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Treatment for Long Gap Esophageal Atresia: Esophageal Elongation and Replacement
With Dr. Daniel von Allmen · hosted by Dr. Rod Gerardo
Chapter 1 of 6 · Fundamentals
Gap measurement
Introduction and Gap Measurement Protocol
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
At Cincinnati Children's, if a child has no gas in the abdomen, they place a G-tube and may put something up the distal esophagus for a fluoro shot, then wait a couple of weeks for a protocol gap measurement in interventional radiology.
Stretch is a very strong promoter of growth, and if you put things on tension they will actually grow over time, which is how the cardiovascular system develops in utero.
The philosophy of traction-based elongation is that with traction, you can get the two ends of the esophagus to grow, and if you can get them to grow far enough, you can put them together.
The colon can be used as an interposition for esophageal replacement.
Common problems with colonic interposition are that the colon can dilate and become tortuous, and it's not uncommon to get a sigmoid sink drain deformity just above the diaphragm.
Von Allman was initially taught that sigmoid redundancy in colonic interposition can't be fixed and that it's too dangerous because it will risk the blood supply, but he found that this is not really true.
Von Allman passes the colonic interposition posterior to the stomach, which leaves the vascular pedicle along the spine, allowing mobilization of the colon by dividing the gastric duodenotomy and colon attachment to the stomach, then mobilizing the sigmoid redundancy transhiatally and reanastomosing the colon to the stomach.
Colonic interpositions sometimes need to be revised, but it is not impossible to revise them and the kids tend to do pretty well.
You can get extraordinary length with colonic interposition, allowing treatment of cases with caustic injuries extending to the pharynx by sewing the colon to the pharynx and down to the stomach, which is tough to do with a gastric pull-up.
Von Allman states we don't know whether esophageal elongation is growth or stretch and should do studies to understand that, but notes that tension is a very good physiologic growth promoter in other organs, making this an area ripe for more basic science.
In the Foker technique, the surgeon ties sutures to either end of the esophagus through an open incision, brings them out through the chest wall, ties them together in a knot, and places spacers underneath periodically (about every day) until the ends come together for primary anastomosis.
The van der Zee technique uses the same concept as Foker but is done thoracoscopically with no external sutures, so all tension is inside the thorax.
The Kimura technique involves creating a spit fistula and periodically moving it down the chest wall over time to stretch the proximal pouch closer to the distal pouch, but is not used all that often anymore.
In the Boston Group 2015 study, the primary group (de novo cases) achieved an intact esophagus in 96% of patients, while the secondary group (patients with previous operations) achieved this in about two thirds of patients.
In the Boston Group 2015 study, the primary group had a median ICU stay of 70 days with a couple of weeks being paralyzed, and the secondary group had a median ICU stay of 110 days with a month of being paralyzed.
In the Boston Group 2015 study, about two thirds of patients with primary repair were able to get full oral nutrition and about 10% of patients who had secondary repair achieved full oral nutrition.
The surgical group from INOEA recommends gastric pull up as the first option for esophageal replacement, dividing the esophageal stump at the esophageal hiatus and mobilizing the fundus to pull it up in either the anterior or posterior mediastinum.
In colonic interposition, the piece of colon chosen is based on the blood supply and the diameter needed.
In colonic interposition, a pyloroplasty is performed to help with gastric emptying.
The short interval between time of traction and anastomosis (less than five days) raises the question of whether the mechanism is stretching or growth, which will impact results and likely reflects on complications.