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CASE REPORT
Year : 2022  |  Volume : 5  |  Issue : 2  |  Page : 91-94

Obstructed inguinal hernia in an adult male: A rare presentation of persistent Müllerian duct syndrome (internal male pseudohermaphroditism): A case report


1 Department of Surgery, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India
2 Department of Pathology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India
3 Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India

Correspondence Address:
Dr. Sabina Khan
Department of Pathology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi 110062
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJAWhs.ijawhs_47_21

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Persistent Müllerian duct syndrome (PMDS) is an unusual form of internal male pseudohermaphroditism in which Müllerian duct derivatives are seen in phenotypically normal males, with 46, XY karyotype. PMDS is an outcome of defective functioning of the Müllerian inhibiting factor (MIF) or its receptor. MIF causes degeneration of the Müllerian duct in the fetus. PMDS clinically manifests as cryptorchidism or inguinal hernia, although it is mostly undetected until puberty. We report a rare case of PMDS in a 45-year-old male patient with right-sided cryptorchidism and contralateral inguinal hernia. Upon exploration of the hernial sac, it contained a uterus-like mass with attached left testis. Biopsy and subsequent histopathological examination of the mass confirmed the presence of endometrial glands.


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