Mayer Rokitansky Kuster Hauser syndrome with urogenital sinus anomaly.
Mayer Rokitansky Kuster Hauser (MRKH) syndrome is a rare disorder, characterized by the congenital absence of uterus and associated renal tract anomalies. The case presented with primary amenorrhea and primary infertility, despite development of normal female secondary sexual characteristics. CT scan revealed absent uterus, a solitary left sided pelvic kidney and a vesicovaginal communication that, on cystoscopy, revealed urogenital sinus anomaly manifesting as a common channel formed due to absent anterior wall of vagina and posterior wall of urethra. The urogenital sinus anomaly in MRKH syndrome has not been reported earlier.
Amin
Muhammad Umar
MU
Department of Radiology, CMH, Bahawalpur. umar1971@hotmail.com
Shafique
Mobeen
M
Ahmed
Faizan
F
Mahmood
Rabia
R
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D000328:Adult; D005260:Female; D006801:Humans; D007668:Kidney / Q000002:abnormalities* / Q000000981:diagnostic imaging; D009095:Mullerian Ducts / Q000002:abnormalities*; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014719:Vesicovaginal Fistula / Q000000981:diagnostic imaging / Q000209:etiology