Surgery is not superior to dilation for the management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome: a multicenter comparative observational study in 131 patients

Cheikhelard, Alaa; Bidet, Maud; Baptiste, Amandine; Viaud, Magali; Fagot, Christine; Khen-Dunlop, Naziha; Louis-Sylvestre, Christine; Sarnacki, Sabine; Touraine, Philippe; Elie, Caroline; Aigrain, Yves; Polak, Michel

doi:10.1016/j.ajog.2018.07.015

Cited by 56 publications

(43 citation statements)

References 19 publications

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“…Therapeutical education throughout the treatment is pivotal to achieve success and the patient should be followed closely in order to monitor progress and provide good support and guidance. An alternative non-invasive option is dilation by intercourse (d'Alberton's method) [138] which has been reported with good anatomical and functional outcome compared with self-dilation and surgery [118,139]. This method, however, requires regular coital activity with a partner and thus, this approach is not an option for all patients.…”

Section: Management Of Vaginal Agenesismentioning

confidence: 99%

“…Knowledge of the psychological aspects in MRKH syndrome remains limited but studies have agreed on presence of higher levels of psychological distress in patients compared with women without MRKH syndrome [ 114 , 115 ]. Most studies have been focusing on sexual function and well-being using quantitative survey testing such as Female Sexual Functioning Index (FSFI) and Female Sexual Distress Scale-Revised (FSDS-R), which particularly have been used measuring functional outcome following neovaginal therapy [ 116 – 118 ]. Other studies have also focused on specific psychological/psychiatric symptoms such as self-esteem, depression and anxiety [ 114 , 115 , 119 , 120 ].…”

Section: Main Textmentioning

confidence: 99%

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Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update

Herlin

Petersen

Brännström³

2020

Orphanet J Rare Dis

191

160

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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.

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Section: Management Of Vaginal Agenesismentioning

confidence: 99%

Section: Main Textmentioning

confidence: 99%

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update

Herlin

Petersen

Brännström³

2020

Orphanet J Rare Dis

191

160

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“…Numerous instrumental and surgical therapies have been described treating vaginal aplasia [1,3,9]. The main objective is to restore a sexually active life by restoring a conventional vaginal size, which is usually defined by a length ≥ 6-7 cm and a sufficient diameter between 3-3.5 cm (admitting two fingers) [9][10][11][12][13].…”

Section: Discussionmentioning

confidence: 99%

“…These vaginal dilations must be performed before considering any surgical procedure [1,3,10]. Regular sexual intercourse may be just as effective as vaginal dilatation in some cases [9,10,12,13].…”

Section: Discussionmentioning

confidence: 99%

Sigmoid vaginoplasty in Mayer-Rokitansky-Kuster-Hauser syndrome

et al. 2020

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Introduction: Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is characterized by congenital aplasia of the uterus and upper 2/3 of the vagina, with normal female secondary sexual characteristics and a normal karyotype (46, XX). The frequency is 1/4500-5000 female births. The aim of this study was to report the management of MRKH syndrome with sigmoid vaginoplasty. Patients and method: This study included 4 patients recruited over a 4-year period from February 2016 to January 2019. MRKH syndrome was retained in the presence of normal secondary sexual characteristics with normal external genitalia associated with vaginal aplasia and uterine agenesis. The approach was a laparotomy and a perineal approach under general anesthesia. The procedure involved the removal of a sigmoidal colonic graft that was anastomosed with the vaginal dimple. Results: The average age was 23 years. All patients had consulted for primary amenorrhea, infertility, and/or difficulties in sexual intercourse. The diagnosis of MRKH type 1 was retained in all patients. The average length of the vagina was 3.25 cm before surgery and 13.63 cm after surgery. The postoperative outcomes were uneventful in 3 patients. One patient developed anastomotic stenosis that was successfully treated with vaginal dilation for 2 weeks. The average postoperative follow-up was 30 months. Conclusion: In the context of a low-resource setting, sigmoid transposition represents a good procedure to treat vaginal aplasia and restore a satisfactory sexual activity to patients with MRKH type 1.

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“…8e14 All vaginoplasties are at risk for strictures and stenosis, and long-term reoperation rates might be as high as 40%. 15 Postoperative and ongoing vaginal dilation is typically required for most procedures to decrease the risk of stenosis. Conversely, primary dilation alone has less than a 1% risk of complication, primarily prolapse and bleeding.…”

Section: Introductionmentioning

confidence: 99%

Vaginal Dilator Therapy: A Guide for Providers for Assessing Readiness and Supporting Patients Through the Process Successfully

Oelschlager

Debiec

2019

Journal of Pediatric and Adolescent Gynecology

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Vaginal dilator therapy is used to increase vaginal length for vaginal agenesis, to increase vaginal width for vaginal narrowing, and to prevent or treat stenosis after vaginal surgery. Although it is an effective therapy, many reproductive health providers have had little training on how to guide patients through this therapy. The purpose of this review is to educate providers on how to assess patient readiness and how to support patients through the process of vaginal dilation.

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Surgery is not superior to dilation for the management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome: a multicenter comparative observational study in 131 patients

Cited by 56 publications

References 19 publications

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update

Sigmoid vaginoplasty in Mayer-Rokitansky-Kuster-Hauser syndrome

Vaginal Dilator Therapy: A Guide for Providers for Assessing Readiness and Supporting Patients Through the Process Successfully

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