Exomphalos

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What is exomphalos?

Exomphalos is a condition where the bowel (and sometimes the liver or stomach) grows in a sac at the base of the umbilical cord, near the belly button.

Exomphalos affects around 1 in 8,000 babies.

Causes

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

Although the cause of exomphalos is not known it can sometimes be associated with other conditions such as:

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

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

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 2 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

 

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 exomphalos

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

The sac covering the bowel is removed and the bowel is pushed back in to the abdomen. If part of the bowel is stuck to the sac it is removed and the bowel will be repaired. The opening in the abdominal wall is then closed. Following the operation your baby will recover on NICU and should be able to feed within the first few days after surgery. Your baby’s progress will be reviewed everyday and they will go home when there are no ongoing problems.

Major 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 and it is better to wait until your baby has grown larger before surgery takes place. Initially the sac is protected by special dressings until skin grows over it.  Instead, surgery may be months or years later however your baby can be at home until operation.

Outlook

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

Babies with genetic conditions associated with exomphalos usually have no ongoing issue with the exomphalos but can have problems associated with their other condition. These babies will need ongoing medical care.