In a freak medical case, a 16-year-old, who is otherwise a male, was found with sexual organs that are typically found only in women. He had a uterus (womb), ovaries (which produce eggs), fallopian tubes (which carry eggs to the uterus during for fertilisation by sperms from a male) and a rudimentary vagina.The condition is supposed to be so rare that it is only the tenth such reported case from all over the world.A series of operations were conducted on the boy between June and August this year at Fortis Hospital on Cunningham Road to correct the rare condition called Persistent Müllerian Duct Syndrome (PMDS).Urologists and transplant surgeons Dr Mohan Keshavmurthy and Dr Shakhir Tabrez performed the surgeries on the boy, a native of West Bengal Dr Keshavmurthy explained that in this rare condition the patients (basically males) have normal male reproductive organs although they also have uterus and fallopian tubes which are female reproductive organs.According to the US National Library of Medi­cine, PMDS is a disorder of sexual development that affects males. The uterus and fallopian tubes are derived from a structure called the Müllerian duct during development of the foetus.The Müllerian duct usually breaks down during early development in males, but it is retained in those with PMDS. But the affected individuals have the normal XY chromosomes of a male and normal external male genitalia, which makes them basically males.In this case, the boy also had a missing left testicle and his penis was curved at the tip, which made passing urine difficult. This condition is called hypospadias, a congenital condition in males in which the opening of the urethra is on the underside of the penis. The urethra is a duct through which urine is passed out of the body from the bladder and, in male vertebrates, semen from the testicles, too.Dr Keshavmurthy said, “This is a case of hormonal imbalance; and on further evaluation we saw that while his right testicle was normal, his left testicle was present inside the groin region. It is referred as ‘undescended testis’, a condition wherein the testicle has not normally descended into the scrotum.”The testicles are responsible for the production of sperm cells and the male sex hormone testosterone.The doctors had to treat both the conditions – the PMDS and hypospadias – in two stages of surgeries.Dr Keshavmurthy said, “The patient and his mother first came to us in June for a urinary abnormality, but they had no clue about the presence of internal female sexual organs which were the size of any 16-year-old girl. Only on conducting a diagnostic laparoscopy, he was diagnosed with PMDS. When we informed them, they were horrified. We had to counsel them considerably. In his case, we had to recreate the urethra and remove the female genital organs. If they were not removed, there were chances of him developing severe abdominal pains during menstruation, as there was no way for it to come out.”The boy’s penis was lengthened and made straight in such a way that he would not have problems with erection in the future.Following this, the uterus, fallopian tubes, the hidden testis and the ovaries were removed.“He will now be able to lead a normal life…even a normal sex life,” Dr Keshavmurthy said.WHAT IS PMDS?Persistent Müllerian Duct Syndrome (PMDS) is a disorder of sexual development that affects males. Males with this disorder have normal male reproductive organs, though they also have a uterus, ovaries, fallopian tubes and vagina, which are female reproductive organs. The uterus and fallopian tubes are derived from a structure called the Müllerian duct during development of the foetus. The Müllerian duct usually breaks down during early development in males, but it is retained in those with persistent Müllerian duct syndrome. Affected individuals have normal chromosomes of a male and normal external male genitalia.WHAT IS HYPOSPADIAS?Hypospadias is a birth defect of the urethra where the urinary opening is not at the usual location on the head of the penis, but either under the penis or at the base of the penis from the underside. It is the second most common birth abnormality of the male reproductive system, affecting approximately 1 in every 250 males. Hypospadias is thought to result from failure of the urinary channel to completely tubularize to the end of the penis. The cause of hypospadias is not known. Most often, it is the only abnormal finding, although in about 10 per cent of cases hypospadias may be part of a syndrome with multiple abnormalities