Ma Irene, Williamson Amy, Rowe Dorothy, Ritchey Michael, Graziano Kathleen
Phoenix Children's Hospital, Phoenix, AZ.
Phoenix Children's Hospital, Phoenix, AZ.
J Pediatr Adolesc Gynecol. 2014 Apr;27(2):104-6. doi: 10.1016/j.jpag.2013.06.015. Epub 2013 Sep 26.
OHVIRA syndrome is a rare diagnosis involving the triad of obstructed hemivagina, uterine anomaly, and ipsilateral renal anomaly. OHVIRA syndrome can be associated with other anomalies due to abnormal embryologic development of the urogenital system.
A 14-year-old female with known left renal agenesis, long-standing urinary incontinence, and history of recurrent urinary tract infections presented with abdominal distention. A 4-year-old female with known right renal agenesis and urinary incontinence was found to have a single common channel at the introitus that communicated with the bladder and a hemivagina on the left.
It is important to identify the presence of a urogenital sinus in the OHVIRA setting, as surgical management for these patients may be affected. In both cases, the urogenital sinus was preserved as the vaginal opening.
OHVIRA综合征是一种罕见的诊断，涉及梗阻性半阴道、子宫异常和同侧肾脏异常三联征。由于泌尿生殖系统胚胎发育异常，OHVIRA综合征可伴有其他异常。
一名14岁女性，已知左肾缺如，长期尿失禁，有反复尿路感染病史，出现腹胀。一名4岁女性，已知右肾缺如和尿失禁，发现阴道口有一个与膀胱相通的单一共同通道，左侧有半阴道。
在OHVIRA综合征情况下识别泌尿生殖窦的存在很重要，因为这可能会影响这些患者的手术治疗。在这两个病例中，泌尿生殖窦均保留为阴道口。
