Diagnosis and laparoscopic surgery in the female form of persistent Müllerian duct syndrome

Abstract

Persistent Müllerian duct syndrome (PMDS) is a rare anomaly characterized by the presence of a combination of Müllerian derivatives, including a uterus, fallopian tubes, cervix or vagina in otherwise normally differentiated men. The female form of PMDS is particularly uncommon, making up only 5–10% of the 150 reported cases. In this case, PMDS was not diagnosed until the patient was well into the fifth decade of life. He presented with haematuria and haematospermia and was referred for sonographic examination. During the transrectal prostate ultrasound examination the Müllerian remnants were discovered, including uterus, cervix and vagina. In this report we describe the sonographic and magnetic resonance appearances of PMDS and discuss management via laproscopic hysterectomy and bilateral ovotestectomy.