Investig Magn Reson Imaging.  2015 Sep;19(3):196-199. 10.13104/imri.2015.19.3.196.

MRI Findings of Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA syndrome) with a Blind Megaureter: Case Report

• Cho YH
• Sung DJ
• Han NY
• Park BJ
• Kim MJ
• Sim KC
• Cho SB

• KMID: 2175616
• DOI: http://doi.org/10.13104/imri.2015.19.3.196

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is an uncommon congenital abnormality of the female urogenital tract characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. A 13-year-old female presented with acute lower abdominal pain. Magnetic resonance imaging (MRI) revealed uterine didelphys, hematometrocolpos, obstructed hemivagina, and right ipsilateral agenesis, consistent with OHVIRA syndrome. Also, a well-defined mass with fluid signal intensity, mimicking adnexal neoplasm was seen in the right lower pelvic cavity adjacent to the posterior wall of the bladder. Vaginal septotomy and drainage of hematometrocolpos were done initially, but unilateral hysterectomy was later performed to relieve the patient's symptoms. The cystic mass in the right lower pelvic cavity was also excised and confirmed as a blind megaureter.

Keyword

Mullerian duct anomaly; Renal anomaly; Blind megaureter; Magnetic resonance imaging (MRI)

MeSH Terms

Abdominal Pain
Adolescent
Congenital Abnormalities
Drainage
Female
Humans
Hysterectomy
Magnetic Resonance Imaging*
Urinary Bladder

Figure

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  Fig. 1 Axial (a-c), coronal (d), and sagittal (e) turbo spin-echo T2-weighted images show two uterine horns (thick arrows), hematocolpos (H), hematosalpinx (curved arrow), and a blind megaureter (U). The right uterus was connected to the hematocolpos (H), and the left uterus and hemivagina (thin arrows) were seen on the left side of the hematocolpos (H). The right kidney was absent on the coronal turbo spin-echo T2-weighted image. A blind ectopic megaureter (U) was seen as a well-defined lobulated cystic mass posterior to the right aspect of the urinary bladder.

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