Scholarlink

Background <p>Omphalocele is a congenital defect of the abdominal wall with high morbidity and high practice variation. Evidence based guidance on its management is currently absent. The European Reference Network for Rare Inherited and Congenital Anomalies (ERNICA) developed this guideline to aid clinical decision-making.</p> Methods <p>This guideline was developed in accordance with the Guidelines 2.0 checklist and GRADE methodology. After a bottleneck analysis and prioritization, a systematic review of the literature and critical appraisal of the evidence was performed. Additionally, registry data from the European Pediatric Surgery Audit (EPSA) was provided in clinical questions for which published evidence was scarce. The Evidence to Decision framework was used as a guide to structure the consensus meetings and draft the recommendations.</p> Results <p>The panel developed 12 recommendations on the following topics: Genetic screening, mode of delivery, prognostic factors, enteral feeding and ventilation during staged reduction, type of closure and timing of surgery in giant and non-giant omphalocele. The panel weighed up the benefits and harms, informed by all relevant arguments and expert opinion, to decide on a recommendation. The supplementary data from the EPSA contributed to the panel’s decision on a recommendation in four topics.</p> Conclusion <p>This guideline provides recommendations for the perinatal care of patients with omphalocele. These recommendations support clinicians in making care decisions and help inform families about treatment options and relevant considerations. This guideline will be revised every five years to ensure it remains up to date.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

ERNICA evidence based guideline on omphalocele

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年3月7日

查看全文
  • Willemijn Irvine,
  • Linde Margriet van der Kamp,
  • Olivia Spivack,
  • René Wijnen,
  • Alberto Sgrò,
  • Julia Brendel,
  • Katrin Zahn,
  • Lucas Matthyssens,
  • Elisabet Gustafson,
  • Henrik Røkkum,
  • Lucia Migliazza,
  • Rony Sfeir,
  • Annika Mutanen,
  • Udo Rolle,
  • Anne Dariel,
  • Marc Miserez,
  • Ausra Lukosiute-Urboniene,
  • Alexandre Vivanti,
  • Nina Peters,
  • Peter Conner,
  • Eglė Machtejevienė,
  • Francesca Russo,
  • Ana Sanchez Torres,
  • Alena Kokešová,
  • Hans Jorgen Stensvold,
  • Florian Kipfmueller,
  • Mohamed Riadh Boukhris,
  • Costanza Tognon,
  • Simon Eaton,
  • Iris den Uijl,
  • Alexandra Benachi,
  • Carmen Mesas Burgos

摘要

Background

Omphalocele is a congenital defect of the abdominal wall with high morbidity and high practice variation. Evidence based guidance on its management is currently absent. The European Reference Network for Rare Inherited and Congenital Anomalies (ERNICA) developed this guideline to aid clinical decision-making.

Methods

This guideline was developed in accordance with the Guidelines 2.0 checklist and GRADE methodology. After a bottleneck analysis and prioritization, a systematic review of the literature and critical appraisal of the evidence was performed. Additionally, registry data from the European Pediatric Surgery Audit (EPSA) was provided in clinical questions for which published evidence was scarce. The Evidence to Decision framework was used as a guide to structure the consensus meetings and draft the recommendations.

Results

The panel developed 12 recommendations on the following topics: Genetic screening, mode of delivery, prognostic factors, enteral feeding and ventilation during staged reduction, type of closure and timing of surgery in giant and non-giant omphalocele. The panel weighed up the benefits and harms, informed by all relevant arguments and expert opinion, to decide on a recommendation. The supplementary data from the EPSA contributed to the panel’s decision on a recommendation in four topics.

Conclusion

This guideline provides recommendations for the perinatal care of patients with omphalocele. These recommendations support clinicians in making care decisions and help inform families about treatment options and relevant considerations. This guideline will be revised every five years to ensure it remains up to date.