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Persisting Müllerian Duct Syndrome
Males with normal external genitalia and additional internal müllerian duct structures: fallopian tubes, uterus or a vagina draining into the prostatic utricle. Unilateral or bilateral undescended testes (cryptorchidism) is the typical presentation. The surgical treatment of undescended testes reveals Müllerian tissue. Late presentation with pelvic masses in adulthood is also possible.
The persisting Müllerian duct syndrome is caused either by deficiency of the anti-Müllerian hormone or by resistance of the Müllerian duct structures to the anti-Müllerian hormone. Treatment consists in early orchidopexy and removal of Müllerian duct structures as necessary.
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Deutsche Version: Persistierendes Müller-Gang-Syndrom