Paediatrics: Exomphalos (omphalocele)

Exomphalos (omphalocele)

The incidence of exomphalos is 1/3000 live births (see Fig. 23.15). It is usu-ally identified on prenatal US. It is characterized by:

•   Hernia into the base of the umbilical cord—the herniated bowel is covered by a sac (amnion).

•   Exomphalos major: defect >5cm diameter.

•   Exomphalos minor: defect <5cm diameter.

Malformations in association with exomphalos are found in 50% of cases:

•   Chromosomal defects: trisomies 18, 13, 21 and Turner’s syndrome.

•   Cardiac defects.

•   Syndromes: BWS.

Surgical treatment

•   Closure of the defect in one or more stages.

The prognosis depends on associated malformations.