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Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2024

Open Access 01-12-2024 | Review

European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis

Authors: Carmen Mesas Burgos, Willemijn Irvine, Alexandre Vivanti, Peter Conner, Egle Machtejeviene, Nina Peters, Joan Sabria, Ana Sanchez Torres, Costanza Tognon, Alberto Sgró, Antti Kouvisalo, Hester Langeveld-Benders, Rony Sfeir, Marc Miserez, Nils Qvist, Ausra Lokosiute-Urboniene, Katrin Zahn, Julia Brendel, Jordi Prat, Simon Eaton, Alexandra Benachi

Published in: Orphanet Journal of Rare Diseases | Issue 1/2024

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Abstract

Background

The European Reference Network for rare Inherited Congenital Anomalies, ERNICA, guidelines for gastroschisis cover perinatal period to help teams to improve care.

Method

A systematic literature search including 136 publications was conducted. Research findings were assessed following the GRADE methodology. The evidence to decision framework was used to determine the strength and direction of recommendations.

Results

The mode or timing of delivery do not impact neonatal mortality, risk of NEC or time on parenteral nutrition (PN). Intra or extra abdominal bowel dilatation predict complex gastroschisis and longer length of hospital stay but not increased perinatal mortality. Outcomes after Bianchi procedure and primary fascia closure under anesthesia are similar. Sutureless closure decreases the rate of surgical site infections and duration of ventilation compared to surgical closure. Silo-staged closure with or without intubation results in similar outcomes. Outcomes of complex gastroschisis (CG) undergoing early or delayed surgical repair are similar. Early enteral feeds starting within 14 days is associated with lower risk of surgical site infection.

Recommendations

The panel suggests vaginal birth between 37 and 39 w in cases of uncomplicated gastroschisis. Bianchi’s approach is an option in simple gastroschisis. Sutureless closure is suggested when general anesthesia can be avoided, sutured closure. If anesthesia is required. Silo treatment without ventilation and general anesthesia can be considered. In CG with atresia primary intestinal repair can be attempted if the condition of patient and intestine allows. Enteral feeds for simple gastroschisis should start within 14 days.
Appendix
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Metadata
Title
European reference network for rare inherited congenital anomalies (ERNICA) evidence based guideline on the management of gastroschisis
Authors
Carmen Mesas Burgos
Willemijn Irvine
Alexandre Vivanti
Peter Conner
Egle Machtejeviene
Nina Peters
Joan Sabria
Ana Sanchez Torres
Costanza Tognon
Alberto Sgró
Antti Kouvisalo
Hester Langeveld-Benders
Rony Sfeir
Marc Miserez
Nils Qvist
Ausra Lokosiute-Urboniene
Katrin Zahn
Julia Brendel
Jordi Prat
Simon Eaton
Alexandra Benachi
Publication date
01-12-2024
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2024
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-024-03062-8

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