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))To read this article in full you will need to make a payment
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References
- A case of unilateral hæmatokolpos, hæmatometra and hæmatosalpinx.BJOG An Int J Obstet Gynaecol. 1922; 29: 643https://doi.org/10.1111/j.1471-0528.1922.tb16100.x
- 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
- Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome).J Pediatr Surg. 2006; 41: 987-992https://doi.org/10.1016/j.jpedsurg.2006.01.021
- 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
- A case of uterus didelphys with unilateral gynatresia.Br Med J. 1950; : 820https://doi.org/10.1136/bmj.1.4657.820
- Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up.Fertil Steril. 2007; 87: 918-922https://doi.org/10.1016/j.fertnstert.2006.11.015
- 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: 944-955https://doi.org/10.1016/s0015-0282(16)59942-7
- The ESHRE-ESGE consensus on the classification of female genital tract congenital anomalies.Gynecol Surg. 2013; 10: 199-212https://doi.org/10.1007/s10397-013-0800-x
- Complex malformations of the female genital tract.Hum Reprod. 2004; 19: 2377-2384https://doi.org/10.1093/humrep/deh423
- Obstructed hemivagina with ipsilateral renal anomaly.J Pediatr Adolesc Gynecol. 2016; 29: 7-10https://doi.org/10.1016/j.jpag.2014.09.008
- Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA): early diagnosis, treatment and outcomes.Eur J Obs Gynecol Reprod Biol. 2021; 261: 12-16https://doi.org/10.1016/j.ejogrb.2021.03.018
- Clinical implications of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome in the prepubertal age group.PLoS ONE. 2016; 18https://doi.org/10.1371/journal.pone.0166776
- Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases.S Hum Reprod. 2013; 28: 1580-1583https://doi.org/10.1093/humrep/det081
- Obstructive reproductive tract anomalies.J Pediatr Adolesc Gynecol. 2014; 27: 396-402https://doi.org/10.1016/j.jpag.2014.09.001
- Comparison between prepubertal and postpubertal patients with obstructed hemivagina and ipsilateral renal anomaly syndrome.J Pediatr Urol. 2021; https://doi.org/10.1016/j.jpurol.2021.06.008
- Herlyn-Werner-Wunderlich syndrome: report of a prenatally recognised case and review of the literature.Urology2. 2019; 125: 205-209https://doi.org/10.1016/j.urology.2018.12.022
- 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: 380-382https://doi.org/10.1002/jcu.21950
- 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: 181-184https://doi.org/10.1016/j.jpag.2011.12.069
- 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: 1377-1380https://doi.org/10.1016/j.jpedsurg.2019.06.006
- Obstructive reproductive tract anomalies: a review of surgical management.J Minim Invasive Gynecol. 2017; 24: 901-908https://doi.org/10.1016/j.jmig.2017.04.020
- Clinical and radiological findings for early diagnosis of Herlyn-Werner-Wunderlich syndrome in pediatric age: experience of a single center.Gynecol Endocrinol. 2018; 34: 56-58https://doi.org/10.1080/09513590.2017.1332178
- Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey.Ann Surg. 2004; 240: 205-213https://doi.org/10.1097/01.sla.0000133083.54934.ae
- Clinical characteristics of 70 patients with Herlyn-Werner-Wunderlich syndrome.Int J Gynaecol Obs. 2013; 121: 173-175https://doi.org/10.1016/j.ijgo.2012.11.023
- Clinical characteristics and treatment of Herlyn-Werner-Wunderlich syndrome.Arch Gynecol Obs. 2014; 290: 947-950https://doi.org/10.1007/s00404-014-3286-5
- Novel findings in floating-harbor syndrome and a mini-review of the literature.Mol Syndr. 2021; 12: 52-56https://doi.org/10.1159/000512050
- Floating-harbor syndrome treated with recombinant human growth hormone: a case report and literature review.Front Pediatr. 2021; 5https://doi.org/10.3389/fped.2021.747353
- Persistence of endometriosis after correction of an obstructed reproductive tract anomaly.J Pediatr Adolesc Gynecol. 2013; 26: 93-94https://doi.org/10.1016/j.jpag.2013.01.002
- ESHRE guideline: management of women with endometriosis.Hum Reprod. 2014; 29: 400-412https://doi.org/10.1093/humrep/det457
- 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: 172-178https://doi.org/10.1016/j.clinimag.2019.11.013
Article info
Publication history
Published online: April 15, 2022
Accepted:
April 4,
2022
Received in revised form:
March 18,
2022
Received:
November 17,
2021
Identification
Copyright
© 2022 Elsevier Inc. All rights reserved.
