PERLMAN-SYNDROME - A CASE-REPORT EMPHASIZING ITS SIMILARITY TO AND DISTINCTION FROM BECKWITH-WIEDEMANN AND PRUNE-BELLY SYNDROMES

Perlman syndrome is a rare entity characterized by hypotonia, facial d ysmorphism, gigantism, and visceromegaly including nephromegaly. The c urrent case illustrates the ultrasonographic and computed tomographic appearances of renal abnormalities in this condition. Findings in Perl man syndrome can overlap with and need to be distinguished from two mo re common clinical entities: prune-belly and Beckwith-Wiedemann syndro mes. Additional cranial findings previously unreported are also descri bed.