There have been less than 300 cases reported in the world for this rare condition

In an intricate, one-of-a-kind case seen rarely in the world, a man in his thirties from Uttar Pradesh; who was born with both male and female genitalia in his body, was successfully operated upon by a team of specialists at Amrita Hospital, Faridabad.

The patient was suffering from Persistent Müllerian Duct Syndrome (PMDS), a rare genetic disorder of sexual development that affects men. In this condition, a biological male develops both male and female reproductive parts. This happens because the gonadal structures that are forming during pregnancy undergo defective development. The female structures like the uterus, fallopian tubes, and sometimes the ovaries, rather than disappearing as they normally would, develop in a male child and persist into adulthood.

The man was facing social and emotional disability due to this anomaly. Despite being married for five years, he was unable to become a father. The patient had been to several hospitals in India where he underwent examinations for his endocrinological, genetic, and psychological profiles. He then visited Amrita Hospital in Faridabad.

Said Dr. Manav Suryavanshi, Head of Uro-Oncology and Robotic Surgery at Amrita Hospital, Faridabad: "The patient came to us seeking a redressal for his predicament at having both male and female reproductive organs. On examination, we found that his testes were still in his abdomen. An MRI scan revealed that he also had female reproductive organs like the uterus and fallopian tubes inside his body. The patient was suffering from persistent Müllerian Duct Syndrome (PMDS) since his birth and was completely unaware of it. PMDS is a rare condition with less than 300 cases reported in medical history worldwide.”

The patient was advised to opt for surgery as there were psychological issues and risks of malignancy in gonads, if left untreated. The Amrita doctors decided to perform surgery robotically to remove the persistent Mullerian duct remnants. Robotic surgery is a team effort in which a Robotic surgeon operates on the console and the bedside surgeon performs the laparoscopic work. This requires training, coordinated teamwork, and precise mastery of the advanced technology.

Said Dr. Manav Suryavanshi: "The case was particularly complex as it involved combined male and female anatomy in a single person in the same area of the body with intermingled and fused anatomical structures. Such surgeries need sound anatomical knowledge, as the risk of damage to normal male structures and ureters draining urine in the vicinity of female structures is high. However, with our planning and a robotic surgery approach, we could achieve a result that was satisfactory to the patient. The robotic technology helps due to its deeper reach in the pelvis, magnification, and human hand-like dexterity, while still allowing the surgery to be performed through keyholes.”

He added: "All the abnormal structures like the uterus, fallopian tubes, round ligaments, and defective gonads like intra-abdominal testes were successfully removed in the patient. Intra-abdominal testes carry a four-fold higher than normal risk of developing malignancy. The overall incidence of malignant transformation in undescended testes of PMDS is 18%. Apart from this, retained Müllerian remnants are known to cause recurrent urinary tract infection, stones, and voiding disturbances in which the muscles that control the flow of urine out of the body don't relax completely, and the bladder never fully empties.”

Robotic keyhole surgery meant a faster recovery for the patient, who was comfortably discharged two days after the surgery. He was counseled about the need for hormonal replacements and has recovered to lead a normal lifestyle. The surgery was performed by Dr. Manav Suryavanshi along with two other doctors from Amrita Hospital, Faridabad, including Dr. Ritesh Goel and Dr. Gautam Khanna.

Said the patient after the surgery: "I am very happy with the results of the surgery, and now I look forward to leading a normal life. This is a big burden off my chest. I thank the doctors at Amrita Hospital for attempting surgery for this rare condition and making it a success."