Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis) : Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome
Copyright © 2019 Elsevier Inc. All rights reserved..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2020 |
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| Erschienen: |
2020 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:55 |
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| Enthalten in: |
Journal of pediatric surgery - 55(2020), 7 vom: 27. Juli, Seite 1377-1380 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Tan, Yu Guang [VerfasserIn] |
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| Links: |
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| Themen: |
Herlyn–Werner–Wunderlich syndrome |
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| Anmerkungen: |
Date Completed 29.12.2020 Date Revised 29.12.2020 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.jpedsurg.2019.06.006 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM298803100 |
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| 245 | 1 | 0 | |a Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis) |b Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome |
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| 500 | |a Date Completed 29.12.2020 | ||
| 500 | |a Date Revised 29.12.2020 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2019 Elsevier Inc. All rights reserved. | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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 | ||
| 520 | |a STUDY DESIGN: Case series, level IV evidence | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Herlyn–Werner–Wunderlich syndrome | |
| 650 | 4 | |a OHVIRA syndrome | |
| 700 | 1 | |a Laksmi, Narasimhan K |e verfasserin |4 aut | |
| 700 | 1 | |a Yap, Te-Lu |e verfasserin |4 aut | |
| 700 | 1 | |a Sadhana, Nadarajah |e verfasserin |4 aut | |
| 700 | 1 | |a Ong, Caroline C P |e verfasserin |4 aut | |
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