Examophalos (Omphalocoele)

  • Mohamed Fahmy


Ventral body wall defects comprise a group of congenital malformations that includes congenital umbilical cord hernia, gastroschisis and omphalocele, which are relatively common, and ectopia cordis, bladder exstrophy and cloacal exstrophy, which are relatively rare, along with another extremely rare defect which was only present as a case report in literature, such as body stalk anomalies, vesico-intestinal fissure, sternal defect and OEIS syndrome.

Recently, there is an increase in the occurrence of gastroschisis; cases with omphalocele had more syndromic and nonsyndromic anomalies, more chromosomal anomalies, a higher mortality rate and more common with an older mothers. The natural history of omphalocele and gastroschisis covaries with race, black infants with gastroschisis have worse survival outcomes, while those with omphalocele have better chances of survival than their White or Hispanic counterparts. Gastroschisis and omphalocele are the two most common congenital abdominal wall defects; both are frequently detected prenatally. Prognosis for gastroschisis is primarily determined by the degree of bowel injury, whereas prognosis for omphalocele is related to the number and severity of associated anomalies. The surgical management of both conditions consists of closure of the abdominal wall defect while minimizing the risk of injury to the abdominal viscera either through direct trauma or due to increased intra-abdominal pressure. Options include primary closure or a variety of staged approaches. Long-term outcome is favourable in most cases; however, significant associated anomalies (in the case of omphalocele) or intestinal dysfunction (in the case of gastroschisis) may result in morbidity and mortality.

Cases of congenital hernia of the cord are usually unrecognizable or misdiagnosed as an examophalos minor, so this entity discussed separately with special emphasis about the proper diagnosis of the classical cases and their variants.


Omphalocoele Beckwith–Wiedemann syndrome Polyhydramnios Pentalogy of Cantrell Vesico-intestinal Fissure Pentalogy of Cantrell and silo 


  1. 1.
    Pare A. The works of that famous Chirurgeon. London: Th. Cotes and R. Young; 1634. p. 59. Book 24.Google Scholar
  2. 2.
    Hey W. Practical observations in surgery. London: Cadell and Davies; 1803. p. 226.Google Scholar
  3. 3.
    Hamilton J. In Cooper, A. (1807). The anatomy and surgical treatment of Crural and umbilical hernia, part 2. London: Longman; 1806. p. 56.Google Scholar
  4. 4.
    Scarpa A. Traite pratique des Hernies. Paris: Gabon; 1812.Google Scholar
  5. 5.
    Ahifeld F. Der Alkohol bei der Behandlung inoperabeler Bauchbriiche. Mschr Geburtsh Gynak. 1899;10:124.Google Scholar
  6. 6.
    Dillon PW, Cilley RE. Newborn surgical emergencies. Gastrointestinal anomalies, abdominal wall defects. Pediatr Clin N Am. 1993;40:1289–314.CrossRefGoogle Scholar
  7. 7.
    McNair C, Hawes J, Urquhart H. Caring for the newborn with an omphalocoele. Neonatal Netw. 2006;25(5):319–32. doi: 10.1891/0730- 0832.25.5.319.CrossRefPubMedGoogle Scholar
  8. 8.
    Quemelo PRV, et al. Teratogenic effect of retinoic acid in swiss mice. Acta Cir Bras. 2007;22(6):451.CrossRefGoogle Scholar
  9. 9.
    Singh S, Madaree A. Omphalocoeles: A decade in review. S Afr J Child Health. 2016;10(4):211–4. doi: 10.7196/SAJCH.2016.v10i4.1149.CrossRefGoogle Scholar
  10. 10.
    Salihu HM, et al. Omphalocele and Gastroschisis: black-white disparity in infant survival. Birth Defects Res A. 2004;70:586–91.CrossRefGoogle Scholar
  11. 11.
    Van Eijck FC, Hoogeveen YL, van Weel C, Rieu PN, Wijnen RM. Minor and giant omphalocele: long-term outcomes and quality of life. J Pediatr Surg. 2009;44:1355–9.CrossRefGoogle Scholar
  12. 12.
    Hamid R, Mufti G, Wani SA, Ali I, Bhat NA, et al. Importance of the early management of omphalocele minor. J Neonatal Biol. 2015;4:169. doi: 10.4172/2167-0897.1000169.CrossRefGoogle Scholar
  13. 13.
    Mayer T, et al. Gastroschisis and omphalocele, an eight-year review. Ann Surg. 1980;192(6):783–7.CrossRefGoogle Scholar
  14. 14.
    Schwalbe E. Die Morphologie der Missbildungen des Menschen und der Tiere. Jena: Fischer; 1909.Google Scholar
  15. 15.
    Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 1958;107(5):602–14.PubMedGoogle Scholar
  16. 16.
    Groves R, Sunderajan L, Khan AR, et al. Congenital anomalies are commonly associated with exomphalos minor. J Pediatr Surg. 41(2):358–61. doi: 10.1016/j.jpedsurg.2005.11.013.CrossRefPubMedGoogle Scholar
  17. 17.
    How HY, Harris BJ, Pietrantoni M, Evans JC, Dutton S, Khoury J, et al. Is vaginal delivery preferable to elective cesarean delivery in fetuses with a known ventral wall defect? Am J Obstet Gynecol. 2000;182(6):1527–34. doi: 10.1067/mob.2000.106852.CrossRefPubMedGoogle Scholar
  18. 18.
    Hamid R, Mufti G, Wani SA, Ali I, Bhat NA, et al. Importance of the early Management of Omphalocele Minor. J Neonatal Biol. 2015;4:169. doi: 10.4172/2167-0897.1000169.CrossRefGoogle Scholar
  19. 19.
    Cohen-Overbeek TE, Tong WH, Hatzmann TR, et al. Omphalocele: comparison of outcome following prenatal or postnatal diagnosis. Ultrasound Obstet Gynecol. 2010;36(6):687–92. doi: 10.1002/uog.7698.CrossRefPubMedGoogle Scholar
  20. 20.
    Wakhlu A, Wakhlu AK. The management of exomphalos. J Pediatr Surg. 2000;35:73–6.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  • Mohamed Fahmy
    • 1
  1. 1.Pediatric SurgeryAl Azher University Pediatric SurgeryCairoEgypt

Personalised recommendations