Operative considerations for late-presenting persistent Müllerian duct syndrome

Persistent Müllerian duct syndrome (PMDS) is a condition in which a 46, XY male displays masculine external genitalia, but internally retains developed Müllerian duct structures (uterus, fallopian tubes, and upper two-thirds vagina). Thoughtful operative consideration is needed to maximize the therapeutic benefit while minimizing the risk of hypogonadism, infertility, and erectile dysfunction. We report a 53-year-old male with a pelvic mass incidentally discovered on routine ultrasound, intra-operatively discovered to be PMDS. PMDS is a rare condition that may present late in life. The primary operative consideration is performing orchiopexy for cancer surveillance or orchiectomy if orchiopexy is not possible. Additional considerations include surveillance and counseling of infertility, hypogonadism, and assessment of the potential need for involvement of psychiatry. Removal of Müllerian remnants is a subject to debate. If possible, discuss with the patient their risks and options in the preoperative setting to guide operative planning.