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. 2010 Feb 5;72(1):16–19. doi: 10.1007/s12262-010-0003-x

Persistent müllerian duct syndrome: How to deal with the müllerian duct remnants — a review

B G Manjunath 1,✉, Vasanth G Shenoy 2, Preetham Raj 1
PMCID: PMC3452545  PMID: 23133198

Abstract

Persistent müllerian duct syndrome (PMDS) is usually a surprise finding either during orchidopexy or during routine inguinal hernia repair in male patients. Often, the surgeon would face a dilemma about what is to be done with the remnants of müllerian duct, i.e. the fallopian tubes, uterus and proximal vagina. Till recently, it was advised to retain these structures whenever it was felt that the complete excision of these structures may jeopardise the blood supply and integrity of the vas deferens. Recent reports of malignancy in these retained structures would justify more aggressive approach. In our patients with PMDS, we have removed the mucosa of the retained müllerian structures, without compromising the integrity and vascularity of the vas deferens, thus reducing the chances of malignancy.

Keywords: Persistent müllerian duct syndrome, Transverse testicular ectopia, Hernia uteri inguinale, Müllerian inhibiting substance

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References

  • 1.El-Gohary M.A. Laparoscopic management of persistent müllerian duct syndrome. Pediatr Surg Int. 2003;19(7):533–536. doi: 10.1007/s00383-003-0984-7. [DOI] [PubMed] [Google Scholar]
  • 2.Josso N., Belville C., Clemente N. AMH and AMH receptor defects in persistent müllerian duct syndrome. Hum Reprod Update. 2005;11(4):351–356. doi: 10.1093/humupd/dmi014. [DOI] [PubMed] [Google Scholar]
  • 3.Hoshiya M., Christian B.P., Cromie W.J. Persistent müllerian duct syndrome caused by both a 27-bp deletion and a novel splice mutation in the MIS type II receptor gene. Birth Defects Res A Clin Mol Teratol. 2003;67(10):868–874. doi: 10.1002/bdra.10091. [DOI] [PubMed] [Google Scholar]
  • 4.Loeff D.S., Imbeaud S., Reyes H.M. Surgical and genetic aspects of persistent müllerian duct syndrome. J Pediatr Surg. 1994;29(1):61–65. doi: 10.1016/0022-3468(94)90525-8. [DOI] [PubMed] [Google Scholar]
  • 5.Berkman F. Persistent müllerian duct syndrome with or without transverse testicular ectopia and testis tumours. Br J Urol. 1997;79(1):122–126. doi: 10.1046/j.1464-410x.1997.27226.x. [DOI] [PubMed] [Google Scholar]
  • 6.Vandersteen D.R., Chaumeton A.K., Ireland K. Surgical management of persistent müllerian duct syndrome. Urology. 1997;49(6):941–945. doi: 10.1016/S0090-4295(97)00104-0. [DOI] [PubMed] [Google Scholar]
  • 7.Brandli D.W., Akbal C., Eugsster E. Persistent müllerian duct syndrome with bilateral abdominal testis: Surgical approach and review of the literature. J Pediatr Urol. 2005;1(6):423–427. doi: 10.1016/j.jpurol.2005.03.011. [DOI] [PubMed] [Google Scholar]
  • 8.Lima M., Morabito A., Libri M. Laparoscopic removal of persistent müllerian duct in a male: Case report. Eur J Pedtr Surg. 2000;10(4):265–269. doi: 10.1055/s-2008-1072371. [DOI] [PubMed] [Google Scholar]
  • 9.Thiel D.D., Erhard M.J. Uterine adenosarcoma in a boy with persistent müllerian duct syndrome: first reported case. J Pedtr Surg. 2005;40(9):e29–31. doi: 10.1016/j.jpedsurg.2005.05.071. [DOI] [PubMed] [Google Scholar]
  • 10.Romereo F.R., Fucs M., Castro M.G. Adenocarcinoma of persistent müllerian duct remnants, case report and differential diagnosis. Urology. 2005;66(1):194–195. doi: 10.1016/j.urology.2005.01.024. [DOI] [PubMed] [Google Scholar]
  • 11.Shinmura Y., Yokoi T., Tsutsui Y. A case of clear cell adenocarcinoma of the müllerian duct in persistent müllerian duct syndrome: The first reported case. Am J Surg Pathol. 2002;26(9):1231–1234. doi: 10.1097/00000478-200209000-00014. [DOI] [PubMed] [Google Scholar]
  • 12.Lima M., Domini M., Libri M. Persistent müllerian duct syndrome associated with transverse testicular ectopia: A case report. Eur J Pedtr Surg. 1997;7(1):60–62. doi: 10.1055/s-2008-1071055. [DOI] [PubMed] [Google Scholar]
  • 13.Martin E.L., Benette A.H., Cromie W.J. Persistent müllerian duct syndrome with transverse testicular ectopia and spermatogenesis. J Urol. 1992;147(6):1615–1617. doi: 10.1016/s0022-5347(17)37646-2. [DOI] [PubMed] [Google Scholar]
  • 14.Imbeaud S., Rey R., Berta P. Testicular degeneration in three patients with the persistent müllerian duct syndrome. Eur J Pediatr. 1995;154(3):187–190. doi: 10.1007/BF01954268. [DOI] [PubMed] [Google Scholar]
  • 15.Shirasaki Y., Nagai A., Nasu Y. Laparoscopic removal of müllerian structures and orchiopexy for persistent müllerian duct syndrome. Urology. 2003;62(6):1121. doi: 10.1016/j.urology.2003.07.005. [DOI] [PubMed] [Google Scholar]
  • 16.Turaga K.K., St Peter S.D., Calkins C.M. Hernia uterus inguinale: a proposed algorithm using laparoscopic approach. Surg Laparosc Endosc Percutan Tech. 2006;16(5):366–367. doi: 10.1097/01.sle.0000213722.49838.44. [DOI] [PubMed] [Google Scholar]
  • 17.Ng J.W., Koh G.H. Laparoscopic orchidopexy for persistent müllerian duct syndrome. Pediatr Surg Int. 1997;12(7):522–525. doi: 10.1007/BF01258717. [DOI] [PubMed] [Google Scholar]
  • 18.Otsuka T., Nakasono T., Matsuo Y. Persistent müllerian duct syndrome suggested by magnetic resonance imaging. Radiat Med. 2003;21(6):277–299. [PubMed] [Google Scholar]
  • 19.Cesare E., Bartolo, DeVincentiis US and MRI in a case of persistent müllerian duct syndrome. Pediatr Radiol. 1998;28(11):865–867. doi: 10.1007/s002470050484. [DOI] [PubMed] [Google Scholar]

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