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Research Article| Volume 57, ISSUE 11, P696-701, November 2022

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OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome): Is it time for age-specific management?

  • Angelo Zarfati
    Correspondence
    Corresponding author at: Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Roma 00165, Italy.
    Affiliations
    Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Roma 00165, Italy

    University of Rome Tor Vergata, Via Cracovia, 50, Roma 00133, Italy
    Search for articles by this author
  • Maria Chiara Lucchetti
    Affiliations
    Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Roma 00165, Italy
    Search for articles by this author

      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.

      Aims

      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.

      Level-of-Evidence

      IV.

      Keywords

      Abbreviations:

      HWWS ((Herlyn-Werner-Wunderlich syndrome)), MCDK ((multicystic-dysplastic kidney)), OHVIRA ((obstructed hemivagina and ipsilateral renal anomaly))
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