A visual pitfall: Persistent Mullerian duct syndrome (PMDS).

A visual pitfall: Persistent Mullerian duct syndrome (PMDS). Research Abstract Details 

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A visual pitfall: Persistent Mullerian duct syndrome (PMDS). Abstract Text:

K A Marcus,F J J Halbertsma,J Y Picard,B J Otten,

Persistent Müllerian Duct Syndrome (PMDS) is a rare disorder of the anti-mullerian hormone (AMH) synthesis or receptor, which due to the visual contrast of normal masculine external genitalia and female internal genitalia can raise confusion, sometimes during surgery for cryptorchidism or hernia inguinalis. For an acute and accurate analysis of such a situation a thorough knowledge of gonadal embryology is mandatory. The diagnosis is made on finding Müllerian structures in an individual with complete virilization without signs of hypocortisolism or exposition to maternal androgens during foetal life. Karyotyping and gonadal biopsy provide additional information to confirm the diagnosis. As the risk of malignant transformation is not clear, orchidopexy is advised in patients with cryptorchidism, with lifelong palpatory follow-up. In case of urologic symptoms, surgical removal of the Müllerian remnants can be considered, with careful attention for the vulnerable ductus deferens. Despite optimal treatment the prognosis regarding fertility remain uncertain.

A visual pitfall: Persistent Mullerian duct syndrome (PMDS). Publishing Authors By Initials

KA Marcus,FJ Halbertsma,JY Picard,BJ Otten,

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A visual pitfall: Persistent Mullerian duct syndrome (PMDS). Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Acta paediatrica (Oslo, Norway : 1992)

VOLUME: 97

Page Numbers: 129-32

Journal Abbreviation: Acta Paediatr.

ISSN: 0803-5253

DAY: 3

MONTH: 12

YEAR: 2007

A visual pitfall: Persistent Mullerian duct syndrome (PMDS). Information

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LANGUAGE: eng

NlmUniqueID: 9205968

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Grant and Affiliation Information for A visual pitfall: Persistent Mullerian duct syndrome (PMDS).

AFFILIATION: Department of Paediatrics, Màxima Medical Centre Veldhoven, The Netherlands.

Country: Norway

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MEDLINETA: Acta Paediatr

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