![]() Consistent with these functional alterations are our morphological findings by spectral-domain OCT indicating an aplasia of the retinal pigment epithelium and a hypoplasia of the outer receptor segments in this patient. For the first time we performed high resolution spectral domain OCT in this pathology and detected an aplasia of the retinal pigment epithelium and a hypoplasia of the outer photoreceptor segments.Ĭonclusion: Congenital albinotic spots of the retinal pigment epithelium or „polar bear tracks“ can be associated with visual dysfunction like in our case with absolute scotomas. These have not been reported to have the potential to transform into adenocarcinoma but yearly evaluations may be prudent. ![]() These visual field defects were not registered by the patient at all which points to a congenital genesis. Several tumors and pseudotumors can arise from the iris pigment epithelium (IPE), ciliary pigment epithelium (CPE), and retinal pigment epithelium (RPE), including cysts of the IPE, solitary congenital hypertrophy of the RPE (CHRPE), multifocal CHRPE ('bear tracks'), congenital simple hamartoma of the RPE, combined hamartoma of the retina and RP. Home Recognizing Pathology Bear Tracks Bear tracks (animal tracks, grouped pigmentation spots) are simply many small CHRPEs located in isolated small area of the retina. Perimetry demonstrated absolute scotomas in the central visual field consistent with the lesions. The autofluorescence imaging showed in two of these lesions a typical „bear track“ configuration. Fundus examination revealed multiple, sharply outlined, non-confluent, asymmetrical, hypopigmented spots involving the macular area of both eyes. Results: The patient had 20/20 vision and regular findings at slit lamp-examination. Methods: Case report of a 54-year old man with bilateral, asymptomatic fundus findings, which were evaluated using autofluorescence imaging, perimetry, and high resolution spectral domain OCT. Multiple areas of grouped congenital hypertrophy of the retinal pigment epithelium (CHRPE) which are. Functional as well as morphological changes of these lesions have been previously discussed controversially. It appears that solitary CHRPE and congenital grouped pigmentation differ clinically from the multiple pigmented lesions seen with familial adenomatous polyposis and that patients with these conditions, as well as their relatives, are not at a greater risk of developing intestinal cancer.Purpose: Congenital albinotic spots of the retina are a very rare anomaly of the retina. This is much lower than would be expected from a survey of patients with the typical fundus lesions seen with familial adenomatous polyposis. When there is an isolated lesion, we are not concerned about the association with polyps or bowel cancer. However, this occurs when there are multiple CHRPE lesions called 'bear tracks,' not with a single isolated CHRPE spot. Among more than 2000 of their blood relatives, only 20 had intestinal polyposis or colonic cancer (1%). Answer: There is a rare condition where CHRPE lesions are associated with colon polyps that can be cancerous. When comparing front and rear tracks, the fore prints of all members of the dog family are significantly larger than the hind print. These freckles are called CHRPEs, which stands for Congenital Hypertrophy of the Retinal Pigment Epithelium. Of the 132 patients with previously diagnosed CHRPE, there were none with familial adenomatous polyposis, Gardner syndrome, or intestinal cancer, and only one patient had a history of intestinal polyps. ![]() Patients and their physicians were contacted by telephone to complete a detailed questionnaire designed to detect signs or symptoms of familial adenomatous polyposis or Gardner syndrome among these patients with CHRPE and their relatives. There are usually no related ocular abnormalities, but it has been observed in the opposite eye of a patient with persistent hyperplastic primary vitreous retinoblastoma. It is generally a nonhereditary, sporadic condition. Review of charts and follow-up studies were performed on all patients diagnosed and coded as having solitary CHRPE or its multifocal variant (congenital grouped pigmentation bear tracks). The multifocal variant of CHRPE is also known as congenital grouped pigmentation of the retina or bear tracks. This study was undertaken to determine whether the typical lesions of CHRPE, seen frequently by ophthalmologists, also were indicators of familial adenomatous polyposis. Although atypical, such lesions have been called congenital hypertrophy of the retinal pigment epithelium (CHRPE). It has been recently documented that multiple bilateral pigmented lesions at the level of the retinal pigment epithelium may be an indicator of patients with familial adenomatous polyposis who are prone to develop intestinal cancer, particularly if there is a positive family history of these intestinal disorders. ![]()
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