Abdominal Wall Defects: Exomphalos (Omphalocoele)

  • What is it?

This is a hernia (protrusion) at the base of the umbilical cord (belly button). Until about 10 weeks of pregnancy the intestines of the developing baby are located outside the abdomen in the umbilical cord. At this stage they start to move back into the baby's abdomen through an opening in the muscle at the base of the umbilical cord. If the muscles in the centre of the baby's abdomen are weak, some of the intestines can fail to move into the abdomen resulting in a protruding sac, or exomphalos, containing liver and bowel.

  • How common is it and will it happen again?

About one in every 5,000 babies are born with exomphalos. It is extremely unlikely that this will happen again in a future pregnancy and the chances are usually no greater than those of any other couple.

  • What causes it to happen?

Exomphalos usually occurs 'out of the blue'. If is not due to a defect of either of the parents or because the mother has done something wrong during or before the pregnancy.

  • Is there anything else wrong with the baby?

In approximately four out of every ten fetuses with exomphalos there are other serious abnormalities. The exomphalos can arise as a result to a chromosomal abnormality, for example Trisomy 18, which is lethal, or can be associated with heart, kidney or spinal defects.

A detailed ultrasound scan will help detect most of these defects but it is also necessary to take a sample of fetal blood by cordocentesis, to check for chromosomal abnormalities. Cordocentesis carries a small (1%) risk of miscarriage.

  • What are my options?

Provided that exomphalos is the only obvious abnormality found, the outlook is generally very good and so most parents decide to continue with the pregnancy. However you will also be given the option of a termination of pregnancy, especially if other problems are found.

  • What are the chances that the baby will survive?

This depends on how big the exomphalos is and if there are any other defects. If the baby is not born very prematurely and there are no other defects, the chances of survival are about 80%-90%. Growth and development are usually normal.

  • Where should I have the baby?

The baby will need to have an operation when it is born and so it is best if you are delivered in a hospital with special facilities for intensive care and neonatal surgery. We have these facilities at King's.

  • Do I need to have a Caesarean section?

Provided that there are not other problems with your pregnancy you can have a normal delivery. However it is best if everything is planned in advance to ensure that the appropriate paediatricians and surgeons are available, and we usually prefer to induce labour at around 38 weeks.

  • What will happen to the baby after if is born?

After birth the baby will be taken to the neonatal intensive care unit to be prepared for the operation. Sometimes the babies have breathing difficulties and may need to be put on a ventilator.

The operation is usually carried out during the first day of life. In the operating theatre the baby is given a general anaesthetic. The surgeon tries to push the sac back inside the abdomen and close the defect with stitches. When the sac is very big and the abdomen very small, the surgeon puts a plastic bag over the hernia. During the next 5-10 days the bag is gradually squeezed and when all the organs are in the abdomen the final operation is carried out to close the defect with stitches.

After the operation the baby is nursed in the intensive care unit and for the first few days will need to be on a ventilator. It takes 2-3 weeks for the abdomen to heal and during this time the baby receives fluid and nutrients through a drip. They usually stay in hospital for a total of 3-6 weeks.

If you have any further questions we will be happy to talk to you.


Support Group

GEEPS (Gastroschisis, Exomphalos, Extrophies Parent's Support)
2 Fairholme Rd
Mount Pleasant
East Sussex BN9 ONY
United Kingdom

Tel: +44 (0)1273 516840

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