How long does it take for a patient with long-gap EA to grow a "new" esophagus?
The length of time it takes to grow the esophagus depends on the length of the gap, whether there have been past operations, and the presence of other medical conditions or problems. In cases where the Foker process is the first form of treatment, the average duration for the cases without prior surgery between placement of the sutures through an opening in the chest (thoracotomy) to repair of the esophagus was 16 days (range six to 34), and for children with prior attempts at repair, it was 31 days (range eight to 57). If the connection leaks or narrows, additional procedures may be necessary, sometimes substantially lengthening hospitalization.
During the stretching phase, children are kept on a ventilator and are heavily sedated and often motionless, so they do not tear the traction sutures. The esophagus will grow an average of 1 to 2 millimeters per day on each end. When the ends are close enough together, the child is returned to the operating room and the esophagus is repaired. Even the very longest gaps respond to growth stimulus, so a child with a very long gap can expect to be connected.
What treatment is used when the Foker process is not the best option?
If the Foker process is not ideal, the EA/TEF may be treated using a jejunal nterposition. In Jejunal Interposition surgeons use a section of the jejunum (the middle part of the small intestine) as a replacement for the missing section of esophagus.
This works because the jejunum is thin enough to be placed behind the trachea, mimicking the course of an esophagus and doesn't dilate or "kink" in the manner of a transposed colon segment, so no surgical revisions are required.
Number of Operations
How many surgeries will it take to fix our child's long-gap EA?
The number of operations required to repair a long-gap EA is directly related to whether this is the first attempt at repair, or whether surgery has been performed prior to the infant coming to Boston Children’s Hospital. At a minimum, two operations are required: one thoracotomy (operation opening the chest) to place sutures, which will apply the tension, into the two ends of the esophagus, and a second thoracotomy to join the two esophageal segments.
Once the sutures are in place, other surgeries may still be required to replace the traction sutures if they pull out, close a leak in the esophagus if it develops, or modify the direction of the traction. How many additional surgeries are needed will be related to whether the patient is receiving the Foker process as an initial treatment or a second treatment. If it is used as the first treatment, children typically need anywhere from two to seven procedures. Patients who receive the Foker process as a second treatment option typically undergo two to 15 procedures.
Most children will require dilations after the esophagus ends are connected to minimize the chance of severe stricture formation at the point where the two ends are sewn together.
The length of time that the children are maintained motionless on a ventilator to allow the induction of growth of the esophagus, and the length of time infants and children stay in the intensive care unit is also dependent upon whether this is a first or a repeat procedure. The interval between placement of the sutures and first surgery to join the two ends was an average of 16 days (range six to 34) for first attempt at repair and 31 days (range eight to 57) for those with subsequent attempt at repair. Of the latter group, 17 of 22 achieved connection of the two ends.
If the two ends of the esophagus can be connected, there may be further surgeries required after this point to repair esophageal leaks or to remove sections of the esophagus that have become severely narrowed with scar tissue.
Also, children often require an operation for gastroesophageal reflux (backflow of gastric contents up into the esophagus), called a Nissen fundoplication. In this procedure the upper part of the stomach is wrapped around the lower end of the esophagus and stitched in place to provide resistance to gastric contents flowing up into the esophagus. This surgery is needed because the lower portion of the esophagus in infants and children born with EA does not have the normal muscle movements (peristalsis) to push fluids and food down the esophagus into the stomach. Unfortunately, attempts to increase the resistance of contents in the stomach from flowing back up into the esophagus also produce resistance for fluid and food going down the esophagus in the normal direction, which can result in difficulty swallowing.
Eating Normal Food
When will my child be able to eat normal foods?
Over a span of three to six months, children usually learn how to eat food by mouth. This is very dependent on the age when they started eating. Eating food seems to be an instinct until about 2 to 3 months of age. After that, learning to eat can be a challenge due to lack of an instinct or development of an oral aversion. This may partially account for the higher frequency of infants and children not eating normally in the reoperative group. They have often gone months, if not years, before they are first able to eat.
So far, 14 out of 19 patients who have received the Foker process as primary treatment are now eating all their meals by mouth. Five out of 22 of our patients who received the Foker process after receiving another form of treatment for EA are now eating by mouth. Others patients are expected to do so in subsequent months.