PMC4898179_01
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Longitudinal transabdominal sonographic image through the pelvis demonstrates a markedly dilated vagina, approximately 18x11 cm, containing echogenic material consistent with blood product.
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Transabdominal sonography demonstrated an absent left kidney and a large, tubular, cystic pelvic mass, at least 18x11 cm, with low-level internal echoes displacing the pelvic organs to the right (Fig. 1).
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Coronal-reformatted, contrast-enhanced CT image demonstrates a blood-filled, markedly dilated vagina (large single arrow), normal right hemiuterine horn (arrowhead), and distended left hemiuterine horn (small paired arrows) with an attenuation of 70 Hounsfield units, suggesting the presence of blood in it.
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This was connected with a low-attenuating, nonenhancing convoluted longitudinal structure in the left pelvis, consistent with a hydrometrocolpos and left hydrosalpinx (Fig. 2).
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Axial contrast-enhanced CT image (top) and coronal-reformatted, contrast-enhanced CT image (above) demonstrate a mildly hypertrophied right kidney (arrows) and an absent left kidney.
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The hydrometrocolpos displaced and partially obstructed the distal right ureter, with attendant mild right hydronephrosis (Fig. 3).
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Sagittal T2-weighted MR image demonstrates a dilated hemivagina with low T2-signal-intensity contents and corresponding high T1-signal intensity consistent with blood product.
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T1- and low T2-signal intensity was compatible with a markedly dilated vagina containing blood products and an obstructing low transverse vaginal septum. The dilated vagina was compressing and displacing both the urinary bladder and sigmoid colon (Fig. 4).
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Axial T1-weighted MR image demonstrates a left hydrosalpinx (arrowhead) containing high T1-signal-intensity contents consistent with blood products, with an axial diameter of 5.2 cm, originating from a dilated hemivagina (arrow).
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Thus, the lesion was most consistent with a left-sided dilated uterus and/or a dilated left-sided adnexa. The lesion contained fluid with the same MR characteristics as the midline structure, suggesting an open connection (Fig. 5).
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Axial T2-weighted MR image demonstrates a dilated vagina with low T2-signal-intensity contents that had corresponding high T1-signal intensity consistent with blood products (large single arrow). Normal-appearing right uterine horn (arrowhead) and dilated left uterine horn with low T2-signal-intensity blood products (small paired arrows) also appear.
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The lesion was not dilated and did not contain fluid, suggesting no connection with the midline tubular structure (Fig. 6).
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Coronal T1-weighted MR image demonstrates an ectopic insertion of a dilated ureter (paired arrows), containing high T1-signal-intensity contents consistent with refluxed blood products, into a dilated left vaginal canal (arrowhead).
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This tubular lesion displaced the left psoas muscle laterally (Fig. 7).
17-year-old girl with uterine didelphys associated with OHVIRA syndrome. Diagrammatic representation of the anatomy of this patient. LV: obstructed left hemivagina dilated with blood product. RU: nondilated right uterine horn. LU: left uterine horn dilated with blood product. Ur: ectopic ureter inserting into the left hemivagina. RK: right kidney.
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An overall diagrammatic representation of the anatomy of this patient makes this clearer (Fig. 8).