Michael D. Ober

Acute central serous chorioretinopathy and fundus autofluorescence

Objectives: To describe fundus autofluorescence (FAF) in a series of patients with acute central serous chorioretinopathy (CSC). Methods: Nine eyes of six patients with acute CSC were evaluated with fluorescein angiography (FA) and FAF imaging to evaluate the nature of the focal retinal pigment epithelial (RPE) leak evident with FA. Results: All nine eyes in this series demonstrated hypoautofluorescence corresponding precisely to the site of the focal RPE leak seen on FA. Conclusions: In this group of patients, the acute focal RPE leaks seen with FA corresponded precisely to an area of hypoautofluorescence imaged with FAF. This observation supports the concept that a mechanical defect or absence of the RPE accounts for the leakage from the inner choroid to the subneurosensory space in CSC. FAF is also a useful noninvasive diagnostic adjunct to identify the focal RPE leak in patients with acute CSC.

Selective photodynamic therapy for neovascular age-related macular degeneration with polypoidal choroidal neovascularization.

Purpose: To evaluate the efficacy of selective treatment with indocyanine green (ICG) angiography-guided photodynamic therapy (PDT) with verteporfin for polypoidal choroidal vasculopathy (PCV). Methods: In this retrospective consecutive series, 30 eyes of 30 patients with PCV were included. Complete ocular examination, digital fluorescein angiography (FA), ICG angiography, and optical coherence tomography were performed at baseline and at standard intervals thereafter. ICG angiography-guided PDT was performed on all eyes. Only the area of the active PCV or “hot spot” evident on the ICG angiogram was treated. A spot size was chosen to cover the active neovascular lesion with a 200-&mgr;m border. Retreatment was performed when angiography revealed a recurrent lesion. Results: Thirty eyes with PCV were treated and followed for 1 year. Mean age of the patients was 75 years (range, 55–90 years). These patients were all classified as having occult choroidal neovascularization (CNV) with FA and polypoidal CNV with ICG angiography. Improvement of vision (≥3 lines) was achieved in 15 eyes (50%). Nine eyes had stable vision (30%), and 6 eyes (20%) had a decrease in vision (≥3 lines). Repeated treatment was required in 15 eyes (50%) for an average of 2.2 treatments in 1 year. Conclusion: This study indicates that stabilization or improvement of vision is achieved in most eyes (80%) with neovascular AMD from PCV after selected ICG angiography-guided PDT. These outcomes compare very favorably with those in previous reports on the treatment of occult CNV. Reduced collateral damage to the choriocapillaris and reduced upregulation of vascular endothelial growth factor are presumed to be the explanation for this apparently better outcome. Further studies with longer follow-up are warranted to investigate the long-term efficacy in these conditions.

Surgical hypothesis: inner retinal fenestration as a treatment for optic disc pit maculopathy.

Optic nerve pits are associated with serous detachment of the macula,1 often with a concomitant ophthalmoscopic appearance of the inner retina mimicking retinoschisis.2 Optical coherence tomography of the maculopathy has demonstrated gross thickening and cystoid degeneration of the inner retina, particularly the nasal macula, with fluid also accumulating in, and under, the central macula.3 The source of the fluid has been open to discussion because there does not appear to be a breakdown in the blood-retina barrier as demonstrated by the lack of leakage during fluorescein angiography. Cerebral spinal fluid (CSF) may or may not be the source, but seems unlikely because of the differences in hydrostatic pressure between the CSF and the intraocular pressure and the inability to displace the macular fluid back to the CSF.1 Histopathologic analysis of a collie dog model of optic disk pits suggested there may be a connection between the vitreous and the macular fluid,4 although this has not been demonstrated in humans.

Congenital uveal malignant melanoma : a case report

CASE REPORT We present a case of congenital choroidal mass in a male infant with multiple cutaneous pigmented lesions. Enucleation performed in the first weeks of life confirmed the diagnosis of diffuse uveal malignant melanoma with extraocular extension. The patient was also treated with 5 cycles of chemotherapy. He subsequently developed cutaneous and ocular pigmented lesions, including 2 choroidal nevi located within the posterior pole and a benign conjunctival lesion in the opposite eye, in addition to malignant melanoma of the skin. COMMENTS At 10 years follow-up, the child is clinically well with no evidence of further malignancy. We believe this represents the fourth case ever reported in the literature and the longest follow-up of a congenital malignant melanoma originating within the eye.

Anecortave Acetate For The Treatment Of Idiopathic Perifoveal Telangiectasia: A Pilot Study

Purpose: To investigate the use of anecortave acetate, a new angiostatic cortisene, for the treatment of the leakage and/or neovascularization associated with idiopathic perifoveal telangiectasia (IPT) in an open label prospective pilot study. Methods: Seven eyes of six patients were treated with posterior juxtascleral administration of anecortave acetate delivered adjacent to the macula using a specially designed curved cannula. A full clinical examination and fluorescein angiography were performed at baseline and at 3-month intervals. The primary efficacy outcome for this pilot study was the mean change in visual acuity (Early Treatment Diabetic Retinopathy Study) from baseline. Results: The visual acuity remained unchanged in two eyes of two patients with nonproliferative disease after 24 months. The five eyes of four patients presenting with subretinal neovascularization, the proliferative stage of IPT, showed stabilization or improvement of lesion size, resolution of leakage, and stabilization of vision at last follow-up. Conclusion: The results of this study suggest that anecortave acetate may inhibit retinal and subretinal permeability as well as neovascular proliferation in patients with IPT. A larger study accordingly should be designed in the future to evaluate the effectiveness and treatment of IPT with this drug.

Optical Coherence Tomography in Asteroid Hyalosis

Purpose: To demonstrate the clinical utility of optical coherence tomography (OCT) in diagnosing macular structural abnormalities in patients with asteroid hyalosis. Methods: Case series. Results: Seven eyes of seven patients underwent OCT due to inadequate funduscopic visualization secondary to asteroid hyalosis. Fluorescein angiography and B-scan ultrasonography were conducted for two patients but failed to provide diagnostic clarity. OCT provided definitive anatomical diagnoses that included macular epiretinal membrane, macular hole, traction retinal detachment, cystoid macular edema, and drusen. On the basis of OCT-assisted diagnoses, three patients elected to undergo surgical intervention. Conclusion: OCT can be critical to diagnose macular conditions when retinal visualization is limited by asteroid hyalosis.

Stellate Nonhereditary Idiopathic Foveomacular Retinoschisis

PURPOSE To describe a new classification of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR). DESIGN Retrospective case series and literature review. PARTICIPANTS A total of 17 patients from 5 institutions. METHODS Detailed case history, multimodal imaging, and genetic testing were reviewed for patients with macular schisis without a known predisposing condition. Patients with a stellate appearance centered on the fovea with correlating confirmed expansion of the outer plexiform layer (OPL) by optical coherence tomography (OCT) were included. Exclusion criteria included a family history of macular retinoschisis, a known genetic abnormality associated with retinoschisis, myopic traction maculopathy, epiretinal membrane, vitreoretinal traction, optic or scleral pit, or advanced glaucomatous optic nerve changes. MAIN OUTCOME MEASURES Clinical features, anatomic characteristics, and visual acuity. RESULTS A total of 22 eyes from 16 female patients and 1 male patient with foveomacular schisis were reviewed from 5 institutions. Initial visual acuity was ≥ 20/50 in all eyes (mean, 20/27), but visual acuity in a single eye decreased from 20/20 to 20/200 after the development of subfoveal fluid. The refractive status was myopic in 16 eyes, plano in 3 eyes, and hyperopic in 2 eyes. Three eyes had a preexisting vitreous separation, and 19 eyes had an attached posterior hyaloid. Follow-up ranged from 6 months to >5 years. CONCLUSIONS In this largest known series of patients with SNIFR, all patients demonstrated splitting of the OPL in the macula with relatively preserved visual acuity (≥ 20/40) except in a single patient in whom subretinal fluid developed under the fovea.

Immunogammopathies and acquired vitelliform detachments: a report of four cases.

PURPOSE To describe the nature and evolution of acquired macular detachments in patients with immunogammopathies and to propose a mechanism for their development. DESIGN Retrospective observational case series. METHODS Three patients with multiple myeloma and 1 with light chain deposition disease were diagnosed with vitelliform macular detachments based on clinical examination, fundus autofluorescence, fluorescein angiography, and optical coherence tomography. These patients were followed over time and their clinical examinations and imaging studies were compared and contrasted. RESULTS Three patients (5 eyes) with multiple myeloma and 1 patient (2 eyes) with light chain deposition disease presented with acquired macular yellowish subretinal deposits on funduscopic examination that corresponded to hyperautofluorescent lesions on fundus autofluorescence imaging and subretinal hyperreflective material on spectral-domain optical coherence tomography. One patient (2 eyes) had diffuse serous retinal detachments involving not only the macular region but also the midperiphery of the retina. These acquired macular vitelliform detachments were not associated with signs of hyperviscosity retinopathy in 5 eyes and resolved after successful treatment of the multiple myeloma in 6 eyes. CONCLUSION Patients with an immunogammopathy such as multiple myeloma or light chain deposition disease may develop serous elevations of the macula that we classify as acquired vitelliform detachments using multimodal imaging. Appropriate evaluation including serum protein electrophoresis and hematology consultation should be considered in the management of patients with acquired vitelliform detachments of uncertain etiology.

Central serous chorioretinopathy in patients with keratoconus.

Purpose: To report the occurrence of central serous chorioretinopathy in patients with keratoconus and discuss the possible connection between these two conditions. Methods: Observational case series. Results: The authors identified three subjects with keratoconus and central serous chorioretinopathy. All patients underwent visual acuity measurement, fundus examination, digital fluorescein angiography, and optical coherence tomography. Conclusions: Keratoconus and central serous chorioretinopathy are two uncommon diseases, possibly due to dysfunction of epithelium and its basement membrane, which can occur together in some individuals. The authors discuss the possible connection between these two conditions.

Focal Retinal Pigment Epithelium Breaks In Central Serous Chorioretinopathy

PURPOSE To describe new evidence supporting a theory on the etiology of the focal retinal pigment epithelium (RPE) leak in central serous chorioretinopathy (CSC). METHODS The records of two patients with CSC were reviewed, including examination details and results of fluorescein angiography, fundus autofluorescence imaging, and optical coherence tomography (OCT). RESULTS A defect in the RPE monolayer demonstrated with OCT that corresponded to the site of the focal leak shown by fluorescein angiography was found in both patients. CONCLUSION The focal leak shown by fluorescein angiography in patients with active CSC is caused by an RPE defect in at least some cases. This finding supports a previously reported theory on the pathogenesis of CSC.