Mayer-Rokitansky-Küster-Hauser syndrome associated with a urogenital sinus anomaly in a 4-year-old : report of a case
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Date
Authors
Adam, Ahmed
Ebrahim, Zaeem I.
Engelbrecht, Matthys J.
Van Heerden, Izak J.
Moshokoa, Evelyn M.
Journal Title
Journal ISSN
Volume Title
Publisher
Karger
Abstract
The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare entity characterized by congenital aplasia of the vagina and uterus in the presence of normal ovarian function, in conjunction with a 46 XX karyotype. This condition is mostly signalled by primary amenorrhoea around the season of puberty. We report on its diagnosis in a 4-year-old child presenting with urinary incontinence. Also of interest in our index patient is the presence of a pure urogenital sinus anomaly. This extremely rare association has not been previously diagnosed in childhood.
Description
Keywords
Mayer-Rokitansky-Küster-Hauser syndrome, Urogenital sinus anomaly
Sustainable Development Goals
Citation
Adam, A, Ebrahim, ZI, Engelbrecht, MJ, Van Heerden, IJ & Moshokoa, EM 2009, 'Mayer-Rokitansky-Küster-Hauser syndrome associated with a urogenital sinus anomaly in a 4-year-old : report of a case', Current Urology, vol. 3, no. 2, pp. 103-105. [http://content.karger.com/ProdukteDB/produkte.asp?Aktion=JournalHome&ProduktNr=231997]