Three patients were identified and medical records were reviewed. Harkness Eye Institute of Columbia University Medical Center (New York, NY). Patients diagnosed with CGAS were identified retrospectively from January 2004 to December 2008 at the Moorfields Eye Hospital, Medical Retina Service (London, UK) and the Edward S. The purpose of this case series is to report FAF and OCT findings in CGAS, which can assist the diagnosis and strengthen our understanding of the pathophysiology of CGAS. To date, FAF and OCT findings have not been characterized for CGAS. 6, 8įundus autofluorescence (FAF) as well as optical coherence tomography (OCT) have become powerful noninvasive diagnostic imaging modalities in evaluating the photoreceptor-RPE complex. FA has shown variable degrees of transmission of choroidal fluorescence through the lesions without leakage or late staining 4, 7, 8 ICGA has demonstrated both increasing hyperfluorescence as well as hypofluorescence of the lesions. However, Gass 1 reported a case of CGAS with severe visual loss caused by choroidal neovascularization, Parke et al 5 reported a syndrome of microcephaly without mental retardation associated with spots quite similar to those in CGAS, and Battaglia and Iacono 6 described a wide atrophic macular lesion and 20/400 vision in a patient with CGAS.įluorescein angiography (FA) and indocyanine green angiography (ICGA) characteristics of CGAS have been described. The disorder is considered to be without functional consequence and patients typically have normal visual acuity, visual fields, color vision, dark adaptation, electroretinography, and electrooculography findings. It is speculated that the spots are focal changes of RPE cells in which a white material, possibly a precursor of melanin, is deposited instead of melanin granules.

The lesions occur in one or both eyes and are generally more numerous and larger in the peripheral retina. Congenital grouped albinotic spots (CGAS), or “polar bear tracks,” is a disorder of the retinal pigment epithelium (RPE) characterized by multiple, variably-sized, sharply circumscribed, placoid, white lesions organized in patterns often resembling animal footprints.

Bear tracks retina child