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E1924. Unclogging Misunderstandings and Half-Truths in Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome
Authors
  1. Trenton Bawcum; Children's Medical Center Dallas; University of Texas Southwestern Medical Center
  2. Mary Yang; Children's Medical Center Dallas; University of Texas Southwestern Medical Center
  3. Cory Pfeifer; Children's Medical Center Dallas; University of Texas Southwestern Medical Center
Background
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare Müllerian duct disorder that often does not become symptomatic until puberty. It can be a source of frequent urinary tract infections and can lead to serious intra-abdominal pathology if not appropriately recognized. This exhibit discusses this rare syndrome including embryonic origins, symptomatology, treatment, and potential morbidity. Specifically, the role of the radiologist is discussed using imaging to drive the diagnosis of this rare entity and its potential complications.

Educational Goals / Teaching Points
The embryological basis, incidence, pertinent anatomy and clinical relevance of OHVIRA are discussed. Clinical and radiological variants, mimics, prognostic considerations and potential therapeutic options are also reviewed. Using multiple cases, OHVIRA is depicted in the asymptomatic patient, a patient presenting with clinical complications, and in the post-treatment setting.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
OHVIRA consists of the combination of a Müllerian duct disorder (uterine didelphys with hemivagina) and ipsilateral renal anomaly. The renal anomaly varies from agenesis to duplication, with complete agenesis being the most common. Patients with OHVIRA are often asymptomatic until puberty. The presenting symptom is often generalized lower abdominal pain in the setting of normal menstruation. This key point can usually differentiate this entity clinically from imperforate hymen however, may result in further delay of the proper diagnosis. Fortunately, the anatomy of the disorder is well delineated via ultrasound, CT, or MRI. Early detection and diagnosis is very important and the radiologist serves a critical role given the clinical challenges discussed. Given this, in a pre-pubescent child with a solitary kidney and no known history of renal agenesis, multicystic dysplastic kidney, or prior surgery, a screening pelvic ultrasound may be appropriate to exclude this rare entity. Although severe complications of this syndrome are rare, they do occur and can result in significant morbidity. The most common complications include infection of the retained menstrual products which can lead to fever, nausea/vomiting, sepsis or even tubo-ovarian/uterine abscess formation, an example of this being presented.

Conclusion
OHVIRA syndrome is a rare disorder that can result in significant morbidity if not appropriately recognized and diagnosed in a timely fashion. The radiologist plays a key diagnostic role in recognizing this rare entity as well as its complications.