Abstract
The persistent Müllerian duct syndrome is characterized by the presence of uterus and fallopian tubes in otherwise normally virilized 46, XY men. PMDS is a recessive autosomal condition usually due to mutations of the AMH or AMH receptor type 2 genes. Infertility and/or testicular malignancy are the main complications. Treatment consists in the repositioning of testes in the scrotum, uterine resection is not recommended.
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