Congenital Hypertrophy of the RPE (CHRPE)

Optomap (1), red separation (2) and green separation (3) images - Left eye

Optomap imaging shows a dark-grey lesion with defined scalloped borders and islets of internal atrophy in the temporal midperiphery of the left eye. It is approximately 4.5DD in size. The lesion is visible on both green and red separation images.

Spectralis OCT line scans taken through the lesion

OCT imaging through the lesion shows thinning of the outer retinal layers and focal areas of marked RPE loss corresponding to the islets of internal atrophy.

Spectralis OCT line scans taken through the lesion

OCT imaging through the lesion shows thinning of the outer retinal layers and focal areas of marked RPE loss corresponding to the islets of internal atrophy.

Fundus photograph (1) green free (2) and red free (3) images - Right eye

Fundus photos show an oval, densely pigmented RPE lesion with distinct margins located in the temporal periphery of the right eye. There is no lacunae.

Optomap (1), red separation (2) and green separation (3) images - Right eye

Optomap imaging show an oval, densely pigmented RPE lesion with distinct margins located in the temporal periphery of the right eye. There is no lacunae.

Fundus Autofluorescence Imaging (right eye)

FAF imaging showed marked hypo-autofluorescence

Spectralis OCT line scans taken through the lesion

OCT imaging shows a flat lesion with subtle outer retinal thinning and RPE thickening. There are no signs of lacunae or subretinal fluid.

Optomap (1), red separation (2) and green separation (3) images - Right eye

Optomap imaging shows a large, solitary pigmented lesion with sharp borders and internal atrophy (lacunae). The large choroidal vessels are visible through the lacunae.

Optomap (1), red separation (2) and green separation (3) images - Left eye

Optomap imaging shows a large pigmented lesion with well defined borders in the superior midperiphery of the left eye. There are also multiple regions of internal atrophy (lacunae) with visibility of the large choroidal vessels.

Fundus autofluorescence (left eye)

FAF imaging shows marked hypo-autofluorescence

Spectralis OCT line scans taken through the lesion

OCT imaging shows thickening of the RPE and thinning of the overlying retina. The lacunae shows a focal absence of RPE with posterior hypertransmission.

Fundus photograph (1) green free (2) and red free (3) images - Left eye

Fundus photos shows a well defined dark-grey lesion at the inferotemporal vascular arcade of the left eye. There is no lacunae but the outer region of the lesion appears less pigmented.

Optomap (1), red separation (2) and green separation (3) images - Left eye

Optomap imaging shows a well defined dark-grey lesion at the inferotemporal vascular arcade of the left eye. There is no lacunae but the outer region of the lesion appears less pigmented.

Fundus autofluorescence (left eye)

FAF imaging shows marked hypo-autofluorescence

Spectralis OCT line scans taken through the lesion

OCT imaging shows a thickened RPE with thinning of the overlying retina and a subretinal cleft.

Subretinal clefts can occur in 33% of cases and is thought to be an empty space left by photoreceptor atrophy and a thinned excavated posterior retina.

Optomap (1), red separation (2) and green separation (3) images - Right eye

Optomap imaging shows a large pigmented lesion in the superonasal midperiphery of the right eye. It displays a slightly irregular surface centrally and the large choroidal vessels are visible inferiorly.

Fundus autofluorescence imaging (right eye)

FAF imaging shows a marked hypo-autofluorescence with pinpoints of hyper-autofluorescence centrally.

Spectralis OCT line scans taken through the lesion

OCT line scan (top) at the temporal border shows a focal region of RPE attenuation with posterior hypertransmission.
OCT line scan (bottom) shows a thickened RPE with overlying disturbance/ thinning of the retina. There are also hyper reflective spots within the retina representing migrated pigment clumps.