The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital abnormality in which agenesis of the müllerian duct is associated with a short or aplastic vagina and a rudimentary or absent uterus despite functioning ovaries. Those affected have primary amenorrhea and are unable to have normal intercourse. One of several conservative and surgical treatments proposed for these patients is the technique described by Vecchietti that combines surgery and dilatation. The present investigators used a minimally invasive laparoscopic version of the Vecchietti procedure in 8 patients with MRKH syndrome in the years 1996–2005, with the goal of creating a functioning neovagina. Outcomes were assessed by semistructured telephone interviews. The median postoperative follow-up period was 40 months.
The patients, whose mean age at presentation was 22 years, had primary amenorrhea and wished to be sexually active. All were operated on by the same surgeon. A vagina of adequate length, 7 cm or more, was achieved in all patients but one. The exceptional patient failed to begin regular sexual activity and did not regularly use vaginal dilators. The median vaginal length at follow-up was 1.5 cm. Six patients began having sexual intercourse 2 to 24 months after surgery, the median interval being 6.8 months. Intercourse was consistently described as satisfactory; the mean value on a 10-point scale was 7.8. Nevertheless, 4 of 6 patients sometimes had minor pain during sexual activity. All patients but one asserted that they would have the procedure again.
These findings demonstrate that the laparoscopic version of the Vecchietti procedure is an effective and safe means of creating a neovagina in patients with MRKH syndrome. Good long-term results require regular dilation of the reconstructed vagina by either intercourse or the use of dilators.