Tsai Jun-Li, Tsai Shang-Feng
MD, Department of Family Medicine, Cheng Ching General Hospital, Taichung, Taiwan.
MD, Division of Nephrology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan; School of Medicine, China Medical University, Taichung, Taiwan; Department of Life Science, Tunghai University, Taichung, Taiwan.
Iran Red Crescent Med J. 2016 Oct 30;18(11):e40267. doi: 10.5812/ircmj.40267. eCollection 2016 Nov.
Urinary tract infection is a common disease in the general population. However, in patients with frequent urinary tract infection, it is important to determine any treatable cause to avoid recurrence.
Herlyn-Werner-Wunderlich syndrome or OHVIRA syndrome is a very rare congenital anomaly with uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The earliest presentation of this syndrome is hematocolpos that develops during menstruation and results in dysmenorrhea and a pelvic mass shortly after menarche. Herein, we report a patient with Herlyn-Werner-Wunderlich syndrome manifested with unusual symptoms, delayed onset and without surgery. The unique point of this patient is the partial obstruction of cervico-vaginal junction.
Early diagnosis and timely treatment of OHVIRA syndrome can prevent long-term complications, such as recurrent urinary tract infection and infertility. A high index of suspicion is required, even though OHVIRA syndrome is extremely rare and may have an atypical presentation.
尿路感染在普通人群中是一种常见疾病。然而,对于频繁发生尿路感染的患者,确定任何可治疗的病因以避免复发很重要。
赫林-韦纳-温德利希综合征(Herlyn-Werner-Wunderlich syndrome)或OHVIRA综合征是一种非常罕见的先天性异常,表现为双子宫、阴道半梗阻和同侧肾缺如。该综合征最早的表现是月经期间出现阴道积血,初潮后不久导致痛经和盆腔肿块。在此,我们报告一例表现出不寻常症状、发病延迟且未接受手术治疗的赫林-韦纳-温德利希综合征患者。该患者的独特之处在于宫颈-阴道交界处存在部分梗阻。
OHVIRA综合征的早期诊断和及时治疗可预防长期并发症,如复发性尿路感染和不孕。即使OHVIRA综合征极为罕见且可能有非典型表现,也需要高度怀疑。