case_images: PMC10481987_01
case_images: Color fundus photo of the left eye disclosing epiretinal membrane involving the papillomacular bundle and areas of hyperpigmentation along with proliferative glial tissue at the inferior border of the lesion that is compatible with diagnosis of combined hamartoma of the retina and retinal pigment epithelium (a). Fluorescein angiography of the same eye. Tortuous vessels with intervening areas of blocked fluorescence are evident in early phases (b). In the late angiograms, hyper fluorescence is seen due to leakage from the intrinsic vasculature of the lesion (c)
case_images: JCO-35-90-g001.jpg
case_images: PMC10481987_01_JCO-35-90-g001.jpg
case_images: joco_30_22-f001
case_images: A dense white glial proliferation was also noted at the inferior border of the tissue [Figure 1a]. Dye leakage from the tortuous vasculature was evident in the late angiograms [Figure 1b and c].
case_images: The horizontal optical coherence tomography (OCT) scan of the inferior part of the lesion showing the folding of the redundant retina on itself (arrow). By following the contour of the retinal fold all retinal layers are recognizable (e.g., the red line follows the contour of the inner plexiform layer). The defect is filled by the glial tissue (asterisk). It is of note that the outer retina is intact nasal to the defect (blue line). In addition, omega sign a characteristic sign of combined hamartoma of the retina and retinal pigment epithelium is evident in the OCT (dashed circle) (a). OCT angiography of the corresponding area visualized the disorganized and tortuous vessels in the superficial (b) and deep capillary plexuses (c). The structural OCT with angio-overlay (d) shows blood flow signal in the area of folded retina (dashed eclipse) which supports the double retina sign
case_images: JCO-35-90-g002.jpg
case_images: PMC10481987_01_JCO-35-90-g002.jpg
case_images: joco_30_22-f002
case_images: Proliferation of RPE layer was also seen beneath the areas of retinal thickening [Figure 2a]. The disorganized vessels were also disclosed in OCT angiography scans [Figure 2b and c]. The OCT B-scan with angio-overlay disclosed areas of flow signal indicative of the folded retina [Figure 2d].