Creation of a Neovagina in a Patient with Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome and Previously Corrected Rectovestibular Fistula Concomitant with Imperforate Anus

Abstract 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.