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OHVIRA Syndrome: A Rare Congenital Anomaly OHVIRA syndrome, also known as Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare congenital condition characterized by a combination of uterine and renal abnormalities. It is a type of Müllerian duct anomaly, which refers to developmental defects of the female reproductive system. The acronym OHVIRA stands for Obstructed Hemivagina with Ipsilateral Renal Anomaly. Key Features of OHVIRA Syndrome The defining features of OHVIRA syndrome include: Uterus didelphys: This refers to a complete or partial duplication of the uterus, resulting in two separate uterine horns, each with its own cervix and vagina. Obstructed hemivagina: One of the vaginal canals is blocked, preventing the flow of menstrual blood. This can lead to a buildup of blood in the obstructed hemivagina, known as hematocolpos. Ipsilateral renal agenesis: The kidney on the same side as the obstructed hemivagina is absent. Symptoms and Complications OHVIRA syndrome often presents with symptoms that appear during puberty, including: Pelvic pain: This is a common complaint, particularly during menstruation due to the buildup of blood in the obstructed hemivagina. Dysmenorrhea: Painful menstrual cramps. Abdominal swelling: This can occur due to the accumulation of menstrual blood in the obstructed hemivagina. Nausea and vomiting: These symptoms may occur during menstruation in some cases. Infertility: The condition can affect fertility due to the abnormal uterine anatomy and the presence of only one functioning kidney. Diagnosis The diagnosis of OHVIRA syndrome is typically made through a combination of imaging studies, including: Ultrasound: This can reveal the presence of a double uterus and the obstructed hemivagina. Magnetic resonance imaging (MRI): This provides a more detailed view of the uterine anatomy and can confirm the absence of the ipsilateral kidney. Treatment Treatment for OHVIRA syndrome aims to relieve symptoms and improve reproductive outcomes. It typically involves: Drainage of hematocolpos: The obstructed hemivagina is opened surgically to drain the accumulated blood. Vaginal reconstruction: The obstructed hemivagina may be surgically repaired to create a functional vaginal canal. Laparoscopy: This minimally invasive procedure may be used to confirm the diagnosis and to address other associated abnormalities. Management of Pregnancy Pregnancies in women with OHVIRA syndrome are considered high-risk due to the abnormal uterine anatomy and the reduced kidney function. Careful management is required, often involving: Cervical sutures: These may be placed to help prevent premature labor. Cesarean section: This is often recommended to reduce the risk of complications during labor. Research and Future Directions While OHVIRA syndrome is a rare condition, ongoing research aims to improve understanding of its pathogenesis, develop more effective treatment strategies, and enhance the reproductive outcomes for affected individuals. Conclusion OHVIRA syndrome is a complex congenital anomaly that can significantly impact a woman's reproductive health. Early diagnosis and appropriate treatment are essential for managing the condition and improving quality of life. Further research is needed to better understand the underlying mechanisms of this syndrome and to develop more effective treatment approaches.