Exomphalos

Your antenatal scans have indicated your baby has exomphalos. This is sometimes called omphacele. This leaflet will help you to understand the condition and what will happen when your baby comes to the Neonatal Surgical Unit (NSU) at Sheffield Children’s NHS Foundation Trust hospital for treatment and care.

What is exomphalos?

Exomphalos is when the abdominal wall (tummy wall) does not develop fully during pregnancy. Early in the pregnancy the baby’s intestines (bowel) develop inside the umbilical cord and a few weeks later it moves inside to the abdomen.

For babies with exomphalos the intestines (and sometimes other organs like the liver) do not move back into the abdomen. The abdomen then fails to close round the base of the umbilical cord.

The size of the exomphalos depends on the number of organs in side it. Most exomphalos have just the bowel inside. This is known as exomphalos minor. If the liver or other organs are inside the exomphalos, this will be referred to exomphalos major or giant exomphalos.

The organs are covered by a loose sac that surrounds the umbilical cord. The abdominal cavity is sometimes smaller in exomphalos babies due to the organs growing externally. Exomphalos can be associated with other birth defects.

What causes exomphalos?

It is not known exactly what causes exomphalos. It is not caused by something you have or have not done. It happens in about every 4 babies out of 10,000. Sometimes it is linked with genetic abnormalities or other medical conditions.

When, how and where will my baby be born?

The aim will be for your baby to be born at the Jessop Wing, Royal Hallamshire Hospital in Sheffield. Your obstetrician will closely monitor your baby. They may recommend your baby is born by caesarean section. This will be discussed with you.

What will happen when my baby is born?

When your baby is born they will be seen by the neonatologists (baby doctors) and taken to the Neonatal Intensive Care Unit (NICU) at the Jessops Wing to be assessed and monitored. The exomphalos will be covered by a protective plastic dressing (like Clingfilm) to keep it clean, prevent heat loss and keep it moist. Your baby will be continuously monitored to check their heart rate, breathing rate, oxygen level and blood pressure.

A plastic tube, often called a ‘drip’ (intravenous cannula) will be inserted in to your baby’s vein to give intravenous fluids, antibiotics and other medicines your baby may need. Your baby will not be able to feed initially. The nurses will pass a tube called a gastric tube, in through your baby’s nose to their stomach. This is to drain the contents of the stomach to keep your baby comfortable and to stop them being sick. The surgeons will liaise with the neonatologists at the Jessops Wing and may see your baby at there.

When will my baby go to the Neonatal Surgical Unit (NSU)?

When your baby is stable and there is a bed for your baby on NSU they will be transferred there by Embrace. Embrace are a specialist transport team who are experienced in transferring babies, including those who have Exomphalos.

Sometimes your baby may need to stay on NICU for a longer period of time before being transferred to NSU. In this situation the surgeons will come to NICU to review and be involved in your baby’s treatment there.

How will my baby’s exomphalos be treated?

Treatment depends on the size of the exomphalos and any other associated problems or conditions. Exomphalos minor will require surgery known as primary repair. The surgeons will explain the operation to you and you will need to give consent.

You will also be seen by an anaesthetist because your baby will need a general anaesthetic for the operation. During the operation the surgeons will move the intestines back into the abdomen and close the muscle and skin making a belly button.

For babies with large or giant exomphalos they will usually have conservative (non-surgical) treatment but may require one or more operations when they are older. The surgeons will discuss this with you at the time.

The treatment will depend on how well your baby is, how big the baby and the exomphalos are, what organs are in the sac (exomphalos) and if the sac is intact (no holes in it).

If my baby has an operation what happens afterwards?

Your baby will be given pain relief and will be continued to be monitored closely. Sometimes after the operation your baby may need help with their breathing for a short period time to help with pain, healing and to enable your baby to rest.

Your baby will be able to start small amounts of milk soon after the operation. The amount of milk your baby has will be slowly increased as they recover and show us signs they can take and digest their milk well. As your baby recovers you will be able to commence breast feeding if you wish to do so.

Occasionally some babies need to have intravenous feeding whilst having milk feeds introduced. This is called parenteral nutrition (PN) which can provide all the nutrients your baby needs to grow which they would normally get from milk. PN is given via a special ‘drip’ in their veins. This will all be explained to you at the time.

If my baby has conservative treatment what will happen?

Conservative treatment involves the regular application of a special dressing with gentle pressure to help reduce the organs back in to the abdomen over a period of weeks. The babies do not need a general anaesthetic for this.

It takes place on NSU with your baby having comfort measures whilst the dressing is being applied. You can be with your baby during this time. Your baby will be monitored and given pain relief as necessary. They may require some oxygen for a short period of time.

Your baby will be able to start having small amounts of milk which will be slowly increased as your baby show signs of tolerating it well. Occasionally your baby may need to have their milk via a nasogastric tube (NGT) for a period of time. This tube is passed through your baby’s nose into their stomach. This will be explained to you at the time. As your baby recovers you will be able to commence breast feeding if you wish to do so.

What milk can my baby have?

Breast milk is very important for every baby but especially if your baby is poorly.

If you wish to breast feed or give you baby breast milk we will support and help you in expressing your breast milk and establishing breast feeding. Your midwife can help you to start expressing after your baby has been born. We have facilities on NSU for expressing and there are infant feeding specialists in the hospital who can advise and support you with this as well as the NSU nurses. Your breast milk can be frozen until your baby is ready to have it. You can also use your colostrum and breast milk for your baby’s mouth care.

If you do not wish to express your breast milk or are unable to do so, your baby can have formula milk of your choice.

Occasionally the surgeons may wish your baby to have a special prescription milk formula. This will be discussed with you at the time.

Will my baby have other problems?

Sometimes babies with exomphalos have associated medical problems, genetic problems or both. These can include problems with their heart and lungs. You may be aware of these from your antenatal scans and other tests you may have had.

When your baby is born they will be carefully examined. They will have ultrasound scans to check their abdomen and heart, and other investigations as necessary. These will all be explained to you at the time.

How long will my baby be in hospital?

We are unable to predict how long your baby will be in hospital. Your baby’s progress will be closely monitored by the surgeons, nurses and the other teams involved with your baby’s care. You will be updated regularly on your baby’s progress and will be able to ask and have questions answered throughout your stay on NSU.

When your baby is tolerating milk feeds and gaining weight they can go home. Your baby will be followed up by the paediatric surgeons in our outpatient clinics here at the Sheffield Children’s Hospital or at your local hospital.

What is the long term outlook for my baby?

The outlook for babies born with exomphalos varies. It depends on the size of the exomphalos and other problems they may have when they are born or go on to develop.

Many babies with exomphalos go onto to lead normal lives.

Your baby’s progress will be monitored by the surgeons and other medical teams if involved.

Are there support groups?

GEEPS
Gastroschisis, Exomphalos, and other Exstrophy Parents Support Group
www.geeps.org

BLISS
Group which offers general support to families of babies born early or sick
68 South Lambeth Road, London SW8 1RL
Website: www.bliss.org.uk

Contact A Family
www.contact.org.uk

For further information

www.gov.uk
Exomphalos:Information for parents

NSU
Telephone: 0114 271 7324 or 271 7705