Folia Medica 63(5): 815-818, doi: 10.3897/folmed.63.e63325
Renal abnormalities associated with Mayer-Rokitansky-Küster-Hauser syndrome
Emil Dorosiev‡, Galya Muzikadzhieva‡, Boris Mladenov‡, Ivan Stoev‡, Dimiter Velev‡‡ UMHATEM N.I. Pirogov, Sofia, Bulgaria
Corresponding author: Boris Mladenov ( boris_mladenov@abv.bg ) © Emil Dorosiev, Galya Muzikadzhieva, Boris Mladenov, Ivan Stoev, Dimiter Velev. This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Citation:
Dorosiev E, Muzikadzhieva G, Mladenov B, Stoev I, Velev D (2021) Renal abnormalities associated with Mayer-Rokitansky-Küster-Hauser syndrome. Folia Medica 63(5): 815-818. https://doi.org/10.3897/folmed.63.e63325 |  |
Abstract
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital disorder affecting female reproductive system (agenesis of uterus and upper part of vagina) alone (type 1), or associated with abnormalities of other organs and systems (type 2). We report a case of a 21-year-old woman diagnosed with MRKH due to primary amenorrhea during puberty and operated for formation of neovagina. She was admitted to the Department of Urology with abdominal pain and oligoanuria, where the physical examination and imaging studies revealed a malformation of the upper urinary tract: a solitary dystopia of kidney in the pelvis with stenosis of ureteropelvic junction and hydronephrosis. After initial desobstruction with a DJ stent, a pyeloplasty was performed. Females with primary amenorrhea are often delayed with the diagnosis of potential MRKH syndrome, and those diagnosed with the syndrome should undergo detailed examination to exclude concomitant urinary abnormalities.
Keywords
Anderson-Hynes pyeloplasty, hydronephrosis, kidney dystopia, MRKH syndrome, primary amenorrhea