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.
•
Closure
of the defect in one or more stages.
The prognosis depends on associated malformations.
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