persistent mullerian duct syndrome

Persistent Mullerian duct syndrome (PMDS) is a rare form of internal male pseudohermaphroditism in which Mullerian duct derivatives are seen in a male patient.

This syndrome is characterized by the persistence of Mullerian duct derivatives (i.e. uterus, cervix, fallopian tubes and upper two thirds of vagina) in a phenotypically and karyotypically male patient.

Synopsis

 bilateral cryptorchidism
 inguinal hernia containing the uterus and fallopian tubes
 transverse testicular ectopia (TTE) is rare.
 Persistent mullerian duct syndrome with TTE may be encountered when performing laparoscopy for patients with nonpalpable testis.
 The persistent mullerian remnants vary among individuals and alter the normal anatomy, thus may complicate diagnosis and surgical management.

Associations

 uterine adenosarcoma in boy (16150330)
 clear cell adenocarcinoma of the müllerian duct (12218580)

References

 Persistent mullerian duct syndrome. Renu D, Rao BG, Ranganath K, Namitha. Indian J Radiol Imaging. 2010 Feb;20(1):72-4. PMID: 20352001[Free]

 Tuberculosis of transverse testicular ectopic testis associated with persistent mullerian duct syndrome. Kella N, Rathi PK, Qureshi MA. J Coll Physicians Surg Pak. 2008 Apr;18(4):250-1. PMID: 18474165

 Persistent mullerian duct syndrome and transverse testicular ectopia: embryology, presentation, and management. Wuerstle M, Lesser T, Hurwitz R, Applebaum H, Lee SL. J Pediatr Surg. 2007 Dec;42(12):2116-9. PMID: 18082721

 Uterine adenosarcoma in a boy with persistent müllerian duct syndrome: first reported case. Thiel DD, Erhard MJ. J Pediatr Surg. 2005 Sep;40(9):e29-31. PMID: 16150330

 A case of clear cell adenocarcinoma of the müllerian duct in persistent müllerian duct syndrome: the first reported case. Shinmura Y, Yokoi T, Tsutsui Y. Am J Surg Pathol. 2002 Sep;26(9):1231-4. PMID: 12218580