Persistent Mullerian duct syndrome: A surgical surprise and management during laparoscopic transabdominal pre-peritoneal repair

Persistent Mullerian duct syndrome is an intersex disorder which occurs due to the failure of regression of Mullerian structures in genotypical and phenotypical males. It is a rare disorder of male internal pseudohermaphroditism with normal secondary sexual characters and XY karyotype. We report a male patient with the diagnosis of bilateral inguinoscrotal hernia with cryptorchidism scheduled for laparoscopic bilateral transabdominal pre-peritoneal (TAPP) mesh repair with bilateral orchidopexy. Intra-operatively, there was a rudimentary uterus with tubes on both sides. Bilateral testicular biopsy was taken to confirm the presence of testicular tissue and to exclude malignancy and ovarian tissue. Hernia surgery was deferred in the first stage. After 2 weeks, he underwent laparoscopic bilateral orchidopexy with TAPP mesh repair. At 6-year follow-up, there is no recurrence of hernia or features of malignancy in the retained testis.