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Mayer–Rokitansky–Küster–Hauser syndrome: diagnosis and management
Author(s) -
Valappil Saudabi,
Chetan Uma,
Wood Nick,
Garden Anne
Publication year - 2012
Publication title -
the obstetrician and gynaecologist
Language(s) - English
Resource type - Journals
eISSN - 1744-4667
pISSN - 1467-2561
DOI - 10.1111/j.1744-4667.2012.00097.x
Subject(s) - mayer rokitansky kuster hauser syndrome , medicine , presentation (obstetrics) , gynecology , agenesis , excellence , vagina , infertility , surgery , pregnancy , political science , law , biology , genetics
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?