University Hospital Southampton NHS Foundation Trust|

Exomphalos

Exomphalos is a weakness of the baby’s abdominal wall where the umbilical cord joins it. This weakness allows the abdominal contents, mainly the bowel and the liver to protrude outside the abdominal cavity where they are contained in a loose sac that surrounds the umbilical cord. As the contents are lying outside it, the abdominal cavity often does not develop properly and remains small in size.

Why does Exomphalos occur?

The cause of this condition is unknown and is a rare problem occurring in about 1 in 5000 pregnancies. About half of all babies with Exomphalos will have problems affecting other body systems. Most commonly affected are the heart, lungs, and kidneys. Detailed scans will be performed but it is not always possible to detect problems before the baby is born. There is also an increased risk of a chromosomal abnormality and you will be offered an amniocentesis.

What happens at the delivery?

It should be possible for you to deliver your baby in the normal way unless the Exomphalos is very large, or there are other reasons for requiring a Caesarean Section. We would recommend, however, that your baby be delivered here at the Princess Anne Hospital. Your baby will be transferred to the neonatal unit soon after delivery.

Initial treatment

After your baby is born, the sac will be wrapped in a protective film to reduce heat and fluid loss. A drip will be placed into a small vein so that intravenous fluids can be given. A tube will be passed through your baby’s nose into the stomach to drain away the green fluid (bile) that collects in the stomach. This lessens the risk of your baby vomiting and reduces discomfort.

Your baby will be examined in order to identify any problems with other body systems and may need further tests. If there are associated lung problems assistance may be required with breathing.

Surgery

The size of the sac and its contents can vary greatly from being very small to very large. It is therefore impossible to say exactly what type of operation is required for Exomphalos until your baby is born and the actual size can be seen. However there are a number of different treatments any of which can be used depending upon the size of the Exomphalos. These are:-

Primary repair - If small, it is possible in one operation to replace the Exomphalos inside the abdominal cavity and repair the muscles the skin.

Staged repair - If the Exomphalos is very large, it may not be possible to close it in a single operation. Therefore, the baby would require an initial operation to construct a temporary envelope of plastic sheeting (silo) outside the abdomen. The silo would then be made smaller over a period of about ten to fourteen days, so that the abdominal contents are gradually pushed back inside the abdomen. This will probably be done on the ward with sedation as necessary.A final operation is necessary to completely replace the abdominal contents and repair the muscles and skin. If the closure is very tight the baby may need to be on a ventilator for some days to help with breathing.

The surgeon may opt just to close the skin of the abdomen over the Exomphalos and not to repair the muscles. Following this your baby will be left with a ventral hernia (a bulge of the tummy wall) and a further operation will be needed to repair the hernia at about three years of age. In the meantime your baby will should be able to go home and live normally while waiting for the second operation.

For all these procedures painkillers will be given as necessary.

Conservative treatment - If the Exomphalos is very large it may not be possible to close it until your baby is older and the size of the abdominal cavity has grown. Skin will gradually grow over the Exomphalos over a period of several weeks/months. Your baby may be able to return home before the Exomphalos is fully covered by skin. With this type of treatment your baby will be left with a ventral hernia which will be repaired at about three years of age. The overall length of time that your baby spends in hospital with this condition varies depending on the type of treatment needed and the presence of any problems affecting other body systems.

Is there any risk to my baby’s life?

The outlook for babies with this condition depends on the presence and severity of any other associated problems. These problems are not always apparent until after the baby is born. The majority of babies with just an Exomphalos survive and grow up to lead normal lives.

Can I feed my baby?

Some babies can feed within a few days of their operation but the bowels of some babies with Exomphalos can take several weeks to work normally post operatively.

During this time your baby will need drip feeds (Parenteral Nutrition) through a long line. Usually the line is placed in a small vein in an arm or leg and fed through into a large vein. Sometimes an operation under anaesthetic is necessary to insert the line directly into a large vein.

Milk feeds will be slowly introduced and increased as the baby is able to tolerate them. Once recovered the baby should be able to feed normally either by breast or bottle.

Long term and follow up

Following discharge from the ward there will be regular check-ups in order to monitor your baby’s progress. Your baby will be seen in the outpatient’s department. It may be possible for this follow up to take place in your local hospital. Your baby should be able to continue normal progress with feeding and weaning.

Following an operation there is always a small risk of future obstruction occurring. If your baby has a bilious vomit or a distended abdomen medical advice should be sought.

Parent to parent link

Many parents who have experienced this same condition with their baby have indicated that they would be willing to speak to other expectant parents about this. If you would like to speak with another family please contact us|.

Disclaimer

University Hospital Southampton NHS Foundation Trust produce guidelines as an aid to good clinical practice. They represent recognised methods and techniques of clinical practice, based on published evidence. The ultimate judgement regarding a particular clinical procedure or treatment must be made by the clinician in the light of the clinical data presented by the patient and the diagnostic or treatment options available. The guidelines issued are not intended to be prescriptive directions defining a single course of management and departure from the local guidelines should be fully documented in the patient's case notes at the time the relevant decision is taken.

Disclaimer

University Hospital Southampton NHS Foundation Trust produce guidelines as an aid to good clinical practice. They represent recognised methods and techniques of clinical practice, based on published evidence. The ultimate judgement regarding a particular clinical procedure or treatment must be made by the clinician in the light of the clinical data presented by the patient and the diagnostic or treatment options available. The guidelines issued are not intended to be prescriptive directions defining a single course of management and departure from the local guidelines should be fully documented in the patient's case notes at the time the relevant decision is taken.