Special Female Hermaphroditism Associated with Multiple Disorders

Female hermaphroditism of the nonadrenal variety is clinically unusual. Among such patients is a special group of masculinized females in whom the cause of masculinization is unidentified and who also have serious multiple somatic anomalies. These patients have been considered to comprise a special group of female hermaphrodites. Fourteen examples are collected from the literature, and a new case report is recorded. The patient had an enlarged clitoris, double vagina, bicornuate uterus, anomalous position of the urethral meatus, congenital deafness, ectodermal defect, athyreotic cretinism, pancreatic achylia, mental retardation and primordial dwarfism.