BACKGROUND: Management of intra-abdominal gonads (IAG) in patients with Androgen Insensitivity Syndrome (AIS) remains controversial. Although gonads are initially left in place to allow for pubertal feminization, disagreement remains as to whether the gonads should remain in place after this time. Despite the very low risk of tumor development and advantages of maintaining endogenous hormone production, many physicians still advise patients to undergo gonadectomy. However, some patients feel that removal has left them with reduced libido and therefore wish they had been given to option of keeping their native gonads. To provide patients with a greater range of choices, while mitigating concerns over difficulty with monitoring for rare cases of tumor, we have relocated the IAG to an inguinal location where they can be more safely monitored with palpation and ultrasound. METHODS: We queried our DSD patient database from 2008-2022 for all patients with AIS that underwent surgery to preserve IAGs. Preoperatively families were informed of the risks and benefits of gonadal preservation. Patients were then followed with annual ultrasounds and physical examination.
RESULTS: Eight girls with AIS underwent surgery with the goal of preserving an IAG. In six the IAGs were relocated to the inguinal location. In two the IAGs appeared normal on MRI, but at the time of surgery were found to be markedly heterogenous and a choice was made to remove them. All relocated gonads have intact blood flow and have maintained their volume at an average follow up was 5.1 years. Hypertrophic Leydig cell rests have been noted in one patient with biopsy revealing no malignancy. CONCLUSIONS:This is the first reported series of successful relocation of IAG to the inguinal region achieved in patients with AIS. This technique allows for preservation of the gonads and thereby provides the patient with a greater range of choices for how to manage their condition. Gonads are situated in a location where serial monitoring with palpation and ultrasound is made more practical and future biopsies, if needed, can be easily achieved in the outpatient setting.
