Pediatric Case ReportsHerlyn-Werner-Wunderlich Syndrome: Report of a Prenatally Recognised Case and Review of the Literature
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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Cited by (24)
Case Report: Neonate Presenting with Acute Kidney Injury Secondary to Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome: Long-Term Follow-Up
2023, Journal of Pediatric and Adolescent GynecologyOHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome): Is it time for age-specific management?
2022, Journal of Pediatric SurgeryCitation Excerpt :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 ReportsCitation Excerpt :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 BiologyCitation Excerpt :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].
Herlyn-Werner-Wünderlich syndrome of neonatal diagnosis
2021, Anales de Pediatria
Declarations of Interest: None.
Support/Financial Disclosures: The authors declare that they have no relevant financial interests.