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Chapter 142: Omphalocele and Gastroschisis

Srijaya K. Reddy

INTRODUCTION

Omphalocele and gastroschisis are both abdominal wall defects that can occur in a neonate. Each results in a variable extent of herniation of abdominal contents and both are considered urgent surgical procedures. If left untreated, bowel ischemia, bowel obstruction, and sepsis can occur. Although omphalocele and gastroschisis can be similar in appearance, they have distinct characteristics (Table 142-1).

TABLE 142-1Characteristics of Omphalocele and Gastroschisis
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TABLE 142-1 Characteristics of Omphalocele and Gastroschisis
  Omphalocele Gastroschisis
Abdominal wall defect

  • Incomplete return of midgut to abdominal cavity, resulting in exposed viscera covered by a thin, clear membrane (intact abdominal sac)

  • Defect can range from 4 to 12 cm in size

  • Midline defect

  • Umbilical cord is inserted into the sac

  • Evisceration of bowel contents through a paramedian abdominal wall defect

  • Defect usually 2–5 cm in diameter

  • Usually occurs to the right of the umbilicus

  • No intact sac

  • Umbilical cord is separated from defect
Incidence 1:4000 to 1:7000 1:10 000
Gender Incidence is higher in males than females Equal occurrence in males and females
Associations

  • 50%–70% are associated with other anomalies (up to 25% have cardiac defects)

  • Associated with:

    • Trisomies 13, 15, 18, and 21 (20%–30% have chromosomal abnormalities)

    • Beckwith–Wiedemann

    • Prune belly

    • Pentalogy of Cantrell

    • OEIS Complex

  • Usually an isolated lesion

  • Less than 20% have associated anomalies

  • May result from in utero exposure to acetaminophen, aspirin, and/or pseudoephedrine

  • Occurs more often in babies born to younger mothers (age < 20)
Prematurity incidence (%) 33 58

EMBRYOLOGY

In normal development, by the third week of gestational age, the gastrointestinal tract is formed into the foregut, midgut, and hindgut. During 7–12 weeks of gestational age, the midgut is extruded into the umbilical cord due to rapid growth and usually returns to the abdominal cavity during the 10th week of gestational age, once the abdominal cavity is large enough to accommodate the developing gut. It is thought that omphalocele and the accompanying small abdominal cavity results from an incomplete fetal return of the midgut to the abdominal cavity during development.

Gastroschisis is believed to be an isolated abdominal wall defect caused by failure in development of one of the embryonic lateral folds, causing abnormal development of the right omphalomesenteric artery or right umbilical vein. Although both omphalocele and gastroschisis result in an underdeveloped abdominal cavity, the actual etiology of each of these lesions is not completely understood.

ANESTHETIC CONSIDERATIONS

Preoperative

Both omphalocele and gastroschisis pose several challenges to the pediatric anesthesiologist. Although repair of these lesions is considered urgent in nature, a careful and thorough assessment of possible associated defects is imperative. Preoperative management should focus on aggressive fluid resuscitation (150–300 mL/kg/day), avoiding hypothermia and heat loss, treating and preventing infection or sepsis, and protecting the herniated organs from direct ...

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