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Abdominal Wall Defects

Gastroschisis

Omphalocele

Pathophysiology

  • Right-sided defect lateral to the base of the umbilical stalk through which a portion of the GI tract has herniated
  • May be secondary to vascular accident with disruption of body wall formation or weakness at the insertion of the umbilical cord after involution of the right umbilical vein
  • Exposure to amniotic fluid and partial closure of the defect may result in mesenteric vascular constriction → further bowel injury
  • Bowel may be edematous and matted with inflammatory “peel”
  • Small bowel with villous atrophy and blunting, muscular hypertrophy, and serosal inflammation
  • Developmental arrest of somites in the abdominal wall or failure of midgut to return to the abdominal cavity after herniation into the umbilical stalk
  • Central defect; umbilical ring fails to contract and leaves a round defect
  • Defect is covered by a translucent mesenchymal membrane that protects the underlying abdominal organs from exposure to amniotic fluid
  • The sac usually remains intact but occasionally is ruptured during delivery
  • The liver and small intestine usually occupy part of the sac along with variable other abdominal organs

Epidemiology

  • Sporadic; 1.5 of 10,000 live births
  • Risk factors: Young maternal age; low SES; exposure to vasoconstricting decongestants, NSAIDs, cocaine, cigarette smoking, pesticides
  • Can be associated with malrotation; associated with intestinal atresias in 5%–25%
  • Not commonly associated with anomalies unrelated to the GI tract
  • Sporadic; 2–2.5 of 10,000 live births
  • Seen with mothers at extremes of reproductive age
  • Associated with maternal obesity and SSRIs
  • Associated with other structural or genetic defects in 50%–75%
  • Associated syndromes: VACTERL association; Beckwith-Wiedemann syndrome; pentalogy of Cantrell; CHD; pulmonary hypoplasia; congenital malrotation of the colon; EEC syndrome; OEIS complex; trisomies 13, 18, and 21

Presentation and Diagnosis

  • Usually diagnosed antenatally on US
  • Mother has elevated AFP levels
  • Most may still be delivered vaginally without further injury to the bowel
  • Most diagnosed prenatally; should have a thorough evaluation for any other anomalies and consider chromosomal analysis
  • Mother may or may not have elevated AFP levels
  • May be delivered vaginally, but consider cesarean section for giant omphalocele because of concern for rupture

Treatment

  • In delivery room, stabilize airway; wrap intestine with warm, sterile saline dressings; place-large bore orogastric tube to continuous suction; and place peripheral IV for fluid management and broad-spectrum antibiotics
  • Place the infant in clear plastic bowel bag to minimize heat and water loss
  • Inspect bowel for evidence of ischemia, torsion, and atresia
  • Place the infant in lateral decubitus position to avoid mesenteric kinking
  • Reduce or place the bowel in a sterile silo as soon as possible; delay may lead to bowel swelling and resultant ischemia
  • If bowel is forced into abdominal cavity, may cause decreased diaphragmatic excursion, requiring increased ventilator support; may also impair mesenteric, hepatic, and renal perfusion
  • During staged closure, parenteral nutrition, gastric decompression, and spontaneous breathing are preferred
  • When reduction of silo contents is complete, final closure is performed
  • Repair of atresias includes primary anastomosis, or if peel exists, internalization ...

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