PDF(Pubmed)
Abstract：
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome presents with complex diagnostic and therapeutic challenges and is characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. A 14-year-old female with a history of anorectal malformation and urogenital sinus anomaly presented with menstrual blood in her urine, abdominal pain, and distension. Investigations revealed a bicornuate uterus, vesicovaginal fistula, and right ovarian cyst, leading to the diagnosis of OHVIRA syndrome. A multidisciplinary approach resulted in salpingo-oophorectomy and subtotal hysterectomy. This case highlights the diagnostic challenges and emphasizes the role of advanced imaging and a multidisciplinary team in managing such complex conditions. It stresses the importance of patient-centered surgical planning tailored to the individual\'s anatomy and reproductive goals. Early recognition and a tailored, multidisciplinary approach are crucial in managing OHVIRA syndrome and improving outcomes for patients with rare congenital anomalies.
摘要：
阻塞的半阴道和同侧肾异常（OHVIRA）综合征具有复杂的诊断和治疗挑战，以子宫双子宫为特征，阻塞的半阴道,和同侧肾脏异常。一名14岁女性，有肛门直肠畸形和泌尿生殖窦异常病史，尿液中有经血，腹痛,和扩张。调查显示双角子宫,膀胱阴道瘘，右卵巢囊肿,导致OHVIRA综合征的诊断。多学科方法导致输卵管卵巢切除术和次全子宫切除术。此案例突出了诊断挑战，并强调了高级成像和多学科团队在管理此类复杂疾病中的作用。它强调了以患者为中心的手术计划的重要性，适合个人的解剖和生殖目标。早期认可和量身定制，多学科方法对于治疗OHVIRA综合征和改善罕见先天性异常患者的预后至关重要.
