Omphalocele

1. Definition
   1. Covered abdominal wall defect at Umbilicus in newborn
2. See Also
   1. Gastroschisis
3. Epidemiology
   1. Incidence: 2.5 per 10,000 births
4. Pathophysiology
   1. Defect at base of Umbilical Cord (2 to 10 cm)
   2. Normal abdominal viscera and contained within sac
   3. Abdominal contents herniate via defect
      1. Intestine
      2. Liver, Gall Bladder
      3. Stomach
      4. Bladder
      5. Pancreas
      6. Spleen
5. Associated conditions (67% of cases)
   1. Trisomy 13
   2. Trisomy 18
   3. Congenital Heart Disease (up to 25% of cases)
      1. Ventricular Septal Defect
   4. Beckwith-Wiedemann Syndrome (Gigantism, Macroglossia)
   5. Gastrointestinal disorders
      1. Midgut Volvulus
      2. Malrotation
      3. Meckel's Diverticulum
      4. Imperforate anus
      5. Colonic agenesis
   6. Neurologic disorders
   7. Renal anomalies
   8. Pentalogy of Cantrell
   9. Bladder Extrophy
6. Diagnosis
   1. Usually diagnosed by prenatal Obstetric Ultrasound
7. Management
   1. See Gastroschisis for stabilization procedures
   2. Surgical repair on first day of life
      1. Immediate surgery consultation
      2. Abdominal contents replacement is difficult
         1. Small abdominal cavity
         2. Malrotation is usually present
8. Complications
   1. Post-operative risk due to associated conditions
9. Prognosis: Mortality
   1. Associated Comorbid condition: 30% mortality
   2. No associated condition: Minimal mortality
   3. Better prognostic factors
      1. Smaller defects
   4. Worse prognostic factors
      1. Liver evisceration
      2. Chromosomal syndrome
10. References
    1. Feldman (1998) Sleisenger Gastrointestinal, p. 1428
    2. Townsend (2001) Sabiston Surgery, Saunders, p. 1479-80
    3. Fuloria (2002) Am Fam Physician 65(2):265-70