title: Uterus didelphys with unilateral vaginal atresia: multicystic dysplastic kidney is the precursor of "renal agenesis" and the key to early diagnosis of this genital anomaly.
abstract: BACKGROUND
  Uterus didelphys with obstructed hemivagina (UDWOH) and ipsilateral renal agenesis is a distinct but rare entity.
  
  
  OBJECTIVE
  To demonstrate the association between UDWOH and a multicystic dysplastic kidney (MCDK) in neonates. To demonstrate the usefulness of sonography with vaginal fluid instillation in the early and reliable diagnosis of this genital anomaly in girls with MCDK.
  
  
  METHODS
  Our patients were five female neonates with a MCDK, which had been detected in utero. We performed sonography of the internal genitalia in all patients before and after filling the vagina with saline.
  
  
  RESULTS
  In all five girls, sonographic examination revealed uterus didelphys with obstruction of the vagina ipsilateral to the MCDK. In three girls, a dilated ureter originating from the MCDK and extending to the level of the vagina could be clearly demonstrated, with one of these including the ureteric insertion into the atretic vagina.
  
  
  CONCLUSIONS
  MCDK is key to the early diagnosis of UDWOH and will facilitate the provision of appropriate treatment. The neonatal period provides a unique opportunity for detecting uterine anomalies by sonography. An MCDK usually involutes and eventually mimics renal agenesis later in life.
authors: Department of Paediatrics, Division of Neonatology, Neuropaediatrics and Metabolic Diseases, Innsbruck Medical University, Innsbruck, Austria.
authors: Ursula
authors: U
authors: Kiechl-Kohlendorfer
authors: Theresa
authors: T
authors: Geley
authors: Kathrin
authors: K
authors: Maurer
authors: Ingmar
authors: I
authors: Gassner
mesh_terms: D000013:Congenital Abnormalities / Q000175:diagnosis* / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities / Q000000981:diagnostic imaging; D007674:Kidney Diseases / Q000151:congenital* / Q000175:diagnosis / Q000000981:diagnostic imaging; D021782:Multicystic Dysplastic Kidney / Q000175:diagnosis* / Q000000981:diagnostic imaging; D014463:Ultrasonography; D014599:Uterus / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014621:Vagina / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014623:Vaginal Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging