Abstract
Persistent Mullerian Duct Syndrome with Transverse Testicular Ectopia Presenting as an Irreducible Inguinal Hernia
Highlights
persistent müllerian duct syndrome (PMDS) is an uncommon disorder of sex differentiation in male fetuses
Mutations of either anti-Müllerian hormone (AMH) or AMH receptor’s (AMHR) genes are the genetic basis of the syndrome which leads to persistence of Müllerian derivatives as uterus, fallopian tube, cervix and seldom the upper vagina
We present a rare case of a male newborn with a history of bilateral undescended testis which presented with left irreducibleinguinal hernia
Summary
PMDS is an uncommon disorder of sex differentiation in male fetuses. Testosterone and anti-Müllerian hormone (AMH) secreted by the testes during the 7 weeks of gestation are responsible for the prevalence of the Wolffian duct and the regression of Müllerian duct, respectively, in the male embryo.Mutations of either AMH or AMH receptor’s (AMHR) genes are the genetic basis of the syndrome which leads to persistence of Müllerian derivatives as uterus, fallopian tube, cervix and seldom the upper vagina. PMDS is an uncommon disorder of sex differentiation in male fetuses. Testosterone and anti-Müllerian hormone (AMH) secreted by the testes during the 7 weeks of gestation are responsible for the prevalence of the Wolffian duct and the regression of Müllerian duct, respectively, in the male embryo. Mutations of either AMH or AMH receptor’s (AMHR) genes are the genetic basis of the syndrome which leads to persistence of Müllerian derivatives as uterus, fallopian tube, cervix and seldom the upper vagina. We present a rare case of a male newborn with a history of bilateral undescended testis which presented with left irreducibleinguinal hernia
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