Abdelmoula Ghada, Ragmoun Houssem, Bezzine Meriem, Hafsi Montacer, Abdallah Ikram Ben, Sassi Elaa
Department of Gynecology and Obstetrics, Regional Hospital of Menzel Temime, Menzel Temime 8080, Nabeul, Tunisia.
Faculty of Medicine of Sousse, University of Sousse, 4000, Sousse, Tunisia.
Radiol Case Rep. 2025 Jul 22;20(10):5065-5069. doi: 10.1016/j.radcr.2025.06.123. eCollection 2025 Oct.
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital anomaly resulting from abnormal development of the Müllerian and Wolffian ducts, characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We report the case of a 26-year-old woman who presented with chronic pelvic pain and leukorrhea; clinical examination revealed a palpable pelvic mass, and imaging confirmed the diagnosis of OHVIRA syndrome. MRI demonstrated a didelphic uterus, an obstructed hemivagina, and absent ipsilateral kidney. Although commonly diagnosed in adolescence after menarche, this case highlights a delayed presentation. Prompt diagnosis is crucial to avoid complications such as endometriosis or infection. MRI plays a key role in the diagnostic process and surgical planning. Treatment is based on relieving the obstruction and preserving fertility, ranging from septum resection in simple cases to hemi hysterectomy in complex anomalies. This case underscores the importance of early recognition of OHVIRA syndrome to prevent long-term morbidity and optimize reproductive outcomes.
梗阻性半阴道及同侧肾异常（OHVIRA）综合征，也称为赫林 - 韦纳 - 温德利希综合征，是一种罕见的先天性异常，由苗勒管和中肾管发育异常引起，其特征为双子宫、梗阻性半阴道和同侧肾缺如三联征。我们报告一例26岁女性，她因慢性盆腔疼痛和白带增多就诊；临床检查发现可触及盆腔肿块，影像学检查确诊为OHVIRA综合征。磁共振成像（MRI）显示双子宫、梗阻性半阴道和同侧肾脏缺如。尽管OHVIRA综合征通常在初潮后青春期被诊断，但该病例显示了延迟表现。及时诊断对于避免诸如子宫内膜异位症或感染等并发症至关重要。MRI在诊断过程和手术规划中起着关键作用。治疗基于解除梗阻并保留生育能力，从简单病例的隔膜切除术到复杂畸形的半子宫切除术不等。该病例强调了早期识别OHVIRA综合征对于预防长期发病和优化生殖结局的重要性。
