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The togetherness of persistent mullerian duct syndrome, transverse testicular ectopia and hypospadias

Year 2011, Volume: 6 Issue: 2, 48 - 50, 01.05.2011

Abstract

Persistent Müllerian duct syndrome PMDS is characterized by the presence of müllerian duct structures such as fallopian tubes, uterus and 1/3 upper part of vagina in the phenotypic male. It is a rare form of male pseudohermaphroditism. This syndrome is rarely associated with transverse testicular ectopia TTE . PMDS and TTE is extremely rare. Patients are normally virilised, although uni- or bilateral cryptorchidism may be present. In this paper we report a PMDS associated with TTE and hypospadias in 5 years old boy

References

  • Imbeaud S, Belville C, Messika-Zeitoun L, Rey R, di Cle- mente N, Josso N, Picard JY. A 27 base-pair deletion of the anti-mullerian type II receptor gene is the most common cause of the persistent mullerian duct syndrome. Hum Mol Genet 5: 1269–1277, 1996.
  • Cohen-Haguenauer O, Picard JY, Mattéi MG, Serero S, Nguyen VC, de Tand MF, Guerrier D, Hors-Cayla MC, Jos- so N, Frézal J. Mapping of the gene for antimüllerian hor- mone to the short arm of human chromosome 19. Cytoge- net Cell Genet 44: 2–6, 1987.
  • Macedo A Jr, Barroso U Jr, Ottoni SL, Ortiz V. Transverse testikular ectopia and persistent müllerian duct syndrome. J Pediatr Urol 5: 234–236, 2009.
  • Tiryaki T, Hucumenoglu S, Atayurt H: Transverse testi- cular ectopia associated with persistent mullerian duct syndrome. Urol Int 74: 190–192, 2005.
  • Vandersteen DR, Chaumeton AK, Ireland K, Tank ES. Sur- gical management of persistent müllerian duct syndrome. Urology 49: 941–945,1997.
  • Josso N, Picard JY, Imbeaud S, et al: The persistent mulle- rian duct syndrome: A rare cause of cryptorchidism. Eur J Pediatr 152: 76–78, 1993.
  • Karnak I, Tanyel FC, Akcoren Z, et al: Transverse testicular ectopia with persistent müllerian duct syndrome. J Pediatr Surg 32: 1362–1364, 1997.
  • Eastham JA, McEvoy K, Sullivan R, et al: A case of simul- taneous bilateral nonseminomatous testicular tumors in persistent müllerian duct syndrome. J Urol 148: 407–408, 1992.

Persistan müllerian kanal sendromu, transvers testiküler ektopi ve hipospadias birlikteliği

Year 2011, Volume: 6 Issue: 2, 48 - 50, 01.05.2011

Abstract

Persistan müllerian kanal sendromu PMKS , erkek fenotipinde tuba uterina, uterus ve vajenin 1/3 üst kısmı gibi müllerian artıkların bulunduğu bir sendromdur. Erkek psödohermafroditizmin nadir bir şeklidir. Bu sendrom nadiren transvers testiküler ektopi TTE ile birlikte olabilir. PMDS ve TTE birlikteliği çok nadir bir patolojidir. Hastalar genellikle normal erkek görünümünde, tek ya da iki taraflı inmemiş testisle birliktedir. Bu yazıda PMKS, TTE ve hipospadiası bulunan 5 yaşında hasta sunulmaktadır

References

  • Imbeaud S, Belville C, Messika-Zeitoun L, Rey R, di Cle- mente N, Josso N, Picard JY. A 27 base-pair deletion of the anti-mullerian type II receptor gene is the most common cause of the persistent mullerian duct syndrome. Hum Mol Genet 5: 1269–1277, 1996.
  • Cohen-Haguenauer O, Picard JY, Mattéi MG, Serero S, Nguyen VC, de Tand MF, Guerrier D, Hors-Cayla MC, Jos- so N, Frézal J. Mapping of the gene for antimüllerian hor- mone to the short arm of human chromosome 19. Cytoge- net Cell Genet 44: 2–6, 1987.
  • Macedo A Jr, Barroso U Jr, Ottoni SL, Ortiz V. Transverse testikular ectopia and persistent müllerian duct syndrome. J Pediatr Urol 5: 234–236, 2009.
  • Tiryaki T, Hucumenoglu S, Atayurt H: Transverse testi- cular ectopia associated with persistent mullerian duct syndrome. Urol Int 74: 190–192, 2005.
  • Vandersteen DR, Chaumeton AK, Ireland K, Tank ES. Sur- gical management of persistent müllerian duct syndrome. Urology 49: 941–945,1997.
  • Josso N, Picard JY, Imbeaud S, et al: The persistent mulle- rian duct syndrome: A rare cause of cryptorchidism. Eur J Pediatr 152: 76–78, 1993.
  • Karnak I, Tanyel FC, Akcoren Z, et al: Transverse testicular ectopia with persistent müllerian duct syndrome. J Pediatr Surg 32: 1362–1364, 1997.
  • Eastham JA, McEvoy K, Sullivan R, et al: A case of simul- taneous bilateral nonseminomatous testicular tumors in persistent müllerian duct syndrome. J Urol 148: 407–408, 1992.
There are 8 citations in total.

Details

Primary Language Turkish
Authors

Ergün Elaltuntaş This is me

Akif Koç This is me

Publication Date May 1, 2011
Published in Issue Year 2011 Volume: 6 Issue: 2

Cite

Vancouver Elaltuntaş E, Koç A. Persistan müllerian kanal sendromu, transvers testiküler ektopi ve hipospadias birlikteliği. New J Urol. 2011;6(2):48-50.