Omphalocele

Aka: Omphalocele

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II. Definition

  1. Covered abdominal wall defect at Umbilicus in newborn

III. See Also

  1. Gastroschisis

IV. Epidemiology

  1. Incidence: 2.5 per 10,000 births

V. 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

VI. 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

VII. Diagnosis

  1. Usually diagnosed by prenatal Obstetric Ultrasound

VIII. 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

IX. Complications

  1. Post-operative risk due to associated conditions

X. 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

XI. 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 [PubMed]

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Related Studies

Ontology: Congenital omphalocele (C0795690)

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
Derived from the NIH UMLS (Unified Medical Language System)

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