Management of the Infant With Gastroschisis: A Comprehensive Review of the Literature

Tracey Williams, MSN, RN, NNP, Rachel Butler, MSN, RN, NNP, Tara Sundem, MSN, RN, NNP

Disclosures

NAINR. 2003;3(2) 

In This Article

Outcomes

Based on the available literature of short- and long-term outcomes of infants with gastroschisis, the overall survival rate is high, and the majority of infants experienced no significant sequelae impacting their perceived quality of life. Interpretation of this data must be made in light of variables such as the decade of treatment, the inclusion of infants with other abdominal wall disorders that have higher rates of associated anomalies, and global differences in management.

Recently, a number of prominent pediatric surgeons called for classifying infants with gastroschisis into two risk categories based on differences in their clinical courses, length of stay, morbidity, and mortality.[1] Infants were classified as low risk if they had a "simple" defect. Infants classified as high risk included those infants with atresias, stenosis, volvulus, or other "complex" gastrointestinal problems. According to a retrospective chart review of over 100 infants in a 5-year period, infants classified as high risk, compared with those classified as low risk, were younger and smaller (mean gestational age 34 weeks; mean birth weight 2.0 kg); had a lower rate of primary closure (65% vs. 71%); stayed on mechanical ventilation longer (22 vs. 7 days); and had a prolonged hospitalization (mean length of stay 85 vs. 26 days). In addition to experiencing more complications, infants in the high-risk category have more severe complications including short bowel syndrome, pneumatosis, pneumonia, and bowel obstruction.[1] The survival rate of low-risk infants was 100% compared with 72% for the high-risk infants.[1]

Published overall survival rates for infants with gastroschisis in the United States range from 83% to 97%.[12,49,53] The reported mean gestational age at the time of birth is 36 weeks gestation (range, 35.9 to 36.6 weeks) with a birth weight of 2.5 kg.[12,49] In a Finnish study of 57 adults ranging in age from 17 to 48 years of age who were born with an abdominal wall defect, 88% described their health as good.[54] The majority of subjects saw no significant difference in their quality of life compared with the general population. The most common long-term complications noted were problems related to the abdominal scar (37% of subjects) and functional gastrointestinal problems such as GER (51% of subjects). According to an Austrian study of 19 children born between 1985 and 1996, 10 of 11 children with gastroschisis were considered developmentally normal.[55] An interesting side note in this study was the majority of mothers had a fear of giving birth to another infant with a birth defect and subsequently decided against having further children. The mothers' decisions were also supported by the fathers.[55] In a similar study of 23 individuals ranging in age from 12 to 23 years conducted in England, 96% were growing normally and considered themselves to be in good health.[56] Of the subjects born with gastroschisis, 35% underwent additional surgeries for small bowel adhesions and scar complications. Over one-half (57%) of the subjects stated the lack of an umbilicus caused significant distress during childhood.[56] In a Swedish study of 61 patients born with an abdominal wall defect who were followed for 10 to 20 years after their hospitalization, 80% of the subjects described their quality of life as good.[53] Twenty-three percent of these individuals underwent additional surgery to correct abdominal wall hernias and/or sequelae of atresias before school age.[53] Late surgical problems noted in another Swedish study of 55 patients with abdominal wall defects included abdominal wall hernias, intestinal obstruction, and revision of an intestinal stoma. Follow-up occurred at a mean of 5 years after the initial hospitalization, and there was no significant difference in outcomes between those with a primary or staged repair.[57]

Financial outcomes were addressed in a University of California at San Francisco study.[58] Hospital costs and clinical outcomes associated with gastroschisis care in a population of 69 infants treated between 1990 to 2000 provide interesting information.[58] The survival rate was 96%. The average length of stay was 47 days, and it took 33 days to achieve full enteral feedings. Factors that negatively impacted the cost of hospitalization were the number of surgical procedures, days on mechanical ventilation, length of stay, and male gender.

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