Long-term outcome of gender assignment in individuals with 46, XY DSD assigned female sex in multicultural society

Background

The decision regarding sex rearing in patients with Disorders of Sex Development (DSD) is heavily connected to the shared decision-making model within multidisciplinary team. Some of these patients might develop gender dysphoria, when they become adults. We have aimed to evaluate the long-term outcomes of patients with XY DSD who underwent female gender assignment at our center.

Methods

We have conducted a retrospective study of all 46, XY DSD patients who underwent female assignment in our institution over the last 30 years.

Results

we have found 25 46, XY patients who were raised as a female after birth. After excluding the Androgen insensitivity syndrome (AIS) patients we have identified 15 patients who have matched study criteria. The decision on gender rearing was made by the parents in 11(74%) and by the surgical team 2(13%) during hernia repair/inguinal exploration. In 2(13%) cases, the patients opted to continue identifying as women after learning about the pathology during adolescence. Nine (60%) out of 15 patients (age17.9 ± 4.7 years (mean ± SD)) agreed to answer questionnaires regarding sexual function and satisfaction from gender assignment. Mean follow up was 11.1 ± 8.2 years (mean ± SD). only one participant consented to respond to a questionnaire regarding sexual intercourse (homosexual). The overall FSFI score was 24 which included the scores 4, 4, 3, 4, 3, 2 in the categories desire, arousal, lubrication, orgasm, satisfaction, and pain respectively. Two patients regretted the decision of female gender assignment. The first with 5α-reductase deficiency, he made the decision for assignment himself as an adult and the other (3β-hydroxysteroid dehydrogenase) who underwent gonadectomy during inguinal exploration as a child. The rest of the patients were satisfied with the choice of gender, 2 need psychological support on the daily basis. In the study group, relationship and cohabitation were significantly later in life compared to the general population.

Conclusions

Despite the sensitivity of the subject and cultural differences, most patients (78%) were satisfied with the decision to undergo female gender assignment. Over the years, patients require meticulous follow-up in order to consider additional interventions, and mental support if it is necessary. The two cases of later regret highlight the importance of proper education of patients, their families and medical providers upon decision on gender assignment.