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]
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| Definition (NCI) | A congenital defect in the muscles of the abdominal wall that results in the intestines and other abdominal organs developing outside the abdominal wall covered in a sac. |
| Definition (MSH) | A congenital defect with major fissure in the ABDOMINAL WALL at the UMBILICUS resulting in the extrusion of VISCERA through the UMBILICUS. Unlike GASTROSCHISIS, omphalocele is covered with PERITONEUM but without overlying SKIN. |
| Concepts | Congenital Abnormality (T019) , Disease or Syndrome (T047) |
| MSH | D006554 |
| ICD9 | 756.72 |
| ICD10 | Q79.2 |
| SnomedCT | 196872004, 271017007, 49324006, 196854005, 18735004 |
| English | Omphaloceles, Congenital omphalocele, Unspecified omphalocele, abdomen omphalocele (___ cm), omphalocele, omphalocele (___ cm), omphalocele (diagnosis), omphalocele (physical finding), Omphalocoele, omphalocoele, omphaloceles, Unspecified omphalocele (disorder), Congenital Omphalocele, Congenital omphalocele (disorder), Omphalocele, Amniocele |
| Dutch | omphalocele, omfalokèle, Omfalokèle |
| Spanish | Onfalocoele, Onfalocele, onfalocele no especificado (trastorno), onfalocele no especificado, onfalocele congénito (trastorno), onfalocele congénito, onfalocele |
| Japanese | 臍帯ヘルニア, ｻｲﾀｲﾍﾙﾆｱ |
| Portuguese | Onfalocele |
| French | Omphalocèle |
| German | Omphalozele |
| Czech | Omfalokéla, omfalokéla |
| Hungarian | ((amniocele)), Omphalocoele |
| Norwegian | Omfalocele |
| Italian | Onfalocele |
