%0 Journal Article
%T Managing giant omphalocele: A systematic review of surgical techniques and outcomes.
%A Ghattaura H
%A Ross A
%A Aldeiri B
%A Mutanen A
%A Saxena A
%J Acta Paediatr
%V 0
%N 0
%D 2024 Jul 11
%M 38992931
%F 4.056
%R 10.1111/apa.17346
%X OBJECTIVE: We analysed closure techniques in the treatment of giant omphalocele. A challenging pathology where there lacks consensus.
METHODS: Cochrane, MEDLINE and EMBASE were searched between 1 January 1992 and 31 December 2022 using terms and variations: omphalocele, exomphalos, giant, closure and outcome. Papers were selected using Preferred Reporting Items for Systematic review and Meta-Analyses 2020 criteria. Data collected included demographics, timing and technique of surgical repair, morbidity and mortality.
RESULTS: We identified 342 papers; 34 met inclusion criteria with a total 356 neonates. Initial non-operative management was described in 26 papers (14 dressings, eight silo, four serial sac-ligation). Operative techniques by paper were as follows: Early closure: nine primary suture closure without patch, two primary closure with patch and four mixed methods. Delayed closure: five simple, four-component separation technique, four tissue expanders, one Botox/pneumoperitoneum and two with patch. Median number of procedures was two (1-6) in the early group versus three (1-4) in the delayed. The most favourable was early primary closure with biological patch. The most unfavourable was delayed closure with patch. Cumulative reported mortality remained high, mostly due to non-surgical causes.
CONCLUSIONS: Definitions of giant omphalocele in the literature were heterogeneous with a variety of management approaches described.