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Gastroschisis and Exomphalos

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Abstract

The earliest description(s) of exomphalos date from antiquity though credit is linked with the French surgeon Ambrose Pare for providing the first accurate account of the malformation and its grave prognosis in the sixteenth century. Exomphalos was considered universally fatal until success with surgical treatment was published in the early 1800s. Scarpa (1814) later emphasized a spectrum of malformation severity. In 1899 Ahfield introduced the concept of conservative management by applying alcohol dressings to the exposed sac. This method was modified by Grob some 60 years later with the introduction of mercurochrome. Creation of a ‘skin silo’ by mobilising abdominal wall skin to cover the intact sac in large exomphalos lesions was advocated by Olshausen (1877), Williams (1930) and later Robert Gross at Children’s Hospital Boston in 1948.

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References

  1. Irving IM. Umbilical abnormalities. In: Lister J, Irving IM, editors. Neonatal Surgery 3rd edn. London: Butterworths; 1990. p. 376–402.

    Google Scholar 

  2. Grob M. Conservative treatment of exomphalos. Arch Dis Child. 1963;54:441–4.

    Google Scholar 

  3. Williams C. Congenital defects of the anterior abdominal wall. Surg Clin North Am. 1930;10:805–9.

    Google Scholar 

  4. Gross RE. A new method for surgical treatment of large omphalocoeles. Surgery. 1948;24:277–92.

    CAS  Google Scholar 

  5. Moore TC, Stokes GE. Gastroschisis. Surgery. 1953;33:112–20.

    CAS  Google Scholar 

  6. Watkins DE. Gastroschisis. Virginia Med Mon. 1943;70:42–4.

    Google Scholar 

  7. Schuster SR. A new method for the staged repair of large omphalocoeles. Surg Gynecol Obstet. 1967;125:837–50.

    CAS  Google Scholar 

  8. Dudrick SJ, Wilmore DW, Vars HM, et al. Can intravenous feeding as the sole means of nutrition support growth in the child and restore weight loss in the adult ? Ann Surg. 1969;169:974–84.

    Article  CAS  Google Scholar 

  9. Redford DH, McNay MB, Whittle MJ. Gastroschisis and exomphalos: precise diagnosis by mid-pregnancy ultrasound. Br J Obstet Gynaecol. 1985;92:54–9.

    Article  CAS  Google Scholar 

  10. Nakayama DK, Harrison MR, Gross BH, et al. Management of the fetus with an abdominal wall defect. J Pediatr Surg. 1984;19:408–13.

    Article  CAS  Google Scholar 

  11. Shermeta DW, Haller JA Jr. A new preformed transparent silo for the management of gastroschisis. J Pediatr Surg. 1975;10:973–5.

    Article  CAS  Google Scholar 

  12. Minkes RK, Langer JC, Mazziota MV, et al. Routine insertion of a silastic spring loaded silo for infants with gastroschisis. J Pediatr Surg. 2000;35:843–6.

    Article  CAS  Google Scholar 

  13. Bax NM, van der Zee D, Pull ter Gunne AJ, et al. Treatment of giant omphalocoele by enlargement of the abdominal cavity with a tissue expander. J Pediatr Surg. 1993;28:1181–4.

    Article  CAS  Google Scholar 

  14. Clifton MS, Heiss KF, Keating JJ, et al. Use of tissue expanders in the repair of complex abdominal wall defects. J Pediatr Surg. 2011;46:372–7.

    Article  Google Scholar 

  15. Almond SL, Goyal A, Jesudason EC, et al. Novel use of skin substitute as rescue therapy in complicated giant exomphalos. J Pediatr Surg. 2006;41:e1–2.

    Article  Google Scholar 

  16. Wilson RD, Johnson MP. Congenital abdominal wall defects: an update. Fetal Diagn Ther. 2004;19:385–98.

    Article  Google Scholar 

  17. Japan Association of Obstetricians and Gynecologists. Annual reports of congenital malformations. 1997–2006.

    Google Scholar 

  18. Kilby MD. The incidence of gastroschisis. BMJ. 2006;332:250–1.

    Article  Google Scholar 

  19. Loanne M, Dolk H, Bradbury I. EUROCAT Working Group. Increasing prevalence of gastroschisis in Europe 1980–2002: a phenomenon restricted to younger mothers ? Paediatr Perinat Epidemiol. 2007;143A:660–71.

    Google Scholar 

  20. Loanne M, Dolk H, Kelly A, et al. Paper 4: EUROCAT statistical monitoring: identification and investigation of ten year trends of congenital anomalies in Europe. Birth Defects Res A Clin Mol Teratol. 2011;91:S31–43.

    Article  Google Scholar 

  21. Torfs CP, Katz EA, Bateson TF, et al. Maternal medications and environmental exposures as risk factors for gastroschisis. Teratology. 1996;54:84–92.

    Article  CAS  Google Scholar 

  22. Draper ES, Rankin J, Tonks AM, et al. Recreational drug use: a major risk factor for gastroschisis ? Am J Epidemiol. 2008;167:485–91.

    Article  Google Scholar 

  23. Root ED, Meyer RE, Emch ME. Evidence of localized clustering of gastroschisis. Births in North Carolina 1999–2004. Soc Sci Med. 2009;68:1361–7.

    Article  Google Scholar 

  24. Gamba P, Midrio P. Abdominal wall defects: prenatal diagnosis, newborn management and long-term outcomes. Semin Pediatr Surg. 2014;23:283–90.

    Article  Google Scholar 

  25. Mastroiacovo P, Lisi A, Castilla EE, et al. Gastroschisis and associated defects: an international study. Am J Med Genet A. 2007;143A:660–71.

    Article  Google Scholar 

  26. Gray SW, Skandalakis JE. Embryology for surgeons. Philadelphia: Saunders; 1972.

    Google Scholar 

  27. Kluth D, Jaeschke-Melli S, Fiegel H. The embryology of gut rotation. Semin Pediatr Surg. 2003;12:275–9.

    Article  CAS  Google Scholar 

  28. Kamata S, Usui N, Sawai T, et al. Prenatal detection of pulmonary hypoplasia in giant omphalocoele. Pediatr Surg Int. 2008;24:107–11.

    Article  Google Scholar 

  29. Danzer E, Victoria T, Bebbington MW, et al. Fetal MRI-calculated total lung volumes in the prediction of short-term outcome in giant omphalocoele: preliminary findings. Fetal Diagn Ther. 2012;31:248–53.

    Article  Google Scholar 

  30. Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet. 1958;107:602–14.

    CAS  Google Scholar 

  31. Devries PA. The pathogenesis of gastroschisis and omphalocoele. J Pediatr Surg. 1980;15:245–51.

    Article  CAS  Google Scholar 

  32. Stevenson RE, Rogers RC, Chandler JC, et al. Escape of the yolk sac: a hypothesis to explain the embryogenesis of gastroschisis. Clin Genet. 2009;75:326–33.

    Article  CAS  Google Scholar 

  33. Palomaki GE, Hill LE, Knight GJ, et al. Second trimester maternal serum alpha-fetoprotein levels in pregnancies associated with gastroschisis and omphalocoele. Obstet Gynecol. 1988;71:906–9.

    CAS  Google Scholar 

  34. Bond SJ, Harrison MR, Filly RA, et al. Severity of intestinal damage in gastroschisis: correlation with prenatal sonographic findings. J Pediatr Surg. 1988;23:520–5.

    Article  CAS  Google Scholar 

  35. Midrio P, Stefanutti G, Mussap M, et al. Amnioexchange for fetuses with gastroschisis: is it effective? J Pediatr Surg. 2007;42:777–82.

    Article  Google Scholar 

  36. Tower C, Ong SSC, Ewer AK, et al. Prognosis in isolated gastroschisis with bowel dilation: a systematic review. Arch Dis Child Fetal Neonatal Ed. 2009;94:F268–74.

    Article  CAS  Google Scholar 

  37. Kuleva M, Khen-Dunlop N, Dumez Y, et al. Is complex gastroschisis predictable by prenatal ultrasound? BJOG. 2012;119:102–9.

    Article  CAS  Google Scholar 

  38. Lato K, Poellmann M, Knippel AJ, et al. Fetal gastroschisis: a comparison of second vs. third-trimester bowel dilatation for predicting bowel atresia and neonatal outcome. Ultraschall Med. 2013;34:157–61.

    Article  CAS  Google Scholar 

  39. Segal SY, Marder SJ, Parry S, et al. Fetal abdominal wall defects and mode of delivery: a systematic review. Obstet Gynecol. 2001;98:867–73.

    Google Scholar 

  40. Grant NH, Dolring J, Thornton JG. Elective preterm birth for fetal gastroschisis. Cochrane Database Syst Rev. 2013;6:CD009394.

    Google Scholar 

  41. Skarsgard ED. Management of gastroschisis. Curr Opin Pediatr. 2016;28:363–9.

    Article  CAS  Google Scholar 

  42. Beckwith JB, Wang CL, Donnell GN, et al. Hyperplastic fetal visceromegaly with macroglossia, omphalocoele, cytomegaly of adrenal fetal cortex, postnatal somatic gigantism and other abnormalities: newly recognized syndrome. Proc Am Pediat Soc, Seattle, WA, June 16–18, 1964 (Abstr 41).

    Google Scholar 

  43. Beasley SW, Jones PG. Use of mercurochrome in the management of the large exomphalos. Aust Paediatr J. 1986;22:61–3.

    CAS  Google Scholar 

  44. Mullins ME, Horowitz BZ. Iatrogenic neonatal mercury poisoning from mercurochrome treatment of a large omphalocoele. Clin Pediatr (Phila). 1999;38:111–2.

    Article  CAS  Google Scholar 

  45. Whitehouse JS, Gourlay DM, Masonbrink AR, et al. Non operative management of giant omphalocoele with topical povidone-iodine and its effects on thyroid function. J Pediatr Surg. 2010;45:1192–7.

    Article  Google Scholar 

  46. Ein SH, Langer JC. Delayed management of giant omphalocoele using silver sulfadiazine cream: an 18 year experience. J Pediatr Surg. 2012;47:494–500.

    Article  Google Scholar 

  47. Khalil BA, Baath ME, Baillie CT, et al. Infections in gastroschisis; organisms and factors. Pediatr Surg Int. 2008;24:1031–5.

    Article  CAS  Google Scholar 

  48. Hoehner JC, Ein SH, Kim PC. Management of gastroschisis with concomitant jejuno-ileal atresia. J Pediatr Surg. 1998;33:885–8.

    Article  CAS  Google Scholar 

  49. Fleet MS, de la Hunt MN. Intestinal atresia with gastroschisis: a selective approach to management. J Pediatr Surg. 2000;35:1323–5.

    Article  CAS  Google Scholar 

  50. Bianchi A, Dickson AP. Elective delayed reduction and no anesthesia: ‘minimal intervention management’ for gastroschisis. J Pediatr Surg. 1998;33:1338–40.

    Article  CAS  Google Scholar 

  51. Dolgin SE, Midulla P, Shlasko E. Unsatisfactory experience with ‘minimal intervention management’ for gastroschisis. J Pediatr Surg. 2000;35:1437–9.

    Article  CAS  Google Scholar 

  52. Kunz SN, Tieder JS, Whitlock K, et al. Primary fascial closure versus staged closure with silo in patients with gastroschisis; a meta-analysis. J Pediatr Surg. 2013;48:845–57.

    Article  Google Scholar 

  53. Shetty S, Kennea N, Desai P, et al. Length of stay and cost analysis of neonates undergoing surgery at a tertiary neonatal unit in England. Ann R Coll Surg Engl. 2016;98:56–60.

    Article  CAS  Google Scholar 

  54. Coletta R, Khalil BA, Morabito A. Short bowel syndrome in children: surgical and medical perspectives. Semin Pediatr Surg. 2014;23:291–7.

    Article  Google Scholar 

  55. Friedmacher F, Hock A, Castellani C, et al. Gastroschisis-related complications requiring further surgical interventions. Pediatr Surg Int. 2014;30:615–20.

    Article  Google Scholar 

  56. Bergholz R, Boettcher M, Reinshagen K, et al. Complex gastroschisis is a different entity to simple gastroschisis affecting morbidity and mortality—a systematic review and meta-analysis. J Pediatr Surg. 2014;49:1527–32.

    Article  Google Scholar 

  57. van Manen M, Hendson L, Wiley M, et al. Early childhood outcomes of infants born with gastroschisis. J Pediatr Surg. 2013;48:1682–7.

    Article  Google Scholar 

  58. Gorra AS, Needelman H, Azarow KS, et al. Long-term neurodevelopmental outcomes in children born with gastroschisis; the tiebreaker. J Pediatr Surg. 2012;47:125–9.

    Article  Google Scholar 

  59. Yardley IE, Bostock E, Jones MO, et al. Congenital abdominal wall defects and testicular maldescent: a 10 year single-center experience. J Pediatr Surg. 2012;47:118–22.

    Article  Google Scholar 

  60. Harris EL, Minutillo C, Hart S, et al. The long-term physical consequences of gastroschisis. J Pediatr Surg. 2014;49:1466–70.

    Article  Google Scholar 

  61. Davis BW, Stringer MD. The survivors of gastroschisis. Arch Dis Child. 1997;77:158–60.

    Article  CAS  Google Scholar 

  62. Koivusalo A, Lindahl H, Rintala RJ. Morbidity and quality of life in adult patients with a congenital abdominal wall defect: a questionnaire survey. J Pediatr Surg. 2002;37:1594–601.

    Article  CAS  Google Scholar 

  63. Van Eijick FC, Hoogeveen YL, van Weel C, et al. Minor and giant omphalocoele: long-term outcomes and quality of life. J Pediatr Surg. 2009;44:1355–9.

    Article  Google Scholar 

  64. Danzer E, Hedrick HL, Rintoul NE, et al. Assessment of early pulmonary function 65. Abnormalities in giant omphalocoele survivors. J Pediatr Surg. 2012;47:1811–20.

    Article  Google Scholar 

  65. Danzer E, Gerdes M, D’Agostino JA, et al. Prospective interdisciplinary follow up of children with prenatally diagnosed giant omphalocoele: short-term neurodevelopmental outcome. J Pediatr Surg. 2010;45:718–23.

    Article  Google Scholar 

  66. Partridge EA, Hanna BD, Panitch HB, et al. Pulmonary hypertension in giant omphalocoele infants. J Pediatr Surg. 2014;49:1767–70.

    Article  Google Scholar 

  67. Pernanteau WH, Tharakan SJ, Partridge E, et al. Systemic hypertension in giant omphalocoele: an underappreciated association. J Pediatr Surg. 2015;50:1477–80.

    Article  Google Scholar 

  68. Danzer E, Gerdes M, D’Agostino JA, et al. Patient characteristics are important determinants of neurodevelopmental outcome during infancy in giant omphalocoele. Early Hum Dev. 2015;91:187–93.

    Article  Google Scholar 

  69. Partridge EA, Peranteau WH, Flake A, et al. Frequency and complications of inguinal hernia repair in giant omphalocoele. J Pediatr Surg. 2015;50:1673–5.

    Article  Google Scholar 

  70. Beaudoin S, Kieffer G, Sapin E, et al. Gastroesophageal reflux in neonates with congenital abdominal wall defect. Eur J Pediatr Surg. 1995;5:323–6.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, SIDRA, Doha, Qatar

    Basem A. Khalil MPH, PhD, FRCS(Paed)

  2. Department of Paediatric Surgery, Institute of Translational Medicine, Alder Hey Children’s Hospital NHS Foundation Trust, University of Liverpool, Liverpool, UK

    Paul D. Losty MD, FRCS(Paed), FEBPS

Authors
  1. Basem A. Khalil MPH, PhD, FRCS(Paed)
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  2. Paul D. Losty MD, FRCS(Paed), FEBPS
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Corresponding author

Correspondence to Paul D. Losty MD, FRCS(Paed), FEBPS .

Editor information

Editors and Affiliations

  1. Division of Child Health, Alder Hey Children’s NHS Foundation Trust, Liverpool, United Kingdom

    Paul D. Losty

  2. University of Pennsylvania , Philadelphia, Pennsylvania, USA

    Alan W. Flake

  3. Childrens Hospital, University of Helsinki, Helsinki, Finland

    Risto J. Rintala

  4. Royal Children’s Hospital, University of Melbourne, Parkville, Victoria, Australia

    John M. Hutson

  5. Department of Surgery, Meiji University of Integrative Medicine, Kyoto, Japan

    Naomi lwai

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Khalil, B.A., Losty, P.D. (2018). Gastroschisis and Exomphalos. In: Losty, P., Flake, A., Rintala, R., Hutson, J., lwai, N. (eds) Rickham's Neonatal Surgery. Springer, London. https://doi.org/10.1007/978-1-4471-4721-3_46

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  • DOI: https://doi.org/10.1007/978-1-4471-4721-3_46

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