Department of Gynecological Surgery and Gynecological Oncology of Adults and Adolescents, Pomeranian Medical University, 70-111 Szczecin, Poland.
Department of Obstetrics and Gynecology, Pomeranian Medical University, 70-111 Szczecin, Poland.
Int J Environ Res Public Health. 2020 Sep 30;17(19):7173. doi: 10.3390/ijerph17197173.
Herlyn-Werner-Wunderlich Syndrome is a rare malformation syndrome characterized by uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. Symptoms appear most often after menarche and are secondary to hematocolpos. We compare clinical symptoms, diagnosis and treatment of two patients, a 13-year-old and a 17-year-old.
Despite the non-uniform clinical symptoms, it should be noted that in both patients, the 13-year-old and the 17-year-old, hematocolpos, which was probably the cause of lower abdominal pain, was diagnosed with ultrasound. The diagnosis was complemented by laparoscopy, which determined the diagnosis of malformation of uterus didelphys with obstructed hemivagina. The patients had a history of kidney agenesis, which, after gynecological diagnosis, turned out to be ipsilateral. In the 13-year-old, agenesis was diagnosed by uroscintigraphy, while in the 17-year-old it was diagnosed by urography. Incision and drainage of the residual vagina was performed in the course of therapeutic management. In both cases, the clinical situation required a repeated widening of the orifice.
Lower abdominal pain accompanying hematocolpos suggested Herlyn-Werner-Wunderlich Syndrome (HWWS) as the cause of symptoms. 3D transvaginal ultrasound enabled the determination of a congenital uterine defect with high probability, although inconclusive cases required confirmation by laparoscopy. Incision of the blocked vagina and drainage of hematocolpos were the key components of treatment. The treatment of HWWS is a multi-step process.
赫尔林-韦纳-旺德雷希综合征是一种罕见的畸形综合征，其特征为双子宫伴半侧阴道闭锁和同侧肾发育不全。症状多在初潮后出现，是由阴道积血引起的。我们比较了两名患者（一名 13 岁和一名 17 岁）的临床症状、诊断和治疗方法。
尽管临床表现不典型，但应注意到，在这两名患者（一名 13 岁和一名 17 岁）中，超声检查均发现了可能引起下腹痛的阴道积血。腹腔镜检查补充了诊断，确定了双子宫伴半侧阴道闭锁的畸形诊断。两名患者均有单侧肾发育不全病史，妇科诊断后发现同侧肾发育不全。在 13 岁患者中，通过尿路闪烁扫描术诊断了肾发育不全，而在 17 岁患者中，通过尿路造影术诊断了肾发育不全。在治疗过程中，对残余阴道进行了切开引流。在这两种情况下，临床情况均需要反复扩大阴道开口。
伴有阴道积血的下腹痛提示可能为赫尔林-韦纳-旺德雷希综合征（HWWS）引起的症状。3D 经阴道超声检查能够高度确定先天性子宫缺陷，但存在不确定的病例需要通过腹腔镜检查确认。切开闭锁阴道和引流阴道积血是治疗的关键组成部分。HWWS 的治疗是一个多步骤的过程。
