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December 2002

Papillorenal Syndrome in a Brazilian Family

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Copyright 2002 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2002

Arch Ophthalmol. 2002;120(12):1772. doi:

The report by Gus et al1 in the October 2001 issue of the ARCHIVES describes a family with "renal-coloboma" syndrome, otherwise known as papillorenal syndrome.2 The optic disc anomalies in this disorder are not true colobomas. Colobomas result from the failure of the optic fissure to close and may manifest as defects in tissues such as the inferonasal iris, choroid, retina, or optic nerve. In a coloboma affecting the optic disc, the origin of the central retinal vessels will thus appear to be displaced superotemporally. Characteristic findings in papillorenal syndrome, on the other hand, consist of an absence or attenuation of the central retinal vessels within the optic nerves, with multiple compensatory cilioretinal vessels present.3,4 These features are suggested in the cases illustrated and described by the authors.1 The term papillorenal syndrome, first employed in 1984,2 effectively pinpoints the optic papilla as the site where this anomalous retinal vascular supply can best be recognized. It also avoids the pathogenetic confusion that habitually occurs with use of the term coloboma.

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