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PMDS or Persistent Müllerian Duct Syndrome is a rare genetic disorder, which occurs in men between the age of 8 till 22, causing the body to have both male and female reproductive organs.  Such  a case was brought for treatment at the GBH American Hospital where a man was operated and uterus and ovaries were removed from his body.

In this rarest of rare syndromes, doctors at Udaipur’s GBH American Hospital, under the supervision of Dr. Shilpa Goyal, operated on a man for undescended testicles, but found female reproductive organs inside, instead of the testicles.  The surgeons removed a uterus, cervix and ovaries from inside the man’s body, whose name has been withheld on request.  The female organs were in the abdomen area.  This case of PMDS is a rare disorder of sexual development, said the doctors.

Undescended testes and fleshy hernia are one of the first symptoms of this rare disease as per Dr. Manish Bhatt, Urologist at GBH, who performed this surgery alongside Dr Goyal.  The 22 year old man is now cured as the female organs have been removed.  Dr Goyal, who is a gynecologist said that being a Gynae, she understands organ positions in a female body, but this was rare as the organs had to be removed from a male body.

This syndrome occurs because the Müllerian duct that forms the uterus and fallopian tubes in the foetus do not break down and the men with this issue have the normal male chromosomes (46, XY) and external male genitalia. The uterus and fallopian tubes are usually discovered during the treatment of the undescended testicles, as it was in this case.

Source: Media Reports