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
    Corresponding author at: Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Roma 00165, Italy.
    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
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  • Maria Chiara Lucchetti
    Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Roma 00165, Italy
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      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.


      To review the experience of a pediatric tertiary center and to propose an age-specific management protocol for patients diagnosed before menarche.


      A retrospective cohort study (review of medical records - period 2009–2021).


      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.


      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.





      HWWS ((Herlyn-Werner-Wunderlich syndrome)), MCDK ((multicystic-dysplastic kidney)), OHVIRA ((obstructed hemivagina and ipsilateral renal anomaly))
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        • Purslow C.
        A case of unilateral hæmatokolpos, hæmatometra and hæmatosalpinx.
        BJOG An Int J Obstet Gynaecol. 1922; 29: 643
        • Wunderlich M.
        Seltene variante einer genitalmissbildung mit aplasie der rechten niere [Unusual form of genital malformation with aplasia of the right kidney].
        Zentralbl Gynakol. 1976; 98: 559-562
        • Gholoum S.
        • Puligandla P.
        • Hui T.
        • Su W.
        • Quiros E.
        • Laberge J.
        Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome).
        J Pediatr Surg. 2006; 41: 987-992
        • Orazi C.
        • Lucchetti M.
        • Schingo P.
        • Marchetti P.
        • Ferro F.
        Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases.
        Pediatr Radiol. 2007; 37 (Pediatr Radiol): 657-665
        • Embrey M.
        A case of uterus didelphys with unilateral gynatresia.
        Br Med J. 1950; : 820
        • Smith N.
        • Laufer M.
        Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up.
        Fertil Steril. 2007; 87: 918-922
        • The American Fertility Society
        The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions.
        Fertil Steril. 1988; 49: 944-955
        • Grimbizis G.
        • Gordts S.
        • Di Spiezio Sardo A.
        • Brucker S.
        • De Angelis C.
        • Gergolet M.
        • et al.
        The ESHRE-ESGE consensus on the classification of female genital tract congenital anomalies.
        Gynecol Surg. 2013; 10: 199-212
        • Acién P.
        • Acién M.
        • Sánchez-Ferrer M.
        Complex malformations of the female genital tract.
        Hum Reprod. 2004; 19: 2377-2384
        • Santos X.
        • Dietrich J.
        Obstructed hemivagina with ipsilateral renal anomaly.
        J Pediatr Adolesc Gynecol. 2016; 29: 7-10
        • Yang M.
        • Wen S.
        • Liu X.
        • He D.
        • Wei G.
        • Wu S.
        • et al.
        Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA): early diagnosis, treatment and outcomes.
        Eur J Obs Gynecol Reprod Biol. 2021; 261: 12-16
        • Han J.
        • Lee Y.
        • Im Y.
        • Kim S.
        • Lee M.
        • Han S.
        Clinical implications of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in the prepubertal age group.
        PLoS ONE. 2016; 18
        • Fedele L.
        • Motta F.
        • Frontino G.
        • Restelli E.
        • Bianchi S.
        Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases.
        S Hum Reprod. 2013; 28: 1580-1583
        • Dietrich J.
        • Millar D.
        • Quint E
        Obstructive reproductive tract anomalies.
        J Pediatr Adolesc Gynecol. 2014; 27: 396-402
        • Kim Y.
        • Han J.
        • Lee Y.
        • Lee I.
        • Han S.
        • Seo S.
        • et al.
        Comparison between prepubertal and postpubertal patients with obstructed hemivagina and ipsilateral renal anomaly syndrome.
        J Pediatr Urol. 2021;
        • Tuna T.
        • Estevão-Costa J.
        • Ramalho C.
        • Fragoso A.
        Herlyn-Werner-Wunderlich syndrome: report of a prenatally recognised case and review of the literature.
        Urology2. 2019; 125: 205-209
        • Han B.
        • Park S.
        • Lee Y.
        • Lee K.
        • Lee Y.
        Uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) suspected on the presence of hydrocolpos on prenatal sonography.
        J Clin Ultrasound. 2013; 41: 380-382
        • Santos X.
        • Krishnamurthy R.
        • Bercaw-Pratt J.
        • Dietrich J.
        The utility of ultrasound and magnetic resonance imaging versus surgery for the characterization of müllerian anomalies in the pediatric and adolescent population.
        J Pediatr Adolesc Gynecol. 2012; 25: 181-184
        • Tan Y.
        • Laksmi N.
        • Yap T.
        • Sadhana N.
        • Ong C.
        Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): early diagnosis and management of asymptomatic Herlyn-Werner-Wunderlich syndrome.
        J Pediatr Surg. 2020; 55: 1377-1380
        • Skinner B.
        • Quint E
        Obstructive reproductive tract anomalies: a review of surgical management.
        J Minim Invasive Gynecol. 2017; 24: 901-908
        • Noviello C.
        • Romano M.
        • Nino F.
        • Martino A.
        • Cobellis G.
        Clinical and radiological findings for early diagnosis of Herlyn-Werner-Wunderlich syndrome in pediatric age: experience of a single center.
        Gynecol Endocrinol. 2018; 34: 56-58
        • Dindo D.
        • Demartines N.
        • Clavien P.A.
        Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey.
        Ann Surg. 2004; 240: 205-213
        • Tong J.
        • Zhu L.
        • Lang J.
        Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome.
        Int J Gynaecol Obs. 2013; 121: 173-175
        • Wang J.
        • Zhu L.
        • Lang J.
        • Liu Z.
        • Sun D.
        • Leng J.
        • et al.
        Clinical characteristics and treatment of Herlyn-Werner-Wunderlich syndrome.
        Arch Gynecol Obs. 2014; 290: 947-950
        • Ercoskun P.
        • Yuce-Kahraman C.
        Novel findings in floating-harbor syndrome and a mini-review of the literature.
        Mol Syndr. 2021; 12: 52-56
        • Bo H.
        • Jiang L.
        • Zheng J.
        • Sun J.
        Floating-harbor syndrome treated with recombinant human growth hormone: a case report and literature review.
        Front Pediatr. 2021; 5
        • Silveira S.
        • Laufer M.
        Persistence of endometriosis after correction of an obstructed reproductive tract anomaly.
        J Pediatr Adolesc Gynecol. 2013; 26: 93-94
        • Dunselman G.
        • Vermeulen N.
        • Becker C.
        • Calhaz-Jorge C.
        • D'Hooghe T.
        • De Bie B.
        • et al.
        ESHRE guideline: management of women with endometriosis.
        Hum Reprod. 2014; 29: 400-412
        • Gungor Ugurlucan F.
        • Dural O.
        • Yasa C.
        • Kirpinar G.
        • Akhan S.
        Diagnosis, management, and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): is there a correlation between MRI findings and outcome?.
        Clin Imaging. 2020; 59: 172-178