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  1. Definition

    1. Full-thickness abdominal wall defect, occurring to the right of the intact umbilical cord, leaving exposed viscera to herniate into amniotic fluid. Ruptured omphalocele may mimic gastroschisis but the omphalocele defect in the abdominal wall is at the umbilicus.

    2. Defect measures 2 to 4 cm. Stomach, bowel, and occasionally gonads are visible. Solid organs (liver and spleen) remain intraperitoneal. (Figure 123–1).

  2. Incidence

    1. Rare malformation but incidence remains on the rise.

    2. Latest estimates range between 1.5 and 5 per 10,000 births.

  3. Pathophysiology

    1. Defect occurs sporadically, but familial clusters suggest genetic component.

    2. Improper involution of right umbilical vein and other vascular accidents have been implicated in pathogenesis, as have environmental influences, vasoactive drugs, and nutritional deficiencies.

  4. Clinical presentation

    1. The infant is born with varying amounts of herniated intestine.

    2. Prenatal exposure to amniotic fluid results in intestinal edema, induration, and foreshortened mesentery.

    3. Inflammatory peel covers the bowel, leading to intestinal dysmotility and delayed feeding tolerance.

    4. Up to 10% have an intestinal atresia, and all are malrotated. Other congenital anomalies are rare.

  5. Diagnosis

    1. Usually identified on prenatal ultrasound and readily apparent at birth.

    2. Ruptured omphalocele may mimic gastroschisis, and the 2 conditions must be distinguished to optimize care.

  6. Management

    1. General considerations

      1. Prenatal diagnosis affords prenatal counseling and planned delivery at a center capable of providing definitive care.

      2. Safety has been established for both vaginal and cesarean deliveries.

      3. Some centers advocate elective, late preterm delivery, with data favoring earlier time to feeds and decreased length of stay, presumably due to decreased inflammatory effects on the bowel. This practice remains controversial.

    2. Specific measures

      1. Care in the delivery room

        1. The exposed bowel is at risk for injury, hypoperfusion, and fluid and heat loss. The baby’s abdomen and legs should be placed in a plastic bag. Alternatively, the viscera may be covered with saline-soaked gauze and wrapped in cellophane.

        2. While awaiting surgical evaluation, the newborn is laid on either flank to minimize occlusion of the narrow mesentery.

        3. Orogastric tube should be placed.

      2. Fluid resuscitation

        1. Due to exposed viscera, insensible losses begin at birth and remain significant until abdominal domain is restored.

        2. Prompt venous access and fluid resuscitation are needed to replace losses leading to further bowel compromise. Delay in care worsens the shock state.

      3. Thermoregulation. Hypothermia risk due to evaporative losses occurs rapidly. Immediate measures are needed to restore and maintain normal temperature.

      4. Prophylactic antibiotics. Broad-spectrum antibiotics are generally recommended, due to unavoidable seeding of the peritoneum during delivery and handling of the defect. Prolonged use is discouraged.

  7. Surgical management

    1. Surgical options include primary closure (when possible) or placement of silastic silo with staged reduction (Figure 123–2).

    2. Operative decision is guided by bowel appearance, defect size, and baby’s general condition. Primary reduction is contraindicated with respiratory distress syndrome, lung hypoplasia, or concern for compartment syndrome.

  8. Nutrition

    1. Gastroschisis places the newborn in a catabolic state, necessitating early focus on nutrition.

    2. Intestinal dysmotility leads to delayed tolerance of enteral feeds, necessitating total parenteral nutrition and early ...

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