Management of the patients with persistent Müllerian duct syndrome: Is the ultimate goal testicular descent?

Turk J Urol. 2018 Mar;44(2):166-171. doi: 10.5152/tud.2018.33407. Epub 2018 Mar 1.

Abstract

Objective: Persistent Müllerian duct syndrome (PMDS) is a rare congenital disease characterized by the presence of rudimentary Müllerian structures within an intra-abdominal or hernial sac in a virilized male, often presenting as undescended testes. In this study, we aim to present a series of the PMDS patients who were managed by orchiopexy without removal of Müllerian remnants (MR).

Material and methods: Between May 2010 and June 2017, we treated six cases diagnosed as PMDS in our department. Laparoscopy and gonadal biopsy were performed in all patients, and vessel ligation was done in four patients for the first session of Stephen-Fowler orchiopexy. After initial diagnosis, genetic analyses and endocrine investigations were performed. Demographic and clinical features of the patients, operative methods and follow-up data were analyzed retrospectively.

Results: Mean age of the patients was 5.5 years. Three boys had undergone inguinal surgery due to hernia or undescended testis, while others were diagnosed during laparoscopic investigation of nonpalpable testis. As a definitive operation, testes and MR were completely removed in an adult patient, and the remaining patients were treated with laparoscopic or open orchiopexy with or without utero-cervical splitting and the MRs were left in situ. Two testes atrophied during follow-up period.

Conclusion: The goal of the approach in PMDS patients is to preserve testes, as well as carry them to their natural location. Leaving the MR in place is a suitable option for blood circulation of the testes but the long-term results are still unknown.

Keywords: Disorders of sex development; Müllerian remnant; laparoscopic orchiopexy; persistent Müllerian duct syndrome.