Congenital grouped pigmentation of the retina. Histopathologic description and report of a case
J. A. Shields and M. O. Tso
Gross examination of an eye removed at autopsy from a 4-year-old child with
congenital heart disease revealed typical grouped pigmentation of the
retina. Routine light microscopy and flat preparation of the retinal
pigment epithelium demonstrated the lesions to consist of focal areas of
increased concentration of pigment granules in otherwise normal retinal
pigment epithelial cells. The pigment granules were large and football
shaped. The overlying photoreceptors showed no abnormalities, possibly
explaining the clinical features of normal visual acuity and visual fields
in this patient. We believe that this is the first histologic description
of congenital grouped pigmentation reported in the English literature. A
differential diagnosis and brief review of the pertinent literature are
also presented.