Omphalocele

Patient Considerations

• Omphalocele is midline abdominal wall defect that is surrounded by a layer of amnion and peritoneum which contains abdominal contents. The prevalence of this disease in the US is 4 in every 10,000 live births and are most commonly found in children of women at extremes of reproductive age. Disease is detected prenatally with ultrasound as early as 11 weeks. The disease is categorized into those involving containing part of the liver and those that don’t with those containing liver making up 80% of cases.

• Pathogenesis

  • The pathology of omphalocele occurs during the embryonic period and is due to failure of the extraembryonic gut to return to the abdominal cavity and begin 270-degree counterclockwise rotation. Identification of omphalocele is usually not made until after the 10^th postmenstrual week.

• Associated Anomalies

  • Omphalocele without liver involvement is associated with chromosomal abnormalities up to 60% of the time. There are also many structural defects associated with this disease including; GI abnormalities, neural tube defects, oral clefts, defects of diaphragm, polyhydramnios, growth restriction, and congenital heart disease. Omphalocele has an association with Beckwith-Weidemann syndrome (Difficult Airway).

Case Planning

• Specific or Unique Room Set-Up Requirements

  • Airway

    • Oral ETT

    • Difficult airway management equipment if patient is suspected to have Beckwith-Weidemann syndrome

  • Drugs/Infusions

    • No specific infusions indicated unless indicated by co-morbid conditions

  • Monitors

    • Standard ASA monitors

    • Consider Somatic NIRS

      • Decreased somatic NIRS may be the first sign of impaired renal perfusion secondary to increased intra-abdominal pressures post-reduction

    • Arterial Access if co-morbid conditions indicate

  • Blood Availability (if indicated)

    • Blood loss is usually low

      • Consider T+S/T+C

  • PICU Bed Availability (if indicated)

    • Not mandatory

      • Discuss case-specific concerns with the surgeon

Anesthetic Considerations

• Induction

  • Obtain baseline NIRs on Room Air

  • IV or Inhalational induction

  • Oral ETT placement

• Positioning

  • Supine

• Maintenance

  • Maintenance of Anesthesia

    • Hemodynamic/Physiologic goals

      • Somatic NIRS should be within 20% of baseline post-reduction

      • Post-reduction tidal volumes should be monitored to ensure that reduction has not impaired ventilation

    • Volatile Anesthesia

    • Non-Depolarizing Muscle Relaxation

    • Narcotic

  • Surgical Considerations (such as neuromonitoring, muscle relaxation, anticipated blood loss)

    • Muscle relaxation may help facilitate reduction and abdominal closure

    • Blood loss is usually low

• Emergence/Disposition

  • Awake v. Deep extubation may be considered

  • Patients with significant co-morbid conditions may require post-op ventilation

• Post-Op Pain Management

  • Appropriate patients may benefit from continuous caudal/epidural analgesia

• Case-Specific Complications

  • Closure of the abdomen may precipitate abdominal compartment syndrome

    • Notify the surgeon if patient is not tolerating closure

  • Patients with congenital heart disease may not tolerate the physiologic stress associated with increasing intra-abdominal pressure

  • Beware comorbid conditions such as Beckwith-Weidemann syndrome

    • Difficult airway