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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.
Abstract:
Persistent Müllerian duct syndrome is a rare form of internal male pseudohermaphroditism caused by defects in synthesis or action of Müllerian-inhibiting factor, due to which Müllerian duct derivatives, such as uterus, fallopian tube, and upper vagina, are normally present in 46XY males. Here, we report a 26-year-old male with right-sided obstructed inguinal hernia with left undescended testis. On exploration, hernial sac containing bowel loops, uterus with fallopian tubes, upper vagina, and testes were present.
Insights
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.
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:
- 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.
