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 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.
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.
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.
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 anastomosis – delayed 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.
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.