A Case Report of Ohvira Syndrome

Das A, Rimabati K, Gowda N, Singh AS and Hari JS

Department of Obstetrics and Gynaecology, NEIGRIHMS, India
Department of Radiology, NEIGRIHMS, India

^*Correspondance to: Rimabati Khulakpam 

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Abstract:

Background: Obstructed Hemivagina Ipsilateral Renal Agenesis (OHVIRA) syndrome is a congenital defect of the Müllerian ducts characterized by obstructed hemivagina, uterine didelphys, and ipsilateral renal agenesis. Case Presentation: A 13-year-old female presented with abdominal pain and a palpable abdominal mass, later diagnosed as hematometrocolpos with primary amenorrhea. Ultrasonography was suggestive, but MRI confirmed uterine didelphys, obstructed hemivagina, and left renal agenesis, consistent with OHVIRA syndrome. She underwent surgical intervention under general anesthesia with drainage of hematocolpos and resection of the obstructive vaginal septum. Conclusion: Intraoperative hysteroscopy and laparotomic exploration confirmed the diagnosis showing two uterine horns with no interconnection. The patient had significant symptom relief and continued with successful follow-up management for reproductive health.

Keywords:

OHVIRA syndrome; Uterine didelphys; Primary amenorrhea; Hematometrocolpos; Renal agenesis.

Cite the Article:

Das A, Rimabati K, Gowda N, Singh AS, Hari JS. A Case Report of Ohvira Syndrome. Clin Case Rep Int. 2025; 9:1736.