Laparoscopic management of persistent mullerian duct syndrome

Abstract Persistent mullerian duct syndrome (PMDS), characterized by the presence of mullerian structures in a virilized male, frequently presents as undescended testis, either intraabdominal or within a hernial sac. We describe a 10-month-old infant with PMDS successfully managed by the laparoscopic approach. At the age of 1.5 months, the patient presented with a left inguinal hernia and bilateral nonpalpable gonads in another center and underwent left inguinal exploration. The uterus and a gonadlike structure along with the hernia sac were found in the inguinal canal. Left inguinal herniotomy was performed after reduction of the uterus and gonadlike structure. No gonadal biopsy was obtained. The patient was further investigated in the same center. His karyotype was 46,XY. Magnetic resonance imaging of the abdomen and pelvis revealed a uterinelike structure posterior to the urinary bladder, but neither testis nor ovaries were visualized. At 10 months of age, he was referred to our department for further management. A laparoscopic single-stage orchiopexy was performed. Both testes were identified and brought to the scrotum by splitting the uterus in the midline and then bringing the testes with the vas and attached uterine tissue into the scrotum. The aim of placement of well-vascularized testes in the scrotum was achieved as confirmed on follow-up color Doppler ultrasound study 6 months postoperatively, which showed normal vascularity. Laparoscopic surgical techniques for this condition are also discussed.