OHVIRA syndrome

Case contributed by Hyeonggi Choi
Diagnosis certain

Presentation

Initially presented to the emergency department with fever and lower abdominal pain. Subsequently underwent O&G review due to a cystic lesion in the left pelvic area seen on ultrasound.

Patient Data

Age: 10 years
Gender: Female
ct

Axial, coronal and sagittal enhanced CT shows two uterine horns (uterus didelphys), left hematometrocolpos (obstructed hemivagina) and left renal agenesis.

Case Discussion

OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome is a rare congenital disorder that affects the female reproductive and urinary systems. It is characterized by the presence of a septum that divides the vagina into two parts, with one side being obstructed, and the other being normal. The condition is often associated with renal anomalies on the same side, such as renal agenesis or duplication.

OHVIRA can lead to various symptoms, such as pelvic pain, dysmenorrhea, recurrent infections, and infertility. Treatment typically involves surgical correction of the vaginal anomaly and management of any associated renal anomalies.

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