Presentation
History of a solitary kidney and irregular menstrual cycles.
Patient Data
There are two divergent uterine horns. A definite communication is not visualized between the 2 horns. There appears to be a thin left vagina, however, there is an additional tubular structure to the right of the thin left hemivagina. There are complex internal echoes.
The ovaries appear normal and demonstrate normal color Doppler flow.
There is a didelphys configuration with two uteri/cervices and vaginas. There is definite separation at the level of the uterine fundus. Both uteri demonstrate normal signal characteristics of the myometrium and the endometrium is normal in appearance.
There is a caliber change with subsequent focal dilation of the right hemivagina, presumably at the level of the cervix with evidence of obstruction. There is no clear communication with the right hemivagina. On the coronal T2-weighted images, there is an apparent blind ending of the right hemivagina adjacent to the nondilated left hemivagina.
The bladder, distal left ureter, and ovaries are normal. A right ureter is not visualized.
Case Discussion
This is a case of OHVIRA (also known as Herlyn-Werner-Wunderlich syndrome).
This patient has a history of a congenitally solitary left kidney and left vesicoureteral reflux status post unilateral ureteroneocystostomy. Despite this, her renal function appears normal. Gynecologically, she has a left hemivagina without a cervix. Her menstrual cycles are irregular. Her growth and development have otherwise been normal.
Co-author:
Travis Bevington, MD
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