Combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) is a rare benign lesion that may present with marked vitreoretinal distortion and simulate other tractional or exudative retinal disorders. A 53-year-old woman was referred to the ophthalmic emergency department for suspected retinal detachment in the left eye and presented with hand-motion visual acuity. Multimodal imaging, including infrared reflectance, blue reflectance, blue-light autofluorescence, multicolor imaging, fluorescein angiography (FA), and spectral-domain optical coherence tomography (SD-OCT), demonstrated a large epi-papillary fibroglial lesion with severe posterior pole distortion. Infrared and multicolor imaging highlighted radially dragged retinal vessels, fibrogliotic extensions spreading temporally from the lesion, and marked corrugation of the posterior pole. Blue-light autofluorescence showed a predominantly hypoautofluorescent mass with multiple focal hyperautofluorescent areas at the temporal margin, corresponding to areas of maximal traction. FA revealed early masking hypofluorescence followed by progressive staining of intrinsic fibrovascular tissue and abnormal vascular loops at the disc margin, without peripheral anomalies. SD-OCT was crucial in clarifying the structural changes, disclosing a massive dome-shaped epipapillary elevation, full-thickness retinal disorganization, broad splitting between inner and outer retinal layers, multiple schitic cavities, and a temporally located posterior pole sector of abnormally hyperreflective, disorganized inner retinal layers compatible with chronic traction‑related ischemia. This case emphasizes the value of multimodal imaging in detecting remarkable retinal alterations in a case of advanced epipapillary CHRRPE. Accurate cross-correlation among imaging modalities is essential for diagnosis, prognosis, and surgical decision-making.
Brocca et al. (Fri,) studied this question.