Mayer-Rokitansky-Küster-Hauser syndrome: fertility counseling and treatment

Reichman, Daniël; Laufer, Marc R.

doi:10.1016/j.fertnstert.2010.01.036

Cited by 20 publications

(10 citation statements)

References 26 publications

(20 reference statements)

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“…The majority (93%) of our cases had normally developed bilateral ovaries, as expected from the difference in embryonic origins of the ovaries and uterus (7, 14). These results are in accordance with those of the previous studies (2, 3, 7-12, 15). …”

Section: Discussionsupporting

confidence: 94%

Magnetic Resonance Evaluation of Müllerian Remnants in Mayer-Rokitansky-Küster-Hauser Syndrome

et al. 2013

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ObjectiveTo analyze magnetic resonance imaging (MRI) findings of Müllerian remnants in young females clinically suspected of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome in a primary amenorrhea workup.Materials and MethodsFifteen young females underwent multiplanar T2- and transverse T1-weighted MRI at either a 1.5T or 3.0T MR imager. Two gynecologic radiologists reached consensus decisions for the evaluation of Müllerian remnants, vagina, ovaries, and associated findings.ResultsAll cases had bilateral uterine buds in the pelvic cavity, with unilateral cavitation in two cases. The buds had an average long-axis diameter of 2.64 ± 0.65 cm. In all cases, bilateral buds were connected with fibrous band-like structures. In 13 cases, the band-like structures converged at the midline or a paramedian triangular soft tissue lying above the bladder dome. The lower one-third of the vagina was identified in 14 cases. Fourteen cases showed bilateral normal ovaries near the uterine buds. One unilateral pelvic kidney, one unilateral renal agenesis, one mild scoliosis, and three lumbar sacralization cases were found as associated findings.ConclusionTypical Müllerian remnants in MRKH syndrome consist of bilateral uterine buds connected by the fibrous band-like structures, which converge at the midline triangular soft tissue lying above the bladder dome.

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Section: Discussionsupporting

confidence: 94%

Magnetic Resonance Evaluation of Müllerian Remnants in Mayer-Rokitansky-Küster-Hauser Syndrome

et al. 2013

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“…There is also concern about transmitting the same congenital abnormality to the offspring. In a study of 58 women with MRKH syndrome undergoing fertility treatment with gestational surrogates, none of the 17 female children born had MRKH syndrome. Adoption is another option.…”

Section: Fertility Optionsmentioning

confidence: 99%

“…Uterine transplantation may hold hope for these women in the future, as studies in animals have been promising. One attempted uterine transplantation in humans has been reported but was unsuccessful . Research is focusing on such novel therapies and the genetic aetiology and pattern of inheritance of MRKH syndrome.…”

Section: Fertility Optionsmentioning

confidence: 99%

Mayer–Rokitansky–Küster–Hauser syndrome: diagnosis and management

Valappil

Chetan

Wood

et al. 2012

The Obstetric & Gynaecologis

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Key content In Mayer–Rokitansky–Küster–Hauser syndrome (müllerian agenesis) there is congenital absence of the upper two‐thirds of the vagina and the uterus is absent or rudimentary. A common presentation is primary amenorrhoea in a female with 46,XX karyotype and normal secondary sexual characteristics. There may be associated abnormalities of the kidneys, skeletal system, heart and auditory system. Magnetic resonance imaging is a useful diagnostic tool with which to assess the anatomical abnormalities. Management involves psychological support and creation of a neovagina for sexual function. Objectives To understand the anatomical abnormalities of this entity. To learn about the clinical presentation and differential diagnosis. To understand the management issues. Ethical issues If, in the future, uterine transplantation becomes safe and effective, will it be ethical for a woman to donate her uterus to her daughter? With many children awaiting adoption, should women with Mayer–Rokitansky–Küster–Hauser syndrome be encouraged to adopt rather than undergo surrogacy? Who should pay for assisted reproduction treatment, given the constraints of health service budgets? Should women with Mayer–Rokitansky–Küster–Hauser syndrome be managed in selected centres of excellence?

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“…Management of AUFI thus requires an integrated, multidisciplinary approach, involving gynaecologists, psychologists and clinical nurse specialists 3 . Additionally, particularly in conditions such as MRKH, when the diagnosis commonly occurs during adolescence, counselling and patient support groups can be particularly beneficial 4 …”

Section: Introductionmentioning

confidence: 99%

“…3 Additionally, particularly in conditions such as MRKH, when the diagnosis commonly occurs during adolescence, counselling and patient support groups can be particularly beneficial. 4 After a diagnosis of infertility, many women experience anxiety, depression, low self-esteem, loss of gender identity, a decrease in their quality of life and an enduring sense of incompleteness and grief. [5][6][7][8] Worse psychological outcomes arise in women experiencing infertility who fail to conceive after assisted reproductive technology (ART) treatment than in those who are successful.…”

Section: Introductionmentioning

confidence: 99%

Options for acquiring motherhood in absolute uterine factor infertility; adoption, surrogacy and uterine transplantation

Jones

Ranaei-Zamani

Vali

et al. 2021

The Obstetric & Gynaecologis

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Following the diagnosis of absolute uterine factor infertility (AUFI), women may experience considerable psychological harm as a result of a loss of reproductive function and the realisation of permanent and irreversible infertility. Adoption enables women with AUFI, and their partners, to experience social and legal parenthood, also often providing benefits for the adopted child. Surrogacy offers the opportunity to have genetically related offspring. Outcomes are generally positive in both surrogates and the children born as a result. Uterine transplantation is the only option to restore reproductive anatomy and functionality. While associated with considerable risk, it allows the experience of gestation and the achievement of biological, social and legal parenthood. Learning objectivesTo gain an understanding of the routes to parenthood available for women with AUFI experiencing involuntary childlessness, such as adoption, surrogacy and, most recently, uterine transplantation To consider a suggested management plan to facilitate counselling in women with AUFI who experience involuntary childlessness. Ethical issuesIn the UK, while the number of children requiring adoption continues to increase, the number being adopted from care is decreasing. Some cultures may hold ethical or religious beliefs that surrogacy is unacceptable, and its legal position in many jurisdictions is problematic. Restrictive selection criteria and high costs may limit future availability of uterine transplantation

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Mayer-Rokitansky-Küster-Hauser syndrome: fertility counseling and treatment

Cited by 20 publications

References 26 publications

Magnetic Resonance Evaluation of Müllerian Remnants in Mayer-Rokitansky-Küster-Hauser Syndrome

Magnetic Resonance Evaluation of Müllerian Remnants in Mayer-Rokitansky-Küster-Hauser Syndrome

Mayer–Rokitansky–Küster–Hauser syndrome: diagnosis and management

Options for acquiring motherhood in absolute uterine factor infertility; adoption, surrogacy and uterine transplantation

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