Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
J Pediatr Adolesc Gynecol. 2022 Jun;35(3):387-390. doi: 10.1016/j.jpag.2021.11.008. Epub 2021 Nov 27.
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly. Several associations of OHVIRA with other anomalies are being reported.
A 13-year-old girl reported with acute onset abdominal pain. Patient was operated on in the past for type IV congenital pouch colon (all stages complete). She was diagnosed with hematometrocolpos and underwent tube vaginostomy. Further workup was suggestive of OHVIRA, which was subsequently managed with surgical repair.
Delineation of reproductive anomalies in female patients with anorectal malformations is of paramount importance. OHVIRA syndrome should be considered at a high index of suspicion in female patients with a solitary functioning kidney. Nonspecific symptoms in an adolescent female can lead to erroneous judgment, leading to unnecessary investigations, which, if not well managed in time, can be detrimental to fertility.
半侧阴道闭锁伴同侧肾发育不全（OHVIRA），也称 Herlyn-Werner-Wunderlich 综合征，是一种罕见的苗勒管畸形。目前已有报道称 OHVIRA 与其他多种畸形存在关联。
一名 13 岁女孩因突发性腹痛就诊。患者曾因 IV 型先天性袋状结肠（所有阶段均完整）接受过手术治疗。该患者被诊断为阴道积血，并接受了阴道造口术。进一步的检查提示为 OHVIRA，随后进行了手术修复。
对于患有肛门直肠畸形的女性，明确其生殖系统异常至关重要。对于单侧有功能肾的女性患者，应高度怀疑 OHVIRA 综合征。青春期女性的非特异性症状可能导致误诊，从而导致不必要的检查，如果不能及时妥善处理，可能会对生育能力造成损害。
