What condition is gastroschisis associated with an increased risk of?

Gastroschisis is Associated with an Increased Risk of Intestinal Atresia

Gastroschisis is most strongly associated with an increased risk of intestinal atresia (option b). 1, 2

Evidence for Association with Intestinal Atresia

• Approximately 10% of infants with gastroschisis have intestinal atresia as a complication, making it the most common significant associated anomaly 2

• The ESPGHAN/ESPEN/ESPR/CSPEN guidelines specifically identify intestinal atresia as a condition associated with gastroschisis, noting that this combination contributes to intestinal failure and disruption of bile acid enterohepatic circulation 1

• Gastroschisis complicated by intestinal atresia represents a more severe clinical presentation than either condition alone, with significantly worse outcomes 3

• The combination of gastroschisis and intestinal atresia is associated with:

  • Longer duration of mechanical ventilation 3
  • Extended hospital stays 3
  • Prolonged parenteral nutrition requirements 3
  • Higher rates of intestinal failure 3
  • More frequent surgical complications and reoperations 3

Pathophysiology of the Association

• The association between gastroschisis and intestinal atresia likely results from:

  • Compromised blood supply to the herniated bowel 4
  • Further impairment of blood flow by a tight abdominal wall defect 4
  • Postnatal increases in gravitational force on exposed bowel 4
  • Potential vascular compromise during fetal development 5

• The atretic segments most commonly involve the ileum and colon, with loss of the ileocecal region being a frequent complication 4, 5

Management Implications

• The presence of intestinal atresia significantly complicates the surgical management of gastroschisis 5

• Surgical options for the combined conditions include:

  • Stoma formation 5
  • Primary anastomosis (when feasible) 5
  • Deferred management with subsequent anastomosis 5

• Intestinal atresia may be missed during the initial gastroschisis repair, highlighting the importance of careful intraoperative assessment 5

Evidence Against Other Options

• Hepatomegaly (option a): Not specifically associated with gastroschisis in the provided evidence 1, 2

• Microcephaly (option c): Not identified as a complication or association of gastroschisis in any of the guidelines or research 1, 2

• Cardiac anomalies (option d): While gastroschisis patients may have other anomalies, cardiac defects are not characteristically associated with gastroschisis, unlike intestinal atresia which has a well-documented association 2

Clinical Implications

• Prenatal ultrasound should carefully evaluate for signs of intestinal atresia in fetuses with gastroschisis 2

• Neonates with both conditions require specialized care and often need referral to experienced pediatric intestinal failure rehabilitation/transplantation centers 1

• The combination of gastroschisis and intestinal atresia significantly increases morbidity compared to either condition alone, requiring more intensive management and follow-up 3