Mayer–Rokitansky–Küster–Hauser syndrome managed with McIndoe’s vaginoplasty: a case series and literature review

Abstract

Introduction: Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a congenital anomaly characterized by the absence of the uterus and the upper two-thirds of the vagina. It is a rare congenital anomaly with an incidence of 1 in 5000 female live births. Case series: The authors describe three cases of females presenting with primary amenorrhoea who were diagnosed with MRKH syndrome. The patients were managed with McIndoe’s vaginoplasty with neovagina creation with an amnion graft. Discussion: Management of MRKH syndrome involves vaginoplasty with neovagina creation. The approach to neovagina creation can be done surgically or non-surgically. Non-surgical creation of the vaginal cavity involves serial use of vaginal dilators, while there are several ways for surgical creation of neovagina. The modified Abbe-McIndoe procedure using amnion to create neovagina is a minimally invasive, rapid, and simple procedure with no risk of immune rejection because the amnion membrane lacks histocompatibility antigens. In addition, the graft is also readily available, storable, and inexpensive. Conclusion: Diagnosis of MRKH syndrome can be made when a young female with primary amenorrhoea and normal secondary sexual characteristics has agenesis of the uterus, and upper two-thirds of the vagina revealed on ultrasonography or magnetic resonance imaging. The patient can be offered treatment with vaginoplasty with neovagina creation.