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Congenital hypertrophy of retinal pigment epithelium: a clinico-pathological case report
  1. M A Parsons1,
  2. I G Rennie2,3,
  3. P A Rundle3,
  4. S Dhingra3,
  5. H Mudhar4,
  6. A D Singh5
  1. 1Ophthalmic Sciences Unit, University of Sheffield, Sheffield, UK
  2. 2Department of Ophthalmology and Orthoptics, University of Sheffield, Sheffield, UK
  3. 3Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, UK
  4. 4Department of Histopathology, Royal Hallamshire Hospital, Sheffield, UK
  5. 5Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH, USA
  1. Correspondence to: Arun D Singh MD, Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA; singhaccf.org

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Congenital hypertrophy of retinal pigment epithelium (CHRPE) is a peculiar congenital anomaly of the retinal pigment epithelium (RPE) diagnosed by its characteristic ophthalmoscopic appearance.1 It is now realised that sporadic CHRPE is distinct from the similar appearing retinal lesions described in patients with Gardner’s syndrome.2–4 We recently enucleated an eye with a choroidal melanoma that also had a distinct area of solitary CHRPE with lacunae formation. This provided us with a unique opportunity to correlate clinical and histopathological features of a solitary CHRPE.

Case report

A 62 year old woman with a large cilio-choroidal melanoma was observed to have an elliptical retinal pigment epithelial lesion about 1 mm temporal to the foveola (fig 1A). The lesion was about 3 mm×2 mm in basal dimension and appeared flat. The lesion was depigmented in the nasal aspect with scalloped hyperpigmentation temporally. The eye was enucleated and processed routinely for histological examination.

Figure 1

 (A) Fundus photograph of the left eye showing an elliptical area of congenital retinal pigment epithelial (RPE) lesion temporal …

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