Table 1

Differential diagnosis of ventral wall defects

Umbilical cord herniaOmphaloceleGastroschisisUmbilical hernia
AetiologyPersistence of physiological mid-gut herniation beyond 10–12 weeks gestation2Large umbilical defect resulting from a failure of closure of the ventral abdominal wall5Several hypotheses; disruption of the right umbilical vein10Herniation of the content of the abdomen secondary to delayed/failed closure of the umbilical ring11
Stage of acquisition12–14 weeks23–5 weeks25–6 weeks2Postnatal11
Incidence1 in 500012
Under-reported
1 in 4000121 in 200012Very common; true incidence unknown; increased incidence in premature and African descent infants11
GeneticsSporadic2Mostly sporadic; few familial cases13Mostly sporadic;
Rare familial cases14
Sporadic11
Associated anomaliesRare case reports of associated bowel anomalies and umbilical cord cysts1 5 650–70% of them have other major anomalies6–8Some studies show an 8–10% association with other major anomalies and 1–3% association with cardiac anomalies10Rare11
Abnormal karyotypeNo association of abnormal karyotype with isolated presentation530–40%6–8No association of abnormal karyotype with isolated presentation10No association of abnormal karyotype with isolated presentation11
Prenatal ultrasound findingsSmall bowel extending into the base of the normally inserted umbilical cord5Fetal midline abdominal mass with the umbilical cord attaching at the apex of the mass8Exteriorised bowel floating freely in the amniotic cavity adjacent to a normally inserted umbilical cord9No herniation seen on prenatal ultrasound
PrognosisIsolated presentations are considered to have a good prognosis5Depends on associated malformations and karyotype abnormalities7Good prognosis; 25% cases associated with bowel complications10Good prognosis11