Faculty Bibliography

PURPOSE: To investigate the anatomic characteristics of peripapillary intrachoroidal cavitation using optical coherence tomography methodologies that are capable of deeper tissue penetration and consider pathophysiologic mechanisms of disease on the basis of the derived imaging information. METHODS: Consecutive eyes with peripapillary intrachoroidal cavitation were imaged with swept source optical coherence tomography with a 1-mm light source and in one eye with enhanced depth imaging spectral domain optical coherence tomography and 3-dimensional rendering. The anatomic layers were identified, and the induced abnormalities were evaluated. RESULTS: There were 16 eyes of 13 patients who had a mean age of 50.3 years and a mean spherical refraction of -12.5 diopters in the affected eyes. The lesion appeared as a yellowish-orange lobular region, usually inferior to the optic nerve, which was invariably tilted. The sclera was bowed posteriorly under the region of the intrachoroidal cavitation, while the overlying retina-retinal pigment epithelium-Bruch membrane complex showed little, if any deformation. Full-thickness defects in the retina at the inferior border of the conus were seen in four eyes and were associated with prominent cavitation within the choroid with marked posterior bowing of the sclera, but negligible deformation of the overlying retina inferior to the nerve. CONCLUSION: Newer imaging modalities provided information about deeper structures in the eye not available in older studies that were performed with time-domain optical coherence tomography. This study demonstrated posterior deformation of the sclera in regions previously thinned by the ocular expansion that occurs in high myopia and imaged the resultant effects on the involved choroid.

PURPOSE: The purpose of this study was to describe the association of acquired vitelliform lesion (AVL) and large drusen in patients with non-neovascular age-related macular degeneration. METHODS: A retrospective review of clinical examination and multimodal imaging data of patients with AVL and large drusen seen over a 12-month period was performed. Acquired vitelliform lesion was defined as subretinal accretion of hyperautofluorescent yellowish material within the macular region not due to vitelliform macular dystrophy. Large drusen were diagnosed by the presence of mounded deposits in the subretinal pigment epithelial space between the retinal pigment epithelium and the Bruch membrane using multimodal imaging analysis (color photography, autofluorescence, and spectral domain optical coherence tomography). RESULTS: Thirteen eyes of 9 white patients with a mean age of 74 years were observed to have AVL associated with large drusen. The median visual acuity was 20/60. All AVLs were hyperautofluorescent and were located in the subretinal space between the retinal pigment epithelium and the photoreceptor inner segment/outer segment junction. The AVL in this series had similar color, autofluorescence, and optical coherence tomographic findings as the AVL seen in association with cuticular drusen and subretinal drusenoid deposits. CONCLUSION: Acquired vitelliform lesions, which have previously been related to cuticular drusen and subretinal drusenoid deposits, can occur in association with large drusen. Abnormalities leading to drusen formation or processes that function in parallel to these may be causative in AVL formation.

Eleven patients, 40 to 71 years old, had a choroidal vasculopathy that led to hemorrhagic and exudative macular degeneration. The patients had peculiar polypoidal, subretinal, vascular lesions associated with serous and hemorrhagic detachments of the retinal pigment epithelium. This macular disorder, which we have named idiopathic polypoidal choroidal vasculopathy (IPCV), appears to represent a distinct entity that differs clinically and demograph-ically from age-related macular degeneration (AMD) and other macular diseases associated with subretinal neovascularization. Recognition of this condition is important because it may have specific risk factors, natural course, and management considerations that differ from those of age-related macular degeneration

BACKGROUND: Most patients with central serous chorioretinopathy (CSC) have spontaneous resolution of exudative macular detachments and a good visual prognosis. Patients with CSC have a primary choroidal hyperpermeability problem evident as multifocal areas of hyperpermeability during indocyanine green (ICG) angiography. A small percentage of patients develop chronic or progressive disease with widespread decompensation of the retinal pigment epithelium and severe vision loss. There is no known treatment for this variant of the disorder. PURPOSE: To study ICG-guided photodynamic therapy (PDT) with verteporfin as a potential treatment for patients with chronic CSC. METHODS: Twenty eyes of 15 patients were studied with fluorescein angiography, optical coherence tomography, and ICG angiography to diagnose the maculopathy, monitor the detachments, and localize the choroidal hyperpermeability of the disorder. PDT with ICG guidance was applied to areas of choroidal hyperpermeability, and the patients were observed to determine the anatomic and functional outcomes. RESULTS: Photodynamic therapy guided by ICG was associated with complete resolution of exudative macular detachments in 12 patients and incomplete resolution in the remaining eight eyes. The vision improved in six eyes and remained unchanged in 14 eyes during a mean follow-up of 6.8 months. Six weeks after treatment, the mean visual acuity improved by 0.55 lines, an amount that was marginally significant. There was a significant inverse correlation between the baseline visual acuity and the amount of improvement in acuity at 6 weeks. No patient had any treatment-related side effects. CONCLUSIONS: Indocyanine green angiography-guided PDT with verteporfin seems to aid in the resolution of exudative detachments in patients with chronic CSC. This treatment was associated with a rapid reduction in subretinal fluid and improvement in visual acuity. Although the follow-up time and number of patients in this pilot study were limited, the encouraging results and lack of complications suggest that further study is indicated.

PURPOSE: To study the effects of photodynamic therapy using verteporfin in the treatment of patients with subfoveal polypoidal choroidal vasculopathy (PCV). METHODS: A retrospective chart review of 16 consecutive patients with subfoveal PCV treated with photodynamic therapy using verteporfin was performed. RESULTS: The mean age of the patients involved was 70.5 years. The mean follow-up time was 12 months. The visual acuity improved in 9 (56.3 %), remained the same in 5 (31.3 %), and decreased in 2 (12.5 %). The mean change in visual acuity was an improvement of 2.38 lines, a difference that was highly significant (P = 0.004). The change in visual acuity was negatively correlated with increasing age. The final visual acuity was positively correlated with initial acuity and negatively correlated with age. These results were confirmed by multiple linear regression. No patient had any lasting complication from the treatment. CONCLUSIONS: Subfoveal PCV has no proven method of treatment. Although the follow-up time and the number of patients in this pilot study were limited, the encouraging results and lack of complications suggest that further study is indicated.

PURPOSE: To examine the subarachnoid space (SAS) of eyes with pathologic myopia and analyze the characteristics of the SAS and the surrounding tissues by swept-source optical coherence tomography (OCT). METHODS: One hundred thirty-three eyes of 76 patients with pathologic myopia (spherical equivalent refractive error of >-8.00 diopters (D) or an axial length >26.5 mm) and 32 eyes of 32 subjects with emmetropia were enrolled. The eyes in both groups were not tested to determine whether glaucoma was present. The papillary and peripapillary areas were examined with a swept-source OCT prototype system that uses a wavelength sweeping laser operated at 100,000 Hz A-scan repetition rate in 1-mum wavelength. RESULTS: In the B-scan images, the arachnoid trabeculae inside the SAS were clearly observed as a pattern of reticular lines and dots interspersed with hyporeflective zones consistent with fluid, whereas orbital fat had more uniform features with gray intervening spaces. The SAS was triangular, with the base toward the eye surrounding the optic nerve in the region of the scleral flange. An SAS was found in 124 highly myopic eyes (93.2%) but not in the emmetropic eyes. The shortest distance between the inner surface of lamina cribrosa and SAS was 252.4 +/- 110.9 mum, and the thinnest region of peripapillary sclera above SAS (scleral flange thickness) was 190.6 +/- 51.2 mum. In one myopic patient, there appeared to be direct communication between the intraocular cavity and SAS through pitlike pores. CONCLUSIONS: Optic SAS is seen in 93% of highly myopic eyes, and the SAS appears to be dilated in highly myopic eyes. The expanded area of exposure to CSF pressure along with thinning of the posterior eye wall may influence staphyloma formation and the way in which certain diseases, such as glaucoma, are manifested.

OBJECTIVE: To describe the clinical and imaging findings in patients with focal choroidal excavation. METHODS: Retrospective observational case series. The medical records of 12 patients (13 eyes) with focal choroidal excavation were reviewed. Clinical histories and imaging findings (including color photography, fundus autofluorescence imaging, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and enhanced depth imaging spectral-domain optical coherence tomography) were analyzed. RESULTS: The mean age of the patients was 45 years (range, 22-62 years). Four patients were Asian. Mean visual acuity was 20/31 (range, 20/20 to 20/100). Mean refractive error was -3.54 diopters (D) (range, 6.00 to -8.00 D). One patient had bilateral involvement. All patients manifested varying degrees of foveal pigmentary changes that were usually hypoautofluorescent on fundus autofluorescence images. Fluorescein angiographic findings varied with degree of retinal pigment epithelial alterations. Indocyanine green angiography revealed relative hypofluorescence. In 7 eyes, spectral-domain optical coherence tomography revealed outer retinal layers conforming to retinal pigment epithelial alterations within the excavation. In the other 6 eyes, spectral-domain optical coherence tomography revealed a separation between the outer retina and the retinal pigment epithelium within the excavation. In 7 eyes studied with enhanced depth imaging spectral-domain optical coherence tomography, there was no evidence of scleral ectasia. Mean choroidal thickness of the uninvolved choroid was thicker than normal at 319 mum (range, 244-439 mum). All lesions remained stable except for in 1 eye, which had findings of central serous chorioretinopathy and secondary type 2 (subretinal) neovascularization. CONCLUSION: Focal choroidal excavation is a newly described idiopathic entity in eyes having 1 or more focal areas of choroidal excavation. In some patients, there may be an association with central serous chorioretinopathy. Although most lesions remain stable, secondary choroidal neovascularization may occur.

PURPOSE: To evaluate the validity of commonly used anatomical designations for the four hyperreflective outer retinal bands seen in current-generation optical coherence tomography, a scale model of outer retinal morphology was created using published information for direct comparison with optical coherence tomography scans. METHODS: Articles and books concerning histology of the outer retina from 1900 until 2009 were evaluated, and data were used to create a scale model drawing. Boundaries between outer retinal tissue compartments described by the model were compared with intensity variations of representative spectral-domain optical coherence tomography scans using longitudinal reflectance profiles to determine the region of origin of the hyperreflective outer retinal bands. RESULTS: This analysis showed a high likelihood that the spectral-domain optical coherence tomography bands attributed to the external limiting membrane (the first, innermost band) and to the retinal pigment epithelium (the fourth, outermost band) are correctly attributed. Comparative analysis showed that the second band, often attributed to the boundary between inner and outer segments of the photoreceptors, actually aligns with the ellipsoid portion of the inner segments. The third band corresponded to an ensheathment of the cone outer segments by apical processes of the retinal pigment epithelium in a structure known as the contact cylinder. CONCLUSION: Anatomical attributions and subsequent pathophysiologic assessments pertaining to the second and third outer retinal hyperreflective bands may not be correct. This analysis has identified testable hypotheses for the actual correlates of the second and third bands. Nonretinal pigment epithelium contributions to the fourth band (e.g., Bruch membrane) remain to be determined.

PURPOSE: To provide a comprehensive description of chorioretinal layer thicknesses in the normal human macula, including two-layer pairs that can produce a combined signal in some optical coherence tomography (OCT) devices (ganglion cell [GCL] and inner plexiform [IPL] layers and outer plexiform [OPL] and outer nuclear [ONL] layers). METHODS: In 0.8-mum-thick, macula-wide sections through the foveola of 18 donors (age range, 40-92 years), 21 layers were measured at 25 locations by a trained observer and validated by a second observer. Tissue volume changes were assessed by comparing total retinal thickness in ex vivo OCT and in sections. RESULTS: Median tissue shrinkage was 14.5% overall and 29% in the fovea. Histologic laminar boundaries resembled those in SD-OCT scans, but the shapes of the foveolar OPL and ONL differed. Histologic GCL, IPL, and OPLHenle were thickest at 0.8. to 1, 1.5, and 0.4 mm eccentricity, respectively. ONL was thickest in an inward bulge at the foveal center. At 1 mm eccentricity, GCL, INL, and OPLHenle represented 17.3% to 21.1%, 18.0% to 18.5%, and 14.2% to 16.6% of total retinal thickness, respectively. In donors >/= 70 years of age, the RPE and choroid were 17.1% and 29.6% thinner and OPLHenle was 20.8% thicker than in donors <70 years. CONCLUSIONS: In this study, the first graphic representation and thickness database of chorioretinal layers in normal macula were generated. Newer OCT systems can separate GCL from IPL and OPLHenle from ONL, with good agreement for the proportion of retinal thickness occupied by OPLHenle in OCT and histology. The thickening of OPLHenle in older eyes may reflect Muller cell hypertrophy associated with rod loss.