Therefore, we considered this condition as congenital grouped pigmentation and albinotic spots in the retina. Their fundus changes were very similar to those observed in the present case. described congenital grouped pigmentation of the retina in a girl and congenital albinotic spots of the retina in her sister. described a case of grouped pigmented lesions with nonpigmented, punctate lesions located within the macula. However, these fundus changes were very similar to those observed in the following 2 reports. In our present case, the size of albinotic spots was smaller than that of the pigmented spots, and the albinotic spots organized in patterns resembling drusen. Congenital grouped albinotic spots are characterized by multiple, variably sized, sharply circumscribed, placoid, white lesions. In addition, a genetic consultation was not performed in this case.Ĭongenital grouped pigmentation of the retina is characterized by multiple, grouped, sharply circumscribed, pigmented spots. Although the patient's mother has undergone a colonoscopy to examine her adenomatous polyposis, abnormal findings were not detected. 3a, b) and an irregular pigmentation in the peripheral retina (fig. Ophthalmoscopic examination of the girl's mother revealed albinotic spots in the posterior pole (fig. 1c, d) and granular hyperfluorescence correlating with the albinotic spots (fig. 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. Fluorescein angiography demonstrated a persistent hypofluorescence correlating with grouped pigmentation of the retina (fig. The albinotic spots were predominantly detected at the posterior pole of both fundi (fig. As a unique co-existing feature, small albinotic spots were identified in the posterior pole (fig. 1a, b), consistent with a grouped pigmentation of the retina or bear track spots. Ophthalmoscopy of both eyes revealed multiple, small, flat, pigmented spots in the peripheral retina (fig. Her visual acuity was 1.2 in both eyes.The anterior segment of both eyes was normal. They both need to be monitored to make sure they are not malignant melanomas. Both conditions are usually benign and not a serious problem.
Her medical and personal histories were unremarkable and her physical examination revealed no abnormalities. It could be a number of things but the most likely are choroidal nevus (benign choroidal melanoma) or CHRPE (congenital hypertrophy of the retinal pigmented epithelium). May be encircled by hyper- or hypo-pigmented halo. Signs: Well-demarcated, round, solitary or multiple gray-brown or black lesions which have flat or scalloped margins. There are several intriguing tumors and pseudotumors of the pigmented epithelium that have major clinical and histopathologic importance.Ĭopyright 2017 Asia-Pacific Academy of Ophthalmology.A 10-year-old asymptomatic Japanese girl was referred to our clinic for an ophthalmological examination of bilateral fundus discoloration. 'Bear Tracks' CHRPE Benign pigmented fundus lesions that commonly discovered during routine eye examination. Torpedo maculopathy is a small stable lesion with typical features, resembling a torpedo. Acquired tumors (adenoma, adenocarcinoma) have features distinct from melanoma clinically and histopathologically. Combined hamartoma, an idiopathic proliferation of RPE cells, blood vessels, and glial cells, is also believed to be a relatively stable lesion but can cause vision loss due to traction. Congenital simple hamartoma of the RPE is a specific lesion that involves the fovea and does not tend to cause complications. Multifocal CHRPE (congenital grouped pigmentation) has no relationship to familial adenomatous polyposis or Gardner syndrome, despite its similarity to the pigmented fundus lesions seen with those conditions. Solitary CHRPE, traditionally believed to be stationary, can show growth in diameter in 83% and can spawn elevated nodular tumors that can progressively enlarge, cause complications, and even evolve into malignant epithelioma (adenocarcinoma). This article describes examples of pigment epithelial tumors and pseudotumors by reviewing the literature and cases on file in the Oncology Service at Wills Eye Hospital. 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 RPE, and acquired epithelioma of IPE, CPE, and RPE.
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