Primary versus Staged Closure of Exomphalos Major: Cardiac Anomalies Do Not Affect Outcome

Abstract

Aim The objective of the study is to describe management of exomphalos major and investigate the effect of congenital cardiac anomalies.

Methods A single-center retrospective review (with audit approval) was performed of neonates with exomphalos major (fascial defect ≥ 5cm ± liver herniation) between 2004 and 2014.

Demographic and operative data were collected and outcomes compared between infants who had primary or staged closure. Data, median (range), were analyzed appropriately.

Results A total of 22 patients were included, 20 with liver herniation and 1 with pentalogy of Cantrell. Gestational age was 38 (30–40) weeks, birth weight 2.7 (1.4–4.6) kg, and 13 (60%) were male. Two were managed conservatively due to severe comorbidities, 5 underwent primary closure, and 15 had application of Prolene (Ethicon Inc) mesh silo and serial reduction. Five died, including two managed conservatively, none primarily of the exomphalos. Survivors were followed up for 38 months (2–71). Cardiac anomalies were present in 20 (91%) patients: 8 had minor and 12 major anomalies. Twelve (55%) patients had other anomalies. Primary closure was associated with shorter length of stay (13 vs. 85 days, p = 0.02), but infants had similar lengths of intensive care stay, duration of parenteral feeds, and time to full feeds. Infants with cardiac anomalies had shorter times to full closure (28 vs. 62 days, p = 0.03), but other outcomes were similar.

Conclusion Infants whose defect can be closed primarily have a shorter length of stay, but other outcomes are similar. Infants with more significant abdominovisceral disproportion are managed with staged closure; the presence of major cardiac anomalies does not affect surgical outcome.

Keywords

exomphalos major - surgical outcome - abdominal wall defect - congenital cardiac defect - silo

• References

• 1 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
• 2 Christison-Lagay ER, Kelleher CM, Langer JC. Neonatal abdominal wall defects. Semin Fetal Neonatal Med 2011; 16 (03) 164-172
• 3 Gilbert WM, Nicolaides KH. Fetal omphalocele: associated malformations and chromosomal defects. Obstet Gynecol 1987; 70 (04) 633-635
• 4 Yazbeck S, Ndoye M, Khan AH. Omphalocele: a 25-year experience. J Pediatr Surg 1986; 21 (09) 761-763
• 5 Rijhwani A, Davenport M, Dawrant M. , et al. Definitive surgical management of antenatally diagnosed exomphalos. J Pediatr Surg 2005; 40 (03) 516-522
• 6 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
• 7 Tsakayannis DE, Zurakowski D, Lillehei CW. Respiratory insufficiency at birth: a predictor of mortality for infants with omphalocele. J Pediatr Surg 1996; 31 (08) 1088-1090 , discussion 1090–1091
• 8 Ooms N, Matthyssens LE, Draaisma JM. , et al. Laparoscopic treatment of intestinal malrotation in children. Eur J Pediatr Surg 2016; 26 (04) 376-381
• 9 Draus Jr JM, Foley DS, Bond SJ. Laparoscopic Ladd procedure: a minimally invasive approach to malrotation without midgut volvulus. Am Surg 2007; 73 (07) 693-696
• 10 Graziano K, Islam S, Dasgupta R. , et al. Asymptomatic malrotation: diagnosis and surgical management: an American Pediatric Surgical Association outcomes and evidence based practice committee systematic review. J Pediatr Surg 2015; 50 (10) 1783-1790
• 11 van Eijck FC, Aronson DA, Hoogeveen YL, Wijnen RM. Past and current surgical treatment of giant omphalocele: outcome of a questionnaire sent to authors. J Pediatr Surg 2011; 46 (03) 482-488
• 12 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
• 13 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
• 14 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
• 15 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
• 16 Charlesworth P, Ervine E, McCullagh M. Exomphalos major: the northern Ireland experience. Pediatr Surg Int 2009; 25 (01) 77-81
• 17 Jackson HJ, Sutherland RM. Effect of povidone-iodine on neonatal thyroid function. Lancet 1981; 2 (8253): 992-992
• 18 Kothari M, Pease PWB. Closure of the ventral hernia in the management of giant exomphalos: a word of caution. Pediatr Surg Int 2005; 21 (02) 106-109
• 19 Gross RE. A new method for surgical treatment of large omphaloceles. Surgery 1948; 24 (02) 277-292
• 20 Schuster SR. A new method for the staged repair of large omphaloceles. Surg Gynecol Obstet 1967; 125 (04) 837-850
• 21 Kumar HR, Jester AL, Ladd AP. Impact of omphalocele size on associated conditions. J Pediatr Surg 2008; 43 (12) 2216-2219
• 22 Partridge EA, Hanna BD, Panitch HB. , et al. Pulmonary hypertension in giant omphalocele infants. J Pediatr Surg 2014; 49 (12) 1767-1770
• 23 Akinkuotu AC, Sheikh F, Olutoye OO. , et al. Giant omphaloceles: surgical management and perinatal outcomes. J Surg Res 2015; 198 (02) 388-392
• 24 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
• 25 Khalil A, Arnaoutoglou C, Pacilli M, Szabo A, David AL, Pandya P. Outcome of fetal exomphalos diagnosed at 11–14 weeks of gestation. Ultrasound Obstet Gynecol 2012; 39 (04) 401-406
• 26 Lakasing L, Cicero S, Davenport M, Patel S, Nicolaides KH. Current outcome of antenatally diagnosed exomphalos: an 11 year review. J Pediatr Surg 2006; 41 (08) 1403-1406