Management of an unusual case of atypical Mayer-Rokitansky-Kuster-Hauser syndrome, with unilateral gonadal agenesis, solitary ectopic pelvic kidney, and pelviureteric junction obstruction.

Abstract

Congenital absence of uterus and vagina, Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, is mullerian agenesis and is the second most frequent cause of primary amenorrhea. Only atypical form of MRKH (type B) is associated with renal skeletal and ovarian abnormalities. We report the management of an unusual case of atypical MRKH, unilateral gonadal agenesis, and solitary ectopic pelvic kidney with pelviureteric junction obstruction (PUJO). After doing thorough Medline search, to the best of our knowledge, this is the first case reported with this combination.

• Authors: Anup Kumar, Saurabh Mishra, P N Dogra
• Journal: International urogynecology journal and pelvic floor dysfunction (Int Urogynecol J Pelvic Floor Dysfunct) Vol. 18 Issue 7 Pg. 823-5 (Jul 2007) England
• PMID: 17093891 (Publication Type: Case Reports, Journal Article)

Topics

• Adolescent
• Female
• Gonadal Dysgenesis (complications, diagnosis)
• Humans
• Kidney (abnormalities, surgery)
• Syndrome
• Ureter (abnormalities, surgery)
• Urinary Bladder (surgery)