Telecan Teodora, Capras Roxana Denisa, Filip Gabriela Adriana, Ionutas Emanuela Maria, Stanca Dan Vasile, Crivii Carmen-Bianca
Department of Anatomy and Embryology, "Iuliu Hatieganu" University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania.
Department of Pathology, County Emergency Clinical Hospital, 400347 Cluj-Napoca, Romania.
Medicina (Kaunas). 2024 Nov 22;60(12):1922. doi: 10.3390/medicina60121922.
Müllerian (paramesonephric) duct anomalies (MDA) are a rare condition, occurring in 5.5% of female newborns. One of the most complex malformations is represented by Obstructed Hemivagina and Ipsilateral Renal Anomalies (OHVIRA) syndrome, also known as Herlyn -Werner-Wunderlich (HWW) syndrome. We present the case of a 7-year-old asymptomatic premenarchal female patient diagnosed with OHVIRA syndrome with ipsilateral renal hypoplasia and ectopic ureteral implantation at the level of the uterus. As the patient developed urinary incontinence after the incision of the vaginal septum, right-sided 3D laparoscopic total nephrectomy was performed. OHVIRA syndrome associated with ureteral ectopy is a rare occurrence, being encountered in 0.0064% of cases. The premenarchal diagnosis represents a challenge, due to the underdeveloped status of the genital tract. However, it should be ruled out in female newborns with unilateral renal agenesia or multicystic dysplastic kidney. Most reported cases describe the obstructed hemivagina as the site of ureteral ectopy. To our knowledge, this is the first reported case of OHVIRA syndrome with ectopic ureter draining at the level of the ipsilateral hemiuterus, diagnosed before the pubertal age. OHVIRA syndrome is one of the rarest Müllerian duct abnormalities. The management of these patients should be conducted in multidisciplinary teams, with long-term urological and gynecological follow-ups.
苗勒管（副中肾管）异常（MDA）是一种罕见疾病，在5.5%的女性新生儿中出现。最复杂的畸形之一是梗阻性半阴道并同侧肾异常（OHVIRA）综合征，也称为赫林-韦纳-温德利希（HWW）综合征。我们报告一例7岁无症状青春期前女性患者，诊断为OHVIRA综合征，伴有同侧肾发育不全和输尿管异位植入子宫水平处。由于患者在阴道纵隔切开术后出现尿失禁，遂行右侧3D腹腔镜全肾切除术。OHVIRA综合征合并输尿管异位很少见，发生率为0.0064%。青春期前诊断具有挑战性，因为生殖道发育不全。然而，在单侧肾缺如或多囊性发育不良肾的女性新生儿中应排除该疾病。大多数报道的病例将梗阻性半阴道描述为输尿管异位的部位。据我们所知，这是第一例在青春期前诊断的OHVIRA综合征，异位输尿管引流至同侧半子宫水平处。OHVIRA综合征是最罕见的苗勒管异常之一。这些患者的管理应由多学科团队进行，并进行长期的泌尿外科和妇科随访。
