The management of the persistent Müllerian duct syndrome

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The management of the persistent Müllerian duct syndrome

OBJECTIVES To report the findings and management of patients with persistent Müllerian duct syndrome (PMDS). PATIENTS AND METHODS Nineteen phenotypically male patients (aged 8 months to 27 years) presented with testicular maldescent. All of them had normal male external genitalia. Two of them had had a previous diagnosis of persistent Müllerian structures. All patients were karyotyped, and ha...

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Persistent müllerian duct syndrome--a case report.

An eighteen month old phenotypically and genotypically normal male child was admitted with a left inguinal hernia and a right undescended testis. At operation, he was found to have a uterus, bilateral fallopian tubes, and a vagina in the left hernial sac. Bilateral orchidopexies and excision of the persistent Mullerian duct structures were carried out. This rare case of persistent Mullerian duc...

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Management of the patients with persistent Müllerian duct syndrome: Is the ultimate goal testicular descent?

Objective Persistent Müllerian duct syndrome (PMDS) is a rare congenital disease characterized by the presence of rudimentary Müllerian structures within an intra-abdominal or hernial sac in a virilized male, often presenting as undescended testes. In this study, we aim to present a series of the PMDS patients who were managed by orchiopexy without removal of Müllerian remnants (MR). Material...

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Persistent Müllerian duct syndrome: A novel mutation in the Αnti-Müllerian Ηormone gene.

BACKGROUND AND OBJECTIVE Persistent Müllerian duct syndrome (PMDS) is a relatively rare form of 46,XY disorder of sex development caused by the failure of formation, release or action of anti-Müllerian hormone (AMH) in intrauterine life. In this report we describe a case diagnosed with PMDS with a novel homozygous mutation in the AMH gene. CASE REPORT A 4-month-old male presented with bilater...

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Bilateral Cryptorchidism, a rare presentation for persistent Müllerian duct syndrome

Persistent Müllerian duct syndrome (PMDS) is a rare, sex-limited, autosomal recessive disorder representing male pseudo-hermaphroditism. It is observed in males with the presence of female reproductive organs such as the uterus, cervix, and bilateral fallopian tubes along with normally developed male reproductive organs. It generally occurs during embryogenesis due to mutation in anti-Müllerian...

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ژورنال

عنوان ژورنال: Arab Journal of Urology

سال: 2014

ISSN: 2090-598X

DOI: 10.1016/j.aju.2014.04.001