Dr. Hidash .
(photo credit: COURTESY HADASH)
Pediatric urologists have surgically removed testicles found inside a 14-year-old girl who was born without a uterus.
The very rare case, treated at Hadassah-University Medical Center in Jerusalem’s Ein Kerem, involved a girl with a female appearance and perception.
A few months ago, the girl’s parents identified male traits in their daughter that were getting stronger – as the girl entered adolescence, the testicles began to grow, and sex hormones they produced made hair grow on her body and her voice to become lower.
She was referred for testing to Prof. David Zangen, director of the pediatric endocrinology and juvenile diabetes unit, who immediately suspected the syndrome included in a rare group of diseases involving sexual development.
Specific hormonal tests and ultrasound confirmed the suspicion of a specific syndrome and therefore a thorough genetic investigation was also carried out. The diagnosis shocked the girl and her family when it became clear that there were testicles in her abdominal cavity, even though her external genitalia were female.
“In our tests it turned out that she had a vagina, but in the internal structure of the body she had no uterus,” explained Zangen. “She was born with the testes that functioned only partially in terms of hormonality, which is very rare, but she has female gender identity. In such cases, a multidisciplinary team at Hadassah have taken her case.
The team, headed by Zangen and senior pediatric urologist Dr. Guy Hidash, includes a psychologist, a geneticist and a social worker to discuss the girl’s gender. “The case of the girl presents us with a situation of relative deficiency in the enzyme, which causes the non-development of male sexual organs in the newborn – but increases the signs during adolescence in adolescence,” Zangen said.
Hidash, who performed the procedure to remove the testes from the abdominal cavity, explained: “The gender of human beings consists of four distinct parameters – genetics (whether it is XY or XX chromosome), the appearance of the genitals, the hormonal state (testosterone or estrogen) and sexual identity (how he or she perceives himself/herself as male or female).
“But if there is a contradiction among the parameters, we must analyze what is the right treatment for the specific patient. In terms of health, testicular removal was an important step once it was decided that she is a girl.”
The teen will need further surgery to complete the anatomy accurately.
Since the establishment of the multidisciplinary team at Hadassah three years ago, 18 cases of gender disorders have been treated surgically, and all have been successful,” Hidash continued, “both in terms of the success of the operation and the patients’ coping with their decision.”
The girl’s family was afraid of the case becoming known in the community in which they live and debated how to raise the funding for the complex treatment. They were finally helped by the Roseana organization in the Hadassah Women’s Zionist Organization in Australia, which covered the cost of the surgery.