Sclerochoroidal calcifications imaged using enhanced depth imaging optical coherence tomography.

PURPOSE: To report a case of sclerochoroidal calcifications and describe the appearance of these lesions using optical coherence tomography with enhanced depth imaging (EDI-OCT) technology. DESIGN: Case Report METHODS: An 85-year-old patient who presented with bilateral retinal lesions suspected to be metastases was evaluated with slit-lamp biomicroscopy, funduscopy, fluorescein angiography, B-scan ultrasonography (U/S), and EDI-OCT. RESULTS: B-scan U/S demonstrated echo-dense consolidations in the choroid bilaterally with acoustic shadowing consistent with sclerochoroidal calcifications. EDI-OCT revealed hyper-reflective lesions with decreased reflectivity posterior to the lesion. CONCLUSIONS: Sclerochoroidal calcifications should be included in the differential of choroidal masses, given their unique appearance on EDI-OCT.

Confirmation of and differentiation among primary vascular lesions using ultrasonography.

PURPOSE: To demonstrate the utility of orbital ultrasound in the evaluation and management of suspected orbital vascular lesions. METHODS: Clinical case series of 15 patients with suspected orbital vascular lesions seen in the Neuro-ophthalmology clinic of the Wilmer Eye Institute. The techniques of standardized echography (combined use of a contact B-scan and standardized A-scan) were performed by an experienced echographer, and data obtained were compared with other available imaging modalities to determine appropriate management. Patients were monitored for improvement, stability, or worsening of symptoms and signs. RESULTS: Based on echographic data obtained, 6 patients underwent surgery, 9 were observed over a follow-up period of 0-14 (mean 3.3) years. All patients demonstrated improvement or remained stable, and no complications developed from misdiagnosis during subsequent follow-up. CONCLUSIONS: Orbital ultrasound provides reliable imaging parameters and can be used as the primary imaging modality when evaluating suspected orbital vascular lesions.

The suboptimal role of optical coherence tomography in diagnosing choroidal metastasis in the macula.

PURPOSE: To describe the clinical, optical coherence tomography, and echographic findings of a choroidal metastasis in the macula. An initial, erroneous diagnosis of a serous pigment epithelial detachment of the macula had been based on ophthalmoscopic and optical coherence tomography findings. METHODS: Case report of a patient with blurred vision in one eye and presumed serous pigment epithelial detachment. RESULTS: An 83-year-old man with blurred vision and presumed serous pigment epithelial detachment was self-referred to our clinic for a second opinion. Optical coherence tomography showed an elevation of the neurosensory macula, but no abnormalities were reflected from the choroid. We performed an ultrasound evaluation, which revealed a solid mass involving the macular region of the choroid. The echo-graphic characteristics, including low internal reflectivity, regular structure, and vas-cularity, were consistent with choroidal melanoma or metastasis from any small cell carcinoma. The patient was not aware of any active primary tumor. An extensive systemic workup was performed. His prostate-specific antigen was found to be elevated to 61.4 ng/mL, and a prostate biopsy was positive for adenocarcinoma. Additional studies revealed metastatic disease involving the bones, pulmonary parenchyma, and mediastinal and retroperitoneal lymph nodes. CONCLUSION: Ultrasound imaging was superior to optical coherence tomography imaging in the clinical setting of a choroidal mass in the macula.

Ultrasound features of orbital granular cell tumor.

The authors report the echographic characteristics of a rare orbital granular cell tumor and correlate these findings with histopathology. A 56-year-old woman presented with proptosis. Complete ophthalmic and ultrasound examinations were performed. Ultrasound revealed an oval, well-outlined orbital mass in the intraconal space with low-medium reflectivity and regular internal structure. An orbitotomy with complete excision of the tumor was performed. Histopathologic evaluation showed sheets and nests of cells with abundant eosinophilic and granular cytoplasm in a uniform distribution throughout the lesion. The echographic characteristics correlated well with the morphologic surgical findings and the histologic architecture. This is the first report describing the echographic characteristics of orbital granular cell tumor.

Epibulbar foreign body granuloma masquerading as extrascleral extension following brachytherapy for choroidal melanoma.

PURPOSE: To describe a patient with epibulbar foreign body granuloma following brachytherapy for choroidal melanoma and to illustrate the echographic features that aided in the differential diagnosis. METHODS: Observational case report. Patient had multiple clinical exams over time to evaluate a treated choroidal melanoma. PATIENTS: A 61-year-old man presented for echographic follow-up examination following treatment for choroidal melanoma. An epibulbar lesion was noted adjacent to the site of the previously treated choroidal melanoma. Incisional biopsy and histologic evaluation were required. RESULTS: Ultrasonography revealed regression of the intraocular tumor; however, an oval epibulbar mass with irregular internal structure, irregular reflectivity, and no vascularity was noted. The echographic features were not typical of extrascleral extension of choroidal melanoma. Histopathologic evaluation showed fibrous connective tissue with an intense infiltrate of histiocytes with occasional giant cells and rare birefringent foreign body materials. CONCLUSION: Foreign body granuloma should be considered in the echographic differential diagnosis of an atypical extrascleral lesion following uveal melanoma brachytherapy.

Prevalence of cystoid macular edema and stability in oct retinal thickness in eyes with retinitis pigmentosa during a 48-week lutein trial.

PURPOSE: To describe, in a group of patients with moderately advanced retinitis pigmentosa (RP), the prevalence of cystoid macular edema (CME), the variation in foveal thickness over a 48-week period, the correlation of visual acuity (VA) with retinal thickness, and the lack of response of CME to lutein administration. METHODS: Optical coherence tomography (OCT) imaging of the macula and clinical examination were evaluated for 77 eyes of 39 patients with RP over 11 months, with a scan done every 6 weeks. RESULTS: The prevalence of CME, defined by cysts visible on OCT, was 49%. Bilateral CME was present in 44% of patients (17 of 39), and an additional two patients had unilateral CME. Central retinal thickness varied little over the 48 weeks. Sixty-six percent of the eyes with CME had VA of 20/40 or better. The eyes with CME with VA worse than 20/40 had either greater degrees of thickening or in fact had lower thickness measures. For the eyes without CME, the eyes with VA worse than 20/40 tended to have lower retinal thickness than the eyes with VA of 20/40 or better. VA was highly concordant between eyes, and did not differ significantly between the groups with and without CME. Lutein did not show a statistically significant effect on retinal thickness in the patients with or without CME, nor was such an effect observed in subgroups of patients with vision better or worse than 20/40. CONCLUSION: The prevalence rate of CME is higher than in previous reports, perhaps because the patients had some preserved macular vision and because of the use of a definition based on OCT findings. Retinal thickness remains fairly stable over time, both in eyes with CME and in eyes without CME.

Recurrent vitreous hemorrhage secondary to haptic-induced chafing.

CASE REPORT: After cataract extraction and placement of a posterior chamber intraocular lens, a 70-year-old man had 5 vitreous hemorrhages over 3 years. Iris transillumination defects and apposition of the nasal haptic and ciliary body were found. COMMENTS: Iris chafing is a rare complication of cataract extraction. Vitreous hemorrhage presumably resulted from iris erosion caused by the IOL haptic.

Optical coherence tomography of enucleated human eye specimens with histological correlation: origin of the outer "red line".

PURPOSE: To further define the origin of the "outer red line" (ORL) present in retinal optical coherence tomograms. DESIGN: Laboratory investigation. METHODS: Eleven formalin-fixed caps of seven human eyes previously enucleated for choroidal melanoma at the Wilmer Eye Institute were studied. The ORL evaluation consisted of sequential, surgical elimination of the retinal pigment epithelium, Bruch's membrane (BM), and choroid in separate, but adjacent, areas. Optical coherence tomography (OCT) images through each of these areas were correlated with corresponding histologic sections. The relative contribution of the retinal pigment epithelium, BM, and choroid to the ORL in retinal OCT3 images was evaluated. RESULTS: In the specimens examined, the ORL correlated anatomically with a highly reflective optical surface at the level of the outer retina, and did not appear to represent a specific anatomic layer. The reflecting layers appeared to be composed of the retinal pigment epithelium, BM, and inner choroid. The relative contribution of BM together with the inner choroid to the ORL appeared greater than that of the retinal pigment epithelium. CONCLUSIONS: This study suggests that the ORL present in retinal OCT3 images and generally associated with the retinal pigment epithelium/BM/inner choroidal complex appears to indicate a highly reflective optical surface which, when eye layers are normally configured, represents this complex of anatomic structures with a predominant contribution from BM and inner choroid. The findings of this study suggest a potential role for OCT in the eye pathology laboratory.

Optical coherence tomography of peripheral retinal lesions in enucleated human eye specimens with histologic correlation.

PURPOSE: To report optical coherence tomography (OCT) histologic correlations of three common peripheral retinal lesions and to explore the feasibility of the use of OCT to image retinal pathologic conditions in fixed tissue specimens. DESIGN: Experimental study. METHODS: We studied peripheral retinal lesions that were available to us in 11 formalin-fixed caps of seven human eyes that had been enucleated previously for choroidal melanoma. OCT scans were performed through these lesions, and the images were correlated with corresponding histologic sections. RESULTS: Three peripheral retinal diseases that included cystoid degeneration, localized retinal detachment, and paving stone degeneration were imaged successfully, and their optical characteristics correlated well with their histologic data. CONCLUSION: We explored potential utility for OCT imaging of peripheral retinal pathologic conditions in fixed tissue specimens. That these OCT images correlate with findings from histologic sections suggests a possible role for OCT in the eye pathology laboratory.

Identification of visually significant operculum without macular hole by optical coherence tomography.

CASE REPORT: We report a case that illustrates the effectiveness of optical coherence tomography (OCT) in identifying visually significant vitreoretinal interface opacities in the absence of a macular hole. A patient presented with a scotoma in the right eye. Fundoscopic examination revealed a small lesion in close proximity to the foveal avascular zone. OCT of the right eye revealed a small operculum in the vitreous anterior to the fovea without evidence of a macular hole. COMMENTS: To our knowledge, ours is the first reported case of the use of OCT to identify the presence of a visually significant operculum without an associated macular hole.

Extreme hyperopia is the result of null mutations in MFRP, which encodes a Frizzled-related protein.

Nanophthalmos is a rare disorder of eye development characterized by extreme hyperopia (farsightedness), with refractive error in the range of +8.00 to +25.00 diopters. Because the cornea and lens are normal in size and shape, hyperopia occurs because insufficient growth along the visual axis places these lensing components too close to the retina. Nanophthalmic eyes show considerable thickening of both the choroidal vascular bed and scleral coat, which provide nutritive and structural support for the retina. Thickening of these tissues is a general feature of axial hyperopia, whereas the opposite occurs in myopia. We have mapped recessive nanophthalmos to a unique locus at 11q23.3 and identified four independent mutations in MFRP, a gene that is selectively expressed in the eye and encodes a protein with homology to Tolloid proteases and the Wnt-binding domain of the Frizzled transmembrane receptors. This gene is not critical for retinal function, as patients entirely lacking MFRP can still have good refraction-corrected vision, produce clinically normal electro-retinograms, and show only modest anomalies in the dark adaptation of photoreceptors. MFRP appears primarily devoted to regulating axial length of the eye. It remains to be determined whether natural variation in its activity plays a role in common refractive errors.

Detection of diabetic foveal edema: contact lens biomicroscopy compared with optical coherence tomography.

OBJECTIVE: To compare contact lens biomicroscopy with optical coherence tomography (OCT) for the detection of diabetic foveal edema. METHODS: Study participants consisted of a convenient cohort of consecutive patients with diabetes mellitus seen at the Wilmer Eye Institute's Retinal Vascular Center, Baltimore, MD. Case characteristics were recorded and eyes were examined by 1 of 4 retina specialists by means of contact lens biomicroscopy. Edema involving the center of the macula was assessed as definitely present, questionably present, or definitely not present. The OCT testing was performed and interpreted by trained technicians, masked to the physicians' assessment of foveal edema. Agreement between OCT and contact lens examination for the absence or presence of foveal edema was evaluated. RESULTS: One hundred seventy-two eyes of 95 patients with diabetes were enrolled in August and September 2002. Foveal thickness was objectively measured by OCT in 170 (99%) of 172 cases. We found excellent agreement between OCT and contact lens examination for the absence or presence of foveal edema when OCT thickness was normal (300 microm). However, agreement was poor when foveal thickness was mildly increased on OCT (201-300 microm). CONCLUSIONS: Agreement between contact lens examination and OCT for the detection of diabetic foveal edema is poor when OCT thickening is mild. This suggests that contact lens biomicroscopy is relatively insensitive for the detection of mild foveal thickening apparent on OCT. Additional studies are needed to investigate the natural course of cases with mildly increased foveal thickness on OCT that do not appear thickened clinically.