PDF(Pubmed)
Abstract：
Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) is a complex congenital malformation characterized by a triad including uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. In this case report, we present a case of HWWS along with the challenges in diagnosis and multi-step treatment processes. A 25-year-old woman presented to Dr. Zainoel Abidin Hospital in Banda Aceh, Indonesia with a chief complaint of lower back pain for the past six months (two months after the marriage). The patient also complained of late menstruation, followed by thick yellow vaginal discharge resembling malodorous pus occurring after menstruation. Additionally, a history of fever and pain during intercourse was reported. Vaginal examination revealed purulent fluid emerging from a pinpoint hole on the right vaginal wall. Vaginal palpation revealed a two-branched uterus, along with a palpable cystic mass on the right vaginal wall. Ultrasound examination indicated the presence of the right and left hemi-uteri, measuring 4.7x1.35 cm and 5.7x1.26 cm in size, respectively with both ovaries appearing normal. Fluid accumulation was observed in the right hemivagina, while the left kidney exhibited normal features, and the right kidney showed signs of hypoplasia. The patient was diagnosed with uterus didelphys, fluid accumulation in the hemivagina, and right kidney hypoplasia. Magnetic resonance imaging (MRI) confirmed uterus didelphys and revealed narrowing of the right hemivagina, suggestive of HWWS. Hysteroscopy was performed to resect the vaginal septum with laparoscopic guidance, along with drainage of hematocolpos, pyocolpos and placement of an intracervical mold. Despite HWWs having conservative gradual management, it was opted to perform vaginal septum resection, hematocolpos and pyocolpos drainage and placement of an intracervical mould in this case, in order to relieve symptoms and restore the reproductive and sexual functions.
摘要：
Herlyn-Werner-Wunderlich综合征（HWWS），也称为OHVIRA综合征（阻塞的半阴道和同侧肾脏异常）是一种复杂的先天性畸形，其特征是三联征，包括子宫，半阴道梗阻,和同侧肾发育不全。在这个案例报告中,我们介绍了一例HWWS病例以及诊断和多步骤治疗过程中的挑战.一名25岁的妇女被送往班达亚齐的ZainoelAbidin医生医院，印度尼西亚在过去六个月（婚后两个月）主要抱怨腰痛。患者还抱怨月经晚期，其次是厚厚的黄色阴道分泌物，类似于月经后出现的恶臭脓液。此外,报告了性交期间的发烧和疼痛史。阴道检查发现脓性液体从右阴道壁上的针孔中出现。阴道触诊显示子宫有两个分支，右侧阴道壁上有明显的囊性肿块。超声检查提示存在左右半子宫,尺寸为4.7x1.35厘米和5.7x1.26厘米，两个卵巢分别正常。在右半阴道观察到液体积聚，而左肾表现出正常特征，右肾有发育不全的迹象.病人被诊断为子宫双子宫，液体在半阴道积聚，和右肾发育不全.磁共振成像（MRI）证实了子宫的差异，并显示了右半阴道狭窄，暗示HWWS。在腹腔镜引导下进行宫腔镜切除阴道隔，随着血结肠的引流，pyocolpos和放置宫颈内模具。尽管HWWs有保守的渐进管理，它选择了阴道隔切除术，在这种情况下，血结肠和血结肠引流和放置宫颈内模具，以缓解症状，恢复生殖和性功能。
