Ureteral switch for bilateral ureteropelvic junction obstruction in a case of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part of the vagina. Secondary sexual characteristics and karyotype are normal. This syndrome affects at least 1 out of 4500 women. MRKH may be isolated (type I) but it is more frequently associated with other malformations (MRKH type II). We present a typical case of MRKH type II associated with bilateral pelvic kidneys ectopia, ureteropelvic junction (UPJ) obstruction and high inserting ureters. The ureteral switch was performed at the time of pyeloplasty to prevent postoperative obstruction secondary to the angulation.