Creation of a neovagina in a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and previously corrected rectovestibular fistula concomitant with imperforate anus.

Background: Congenital absence of uterus and vagina (CAUV) when associated with anorectal malformations is usually diagnosed and repaired in infancy at the time of anorectoplasty. Long-term observations of patients are scarce and do not justify early vaginal reconstruction. Question arises whether creation of a neovagina can be safely and successfully performed when the patient is mature.

Case: The patient, diagnosed with MRKH syndrome at 16 years of age, underwent repair of rectovestibular fistula and imperforate anus ("cut-back" procedure, temporal sigmostomy and sagittal anterior anorectoplasty) in infancy. At 18, modified Wharton vaginoplasty was performed with a good anatomico-functional outcome.

Summary And Conclusions: Early repair of anorectal malformation and postponed vaginal reconstruction seem to be a viable option for patients with congenital rectovestibular fistula and anal atresia concomitant with CAUV.