Diagnostic Dilemma and Management of Rare Mullerian Anomaly: OHVIRA Syndrome

Jesmine Banu, Rebeka Sultana, Suriya Jahan Rolly, Chowdhury Faisal Alamgir, Maliha Darmini, Mostafa Tarique, Nastaran Laskar
In: Scholars Journal of Applied Medical Sciences · 2023 · vol. 11(11) , pp. 1964–1969 · doi:10.36347/sjams.2023.v11i11.018 · W4388975840
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Abstract

Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare female urogenital tract malformation characterized by the triad of uterine didelphys, obstructed hemivagina and ipsilateral renal anomaly. Obstructed anomalies, if not treated timely and adequately, may lead to chronic complications and thereby impairing a woman’s reproductive life. Aims and Objectives: The aim of the study is to evaluate the reproductive outcome of patients with OHVIRA syndrome after treatment. Methods: The present prospective observational study was conducted in Reproductive Endocrinology and Infertility Department of BSMMU from September 2018 to September 2022. Among 84 patients of Mullerian Anomaly, 10 patients presented with obstructive features were enrolled in the study. Cases were analyzed according to detailed history, thorough examination, relevant investigations with TVS and MRI of whole abdomen and laparoscopy maintaining inclusion and exclusion criteria. Results: The mean age was 25.30 ± 5.93 years. In our study 90% patient presented with severe dysmenorrhoea. Right sided obstructed Hemivagina with right renal agenesis were more common 60%. Eighty percent patients had didelphys uterus, twenty percent had bicornuate uterus and twenty percent cases were associated with endometriosis. 60% patients were treated by excision of obstructed vaginal septum and 40% patients required excision of functioning non-communicating uterine horn. Improvement of symptom occurred in 90% patients. Conclusions: When diagnosis of OHVIRA Syndrome is not delayed and indication and timing of surgery are appropriate, complications can be avoided and fertility can be preserved.

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endometriosisdysmenorrheainfertility

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