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Long gap OA – delayed primary anastomosis

Most babies born with oesophageal atresia (OA) undergo a primary repair within the first few days of life. However, approximately 10% of babies have a gap between the upper and lower ends of the oesophagus which is too long for a primary repair.

These babies have long gap oesophageal atresia.

Long gap oesophageal atresia can occur in babies with a distal tracheo-oesophageal fistula (TOF), but is much more common (80%) in babies born with isolated OA, without a distal TOF. Around 10% of babies with long gap OA have an upper pouch TOF instead of a distal TOF.

There are several treatment options for babies with long gap OA.

  • The commonest technique is delayed primary repair
  • If delayed primary repair is not possible then oesophageal replacement is necessary
Variations in long gap oesophageal atresia
Variations in long gap oesophageal atresia

How is long gap OA diagnosed?

The diagnosis of OA is confirmed shortly after birth when a doctor or nurse attempts to pass a feeding tube through the mouth or nose, down the oesophagus, and into the stomach.

  • The tube gets stuck in the upper part of the oesophagus.

  • This is confirmed with an x-ray of the chest.

  • At this point it is not possible to be certain about the gap in the oesophagus.
OA with a distal TOF
Chest x-ray showing OA with a distal TOF. Air has entered the stomach through a distal TOF

If the lower oesophagus is connected to the trachea (a distal tracheo-oesophageal fistula), air can be seen in the stomach on the x-ray.

  • The gap in the oesophagus is usually short and a primary repair takes place within the first few days.
  • Occasionally during this operation the gap in the oesophagus is too long to allow immediate repair and a diagnosis of long gap OA is made. The TOF is divided but the ends of the oesophagus cannot be joined together.
Isolated OA
Chest x-ray showing isolated OA. There is no air in the stomach.

If the initial chest x-ray confirms oesophageal atresia but there is no air in the stomach, then the usual explanation is that there is isolated oesophageal atresia with no distal TOF.

  • Almost always this means a long gap in the oesophagus.

  • A primary repair is not attempted.
delayed primary anastomosis
Delayed primary anastomosis

What happens after long gap OA has been diagnosed?

Once a long gap OA diagnosis has been made, oral feeding must not occur.

A tube called a Replogle tube is inserted into the upper oesophagus to hoover out saliva. This is necessary to prevent saliva spilling over into the airway and causing a chest infection (aspiration pneumonia).

A gastrostomy feeding tube is stitched into the stomach within the first few days of life with an operation on the abdomen. Milk feeds are started through the gastrostomy using either the mother’s expressed breast milk (EBM) or formula milk.

Some surgeons use a camera (a bronchoscope) in the trachea to check that there is no connection between the upper oesophagus and the trachea (an upper pouch TOF). This is rare and some surgeons delay this until the baby is bigger. If there is an upper pouch TOF this will have to be divided at some stage which usually involves an operation on the neck.

With a gastrostomy tube in place for feeding and a Replogle tube to prevent aspiration of saliva into the lungs, the baby is in a safe  position to let them and their oesophagus grow.

The Replogle tube must be kept on continuous suction and flushed with small volumes of water every 15-30 minutes to prevent blockage.

The baby cannot feed by mouth although some surgeons allow a dummy. Sham feeding may also be recommended.

Despite all of this chest infections are fairly common in babies on Replogle suction. Chest infections are not usually dangerous and does not cause long term damage but they do make babies poorly and often put them into oxygen.

Waiting for the oesophagus to grow or, to be more accurate, waiting for the gap to narrow, takes weeks to months. This is always a hugely frustrating time for parents.

During this time, your baby has to remain in hospital and cannot feed. Worse than that, the baby forgets how to feed. So, why do they do this?

The reason is because in most babies the gap will narrow over the first few months to the point that it becomes possible to join the two ends of the oesophagus together.

This is procedure is called a delayed primary anastomosisdelayed because of the wait, primary because it is the first operation on the oesophagus (at least in a baby with isolated OA) and anastomosis meaning the join.

Would you like more information?

Published by TOFS, The TOF Book is the must have guide for anyone affected by, or caring for someone with OA/TOF.

With contributions from medical experts, the TOF Book contains chapters on every aspect of OA/TOF and VACTERL condition, from infancy to adulthood all presented in an easy-to-understand format.