Surgical management of persistent müllerian duct syndrome

To describe the optimal surgical management of the testes and müllerian duct structures in patients with persistent müllerian duct syndrome.

We performed a comprehensive Medline literature search regarding the surgical management of persistent müllerian duct syndrome and extracted information regarding the etiology, pathogenesis, and treatment of this disorder. We specifically assessed the risks of retained müllerian structures versus surgical excision of the infantile uterus and fallopian tubes. Using this information, we formulated a comprehensive strategy for the management of patients with persistent müllerian duct syndrome. An illustrative case is described.

No malignant degeneration of persistent müllerian structures has been reported. The risk of testicular neoplasia in persistent müllerian duct syndrome approximates the risk of neoplasia in other intra-abdominal gonads. Fertility has rarely been reported although virilization is unaffected. Surgical excision of the infantile uterus and fallopian tubes risks damage to vasa deferentia and the deferential blood supply to the testis.

Surgical excision of persistent müllerian duct structure may result in ischemic and/or traumatic damage to the vasa deferentia and testes. Optimal surgical management is orchiopexy leaving the uterus and fallopian tubes in situ. Meticulous proximal salpingectomy and hysterectomy is indicated only in patients whose müllerian structures limit intrascrotal placement of the tests. Orchiectomy is indicated for testes that cannot be mobilized to a palpable location.