[89] Robot-assisted hysterectomy and bilateral orchidopexy for a patient with persistent Müllerian duct syndrome

Objective

To demonstrate robot-assisted hysterectomy and bilateral orchidopexy in a patient with persistent Müllerian duct syndrome (PMDS). PMDS is a relatively rare disorder of sexual development, patients have a 46, XY karyotype and normal male external genitalia, but internal Müllerian duct structures. Usually these phenotypic males have unilateral or bilateral undescended testes, bilateral fallopian tubes, a uterus, and an upper vagina draining into a prostatic utricle.

Methods

We present a 48-year-old gentleman that was referred to our institute as a case of primary infertility and bilateral undescended testes.

Results

Diagnostic laparoscopy revealed a uterus, fallopian tubes with bilateral gonads that were biopsied and showed fibrotic seminiferous tubes. Due to the probable risk of malignant transformation of the remnant Müllerian structures, we counselled the patient for robot-assisted hysterectomy, bilateral salpingectomy and bilateral orchidopexy, upon which he agreed to proceed.

Conclusion

Our video demonstrates this rare syndrome and shows the feasibility of performing such a procedure robotically with good results.