Clinical Features of the Persistent Mullerian Duct Syndrome

Description

The Persistent Mullerian Duct Syndrome (PMDS) occurs when Mullerian duct structures fail to regress in a male. Control of male genital development is determined by action of androgen from Leydig cells and Mullerian regression factor from Sertoli cells.

Synonym: hernia uteri inguinalis

Clinical features:

(1) The patient is a phenotypic male.

(2) There may be an undescended testis on one or both sides.

(3) A hernia is present that contains Mullerian structures (vagina, uterus, and/or fallopian tube).

(4) The patient may be infertile.

Laboratory findings:

(1) genotypically male (XY)

(2) seman analysis may show an absence of spermatozoa

Excision of the Mullerian structures may be difficult since they may be intertwined with normal male structures.

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Description

Specialty

ICD-10