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Related Experiment Video

Updated: May 4, 2026

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Persistent müllerian duct syndrome: a case report.

Vijaya Patil1, Sunilkrishna Muktinaini2, Rashmi Patil2

  • 1Department of Surgery, B.L.D.E.U.'s Shri B.M. Patil Medical College & Research Centre, Bijapur, Karnataka India.

The Indian Journal of Surgery
|January 16, 2014
PubMed
Summary

Persistent Müllerian duct syndrome (PMDS) is a rare condition where 46XY males retain female internal reproductive organs. This case highlights PMDS in a 26-year-old male presenting with an obstructed inguinal hernia and undescended testis.

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Area of Science:

  • Endocrinology
  • Genetics
  • Reproductive Medicine

Background:

  • Persistent Müllerian duct syndrome (PMDS) is a rare disorder of sex development.
  • It results from defects in Müllerian-inhibiting factor (MIF) synthesis or action.
  • This leads to the presence of Müllerian duct derivatives (uterus, fallopian tubes, upper vagina) in 46XY males.

Purpose of the Study:

  • To report a unique case of Persistent Müllerian duct syndrome.
  • To describe the clinical presentation and surgical findings in an adult male with PMDS.

Main Methods:

  • Case report of a 26-year-old male.
  • Surgical exploration for right-sided obstructed inguinal hernia and left undescended testis.

Main Results:

Keywords:
Hernia uteri inguinalePersistent Müllerian duct syndromeTransverse testicular ectopia

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  • Exploration revealed a hernial sac containing bowel loops.
  • Intra-abdominal structures included a uterus, fallopian tubes, upper vagina, and testes.

Conclusions:

  • This case underscores the importance of considering PMDS in adult males with atypical presentations like obstructed inguinal hernia and undescended testes.
  • Surgical management requires careful intraoperative identification of Müllerian remnants in 46XY individuals.