Ileum neovaginoplasty for Mayer–Rokitansky–Küster–Hauser: Review and case series

Abstract

Objective: To review treatment modalities of Mayer–Rokitansky–Küster–Hauser syndrome, and to present further evidence on the successful use of ileum segment as an additional procedure for the creation of a neovagina.

Methods: Five women presented with primary amenorrhea, normal secondary female sexual characteristics, normal external anatomy, shortening of the vagina, with only vaginal dimples. Abdominal ultrasound scans suggested the absence of uterus. Both ovaries were present with normal kidneys. Karyotyping confirmed XX genotype. Pelvi-abdominal MRI confirmed the diagnoses. All patients were started on nonsurgical treatment, in the form of graduated dilators, as a first-line approach. This was not acceptable to the patients. Decisions were made to resort to ileum vaginoplasty.

Results: The mean surgical time was 5 h. Three patients sustained intra-operative bladder injury. All patients reported watery vaginal discharge. Four patients were followed up for 5 years, and were satisfied with their results. This was confirmed by clinical examination. The fifth patient failed to attend for immediate postoperative care. The vagina was 6–7 cm long with introital stricture.

Conclusion: Women with Mayer–Rokitansky–Küster–Hauser syndrome who need vaginal reconstruction have the surgical option of a vascularized free ileum graft that provides a durable, lubricated neovagina without the scarring of skin grafts.

Keywords: Vaginal agenesis; Mayer–Rokitansky– Küster–Hauser syndrome; Ileum segment neovagina