Persistent Müllerian duct syndrome: lessons learned from managing a series of eight patients over a 10-year period and review of literature regarding malignant risk from the Müllerian remnants

Abstract

Study Type--Therapy (case series) Level of Evidence 4. What's known on the subject? and What does the study add? Approximately 200 cases of persistent Müllerian duct syndrome have been reported over the last 50 years and most authors suggest leaving the Müllerian remnant in situ because of the difficulty in dissection and the presumed absence of risk of malignancy. However, with increasing reports of Müllerian malignancies emerging, we report our 10-year experience of managing patients with persistent Müllerian duct syndrome, with removal of müllerian remnants. This case series shows that there is an increased risk of Müllerian malignancy that was previously unknown. With the laparoscopic approach, orchidopexy with simultaneous removal of Müllerian remnants could be accomplished with minimal surgical trauma and the benefit of no malignancy risk in the future. This is a new technique that has not been previously performed. Considering the current evidence of malignancy in the Müllerian remnant, surgeons would need to discuss with families about removal of remnants or long-term monitoring.

Objectives: • To describe the presentation and management of eight patients with persistent Müllerian duct syndrome (PMDS) seen over a 10-year period at our tertiary centre. • To review the literature of Müllerian malignancies reported in PMDS.

Patients and methods: • The hospital records of eight patients with PMDS were retrospectively reviewed between 2001 and 2011. • Extensive PubMed searches for PMDS and Müllerian malignancy were performed.

Results: • Eleven cases with PMDS and malignancy of the Müllerian remnants were identified. • From our own PMDS series: five males presented with bilateral undescended testes and three had unilateral undescended testis. • We found that the Müllerian remnants could be removed by laparoscopy and three patients had simultaneous laparoscopic removal of the Müllerian structures and laparoscopic orchidopexy.

Conclusions: • The principle aim of orchidopexy with simultaneous laparoscopic removal of the Müllerian structures can be accomplished with minimal surgical trauma and the benefit of no malignancy risk in the future. • Surgeons should consider excision of the Müllerian remnants where possible.