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

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

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

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

URI: http://hdl.handle.net/2263/15230

Date: 2009

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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