Omphalocele and gastroschisis

The course and prognosis for gastroschisis and omphalocele depend on the severity of the defect and accompanying anomalies. Major malformations and chromosomal anomalies are common with omphalocele but rare in gastroschisis, with the exception of intestinal atresia. Advances in intensive care practices and postnatal resuscitation have decreased the mortality and morbidity associated with abdominal wall defects.

Gastroschisis

Reported survival rates in gastroschisis range from 91% to 100%, although the condition is associated with lengthy hospital stays.[36]Snyder CL. Outcome analysis for gastroschisis. J Pediatr Surg. 1999 Aug;34(8):1253-6. https://www.jpedsurg.org/article/S0022-3468(99)90162-8/pdf http://www.ncbi.nlm.nih.gov/pubmed/10466606?tool=bestpractice.com [5]Molik KA, Gingalewski CA, West KW, et al. Gastroschisis: a plea for risk categorization. J Pediatr Surg. 2001 Jan;36(1):51-5. http://www.ncbi.nlm.nih.gov/pubmed/11150437?tool=bestpractice.com Prenatal intestinal insult may contribute to small bowel atresia, bowel infarction, and intestinal motility dysfunction. If the insult to the intestine is severe, short gut syndrome may lead to the need for prolonged hyperalimentation and associated complications of total parenteral nutrition, including hepatitis. After closure, infants may develop intestinal obstruction from atresia, bowel perforations, or peritonitis. Additional operative intervention may prolong the hospital stay and delay opportunities to feed orally prior to discharge. The most important prognostic factor for morbidity is the presence of intestinal atresia; preterm delivery and very low birthweight are also associated with worse clinical outcomes.[37]Raymond SL, Hawkins RB, St Peter SD, et al. Predicting morbidity and mortality in neonates born with gastroschisis. J Surg Res. 2020 Jan;245:217-24. https://www.doi.org/10.1016/j.jss.2019.07.065 http://www.ncbi.nlm.nih.gov/pubmed/31421366?tool=bestpractice.com

Omphalocele

If omphalocele is not accompanied by additional structural abnormalities or chromosomal defects, the prognosis is good, with a low mortality.[38]Fogelström A, Caldeman C, Oddsberg J, et al. Omphalocele: national current birth prevalence and survival. Pediatr Surg Int. 2021 Nov;37(11):1515-20. https://www.doi.org/10.1007/s00383-021-04978-z http://www.ncbi.nlm.nih.gov/pubmed/34392395?tool=bestpractice.com However, associated congenital heart disease, genetic syndromes, and intestinal problems are common with omphalocele, and the condition is associated with anomalies in other organ systems in 30% to 80% of cases, with the severity of the anomalies dictating prognosis.[39]Poaty H, Pelluard F, Lucienne IP, et al. Omphalocele: a review of common genetic etiologies. Egypt J Med Hum Genet.  2019 Dec 27;20(37). https://jmhg.springeropen.com/articles/10.1186/s43042-019-0040-3 Most omphaloceles are associated with cardiac and chromosomal anomalies, and more than one half of the fetuses will die. Infections, surgical complications, and low birth weight are responsible for many deaths. Infections, immaturity, hernia rupture, and intestinal obstruction rates are high.