Mayer-Rokitansky-Küster-Hauser syndrome
Alternative eponyms
- 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
Related people
Syndrome characterised by congenital absence of vagina, primary amenorrhoea, rudimentary cornua uteri and morphologically normal ovaries and Fallopian tubes situated on the pelvic sidewall.
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.
