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A. Mashayekhi, C.L. Shields, T. Ho, J. Cater, J.A. Shields; Solitary Congenital Hypertrophy of the Retinal Pigment Epithelium: Clinical Features and Frequency of Enlargement in 330 Patients . Invest. Ophthalmol. Vis. Sci. 2003;44(13):2155.
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Purpose: To describe the clinical features of solitary congenital hypertrophy of the retinal pigment epithelium (CHRPE) and to determine the frequency of enlargement of this lesion. Methods: Review of the clinical records of 330 patients with solitary CHRPE referred to the Ocular Oncology Service at Wills Eye Hospital, Philadelphia, PA between January 1974 and July 2002 . Results: The most common referring diagnosis included choroidal nevus (26%), choroidal melanoma (15%), CHRPE (9%), and unspecified lesion (48%). The median age at diagnosis was 45 years (range 1 to 80 years) and there were no patients with familial adenomatous polyposis or related colon cancer, but a history of cancer was noted in 8% of patients, most commonly breast cancer (3%). The lesion was most frequently located inferotemporally (31%) at the equatorial region (45%). Rarely was it located in the macula (1%) or peripapillary region (1%). The median largest basal diameter was 4.5 mm and the lesion was flat in all cases, except in 5 cases (1.5%) in which there was an intralesional lesion nodule. The lesion was pigmented in 88% of cases and nonpigmented in 12%. Lacunae were noted in 43% of the pigmented CHRPE and the lacunae showed gradual enlargement in 32%. Factors related to lacunae enlargement included number and relative size of lacunae. Flat enlargement of the lesion was documented in 46% of patients with comparative photographic follow up and in 83% of those followed for over 3 years. The median rate of enlargement was 10.2 microns per month. The only factor associated with flat lesion enlargement was relative size of the lacunae within CHRPE. There were no cases of CHRPE that developed a nodule while under observation. Of the 5 lesions that had a nodule, progressive enlargement of the nodule was found in 3. Conclusions: CHRPE has been generally regarded as a benign stable lesion, but subtle flat enlargement was noted in the majority (83%) of patients followed for 3 or more years using meticulous photographic comparison. Flat enlargement of the lesion appeared to be related to percentage of the mass occupied by lacunae.
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