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