Exomphalos

What is exomphalos?

Exomphalos is a condition where the bowel (and sometimes the liver or stomach) grows outside of a baby’s body and this is covered by a thin layer of material called a sac.

This happens because a hole was left in the tummy wall when it formed during pregnancy.

The sac is at the base of the umbilical cord, near the belly button.

Exomphalos affects around 1 in 8,000 babies.

Causes

The cause of exomphalos is not known.

The condition is not caused by anything the parents have done or not done during pregnancy.

The condition is not inherited and any future pregnancy is not at an increased risk.

Sometimes it can be a sign of other health conditions:

  • Beckwith-Wiedemann syndrome
  • Trisomy – genetic conditions caused by an extra copy of a chromosome

Diagnosis

Exomphalos is usually diagnosed during a routine antenatal scan.

Occasionally, babies can be found to have a small exomphalos after being born.

Types of exomphalos

There are 3 types of exomphalos.

Minor exomphalos

  • Minor exomphalos have a diameter smaller than 5 cm
  • They contain bowel (intestines)
  • They do not contain the liver

Major exomphalos

  • Major exomphalos are larger that 5 cm, or
  • contain the liver

Extremely large exomphalos

[little explanation?]

During pregnancy

If your baby is diagnosed with exomphalos during pregnancy you will be offered:

  • antenatal care with a specialist team
  • regular checks that your baby is growing
  • tests for genetic conditions that are associated with exomphalos
  • an appointment to see a neonatal surgeon and neonatologist at the FANS clinic.

Giving birth

We recommend your baby is born here at St Mary’s Hospital (part of the Manchester Centre for Neonatal Surgery)

You will be offered a planned delivery (induction of labour or caesarean) and you will be given a date for this.

It is likely your baby will be born via caesarean section but in some circumstances a vaginal delivery is possible.

There is a chance your baby may need to be delivered earlier than your due date if the fetal medicine team detect issues that need to be treated.

Your baby will be cared for in the neonatal intensive care unit (NICU).

Treatment

All babies with an exomphalos will need surgery to return the organs to the tummy.

Surgery

Minor and major exomphalos

Babies with minor and major exomphalos will usually need open surgery under general anaesthetic. This normally takes places within the first few days of being born.

The organs are moved from the sac and placed into the tummy. The opening in the abdominal wall is stitched closed.

If part of the bowel is stuck to the sac it is removed and the bowel will be repaired.

Following the operation your baby will usually spend around 2 weeks recovering on NICU and then go home if there are no other medical problems.

Your baby’s progress will be reviewed everyday.

Your baby should be able to feed within the first few days after surgery.

Extremely large exomphalos

Because newborn babies’ tummies are small, there is not always enough room for all of the bowel and liver to fit inside. The lungs would not have enough space to expand, causing breathing problems.

Instead, it is better to wait until your baby has grown larger before surgery takes place. This is usually many months later.

It is important that the exomphalos sac is protected from bumps, so the organs are not damaged. The exomphalos sac is wrapped with dressings to protect and support the organs.

If babies with extremely large exomphalos do not have other health conditions, they can go home from hospital until their operation.

Outlook

After surgery, most babies with exomphalos do not have serious ongoing problems.

Babies with genetic conditions associated with exomphalos may have problems with their heart. These babies would usually need ongoing medical care.