Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn–Werner–Wunderlich Syndrome
jpedsurg.org shows its articles on its own site.
Read the article on jpedsurg.org ↗Article · Jun 2019 · 1 min read
In brief
In brief
Study of 8 HWWS patients shows that early ultrasound screening of female infants with renal agenesis enables presymptomatic diagnosis and elective management, preventing acute complications like hematocolpos. Six of eight patients were diagnosed through postnatal screening rather than symptomatic presentation, avoiding emergency surgery and long-term fertility complications.
Written by the GCMD Library team from the article.
Introduction
Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients.
Methods
We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date.
Results
There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina.
Conclusion
Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment.
Study design
Case series, level IV evidence.