12 - Case report: Persistent mullerian duct syndrome
Canan Tanık, Tuğba Taşkın, Mehmet Yalçın
Persistent mullerian duct syndrome also named as hernia uteri inguinale is a form of internal male pseudohermafroditsm and may result from the failure of synthesis or release of AMH, defect of AMH receptors or the defect in the timing of the release of AMH. This syndrome is an inherited disorder, it is usually transmitted as a recessive autosomal trait or X linked trait.
Patients affected with PMDS are phenotypic males with either bilateral cryptoorchidism or unilateral testicular ectopia associated with inguinal hernia on the contralateral side. The presence of uterus and tubes is usually discovered at surgery. Three month old male child operated for cryptoorchidism and found out to be PMDS after the examination of the operation material is reported. This is a comprehensive review of the literature with evaluation of the clinical features.
Keywords: Persistent Mullerian Duct Syndrome, Anti Mullerian Hormon
To read full article click