Abstract

Background and Objectives: The Mayer–Rokitansky–Küster–Hauser syndrome is a congenital condition in which patients are born with vaginal and uterus agenesis, affecting the ability to have a normal sexual life and to bear children. Vaginal reconstruction is a challenging procedure for plastic surgeons. The aim of this study is to report our experience in the management of twelve patients with congenital absence of the vagina due to the MRKH syndrome. Materials and Methods: We performed a retrospective study on 12 patients admitted to the Plastic Surgery Department of the Clinical Emergency Hospital “Prof. Dr. Agrippa Ionescu”, Bucharest, Romania, for vaginal reconstruction within a period of eleven years (January 2009–December 2019). All patients were diagnosed by the gynaecologists with vaginal agenesis, as part of the Mayer–Rokitansky–Küster–Hauser syndrome. The Abbe‘–McIndoe technique with an autologous skin graft was performed in all cases. Results: The average age of our patients was 20.16 (16–28) years. All patients were 46 XX. The average surgical timing was 3.05 h (range 2.85–4h). Postoperative rectovaginal fistula was encountered in 1 patient. Postoperative average vaginal length was 10.4 cm (range 9.8–12.1 cm). Regular sexual life was achieved in 10 patients. Conclusion: Nowadays, there is no established standard method of vaginal reconstruction. In Romania, the McIndoe technique is the most applied. Unfortunately, even if the MRKH syndrome is not uncommon, less and less surgeons are willing to perform the procedure to create a neovagina.

Highlights

  • The Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is considered to be the second most frequent cause of primary amenorrhea [1] being characterized by congenital absence of the uterus, and the upper part (2/3 proximal) of the vagina

  • The aim of this study is to report our experience in the management of twelve patients with congenital absence of the vagina due to the MRKH syndrome

  • We conducted a retrospective study on 12 patients admitted to the Plastic Surgery Department of the Clinical Emergency Hospital “Prof. Dr Agrippa Ionescu”, Bucharest, Romania, for vaginal reconstruction within a period of eleven years (January 2009–December 2019)

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Summary

Introduction

The Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is considered to be the second most frequent cause of primary amenorrhea [1] being characterized by congenital absence of the uterus, and the upper part (2/3 proximal) of the vagina. The first one is characterised by solitary absence of the proximal two-thirds of the vagina [5] and agenesis of the uterus [4], two symmetric rudimentary horns are present. They are linked by a peritoneal fold to the fallopian tubes, whereas the ovaries and the renal system have a normal development. The Mayer–Rokitansky–Küster–Hauser syndrome is a congenital condition in which patients are born with vaginal and uterus agenesis, affecting the ability to have a normal sexual life and to bear children.

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