OHVIRA

Congenital condition affecting the female reproductive system

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OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) is a rare congenital disorder that affects the female reproductive and urinary systems. It is characterized by a triad of anomalies: a double uterus (uterus didelphys), an obstructed hemivagina, and an ipsilateral renal anomaly, typically renal agenesis. This condition is also known as Herlyn-Werner-Wunderlich syndrome.

Anatomy and Pathophysiology

OHVIRA syndrome involves the malformation of the Müllerian ducts, which are embryonic structures that develop into the female reproductive tract. In OHVIRA, there is a failure of the ducts to fuse properly, resulting in a double uterus. One of the vaginas is obstructed, leading to hematocolpos (accumulation of menstrual blood) in the obstructed side. The renal anomaly, often renal agenesis, occurs on the same side as the obstructed hemivagina.

Uterus Didelphys

Uterus didelphys is a condition where a woman has two separate uterine cavities, each with its own cervix. This occurs due to incomplete fusion of the Müllerian ducts during embryonic development. In OHVIRA, one of these uterine cavities is associated with an obstructed hemivagina.

Obstructed Hemivagina

The obstructed hemivagina is a result of a transverse vaginal septum or other obstruction that prevents normal menstrual flow from exiting the body. This can lead to the accumulation of menstrual blood, causing pain and potential infection.

Ipsilateral Renal Anomaly

The renal anomaly in OHVIRA is typically renal agenesis, where one kidney fails to develop. This is due to the close embryological relationship between the development of the reproductive and urinary systems.

Clinical Presentation

Patients with OHVIRA often present during adolescence with symptoms such as pelvic pain, dysmenorrhea (painful menstruation), and a palpable pelvic mass due to hematocolpos. Some may also experience urinary symptoms due to the associated renal anomaly.

Diagnosis

Diagnosis of OHVIRA is typically made through imaging studies. Ultrasound is often the first-line imaging modality used to identify the presence of a double uterus and hematocolpos. Magnetic resonance imaging (MRI) can provide more detailed information about the anatomy of the reproductive tract and confirm the diagnosis.

Ultrasound image showing OHVIRA syndrome

Management

The primary treatment for OHVIRA is surgical intervention to relieve the obstruction of the hemivagina. This typically involves resection of the vaginal septum to allow normal menstrual flow. Post-surgical follow-up is important to ensure proper healing and to monitor for any complications.

Prognosis

With appropriate surgical treatment, the prognosis for patients with OHVIRA is generally good. Most patients experience relief from symptoms and can lead normal reproductive lives. However, the presence of a renal anomaly may require ongoing monitoring of renal function.

Related Pages

• Müllerian duct anomalies
• Uterus didelphys
• Renal agenesis
• Congenital disorders