Kirk Kerkorian School of Medicine at UNLV, Department of Emergency Medicine, Children's Hospital of Nevada at University Medical Center, 901 Rancho Lane, Suite 135, Las Vegas, NV 89106, USA.
Kirk Kerkorian School of Medicine at UNLV, Department of Emergency Medicine, Children's Hospital of Nevada at University Medical Center, 901 Rancho Lane, Suite 135, Las Vegas, NV 89106, USA.
Am J Emerg Med. 2022 Sep;59:218.e1-218.e3. doi: 10.1016/j.ajem.2022.06.032. Epub 2022 Jun 25.
Abdominal pain is a common presenting complaint to the Emergency Department (ED). Often, rare etiologies can be discovered in the work up of this common complaint. Here we present the case of an adolescent female who presented with abdominal pain and was found to have obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) or Herlyn-Werner-Wunderlich Syndrome. A 12 year old female with known renal agenesis presented with 5 days of left sided abdominal pain that then developed into right lower quadrant pain. She had regular menses for the last 2 years. Ultrasound (US) showed a fluid collection in the lower uterine segment and a complex cystic structure anterior to the uterus. Magnetic resonance imaging (MRI) showed the patient to have didelphys uterus with "severe dilatation of the cervix/vaginal canal… extending from the right uterine horn" and left-sided ovarian and Fallopian tube torsion. She was taken to the operating room where she underwent vaginal septum excision and a left salpingo-oopherectomy. OHVIRA includes the triad of obstructed hemivagina, uterine didelphys, and ipsilateral renal agenesis. This occurs due to embryologic arrest of the mullerian and mesonephric ducts at 8 weeks of gestation. Most abnormalities are right sided which leads to right lower abdominal and pelvic pain approximately 4 months post-menarche. Diagnosis of OHVIRA is made utilizing US and CT scans. MRI can also be useful to further delineate specific anatomy. It is important for the emergency physician to be aware of this entity as most patients don't present to care until acute, severe symptoms develop. This makes it more likely for them to seek care in the ED as opposed to the outpatient setting.
腹痛是急诊科常见的就诊主诉。在对这种常见主诉进行检查时，常常可以发现罕见的病因。这里我们介绍了一位有腹痛表现的青春期女性病例，经检查发现其存在单侧半隔子宫和同侧肾发育不全（OHVIRA）或 Herlyn-Werner-Wunderlich 综合征。一位 12 岁的女性患者已知存在单侧肾发育不全，因左下腹疼痛 5 天就诊，之后疼痛扩展至右下腹。过去 2 年来，她的月经规律。超声（US）显示下段子宫内有积液，子宫前有一个复杂的囊性结构。磁共振成像（MRI）显示患者存在双子宫，“宫颈/阴道管严重扩张……从右侧子宫角延伸”，左侧卵巢和输卵管扭转。她被送入手术室，接受了阴道隔切除术和左侧输卵管卵巢切除术。OHVIRA 包括单侧半隔子宫、双子宫和同侧肾发育不全三联征。这是由于中肾管和苗勒管在妊娠 8 周时胚胎停滞所致。大多数异常为右侧，大约在初潮后 4 个月出现右下腹部和骨盆疼痛。OHVIRA 的诊断依据是 US 和 CT 扫描。MRI 也可用于进一步描绘特定的解剖结构。急诊医生应意识到这种疾病的存在，因为大多数患者在出现急性、严重症状之前不会就诊。这使得她们更有可能在急诊科而不是在门诊就诊。
