Gastroschisis was first described in 1547 by Conrad Wolffhart followed by a report by J. Calder in 1733. The term wasn’t coined until 1894, when Cesare Taruffi, an Italian pathologist, utilized the term in his congenital malformations book.

Etiology

  • Embryologic
  • Risk factors: tobacco, nitrosamine exposure, COX-inhibitors, decongestants

Epidemiology

  • Young maternal age
  • Low socioeconomic status
  • Change in paternity

Pathogenesis

  • Embryologic failure of migration of the lateral fold on the right side
  • Herniation of midgut into umbilical cord

Presentation

  • Defect to the right of an intact umbilical cord, at the side of the obliterated right umbilical vein
  • No sac covering the abdominal viscera
  • Fascial defect usually 4 cm in diameter
  • More common to have intestinal atresia

Labs

  • Elevated alpha-fetoprotein

Imaging

  • Prenatal ultrasonography
    • Most common diagnostic method
    • Free-flowing bowel loops in amniotic fluid with associated defect in abdominal wall

Treatment

  • Prenatal diagnosis
    • Delivery at hospital with NICU and pediatric surgery
    • Cesarean or vaginal delivery
  • Placement of infant into warm, saline-filled plastic “bowel bag” up to nipple line to minimize heat and fluid losses
  • Placement of NG tube 
  • Broad-spectrum antibiotics x48 hours
  • Reduction of eviscerated bowel
    • Primary reduction successful 50-80% of the time
    • Ringed silo bag is placed if primary reduction is unsuccessful and contents are gradually reduced over several days followed by…
      • Operative suture closure of fascia and skin
      • Sutureless delayed spontaneous closure at bedside by covering defect with or without umbilical cord followed by watertight clear dressing
        • Dressing can then be removed day 4 when intraabdominal contents are adhered
        • Dressing changed to dry dressing over cord remnant or Vaseline dressing over exposed bowel

Relevant Information

  • Safe intraabdominal pressure is <15 mmHg

Complications

  • Postoperative ileus