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Hypofertility in a persistence of mullerian duct syndrome: Case report

INTRODUCTION: Persistent Mullerian duct syndrome is a rare form of pseudohermaphroditism, possibly resulting from the absence of Mullerian duct inhibiting factor. PRESENTATION OF CASE: We report the case of a 36 year-old patient with a syndrome of persistent Müllerian ducts (PMDS) of the female type...

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Autores principales: Jandou, Issam, Mhanna, Tarik, Chennoufi, Mehdi, Aynaou, Mohamed, El houmaidi, Amine, Barki, Ali
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7718311/
https://www.ncbi.nlm.nih.gov/pubmed/33395894
http://dx.doi.org/10.1016/j.ijscr.2020.11.011
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author Jandou, Issam
Mhanna, Tarik
Chennoufi, Mehdi
Aynaou, Mohamed
El houmaidi, Amine
Barki, Ali
author_facet Jandou, Issam
Mhanna, Tarik
Chennoufi, Mehdi
Aynaou, Mohamed
El houmaidi, Amine
Barki, Ali
author_sort Jandou, Issam
collection PubMed
description INTRODUCTION: Persistent Mullerian duct syndrome is a rare form of pseudohermaphroditism, possibly resulting from the absence of Mullerian duct inhibiting factor. PRESENTATION OF CASE: We report the case of a 36 year-old patient with a syndrome of persistent Müllerian ducts (PMDS) of the female type. The Clinical examination revealed avacant scrotum, a normal penis. The spermogram analysis found azoospermia. Imaging using ultrasound and tomotensidometry found the presence of amass in the back of bladder. A Laparoscopic exploration revealed two slightly enlarged testicles, in a pelvic situation intimately confined to a rudimentary uterus with two tubes. The histological examination was in favor of a uterine endometrium and two fallopian tubes. The karyotype requested later was male 46 XY. DISCUSSION: Since the first case, between 200 and 262 cases have been reported in literature around the world, this might reveal that the incidence and prevalence of this type of diseases are not well-estimated. The diagnosis is often a fortuitous discovery during a cryptorchidism surgery or inguinal hernia, more rarely during the management of a state of infertility or the degenerescence of the testis or Mullerian derivative. It is caused by either the anti-Mullerian hormone deficiency (AMH) or a dysfunction of its receptor. CONCLUSION: A multidisciplinary approach is necessary for surgical management. long-term prognosis is favorable, though fertility seem to be decreased and also it might lead to a riskiness of malignancy owing to cryptorchidism and preserved Mullerian remants.
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spelling pubmed-77183112020-12-09 Hypofertility in a persistence of mullerian duct syndrome: Case report Jandou, Issam Mhanna, Tarik Chennoufi, Mehdi Aynaou, Mohamed El houmaidi, Amine Barki, Ali Int J Surg Case Rep Case Report INTRODUCTION: Persistent Mullerian duct syndrome is a rare form of pseudohermaphroditism, possibly resulting from the absence of Mullerian duct inhibiting factor. PRESENTATION OF CASE: We report the case of a 36 year-old patient with a syndrome of persistent Müllerian ducts (PMDS) of the female type. The Clinical examination revealed avacant scrotum, a normal penis. The spermogram analysis found azoospermia. Imaging using ultrasound and tomotensidometry found the presence of amass in the back of bladder. A Laparoscopic exploration revealed two slightly enlarged testicles, in a pelvic situation intimately confined to a rudimentary uterus with two tubes. The histological examination was in favor of a uterine endometrium and two fallopian tubes. The karyotype requested later was male 46 XY. DISCUSSION: Since the first case, between 200 and 262 cases have been reported in literature around the world, this might reveal that the incidence and prevalence of this type of diseases are not well-estimated. The diagnosis is often a fortuitous discovery during a cryptorchidism surgery or inguinal hernia, more rarely during the management of a state of infertility or the degenerescence of the testis or Mullerian derivative. It is caused by either the anti-Mullerian hormone deficiency (AMH) or a dysfunction of its receptor. CONCLUSION: A multidisciplinary approach is necessary for surgical management. long-term prognosis is favorable, though fertility seem to be decreased and also it might lead to a riskiness of malignancy owing to cryptorchidism and preserved Mullerian remants. Elsevier 2020-11-27 /pmc/articles/PMC7718311/ /pubmed/33395894 http://dx.doi.org/10.1016/j.ijscr.2020.11.011 Text en © 2020 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Jandou, Issam
Mhanna, Tarik
Chennoufi, Mehdi
Aynaou, Mohamed
El houmaidi, Amine
Barki, Ali
Hypofertility in a persistence of mullerian duct syndrome: Case report
title Hypofertility in a persistence of mullerian duct syndrome: Case report
title_full Hypofertility in a persistence of mullerian duct syndrome: Case report
title_fullStr Hypofertility in a persistence of mullerian duct syndrome: Case report
title_full_unstemmed Hypofertility in a persistence of mullerian duct syndrome: Case report
title_short Hypofertility in a persistence of mullerian duct syndrome: Case report
title_sort hypofertility in a persistence of mullerian duct syndrome: case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7718311/
https://www.ncbi.nlm.nih.gov/pubmed/33395894
http://dx.doi.org/10.1016/j.ijscr.2020.11.011
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