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

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

Journal: Orphanet journal of rare diseases 2024;19(1):60

PMID: 38347519

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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.

Address: Department of Pediatric Surgery, Karolinska University Hospital, Eugeniavägen 23, C11:33, 17176, Stockholm, Sweden. [email protected].; Department of Evidence Based Medicine and Methodology, Qualicura Healthcare Support Agency, Breda, The Netherlands.; Department of Obstetrics and Gynecology, Antoine Béclère Hospital, Paris Saclay University, Clamart, France.; Center for Maternal and Fetal Medicine, Karolinska University Hospital, Stockholm, Sweden.; Department of Gynecology, Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Kaunas, Lithuania.; Department of Gynecology and Obstetrics, Erasmus MC, Rotterdam, The Netherlands.; Center for Maternal and Fetal Medicine, Hospital St Joan de Dieu, Barcelona, Spain.; Department of Neonatology, University Hospital La Paz, Madrid, Spain.; Department of Neonatology, University of Padua, Padua, Italy.; Department of Pediatric Surgery, University of Padua, Padua, Italy.; Department of Pediatric Surgery, Helsinki University Hospital, Helsinki, Finland.; Department of Pediatric Surgery, Erasmus MC, Rotterdam, The Netherlands.; Department of Pediatric Surgery, Centre Hospitalier Régional Universitaire de Lille, Lille, France.; Department of Surgery, UZ Leuven, Louvain, Belgium.; Department of Pediatric Surgery, Odense University Hospital, Odense, Denmark.; Department of Pediatric Surgery, Lithuanian University of Health Sciences Kauno Klinikos, Kaunas, Lithuania.; Department of Pediatric Surgery, Mannheim, Germany.; Department of Pediatric Surgery, Hannover Medical University, Hanover, Denmark.; Department of Pediatric Surgery, Hospital S Joan de Diu, Barcelona, Spain.; Department of Pediatric Surgery, Erasmus MC, Rotterdam, The Netherlands.; UCL Great Ormond Street Institute of Child Health, London, UK.

DOI: 10.1186/s13023-024-03062-8

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MeSH Terms: Gastroschisis, Humans, Infant, Newborn, Europe, Rare Diseases, Female