Abstract

During the 15-year period from January 1979 through December 1993, 13 unrelated sporadic cases of Poland syndrome were ascertained in Kuwait. Two of 5 cases (40%) of left-sided Poland syndrome were associated with dextrocardia. This study supports the previous findings of male sex predilection, right-sided predominance and the association of left-sided involvement with dextrocardia. An overview of the disease is presented with particular emphasis on the possible pathogenetic mechanism, worldwide familial cases and the broad spectrum of phenotypic heterogeneity.

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