Abstract：
BACKGROUND: OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome) is a rare Müllerian malformation. Usually, symptoms begin with worsening dysmenorrhea in post-menarche adolescents. The management in pre-menarche period is controversial and has only recently been subject of study.
OBJECTIVE: To review the experience of a pediatric tertiary center and to propose an age-specific management protocol for patients diagnosed before menarche.
METHODS: A retrospective cohort study (review of medical records - period 2009-2021).
RESULTS: Twenty-eight patients were diagnosed (mean age 11.9 years), seven (25%) before menarche, one (3%) perinatally. One patient had Floating-Harbor syndrome. Twenty-three patients had ipsilateral renal agenesis, while five had a multicystic-dysplastic kidney. The contralateral kidney showed hypertrophy in 25 patients, pelvicalyceal ectasia in 8 and dysplasia in 1. Twenty-four patients were symptomatic. Three of the seven diagnosed prior to menarche had symptoms. All post-menarche diagnosed patients were symptomatic. Twenty-six patients underwent surgery (one-stage drainage, vaginal septal resection, and vaginoplasty). Asymptomatic pre-menarche patients were followed-up until surgery after menarche onset. No patient underwent surgery prior to menarche solely for OHVIRA diagnosis. At follow-up (median 3.5 years, 3 lost to follow-up), eighteen patients were asymptomatic, one developed endometriosis, one had impaired renal function, two needed reoperations.
CONCLUSIONS: Pre-menarche OHVIRA patients, without symptoms, should undergo regular follow-up until the onset of menarche. Surgery must be considered in post-menarche or symptomatic patients. Post-operative, long-term follow-up is required, evaluating both renal and gynecological issues.
METHODS: IV.
OBJECTIVE: To review the experience of a pediatric tertiary center and to propose an age-specific management protocol for patients diagnosed before menarche.
METHODS: A retrospective cohort study (review of medical records - period 2009-2021).
RESULTS: Twenty-eight patients were diagnosed (mean age 11.9 years), seven (25%) before menarche, one (3%) perinatally. One patient had Floating-Harbor syndrome. Twenty-three patients had ipsilateral renal agenesis, while five had a multicystic-dysplastic kidney. The contralateral kidney showed hypertrophy in 25 patients, pelvicalyceal ectasia in 8 and dysplasia in 1. Twenty-four patients were symptomatic. Three of the seven diagnosed prior to menarche had symptoms. All post-menarche diagnosed patients were symptomatic. Twenty-six patients underwent surgery (one-stage drainage, vaginal septal resection, and vaginoplasty). Asymptomatic pre-menarche patients were followed-up until surgery after menarche onset. No patient underwent surgery prior to menarche solely for OHVIRA diagnosis. At follow-up (median 3.5 years, 3 lost to follow-up), eighteen patients were asymptomatic, one developed endometriosis, one had impaired renal function, two needed reoperations.
CONCLUSIONS: Pre-menarche OHVIRA patients, without symptoms, should undergo regular follow-up until the onset of menarche. Surgery must be considered in post-menarche or symptomatic patients. Post-operative, long-term follow-up is required, evaluating both renal and gynecological issues.
METHODS: IV.
摘要：
背景：OHVIRA（阻塞的半阴道和同侧肾异常或Herlyn-Werner-Wunderlich综合征）是一种罕见的Mülerian畸形。通常,症状始于初潮后青少年的痛经恶化。初潮前期的管理是有争议的，直到最近才成为研究的主题。
目的：回顾儿科三级中心的经验，并为初潮前诊断的患者提出年龄特异性管理方案。
方法：回顾性队列研究（病历回顾-2009-2021年）。
结果：诊断出28例患者（平均年龄11.9岁），初潮前七个（25%），一个（3％）围产期。一名患者患有浮港综合征。23例患者有同侧肾发育不全,而5个患有多囊性发育不良的肾脏。25例患者对侧肾脏表现为肥大，骨盆部扩张8例，发育不良1例。24例患者有症状。在初潮之前被诊断出的七个人中有三个有症状。所有初潮后诊断的患者均有症状。26例患者接受了手术（一期引流，阴道间隔切除术,和阴道成形术)。对初潮前无症状患者进行随访，直至初潮发作后手术。在初潮之前，没有患者仅出于OHVIRA诊断而接受手术。随访时(中位数3.5年，3失去随访），18名患者无症状，一个患有子宫内膜异位症，其中一人肾功能受损，两次需要重新手术。
结论：初潮前OHVIRA患者，没有症状,应定期随访，直到初潮开始。初潮后或有症状的患者必须考虑手术。手术后,需要长期随访，评估肾脏和妇科问题。
方法：IV.
目的：回顾儿科三级中心的经验，并为初潮前诊断的患者提出年龄特异性管理方案。
方法：回顾性队列研究（病历回顾-2009-2021年）。
结果：诊断出28例患者（平均年龄11.9岁），初潮前七个（25%），一个（3％）围产期。一名患者患有浮港综合征。23例患者有同侧肾发育不全,而5个患有多囊性发育不良的肾脏。25例患者对侧肾脏表现为肥大，骨盆部扩张8例，发育不良1例。24例患者有症状。在初潮之前被诊断出的七个人中有三个有症状。所有初潮后诊断的患者均有症状。26例患者接受了手术（一期引流，阴道间隔切除术,和阴道成形术)。对初潮前无症状患者进行随访，直至初潮发作后手术。在初潮之前，没有患者仅出于OHVIRA诊断而接受手术。随访时(中位数3.5年，3失去随访），18名患者无症状，一个患有子宫内膜异位症，其中一人肾功能受损，两次需要重新手术。
结论：初潮前OHVIRA患者，没有症状,应定期随访，直到初潮开始。初潮后或有症状的患者必须考虑手术。手术后,需要长期随访，评估肾脏和妇科问题。
方法：IV.
