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Vaginal Reconstruction in Patients with Mayer-Rokitansky-Kuster-Hauser Syndrome-One Centre Experience.

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.

SUBMITTER: Avino A

PROVIDER: S-EPMC7404697 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature

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Vaginal Reconstruction in Patients with Mayer-Rokitansky-Küster-Hauser Syndrome-One Centre Experience.

Avino Adelaida A Răducu Laura L Tulin Adrian A Gheoca-Mutu Daniela-Elena DE Balcangiu-Stroescu Andra-Elena AE Marina Cristina-Nicoleta CN Jecan Cristian-Radu CR

Medicina (Kaunas, Lithuania) 20200701 7

<i>Background and Objectives:</i> 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. <i>Materials and Methods:</i> We perfor ...[more]

PMID: 32630225

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Project description:BACKGROUND:Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also referred to as Müllerian aplasia, is a congenital disorder characterized by aplasia of the uterus and upper part of the vagina in females with normal secondary sex characteristics and a normal female karyotype (46,XX). MAIN BODY:The diagnosis is often made during adolescence following investigations for primary amenorrhea and has an estimated prevalence of 1 in 5000 live female births. MRKH syndrome is classified as type I (isolated uterovaginal aplasia) or type II (associated with extragenital manifestations). Extragenital anomalies typically include renal, skeletal, ear, or cardiac malformations. The etiology of MRKH syndrome still remains elusive, however increasing reports of familial clustering point towards genetic causes and the use of various genomic techniques has allowed the identification of promising recurrent genetic abnormalities in some patients. The psychosexual impact of having MRKH syndrome should not be underestimated and the clinical care foremost involves thorough counselling and support in careful dialogue with the patient. Vaginal agenesis therapy is available for mature patients following therapeutical counselling and education with non-invasive vaginal dilations recommended as first-line therapy or by surgery. MRKH syndrome involves absolute uterine factor infertility and until recently, the only option for the patients to achieve biological motherhood was through gestational surrogacy, which is prohibited in most countries. However, the successful clinical trial of uterus transplantation (UTx) by a Swedish team followed by the first live-birth in September, 2014 in Gothenburg, proofed the first available fertility treatment in MRKH syndrome and UTx is now being performed in other countries around the world allowing women with MRKH syndrome to carry their own child and achieve biological motherhood. CONCLUSION:Several advances in research across multiple disciplines have been made in the recent years and this kaleidoscopic review provides a current status of various key aspects in MRKH syndrome and provides perspectives for future research and improved clinical care.

Dataset Information

Vaginal Reconstruction in Patients with Mayer-Rokitansky-Kuster-Hauser Syndrome-One Centre Experience.

Publications

Vaginal Reconstruction in Patients with Mayer-Rokitansky-Küster-Hauser Syndrome-One Centre Experience.

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