 Under normal conditions such as ovulation, neovascularization is brief and tightly controlled. However, pathologic angiogenesis, as in ocular neovascularization, psoriasis, hemangiomas, and cancer, can be prolonged and serve

TO THE EDITOR. —In a recent issue of the Archives, Wallow et al1 reported a case of recurrent subretinal neovascularization originating from the retinal circulation following krypton laser photocoagulation. We have also observed this phenomenon clinically,2 particularly originating from the venous side. We feel, however, that this form of recurrent subretinal neovascularization is most common following treatment with a hemoglobin-absorbing wavelength, such as argon blue-green or green laser. Argon laser photocoagulation commonly causes destruction and atrophy of the overlying retina. This procedure damages normal intraretinal structural barriers and displaces the retinal circulation into closer proximity to the choroidal circulation. Retinal-choroidal anastomoses and recurrent subretinal neovascularization originating from the retinal circulation are potential consequences. In our experience, this form of recurrent subretinal neovascularization following krypton red laser photocoagulation occurs under the following conditions:A very intense burn that extends to the overlying retina. In this case, the