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Joseph Junewick, MD FACR
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Giant Gastroschisis

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 4 years ago
Updated: over 4 years ago
Tags: PEDS
Modality/Study Types: CR
Activities:
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History

Newborn female.


Case Images


Diagnosis

Giant Gastroschisis

Findings

CR – Abdominal wall defect with herniation of bowel (including stomach) and liver.

Discussion

Gastroschisis is a full-thickness paraumbilical defect in the abdominal wall. The cause is unclear but involves defective formation or disruption of the abdominal wall. This allows abdominal contents, most commonly bowel, to herniate through. Gastroschisis is strongly associated with young maternal age. Additional anomalies are reported in 14-20%, and 1.2% had chromosomal abnormalities.

The appearance on US is uncovered viscera emanating from an abdominal wall defect, almost always on the right, to a normally inserted umbilical cord. The defect is often small, allowing only intestine to herniate through, which can give a “cauliflower” appearance. With rare large abdominal wall defects (>4cm), other abdominal organs can be involved, including the liver, stomach, spleen and genitourinary system. The liver may be involved in 6% of cases. It’s very unusual for all abdominal organs.

Complications include bowel atresia, stenosis, ischemia, or perforation from chemical irration or volvulus. Bowel atresia occurs in 10-30% and increases perinatal morbidity. Bowel dilatation (>10mm) on prenatal US is a common sign of bowel complication. Intrauterine growth restriction (50% of cases) is predictive of adverse neonatal outcome, but can be difficult to monitor as the abdominal circumference is often inaccurate. Large defects and multiorgan hernias are associated with increased mortality. The mortality rate with liver involvement is 57%, compared to bowel only which is 3%.

After delivery the herniated viscera is wrapped with saline dressings and covered with plastic wrap, and the baby is placed right side down to avoid pinching the blood supply. Uncomplicated cases are reduced over 1-3 days with a silo, and complicated cases are initially treated surgically with subsequent placement of a silo. For very large defects, surgery may be necessary and abdominal wall closure can be challenging often requiring biological patches. The major postnatal complication is poor mucosal function and hypoperistalsis.

Reference

McClellan EB, Shew SB, Lee SS, et al. Liver herniation in gastroschisis: incidence and prognosis. J Pediatr Surg (2011); 46(11):2115-8.

Emanuel PG, Garcia GI, Angtuaco TL. Prenatal detection of anterior abdominal wall defects with US. Radiographics (1995); 15(3):517-30.

Obstetric Imaging. Gastroschisis. Copel JA, D’Alton ME, Gratacos E, et al. Philadelphia: Saunders, 2012, 83-90.

Contributor

Ryan Konwinski, MD



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