Mayer-Rokitansky-Küster-Hauser syndrome associated with a urogenital sinus anomaly in a 4-year-old : report of a case

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Authors

Adam, Ahmed
Ebrahim, Zaeem I.
Engelbrecht, Matthys J.
Van Heerden, Izak J.
Moshokoa, Evelyn M.

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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.

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Keywords

Mayer-Rokitansky-Küster-Hauser syndrome, Urogenital sinus anomaly

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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]