Combined hamartoma of the retina and retinal pigment epithelium

Abstract

We report two cases of combined hamatoma of the retina and retinal pigment epithelium (CHR-RPE), illustrated with ultrasonography, optical coherence tomography, fundus fluorescein angiography and indocyanine green angiography images. CHR-RPE could clinically mimic several other retinal conditions. Failure to distinguish it from serious malignancies such as choroidal melanoma or retinoblastoma has led to unnecessary enucleation in the past. Through these case reports and a review of literature, we show the diagnostic features of CHR-RPE, its key differential diagnoses and the management options.

Figure 1

(A) Colour photograph of the posterior pole showed a grey elevated lesion involving the disc and macula with tortuous blood vessels. An overlying translucent fibrotic membrane was seen extending from 12–6 o'clock in a crescent configuration (red arrows). (B) B-scan ultrasonography showed the lesion to be slightly elevated (by 1 mm) with medium-to-high reflectivity (arrow). (C and D) Optical coherence tomography sections through the macula and disc, respectively, revealed thickened retina with loss of architectural layers, and a pre-retinal membrane causing vitreoretinal traction (arrow).

Figure 2

(A) Colour photograph of the right posterior pole showed an elevated lesion involving the inferior macula with a hyperpigmented margin which blends into the surrounding tissue. A grey-white fibrotic thickening is present over the surface of the lesion. (B) B-scan showed the lesion (arrow) to be slightly elevated with low-to-medium reflectivity. (C) Optical coherence tomography section through the centre of the lesion showed grossly thickened and disorganised retina with a tractional pre-retinal membrane (arrow). (D) Fundus fluorescein angiography demonstrated blockage of choroidal fluorescence by the outer pigmented portion of the lesion. Tortuosity of vessels within the lesion was prominent in the arterial phase. There was patchy dye leakage within the lesion and late staining. (E) Indocyanine green (ICG) did not reveal any significant choroidal vascular abnormality. Mild hyperfluorescence corresponding to the lesion and hypofluorescence outlining its border was seen in the late phase.