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Persistent Müllerian duct syndrome: A surgical surprise and management during laparoscopic transabdominal
K Ganesh Shenoy1, Swetha Dama1, Ramesh Makam1
1Department of Minimal Access, GI and Bariatric Surgery, A V Hospital, Bengaluru, Karnataka, India.
Abstract:
Persistent Müllerian duct syndrome is an intersex disorder which occurs due to the failure of regression of Müllerian 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.
Insights
Persistent Müllerian duct syndrome, a rare intersex disorder, was identified in a male patient with hernia and cryptorchidism. Surgical repair was successful, with no recurrence at six-year follow-up.
Area of Science:
- Endocrinology
- Genetics
- Surgical Urology
Background:
- Persistent Müllerian duct syndrome (PMDS) is a rare intersex disorder characterized by the presence of Müllerian structures in XY males.
- This condition can lead to complications such as hernias and cryptorchidism, requiring careful surgical management.
Observation:
- A case report details a genotypical and phenotypical male patient diagnosed with bilateral inguinoscrotal hernia and cryptorchidism.
- Intraoperative findings revealed a rudimentary uterus and bilateral fallopian tubes, confirming PMDS.
- Surgical intervention involved initial deferral of hernia repair, followed by laparoscopic bilateral orchidopexy and transabdominal pre-peritoneal (TAPP) mesh repair.
Findings:
- Bilateral testicular biopsies confirmed testicular tissue and ruled out ovarian tissue or malignancy.
- The patient underwent successful laparoscopic bilateral orchidopexy with TAPP mesh repair.
- A six-year follow-up showed no hernia recurrence and no signs of malignancy in the retained testes.
Implications:
- This case highlights the importance of intraoperative vigilance for unexpected findings in patients undergoing hernia repair.
- Early diagnosis and appropriate surgical management of PMDS can lead to favorable long-term outcomes.
- Further research into the genetic and developmental aspects of PMDS may improve diagnostic and therapeutic strategies.
