OHVIRA with a twist: obstructed hemivagina ipsilateral renal anomaly with urogenital sinus in 2 patients.
BACKGROUND
OHVIRA syndrome is a rare diagnosis involving the triad of obstructed hemivagina, uterine anomaly, and ipsilateral renal anomaly. OHVIRA syndrome can be associated with other anomalies due to abnormal embryologic development of the urogenital system.


METHODS
A 14-year-old female with known left renal agenesis, long-standing urinary incontinence, and history of recurrent urinary tract infections presented with abdominal distention. A 4-year-old female with known right renal agenesis and urinary incontinence was found to have a single common channel at the introitus that communicated with the bladder and a hemivagina on the left.


CONCLUSIONS
It is important to identify the presence of a urogenital sinus in the OHVIRA setting, as surgical management for these patients may be affected. In both cases, the urogenital sinus was preserved as the vaginal opening.
Phoenix Children's Hospital, Phoenix, AZ.
Irene
I
Ma
Phoenix Children's Hospital, Phoenix, AZ.
Amy
A
Williamson
Phoenix Children's Hospital, Phoenix, AZ.
Dorothy
D
Rowe
Phoenix Children's Hospital, Phoenix, AZ.
Michael
M
Ritchey
Phoenix Children's Hospital, Phoenix, AZ. Electronic address: kgraziano@surgery4children.com.
Kathleen
K
Graziano
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000601:surgery; D000293:Adolescent; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D013577:Syndrome; D014564:Urogenital Abnormalities / Q000175:diagnosis* / Q000601:surgery; D014599:Uterus / Q000002:abnormalities* / Q000601:surgery; D014621:Vagina / Q000002:abnormalities* / Q000601:surgery