Download PDF
Eur J Pediatr Surg
DOI: 10.1055/a-2590-5592
Original Article

European Paediatric Surgeons' Association Consensus Statement on the Management of Giant Omphalocele

Amulya K. Saxena 
1   Department of Paediatric Surgery, Chelsea and Westminster Hospital NHS Foundation Trust, London, United Kingdom
2   Imperial College London, London, United Kingdom
,
Romilly K. Hayward 
2   Imperial College London, London, United Kingdom
,
Annika Mutanen
3   Department of Pediatric Surgery, the New Children's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
,
Ayman Goneidy
1   Department of Paediatric Surgery, Chelsea and Westminster Hospital NHS Foundation Trust, London, United Kingdom
,
Harmit Ghattaura
4   Department of Pediatric Surgery, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, England, United Kingdom of Great Britain and Northern Ireland
,
Ramon Gorter
5   Department of Pediatric Surgery, Emma Children's Hospital UMC, Amsterdam, The Netherlands
,
Rene Weijnen
6   Department of Pediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands
,
Richard Keijzer
7   Division of Pediatric Surgery, Departments of Surgery and Pediatrics & Child Health, Max Rady College of Medicine, Rady Faculty of Heath Sciences, University of Manitoba, Children's Hospital Research Institute of Manitoba, Winnipeg, Manitoba, Canada
,
Tutku Soyer 
8   Department of Pediatric Surgery, Hacettepe Universitesi Tip Fakultesi, Ankara, Turkey
,
The European Pediatric Surgeons' Association› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Abstract

Introduction

Giant omphalocele management had not reached a consensus from the pediatric surgical perspective regarding conservative treatments, surgical approaches, and clinical outcomes. This topic was therefore selected for the 2023 Consensus Session of the European Paediatric Surgeons' Association (EUPSA).

Methods

Literature review was conducted by seven EUPSA members, guided by a set of predefined areas relating to the management of giant omphalocele: (1) conservative management, (2) surgical management, and (3) outcomes. Members were assigned to specific topics, with discrepancies resolved through structured group discussion and further literature review. Consensus was reached through unanimous agreement among the contributing members. Each topic was presented with available evidence to congress participants. Comments from participants were accounted to formulate the final consensus statement.

Results

Giant omphalocele is appropriately defined as viscero-abdominal disproportion preventing primary closure. Regarding (1) conservative management, the “paint and wait” approach is recommended when anatomical constraints or high surgical risk preclude primary closure. Common painting agents include povidone-iodine and silver sulfadiazine, with Manuka honey gaining interest, though consensus on dosing, duration, and complications remains unclear. With regards to (2) surgical management, early closure favors biological meshes, while delayed closure appears most effective using native tissues. High mortality in delayed patch closure likely reflects a population with the most severe defects. Finally, (3) outcomes highlights key prognostic factors including chromosomal abnormalities, cardiac defects, and low birth weight, which may guide counseling, screening, and treatment. Limited data on ruptured omphalocele indicates towards increased mortality, necessitating prompt intervention. Complications following management are relatively rare and typically intervention-related, underscoring the need for long-term, multidisciplinary follow-up.

Conclusion

A consensus statement on the management of giant omphalocele was developed based on current evidence and peer practice, though imitations relating to a scarcity of high-level evidence and significant heterogeneity across studies should be acknowledged. Despite these constraints, this consensus statement provides evidence-based guidance to support pediatric surgeons in informed decision-making for this pathology.

Keywords

consensus statement - giant omphalocele - surgical management - conservative treatment - outcomes


Publication History

Received: 09 January 2025

Accepted: 15 April 2025

Article published online:
19 May 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

  • References

  • 1 Kluth D, Lambrecht W. The pathogenesis of omphalocele and gastroschisis: an unsolved problem. Pediatr Surg Int 1996; 11 (2-3): 62-66
    PubMedSearch in Google Scholar
  • 2 Grosfeld JL, Weber TR. Congenital abdominal wall defects: gastroschisis and omphalocele. Curr Probl Surg 1982; 19 (04) 157-213
    PubMedSearch in Google Scholar
  • 3 Bauman B, Stephens D, Gershone H. et al. Management of giant omphaloceles: a systematic review of methods of staged surgical vs. nonoperative delayed closure. J Pediatr Surg 2016; 51 (10) 1725-1730
    PubMedSearch in Google Scholar
  • 4 Wilcinski SL. Use of a vacuum-assisted device in a neonate with a giant omphalocele. Adv Neonatal Care 2010; 10 (03) 119-126 , quiz 127–128
    PubMedSearch in Google Scholar
  • 5 Verla MA, Style CC, Olutoye OO. Prenatal diagnosis and management of omphalocele. Semin Pediatr Surg 2019; 28 (02) 84-88
    PubMedSearch in Google Scholar
  • 6 Rombaldi MC, Barreto CG, Feldens L. et al. Giant omphalocele: a novel approach for primary repair in the neonatal period using botulinum toxin [Article in English, Portuguese]. Rev Col Bras Cir 2023; 50: e20233582
    PubMedSearch in Google Scholar
  • 7 Whitehouse JS, Gourlay DM, Masonbrink AR. et al. Conservative management of giant omphalocele with topical povidone-iodine and its effect on thyroid function. J Pediatr Surg 2010; 45 (06) 1192-1197
    PubMedSearch in Google Scholar
  • 8 Saxena AK, Raicevic M. Predictors of mortality in neonates with giant omphaloceles. Minerva Pediatr 2018; 70 (03) 289-295
    PubMedSearch in Google Scholar
  • 9 Nelson J, Wachowiak R, Siekmeyer M. et al. Treatment of ruptured giant omphalocele and gastroschisis with liver herniation using a wound retractor as a novel approach. European J Pediatr Surg Rep 2020; 8 (01) e112-e116
    Thieme ConnectPubMedSearch in Google Scholar
  • 10 Campos BA, Tatsuo ES, Miranda ME. Omphalocele: how big does it have to be a giant one?. J Pediatr Surg 2009; 44 (07) 1474-1475 , author reply 1475
    PubMedSearch in Google Scholar
  • 11 Tucci M, Bard H. The associated anomalies that determine prognosis in congenital omphaloceles. Am J Obstet Gynecol 1990; 163 (5 Pt 1): 1646-1649
    PubMedSearch in Google Scholar
  • 12 Kumar HR, Jester AL, Ladd AP. Impact of omphalocele size on associated conditions. J Pediatr Surg 2008; 43 (12) 2216-2219
    PubMedSearch in Google Scholar
  • 13 Shi X, Tang H, Lu J, Yang X, Ding H, Wu J. Prenatal genetic diagnosis of omphalocele by karyotyping, chromosomal microarray analysis and exome sequencing. Ann Med 2021; 53 (01) 1285-1291
    PubMedSearch in Google Scholar
  • 14 Barrios Sanjuanelo A, Abelló Munarriz C, Cardona-Arias JA. Systematic review of mortality associated with neonatal primary staged closure of giant omphalocele. J Pediatr Surg 2021; 56 (04) 678-685
    PubMedSearch in Google Scholar
  • 15 Hatch Jr EI, Baxter R. Surgical options in the management of large omphaloceles. Am J Surg 1987; 153 (05) 449-452
    PubMedSearch in Google Scholar
  • 16 Barrios-Sanjuanelo A, Abelló-Munarriz C, Cardona-Arias JA. Mortality in neonates with giant omphalocele subjected to a surgical technique in Barranquilla, Colombia from 1994 to 2019. Sci Rep 2021; 11 (01) 310
    PubMedSearch in Google Scholar
  • 17 Percy DB, Haddock C, Ma V, Nowak A, Panczuk J, Butterworth S. Dressed for success? Silver impregnated nanocrystalline dressing for initial treatment of giant omphalocele. J Pediatr Surg 2018; 53 (05) 905-908
    PubMedSearch in Google Scholar
  • 18 Peters NCJ, Hijkoop A, Lechner RL. et al. The validity of the viscero-abdominal disproportion ratio for type of surgical closure in all fetuses with an omphalocele. Prenat Diagn 2019; 39 (12) 1070-1079
    PubMedSearch in Google Scholar
  • 19 Wagner JP, Cusick RA. Paint and wait management of giant omphaloceles. Semin Pediatr Surg 2019; 28 (02) 95-100
    PubMedSearch in Google Scholar
  • 20 Oquendo M, Agrawal V, Reyna R, Patel HI, Emran MA, Almond PS. Silver-impregnated hydrofiber dressing followed by delayed surgical closure for management of infants born with giant omphaloceles. J Pediatr Surg 2015; 50 (10) 1668-1672
    PubMedSearch in Google Scholar
  • 21 Pandey V, Gangopadhyay AN, Gupta DK, Sharma SP, Kumar V. Non-operative management of giant omphalocele with topical povidone-iodine and powdered antibiotic combination: early experience from a tertiary centre. Pediatr Surg Int 2014; 30 (04) 407-411
    PubMedSearch in Google Scholar
  • 22 Nicoara CD, Singh M, Jester I, Reda B, Parikh DH. Medicated Manuka honey in conservative management of exomphalos major. Pediatr Surg Int 2014; 30 (05) 515-520
    PubMedSearch in Google Scholar
  • 23 Binet A, Scalabre A, Amar S. et al. Operative versus conservative treatment for giant omphalocele: study of French and Ivorian management. Ann Chir Plast Esthet 2020; 65 (02) 147-153
    PubMedSearch in Google Scholar
  • 24 Eltayeb AA, Mostafa MM. Topical treatment of major omphalocoele: Acacia nilotica versus povidone-iodine: a randomised controlled study. Afr J Paediatr Surg 2015; 12 (04) 241-246
    PubMedSearch in Google Scholar
  • 25 Lawrence L, Gavens E, Reda B. et al. Exomphalos major: conservative management using Manuka honey dressings and an outreach surgical nursing team. J Pediatr Surg 2021; 56 (08) 1389-1394
    PubMedSearch in Google Scholar
  • 26 Mitul AR, Ferdous K. Initial conservative management of exomphalos major with gentian violet. J Neonatal Surg 2012; 1 (04) 51
    PubMedSearch in Google Scholar
  • 27 Burge DM, Glasson MJ. The conservative management of exomphalos major. Aust N Z J Surg 1986; 56 (05) 409-411
    PubMedSearch in Google Scholar
  • 28 Festen C, Severijnen RSVM, Staak FHJVD. Nonsurgical (conservative) treatment of giant omphalocele. A report of 10 cases. Clin Pediatr (Phila) 1987; 26 (01) 35-39
    PubMedSearch in Google Scholar
  • 29 Cosman BC, Schullinger JN, Bell JJ, Regan JA. Hypothyroidism caused by topical povidone-iodine in a newborn with omphalocele. J Pediatr Surg 1988; 23 (04) 356-358
    PubMedSearch in Google Scholar
  • 30 Kouame BD, Odehouri Koudou TH, Yaokreh JB. et al. Outcomes of conservative treatment of giant omphaloceles with dissodic 2% aqueous eosin: 15 years' experience. Afr J Paediatr Surg 2014; 11 (02) 170-173
    PubMedSearch in Google Scholar
  • 31 Ein SH, Langer JC. Delayed management of giant omphalocele using silver sulfadiazine cream: an 18-year experience. J Pediatr Surg 2012; 47 (03) 494-500
    PubMedSearch in Google Scholar
  • 32 Hong AR, Sigalet DL, Guttman FM, Laberge JM, Croitoru DP. Sequential sac ligation for giant omphalocele. J Pediatr Surg 1994; 29 (03) 413-415
    PubMedSearch in Google Scholar
  • 33 Kogut KA, Fiore NF. Nonoperative management of giant omphalocele leading to early fascial closure. J Pediatr Surg 2018; 53 (12) 2404-2408
    PubMedSearch in Google Scholar
  • 34 Mitanchez D, Walter-Nicolet E, Humblot A, Rousseau V, Revillon Y, Hubert P. Neonatal care in patients with giant ompholocele: arduous management but favorable outcomes. J Pediatr Surg 2010; 45 (08) 1727-1733
    PubMedSearch in Google Scholar
  • 35 Roux N, Jakubowicz D, Salomon L. et al. Early surgical management for giant omphalocele: results and prognostic factors. J Pediatr Surg 2018; 53 (10) 1908-1913
    PubMedSearch in Google Scholar
  • 36 Aljahdali AH, Al-Buainain HM, Skarsgard ED. Staged closure of a giant omphalocele with amnion preservation, modified technique. Saudi Med J 2017; 38 (04) 422-424
    PubMedSearch in Google Scholar
  • 37 Belloli G, Battaglino F, Musi L. Management of giant omphalocele by progressive external compression: case report. J Pediatr Surg 1996; 31 (12) 1719-1720
    PubMedSearch in Google Scholar
  • 38 Binet A, Gelas T, Jochault-Ritz S. et al. VAC® therapy a therapeutic alternative in giant omphalocele treatment: a multicenter study. J Plast Reconstr Aesthet Surg 2013; 66 (12) e373-e375
    PubMedSearch in Google Scholar
  • 39 Harjai MM, Bhargava P, Sharma A, Saxena A, Singh Y. Repair of a giant omphalocele by a modified technique. Pediatr Surg Int 2000; 16 (07) 519-521
    PubMedSearch in Google Scholar
  • 40 Hendrickson RJ, Partrick DA, Janik JS. Management of giant omphalocele in a premature low-birth-weight neonate utilizing a bedside sequential clamping technique without prosthesis. J Pediatr Surg 2003; 38 (10) E14-E16
    PubMedSearch in Google Scholar
  • 41 Pacilli M, Spitz L, Kiely EM, Curry J, Pierro A. Staged repair of giant omphalocele in the neonatal period. J Pediatr Surg 2005; 40 (05) 785-788
    PubMedSearch in Google Scholar
  • 42 Pelizzo G, Maso G, Dell'Oste C. et al. Giant omphaloceles with a small abdominal defect: prenatal diagnosis and neonatal management. Ultrasound Obstet Gynecol 2005; 26 (07) 786-788
    PubMedSearch in Google Scholar
  • 43 Sönmez K, Önal E, Karabulut R. et al. A strategy for treatment of giant omphalocele. World J Pediatr 2010; 6 (03) 274-277
    PubMedSearch in Google Scholar
  • 44 Jiang W, Zhang J, Lv X. et al. Use of small intestinal submucosal and acellular dermal matrix grafts in giant omphaloceles in neonates and a rabbit abdominal wall defect model. J Pediatr Surg 2016; 51 (03) 368-373
    PubMedSearch in Google Scholar
  • 45 Vargas-Mancilla J, Torrero-Serrato MA, Palacios-Rodríguez AJ, Rodríguez-de León GB, Montes-Rodríguez MI, Mendoza-Novelo B. Repair of a giant omphalocele in an infant with a pericardial implant crosslinked with oligourethane. Artif Organs 2018; 42 (08) 846-851
    PubMedSearch in Google Scholar
  • 46 Biard JM, Wilson RD, Johnson MP. et al. Prenatally diagnosed giant omphaloceles: short- and long-term outcomes. Prenat Diagn 2004; 24 (06) 434-439
    PubMedSearch in Google Scholar
  • 47 Huang X, Huang H, Liang Y. et al. Modified sequential sac ligation and staged closure technique for the management of giant omphalocele. J Pediatr Surg 2021; 56 (09) 1576-1582
    PubMedSearch in Google Scholar
  • 48 Lelj-Garolla B, Campbell L, Kanungo J, Yoshida N. Use of a neoprene binding to reduce giant omphaloceles followed by delayed closure. World J Pediatr Surg 2021; 4 (04) e000284
    PubMedSearch in Google Scholar
  • 49 Lee SL, Beyer TD, Kim SS. et al. Initial nonoperative management and delayed closure for treatment of giant omphaloceles. J Pediatr Surg 2006; 41 (11) 1846-1849
    PubMedSearch in Google Scholar
  • 50 Akinkuotu AC, Sheikh F, Olutoye OO. et al. Giant omphaloceles: surgical management and perinatal outcomes. J Surg Res 2015; 198 (02) 388-392
    PubMedSearch in Google Scholar
  • 51 Nolan HR, Wagner ML, Jenkins T, Lim FY. Outcomes in the giant omphalocele population: a single center comprehensive experience. J Pediatr Surg 2020; 55 (09) 1866-1871
    CrossrefPubMedSearch in Google Scholar
  • 52 Boglione M, Aleman S, Reusmann A, Rubio M, Marcelo B. Giant omphalocele: delayed closure using the San Martin technique following epithelialization of the membrane. J Pediatr Surg 2021; 56 (06) 1247-1251
    PubMedSearch in Google Scholar
  • 53 Pereira RM, Tatsuo ES, Simões e Silva AC. et al. New method of surgical delayed closure of giant omphaloceles: Lazaro da Silva's technique. J Pediatr Surg 2004; 39 (07) 1111-1115
    PubMedSearch in Google Scholar
  • 54 van Eijck FC, Hoogeveen YL, van Weel C, Rieu PNMA, Wijnen RMH. Minor and giant omphalocele: long-term outcomes and quality of life. J Pediatr Surg 2009; 44 (07) 1355-1359
    PubMedSearch in Google Scholar
  • 55 Kruit AS, Al-Ani SA, Jester I, Jester A. Multilayered flap technique: a method for delayed closure of giant omphalocele. Ann Plast Surg 2016; 76 (06) 680-683
    PubMedSearch in Google Scholar
  • 56 Foglia R, Kane A, Becker D, Asz-Sigall J, Mychaliska G. Management of giant omphalocele with rapid creation of abdominal domain. J Pediatr Surg 2006; 41 (04) 704-709 , discussion 704–709
    PubMedSearch in Google Scholar
  • 57 Adetayo OA, Aka AA, Ray AO. The use of intra-abdominal tissue expansion for the management of giant omphaloceles: review of literature and a case report. Ann Plast Surg 2012; 69 (01) 104-108
    PubMedSearch in Google Scholar
  • 58 Tenenbaum MJ, Foglia RP, Becker DB, Kane AA. Treatment of giant omphalocele with intraabdominal tissue expansion. Plast Reconstr Surg 2007; 120 (06) 1564-1567
    PubMedSearch in Google Scholar
  • 59 Martin AE, Khan A, Kim DS, Muratore CS, Luks FI. The use of intraabdominal tissue expanders as a primary strategy for closure of giant omphaloceles. J Pediatr Surg 2009; 44 (01) 178-182
    PubMedSearch in Google Scholar
  • 60 Rombaldi MC, Neto WFS, Holanda FC, Cavazzola LT, Fraga JC. Ventral hernia secondary to giant omphalocele in a child: combined approach of botulinum toxin and preoperative progressive pneumoperitoneum. Hernia 2020; 24 (06) 1397-1400
    PubMedSearch in Google Scholar
  • 61 Kapfer SA, Keshen TH. The use of human acellular dermis in the operative management of giant omphalocele. J Pediatr Surg 2006; 41 (01) 216-220
    CrossrefPubMedSearch in Google Scholar
  • 62 Saxena AK, van Tuil C. Delayed three-stage closure of giant omphalocele using pericard patch. Hernia 2008; 12 (02) 201-203
    PubMedSearch in Google Scholar
  • 63 Țarcă E, Al Namat D, Luca AC. et al. Omphalocele and cardiac abnormalities—the importance of the association. Diagnostics (Basel) 2023; 13 (08) 1413
    PubMedSearch in Google Scholar
  • 64 Raymond SL, Downard CD, St Peter SD. et al. Outcomes in omphalocele correlate with size of defect. J Pediatr Surg 2019; 54 (08) 1546-1550
    PubMedSearch in Google Scholar
  • 65 Raitio A, Tauriainen A, Syvänen J. et al. Omphalocele in Finland from 1993 to 2014: trends, prevalence, mortality, and associated malformations—a population-based study. Eur J Pediatr Surg 2021; 31 (02) 172-176
    PubMedSearch in Google Scholar
  • 66 Marshall J, Salemi JL, Tanner JP. et al; National Birth Defects Prevention Network. Prevalence, correlates, and outcomes of omphalocele in the United States, 1995-2005. Obstet Gynecol 2015; 126 (02) 284-293
    PubMedSearch in Google Scholar
  • 67 Fogelström A, Caldeman C, Oddsberg J, Löf Granström A, Mesas Burgos C. Omphalocele: national current birth prevalence and survival. Pediatr Surg Int 2021; 37 (11) 1515-1520
    PubMedSearch in Google Scholar
  • 68 Kong JY, Yeo KT, Abdel-Latif ME. et al; New South Wales and Australian Capital Territory Neonatal Intensive Care Units' Data Collection. Outcomes of infants with abdominal wall defects over 18years. J Pediatr Surg 2016; 51 (10) 1644-1649
    PubMedSearch in Google Scholar
  • 69 Schmedding A, Wittekind B, Salzmann-Manrique E, Schloesser R, Rolle U. Decentralized surgery of abdominal wall defects in Germany. Pediatr Surg Int 2020; 36 (05) 569-578
    CrossrefPubMedSearch in Google Scholar
  • 70 Elhedai H, Arul GS, Yong S. et al. Outcomes of patients with exomphalos and associated congenital heart diseases. Pediatr Surg Int 2022; 39 (01) 12
    PubMedSearch in Google Scholar
  • 71 Springett A, Draper ES, Rankin J. et al. Birth prevalence and survival of exomphalos in England and Wales: 2005 to 2011. Birth Defects Res A Clin Mol Teratol 2014; 100 (09) 721-725
    PubMedSearch in Google Scholar
  • 72 Ameh EA, Chirdan LB. Ruptured exomphalos and gastroschisis: a retrospective analysis of morbidity and mortality in Nigerian children. Pediatr Surg Int 2000; 16 (1-2): 23-25
    PubMedSearch in Google Scholar
  • 73 Abdur-Rahman LO, Abdulrasheed NA, Adeniran JO. Challenges and outcomes of management of anterior abdominal wall defects in a Nigerian tertiary hospital. Afr J Paediatr Surg 2011; 8 (02) 159-163
    PubMedSearch in Google Scholar
  • 74 Ogundoyin OO, Ajao AE. Changing trend in the management of omphalocoele in a tertiary hospital of a middle-income country. Afr J Paediatr Surg 2021; 18 (03) 143-147
    PubMedSearch in Google Scholar
  • 75 Baerg JE, Munoz AN. Long term complications and outcomes in omphalocele. Semin Pediatr Surg 2019; 28 (02) 118-121
    CrossrefPubMedSearch in Google Scholar
  • 76 van Eijck FC, Wijnen RMH, van Goor H. The incidence and morbidity of adhesions after treatment of neonates with gastroschisis and omphalocele: a 30-year review. J Pediatr Surg 2008; 43 (03) 479-483
    PubMedSearch in Google Scholar