A male child born via cesarean section at 35 weeks’ gestation presents with protrusion of bowel outside the abdominal cavity.
The patient was prenatally diagnosed with an abdominal wall defect.
Physical examination shows an alert, awake baby with protrusion of the small intestine outside the abdomen with no peritoneal covering.
Major defects in closure of the abdominal wall result in exposure of the viscera. In omphalocele, the viscera herniate through the umbilicus and are covered with the peritoneum. In gastroschisis, the viscera herniate through a defect lateral to the umbilicus, usually to the right, and have no covering.
The incidence is 1 in 6000-10 000 live births for omphalocele and 1 in 30 000 live births for gastroschisis. About two-thirds of patients with omphalocele have associated congenital anomalies, such as cardiovascular, genitourinary, gastrointestinal, or craniofacial anomalies, trisomy 13, or Beckwith-Wiedemann syndrome (visceromegaly, macroglossia, microcephaly, and hypoglycemia).
These patients are cared for expeditiously to minimize the potential for heat loss from the exposed viscera, minimize the possibility of infection, and prevent direct trauma to the herniated organs. The stomach is decompressed with a nasogastric tube, and broad-spectrum antibiotics are initiated.
Aggressive fluid resuscitation with a balanced salt solution (150-300 mL/kg/d) is initiated to maintain urine output at 1-2 mL/kg/h.
Prior to induction, suction the stomach.
Use either awake intubation or rapid-sequence induction with endotracheal intubation.
Ensure adequate fluid resuscitation with a balanced salt solution.
Maintain with a narcotic-based anesthetic and a nondepolarizing muscle relaxant.
Patients require postoperative ventilation with fluid resuscitation along with broad-spectrum antibiotics. Patients are at risk of developing abdominal compartment syndrome if the abdominal closure is too tight.
Criteria used as guidelines to monitor intraabdominal pressure are intragastric pressure <20 cm H2O, intravesical pressure <20 cm H2O, and maximum peak ventilatory pressure <30 cm H2O.
DOs and DON’Ts
✓ Do obtain a preoperative echocardiogram in patients with omphalocele.
⊗ Do not mask ventilate the patient.
✓ Do aggressive fluid resuscitation.
✓ Do communicate with the surgeon if abdominal closure is too tight.
✓ Do give adequate nondepolarizing muscle relaxant to facilitate abdominal closure.
The goal is to place the exposed viscera into the abdomen. If the defect is small, primary complete closure is attempted. If a large amount of viscera is exposed, then a staged closure with a silastic silo is performed; the silo is secured at the edge of the defect and gradually reduced over 3-7 days. The patient is then brought to the operating room for complete closure.