Multiple atrophic lesions in the pigment epithelium of various forms and sizes are seen in the far-periphery of the inferior quadrant of the right eye. Some lesions merge, others are isolated. Pigment clumps of various forms and intensity are located within and between the chorioretinal atrophy lesions. There is a clearly seen choroidal pattern in the atrophic areas.

The retinal surface is irregular and wavy. Multiple areas of pigment epithelium destruction are observed. The reflectivity in the neurosensory retina layers is increased. Neurosensory retina is thinned at the level of the pigment epithelium destruction, and hyperreflective shadows are observed at the choroidal level because of increased retinal echogenicity and light permeability. The ellipsoid zone is not seen. Choroid hyperreflectivity correlates with the areas of pigment epithelium destruction. Multiple hyperreflective deposits are present in the vitreous, there is no evidence of vitreous traction.

An asymptomatic 40-year-old female patient with mild myopia came to have distance glasses prescribed. Fundus examination revealed an atrophic lesion with sharp borders in the inferotemporal quadrant of her left eye. No signs of traction were noted.

A round-shaped area of atrophic pigment epithelium with clearly outlined borders is seen at the 7 o’clock position in the mid-periphery of the inferonasal quadrant of the left eye. Pigment clumps are observed around the borders.

The retinal profile is deformed, the structure of all layers is altered. Neurosensory retina is thinned, neuroepithelial surface is slopy on one side of the lesion and excavated on the other side. The pigment epithelium is hyporeflective and atrophic. The ellipsoid zone is not distinguishable. Choroidal reflectivity and light transmittance in the atrophic area is considerably increased.

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is a common benign retinal condition [6]. According to some authors, CHRPE is characterized by pigment deposits found along the ora serrata, which may extend as far as equator. In early stages pigment spots and clumps reach 1/8 PD, and in advanced stages the lesion occupies 1/4–1.0 PD [3]. Other authors claim that CHRPE can be unifocal or multifocal (Table 6.2). Unifocal CHRPE is a round or oval flat lesion, 1–2 PD in size, with well-defined borders, that can be deep gray or black. The lesion can be surrounded by a hypopigmented halo. Multifocal CHRPE appears as grouped pigmented deep gray or black spots of various sizes, that resemble animal tracks (“bear tracks”) [5].

Histological studies have established CHRPE to be a result of pigment redistribution [2, 3]. Patients with multifocal CHRPE (bear tracks) should be evaluated for familial adenomatous polyposis of colon and rectum, which may lead to colorectal cancer after 50 years of age [5, 6].

As reported by many authors, CHRPE is not usually associated with vitreous traction, changes in the vitreoretinal interface, or risk of retinal detachment. However, there have been reports of retinal tears in 11.9 % of cases of advanced CHRPE [3]. Nonetheless, prophylactic laser treatment for this condition is not indicated [5].