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D. M. Monson, J. R. Smith, M. L. Klein, D. J. Wilson; Clinicopathologic Correlation of Retinal Angiomatous Proliferation. Invest. Ophthalmol. Vis. Sci. 2008;49(13):3407.
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To correlate the clinical and histopathologic features of an eye with retinal angiomatous proliferation (RAP) secondary to age-related macular degeneration (ARMD), and to investigate the expression of von Willebrand factor (VWF) and vascular endothelial growth factor (VEGF) in this condition.
Histopathologic features from serial sections through the globe of an 87-year-old female with RAP were studied and compared to fluorescein angiography (FA) and color fundus photographs obtained 4 months before death. An indirect immunostaining method incorporating an antigen retrieval step and using commercially available antibodies was used to detect expression of VWF and VEGF in tissue sections.
Fundus examination of the right eye revealed an intraretinal vascular lesion nasal to the fovea with associated intraretinal hemorrhages. A pigment epithelial detachment (PED) involving the fovea, large soft drusen, and changes in the retinal pigment epithelium (RPE) were also present.FA revealed a hyperfluorescent intraretinal vascular complex nasal to the fovea, associated telangiectatic vessels and intraretinal blocked fluorescence with late leakage. A large PED, cystoid macular edema, and a small area of defect in the RPE were present. These features are all histopathologically correlated.The pathologic correlate of RAP is a circumscribed intraretinal angiomatous complex within the outer part of the neurosensory retina overlying a large PED. There are no breaks in Bruch’s membrane. There is no choroidal neovascularization present. Endothelial cells within the RAP lesion immunostain positively for VWF and VEGF. Bruch’s membrane expresses VWF adjacent to the RAP.
Fundus examination and FA images of RAP in a patient with ARMD correlate histopathologically to a neovascular intraretinal angiomatous complex, without the presence of sub-RPE neovascularization. Immunostaining demonstrates that RAP expresses VWF and VEGF.
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