Elsevier

Urology

Volume 125, March 2019, Pages 205-209
Urology

Pediatric Case Reports
Herlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature

https://doi.org/10.1016/j.urology.2018.12.022Get rights and content

Abstract

Herlyn-Werner-Wunderlich syndrome, defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian malformation, usually diagnosed after menarche, when symptoms related to hematocolpos arise. Rarely, this malformation is diagnosed in the neonatal period, normally following prenatal diagnosis of renal agenesis. Herein, a case recognized on prenatal imagiology that underwent surgery on the fourth day of life is reported. The records of prepubertal cases were also collected, addressing the clinical and imagiological features. In the presence of a solitary kidney and/or a pelvic mass on prenatal ultrasound, Herlyn-Werner-Wunderlich syndrome should be considered, enabling neonatal treatment.

Section snippets

CASE REPORT

A female newborn was delivered at 38 weeks and 5 days of gestation by an eutocic and unremarkable delivery. Prenatal ultrasound at the 36th week showed an absent right kidney and a cystic lesion in the pelvis (Fig. 1A). Fetal magnetic resonance imaging, 1 week later, confirmed right renal agenesis and a left kidney with pelvicalyceal dilation (10 mm). A cystic lesion measuring 65 × 25 × 22 mm was shown behind the bladder, suggesting hydrocolpos, although an anorectal malformation was not

DISCUSSION

Developmental abnormalities of the female genital tract involve a wide variety of disorders of the fallopian tubes, uterus and vagina, and have a reported mean prevalence of approximately 7%.4 They occur from maldevelopment of the Mullerian or paramesonephric ducts and can be associated with reproductive issues. The development of Mullerian ducts is embryologically interlinked to the development of Wolffian or mesonephric ducts, explaining the frequent association of renal and urologic

CONCLUSION

Our clinical case shows that prenatal suspicion and careful physical examination at birth allows early diagnosis and management of HWWS, which relates to better outcomes and avoidance of potential lifelong complications. The presence of renal agenesis in the prenatal ultrasound, especially when associated with a cystic pelvic mass, should raise the clinician awareness of complex urogenital malformations such as HWWS.

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

    2022, Journal of Pediatric Surgery
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    Pre-menarche patients are usually asymptomatic [12,15]. Distended hemivagina presenting as a protruding vaginal mass occurs rarely in newborns and regresses after the cessation of exposure to maternal estrogen after birth and the end of minipuberty [12,16,17]. Diagnosis can be made with history and physical examination, in combination with appropriate imaging [6].

  • Herlyn–Werner–Wunderlich syndrome and its complications: A report of two cases and literature review

    2021, Radiology Case Reports
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    They occur owing to maldevelopment of the Müllerian or paramesonephric ducts [7]. The development of Müllerian ducts is embryologically interlinked to the development of Wolffian or mesonephric ducts, which could explain the frequent association of urologic abnormalities and Müllerian malformations [3]. Renal agenesis is the most common among these concurrent abnormalities, accounting for up to 30% of cases [9].

  • Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA): Early diagnosis, treatment and outcomes

    2021, European Journal of Obstetrics and Gynecology and Reproductive Biology
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    Some surgeons believe that the thinned vaginal septum is more likely to be removed when a large amount of vaginal blood has accumulated after puberty [24]. Tuna et al. performed a vaginal septal excision in an asymptomatic newborn with Herlyn–Werner–Wunderlich syndrome who recovered well and was asymptomatic at follow-up [25]. Han et al. reported that 13 % of patients were treated surgically before puberty [26].

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Declarations of Interest: None.

Support/Financial Disclosures: The authors declare that they have no relevant financial interests.

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