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Paediatrics: Exomphalos (omphalocele)

Paediatrics: Exomphalos (omphalocele)
The incidence of exomphalos is 1/3000 live births.

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.

 

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