Meacham Syndrome

This syndrome was first described by Meacham et al. in two genetically male (46XY) infants who presented with ambiguous external genitalia and a true double vagina accompanied by cardiac, pulmonary, and diaphragmatic abnormalities (Meacham et al. 1991). The most unique feature of this syndrome was the presence of a double vagina. Although the presence of female structures in a male fetus could result from an arrested differentiation, at no stage of fetal development is the vagina a duplicate structure. To date, only several cases of the Meacham syndrome have been documented leading to a rough estimation of <1/1,000,000 for the prevalence of the disease. In all documented cases of Meacham syndrome, the onset of the symptoms was at birth.