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Mayer-Rokitansky-Küster-Hauser syndrome
Also known as:
Küster’s syndrome
Mayer-Rokitansky syndrome
Mayer-von Rokitansky-Küster malformation complex
Mayer-Rokitansky-Küster syndrome
von Rokitansky-Küster-Hauser syndrome
Rokitansky-Hauser syndrome
Rokitansky-Küster syndrome
Associated persons:
Georges André Hauser
Hermann Küster
August Franz Joseph Karl Mayer
Karl Freiherr von Rokitansky
Description:
Syndrome characterised by congenital absence of vagina, primary amenorrhoea, rudimentary cornua uteri and morphologically normal ovaries and Fallopian tubes situated on the pelvic sidewall. Normal ovulation; normal breast development; normal body and hair. The woman is amenorrhoeic and infertile. Frequently associated with urinary tract anomalies, skeletal abnormalities, congenital heart conditions, and inguinal hernia. Due to a form of dysplasia of the Müllerian ducts. Incidence statistics differ from 1: 4000 (at birth) to 1: 20,000 at female hospital admissions. Recognized usually at time of expected menarche. Usually sporadic. Familial form consistent with autosomal dominant trait.
Bibliography:
- C. A. J. Mayer:
Über Verdoppelungen des Uterus und ihre Arten, nebst Bemerkungen über Harenscharte und Wolfsrachen.
Journal der Chirurgie und Augen-Heilkunde, Berlin, 1829, 13: 525-564.
- K. F. von Rokitansky:
Über die sogenannten Verdoppelungen des Uterus.
Medizinische Jahrbücher des kaiserl. königl. österreichischen Staates, Wien, 1838, 26: 39-77.
- H. Küster:
Uterus bipartitus solidus rudimentarius cum vagina solida.
Zeitschrift für Geburtshilfe und Gynäkologie, Stuttgart, 1910, 67: 692-718.
- G. A. Hauser, M. Keller, T. Koller, et al:
Das Rokitansky-Küster-Syndrom. Uterus bipartus solidus rudimentarius cum vagina solida.
Gynaecologia, Basel, 1961, 151: 111-112.
- G. A. Hauser, W. E. Schreiner:
Das Mayer-v. Rokitansky-Küster-Syndrom.
Schweizerische medizinische Wochenschrift, Basel, 1961, 91: 383-384.
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