Ann Ital Chir. 2023 Mar 20;12:S2239253X23038720.
OHVIRA-syndrome (obstructed hemivagina, ipsilateral renal agenesis/anomaly) is a rare Mullerian duct anomaly that can lead to complications in pubescent children.
We report a case of a 13-year-old patient with acute right-sided lower quadrant abdominal pain who was referred for exclusion of appendicitis. As a result of the examination (transvaginal ultrasound scan and gynecological examination), a female genital tract anomaly was suspected in the form of obstructed hemivagina with hematocolpos and hematometra. The MRI scan showed hematocolpos and hematometra on the right side, uterus didelphys accompanied by right-sided renal agenesis, consistent with OHVIRA-syndrome. Excision of the vaginal septum was performed and the accumulated old menstrual blood, as represented by hematocolpos and hematometra, was evacuated. Postoperative recovery was uneventful.
The early surgical management of this rare Mullerian duct anomaly is important in order to prevent longterm complications. This malformation should be considered in the differential diagnosis of acute lower abdominal pain in pubescent girls.
Abdominal Pain, Genital Anomaly, Obstructed Hemivagina, Renal Anomaly.
OHVIRA 综合征（单侧半阴道闭锁伴同侧肾发育不全/畸形）是一种罕见的苗勒管畸形，可导致青春期儿童出现并发症。
我们报告了一例 13 岁女性患者，因右侧下腹部急性疼痛就诊，以排除阑尾炎。由于检查（经阴道超声扫描和妇科检查），怀疑存在单侧半阴道闭锁伴阴道积血和宫腔积血的女性生殖道畸形。MRI 扫描显示右侧阴道积血和宫腔积血，双子宫伴右侧肾发育不全，符合 OHVIRA 综合征。行阴道纵隔切除术，排出积血，如阴道积血和宫腔积血所代表的陈旧月经血。术后恢复顺利。
为了预防长期并发症，对这种罕见的苗勒管畸形进行早期手术治疗很重要。这种畸形在青春期女孩急性下腹痛的鉴别诊断中应予以考虑。
腹痛、生殖器官畸形、单侧半阴道闭锁、肾畸形。
