Fundus Changes Associated With Congenital Hypertrophy of the Retinal Pigment Epithelium

Things that go bump in the light. The differential diagnosis of posterior uveal melanomas

“…They are flat or minimally elevated, well circumscribed, deeply pigmented lesions which may vary in size from less than 1 mm to occupy up to one quarter of the fundus. [83][84][85] Typically, they are dark grey or black in colour and contain foci of depigmentation known as lacunae, which frequently enlarge with time 84,85 ( Figure 9). A circumferential depigmented ring (marginal halo) has been observed in over 75% of cases.…”

“…A circumferential depigmented ring (marginal halo) has been observed in over 75% of cases. 85 Fluorescein angiography shows a blocking of the normal choroidal fluorescence corresponding to the pigmented areas with hyperfluorescence corresponding to the lacunae and halo regions. 84 Retinal vascular abnormalities overlying the lesion are common and include capillary loss, microaneurysmal capillary ectasias and rarely, retino-choroidal anastamoses.…”

“…88,89 However, in a recent study of 35 patients with solitary CHRPEs, enlargement although often subtle, was observed in 74% of cases. 85 To date, there have been no recorded cases of malignant transformation, Shields et al 90 recently reported five cases of a nodular tumour arising from a pre-existing CHRPE lesion. The lesions ranged in colour from light brown to black and had minimally dilated, nontortuous feeder and drainage blood vessels supplying the tumour.…”

Congenital hypertrophy of the retinal pigment epithelium complicated by a choroidal neovascular membrane

“…Some authors believe that these flatter RPE cells may induce pressure on the hypertrophic RPE cells present within the CHRPE lesion, creating areas of micro-trauma at the junction between the two. 2 Breaks in the RPE associated with these areas of micro-trauma may be associated with RPE cell migration into the inner retina and may allow the subretinal In our case, the lesion was fragmented and associated with three satellite lesions, which may have resulted from breaks in the RPE and possibly Bruch's membrane and subsequent migration of hypertrophic epithelial cells. This contrasts with the behavior of a typical CHRPE plaque, where migration of RPE cells results in enlargement of atrophic lacunae with subsequent concentric enlargement of the lesion.…”

“…Fluorescein angiography often reveals retinal vascular changes, including attenuation of the retinal capillary bed and the presence of microaneurysms. 2,4 Case Report A 66-year-old woman presented to CHUM Hôpital Notre-Dame, Montréal (PQ), Canada, in July 2005 with a 3-month history of decreased visual acuity and a central "dark spot" in her left eye. She denied other ocular symptoms, including metamorphopsia or micropsia.…”