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CC BY-NC-ND 4.0 · Ibnosina Journal of Medicine and Biomedical Sciences 2020; 12(02): 130-133
DOI: 10.4103/ijmbs.ijmbs_35_20
Case Report

Persistence of Müllerian derivatives in an adult male

Souha Elabd
1   Obesity, Endocrine, and Metabolism Center, King Fahad Medical City, Riyadh
,
Mussa Almalki
1   Obesity, Endocrine, and Metabolism Center, King Fahad Medical City, Riyadh
2   King Fahad Medical City, College of Medicine, King Saud bin Abdulaziz University for Health Science, Riyadh
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Persistent Müllerian duct syndrome (PMDS) is a rare congenital abnormality that leads to male disorders of sexual development (DSD) due to the persistence of Müllerian duct derivatives in otherwise normally virilized male patients with a normal karyotype. It is usually diagnosed in the early years of life at the time of surgery for cryptorchidism or repair of an inguinal hernia. Cases of affected adult males have been reported as well. We report a rare case of PMDS in a middle-aged (47-year-old) infertile male who was referred to the endocrine clinic for the evaluation of primary infertility. A high index of suspicion needs to diagnose such conditions as early treatment is necessary to preserve fertility and to reduce the occurrence of neoplastic transformation in remnant Müllerian structures.

Key-words:

Anti-Müllerian hormone/Müllerian-inhibiting factor - cryptorchidism - persistent Müllerian duct syndrome - primary infertility - Disorders of sexual development (DSD)

Financial support and sponsorship

Nil.




Publication History

Received: 31 March 2020

Accepted: 07 April 2020

Article published online:
07 July 2022

© 2020. The Libyan Authority of Scientific Research and Technologyand the Libyan Biotechnology Research Center. All rights reserved. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License,permitting copying and reproductionso long as the original work is given appropriate credit. Contents may not be used for commercial purposes, oradapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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