Congenital malformations of the genital tract and their management

Best Pract Res Clin Obstet Gynaecol. 2003 Feb;17(1):19-40. doi: 10.1053/ybeog.2003.0356.


While congenital malformations of the genital tract are not common, the sequelae of their presence can be serious. The practising gynaecologist must be aware of the range of congenital abnormalities that may occur and the symptoms that may result from them. Failure to manage these patients correctly may have long-term sequelae for their psychological, sexual and reproductive health. The involvement of a multi-disciplinary team in dealing with these patients is imperative, and preparation for surgery-particularly in congenital malformations of the vulva and the vagina-is imperative if the long-term sexual function in these patients is to be fulfilled. Surgical correction of vulval abnormalities in adolescence is related solely to sexual function as most of the reconstructive surgery is done in childhood. For the management of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, the recommendation is now that passive dilatation by Franks' technique is the treatment of first choice and only if that fails should surgical approaches be embarked upon. The results of the surgery are similar in all techniques and the particular surgical centre will have its own preference of which technique it adopts. Congenital absence of the cervix is a complex surgical problem and should be dealt with solely in centres with expertise.

Publication types

  • Review

MeSH terms

  • Cervix Uteri / abnormalities
  • Female
  • Genitalia, Female / abnormalities*
  • Genitalia, Female / surgery*
  • Gynecologic Surgical Procedures / methods*
  • Humans
  • Uterus / abnormalities
  • Vagina / abnormalities
  • Vulva / abnormalities