Sijmons Anne, Broekhuizen Suzanne, van der Tuuk Karin, Verhagen Martijn, Besouw Martine
Department of Pediatrics, Wilhelmina Hospital, Assen, The Netherlands.
Treant Zorggroep, Department of Internal Medicine, Scheper Hospital, Emmen, The Netherlands.
Ultrasound. 2023 Feb;31(1):61-64. doi: 10.1177/1742271X221102576. Epub 2022 Jun 20.
The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is characterized by the triad uterus didelphys, obstructed hemivagina, and ipsilateral renal dysplasia. To make a radiological diagnosis, knowledge of this syndrome is of paramount importance. Early recognition may prevent complications such as unnecessary surgical procedures, endometriosis, and infections, which could adversely affect fertility.
A 1-day-old female newborn in whom a right-sided cystic kidney abnormality was seen on antenatal ultrasound was admitted with anuria and intralabial mass. Besides the multicystic dysplastic right kidney, ultrasound revealed a uterus didelphys with right-sided uterus dysplasia, an obstructed right hemivagina, and an ectopic ureteric insertion. The diagnosis of obstructed hemivagina and ipsilateral renal anomaly syndrome with hydrocolpos was made and the hymen was incised. Later, ultrasound helped in diagnosing a pyelonephritis in the afunctional right kidney that was not draining into the bladder (hence no culture could be obtained), requiring intravenous antibiotics and a nephrectomy.
Obstructed hemivagina and ipsilateral renal anomaly syndrome is an anomaly of the Müllerian and Wolffian ducts of unknown cause. Patients typically present after menarche with (progressive) abdominal pain, dysmenorrhea, or urogenital malformations. In contrast, prepubertal patients can present with urinary incontinence or an (external) vaginal mass. The diagnosis is confirmed by an ultrasound or magnetic resonance imaging. Follow-up includes repeated ultrasounds and monitoring of kidney function. Treatment consists of drainage of the hydrocolpos/hematocolpos; in some cases, further surgery is indicated.
Consider obstructed hemivagina and ipsilateral renal anomaly syndrome in girls with genitourinary abnormalities: early recognition prevents complications later in life.
梗阻性半阴道并同侧肾异常（OHVIRA）综合征的特征为双子宫、梗阻性半阴道和同侧肾发育异常三联征。对于进行放射学诊断而言，了解该综合征至关重要。早期识别可预防诸如不必要的外科手术、子宫内膜异位症和感染等并发症，这些并发症可能对生育能力产生不利影响。
一名1日龄女婴，产前超声检查发现右侧肾囊性异常，因无尿和阴唇内肿物入院。除多囊性发育不良的右肾外，超声检查还发现双子宫伴右侧子宫发育异常、梗阻性右侧半阴道和异位输尿管开口。诊断为梗阻性半阴道并同侧肾异常综合征合并阴道积脓，遂行处女膜切开术。后来，超声检查有助于诊断无功能的右肾发生肾盂肾炎，该肾未向膀胱引流（因此无法获得培养物），需要静脉使用抗生素并进行肾切除术。
梗阻性半阴道并同侧肾异常综合征是苗勒管和中肾管的一种异常，病因不明。患者通常在初潮后出现（进行性）腹痛、痛经或泌尿生殖系统畸形。相比之下，青春期前患者可能表现为尿失禁或（外部）阴道肿物。通过超声或磁共振成像可确诊。随访包括重复超声检查和肾功能监测。治疗包括阴道积脓/积血引流；在某些情况下，需要进一步手术。
对于有泌尿生殖系统异常的女孩，应考虑梗阻性半阴道并同侧肾异常综合征：早期识别可预防日后生活中的并发症。
