Obstructive hemivagina and ipsilateral renal anomaly(OHVIRA)syndrome results from congenital anomalies of Wolff’s and Müller’s canals, which presents with a duplicated uterus, unilateral vaginal closure, and ipsilateral renal dysplasia.
Case: Right multiple renal cysts and dilated lower intestines were suspected in a female fetus on ultrasonography. After birth, a 1cm cystic mass with a smooth surface, which increased in size with crying, was found below the urethral orifice.
The right kidney and urinary tract had a polycystic appearance with dilated ureter on ultrasonography and no function by 99mTc- dimercaptosuccinic acid renal scintigraphy.
Magnetic resonance urography revealed a dilated right ureter connected with the vulvar mass.
The right ureteral orifice was not detected in the urethra and bladder by cystoscopy, and the right kidney and dilated ureter were removed by laparoscopy.
Postoperatively, the vulvar mass disappeared, and uterus didelphys were found on ultrasonography, which could be diagnosed as OHVIRA syndrome.
Discussion: Females with congenital unilateral kidney and urinary tract anomalies can have uterine and vaginal abnormalities, so careful examination is important.
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