Case History:14-year-old nulliparous menstruating female with lower abdominal and pelvic pain.
Case History: A 14-year-old nulliparous menstruating female presented to the ER with lower abdominal and pelvic pain.
Figure 2. Transabdominal ultrasound demonstrating a hyperechoic “mass” in the right adnexa initially thought to represent an endometrioma, however, later proven by MRI to represent hematocolpus resulting from atresia of right hemivagina.
Figure 3. Axial T2-weighted MRI demonstrating a blind ending right hemivagina (atresia) resulting in hematocolpus.
Figure 4. Axial T2-weighted MRI demonstrating uterine didelphys (white arrows) and blind ending right hemivagina (black arrow).
Figure 5. Vaginal septum separating the atretic obstructed right hemivagina from the nonobstructed left hemivagina.
Figure 6. Coronal MRI demonstrating agenesis of the right kidney with compensatory hypertrophy of the left kidney.
MRI findings were crucial in determining the correct diagnosis in this patient with a complex congenital anomaly consisting of uterine didelphys, obstructed right hemivagina (atresia) with hematocolpus, ipsilateral agenesis of right kidney, and non-obstructed left hemivagina.
Typically, patients present during puberty with nonspecific/variable symptoms in addition to pelvic pain shortly following menarche.
Although it contains defects in differing embryonic lineages, OHVIRA syndrome possesses paramesonephric and mesonephric defects (uterine and renal defects), which are due to embryologic arrest in the 8th week of gestation.
Each ureteric bud develops from the Wolffian duct and is essentially responsible for the formation of the kidney.
Awareness of these syndrome variants is important for proper diagnosis and therapeutic management decisions.