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MüLLERIAN DUCT SYNDROME
Category: Pediatric Surgery
Abstract : Müllerian Duct Syndrome (MDS) refers to a genetic disorder of male pseudohermaphroditism (46 XY karyotype) characterized by normal masculinization of the external genitalia and the presence of uterus and fallopian tubes. Most cases are discovered during surgery for undescended testis, inguinal hernia or transverse testicular ectopia. MDS is caused by a deficient activity (most cases) or receptor i
Müllerian Duct Syndrome (MDS) refers to a genetic disorder of male pseudohermaphroditism (46 XY karyotype) characterized by normal masculinization of the external genitalia and the presence of uterus and fallopian tubes. Most cases are discovered during surgery for undescended testis, inguinal hernia or transverse testicular ectopia.
MDS is caused by a deficient activity (most cases) or receptor insensitivity of anti-müllerian hormone (also known as Müllerian inhibitor factor). This hormone is produced by testicular Sertoli cells and is responsible of producing fetal regression of Müllerian structures (uterus & fallopian tubes) in genetic males. Most cases are transmitted as autosomal recessive restricted to males (sex-linked). Anatomic variants include fallopian tube or uterus within the inguinal canal, testis and tubes in a hernia sac or bilateral cryptorchidism with the testes embedded in the broad ligaments. The vas deferens is intimately adhered to the uterus lateral wall. Initial procedure consists of hernia repair, replacement of structure within pelvis and karyotype. After diagnosis follow-up management has been controversial. A few suggest partial removal of the uterus (leaving vas deferens intact on a thin pedicle of myometrium) and fallopian tubes with testicular fixation. Most content that surgical excision of persistent MDS structure may result in ischemic or traumatic damage to the vasa deferentia and testes and optimal management is orchiopexy leaving the uterus and fallopian tubes in situ. The testis in MDS are at risk of malignant degeneration.
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