Long Gap Discussion: EA & TEF

The diaphragm of an infant can be pushed all the way up to the upper part of the chest cavity without causing damage or perforation during blind gap measurement, leading to errors in measuring the gap.

The gastroesophageal junction in pure long-gap esophageal atresia is very tiny, and even under direct vision with a scope, manipulation is required because the scope tends to flip out of the area.

Using an endoscope for gap measurement does not allow objective measurement of the boost force applied to the endoscope.

Gap measurement with a Hegar dilator (number 4, no more) should be performed at least 15 days after gastrostomy placement, after endoscopic confirmation of the cardia location.

Some babies with esophageal atresia have no cardia at all.

A contrast study at 3 weeks after gastrostomy placement may not fill the distal esophageal segment completely even in Trendelenburg position, giving a false sense of the real gap.

For long-gap EA with gasless abdomen, one approach is to wait a couple of weeks for gap assessment (using a Hegar or urethral dilator under fluoroscopic control), and if the gap is more than 5-6 vertebrae at 4-6 weeks, opt for esophageal replacement rather than immediate cervical esophagostomy.

One institutional approach is laparoscopic gastrostomy placement followed by gap measurement every 2 weeks until the gap is less than 2 vertebral bodies; if not achieved by 8 weeks, esophageal replacement is considered.

For inborn babies with long-gap EA, one approach is gastrostomy at birth, gap measurement every 15 days until 6 weeks, at which point delayed anastomosis is generally possible.

One surgeon's approach includes endoscoping all long-gap EA patients to intubate the lower esophageal sphincter and assess both the length and quality of the lumen.

In one approach, the fistula is clipped and left attached during the waiting period, with awareness that it may recanalize.

If endoscopic assessment shows the gap is less than 3 vertebral bodies or centimeters, primary repair is pursued; if not, alternative strategies are considered.

One institutional approach uses interventional radiology for G-tube placement and gap assessment via a wire threaded through the G-tube into the esophagus, with monthly gap checks for up to 3 months.

An unstressed gappogram (contrast above and into the lower end) provides an idea of the gap without pushing structures, avoiding the deceptive results of stressed measurement.

If at operation the two esophageal ends are dissected and pulled together but tension is judged excessive, internal traction can be applied for one week, after which significant growth is achieved and anastomosis becomes feasible.

Even very small distal esophageal ends (2-4 millimeters) with all anatomic information present will grow into a serviceable esophagus when placed on traction.

A case from Boston initially reported a 2-centimeter gap, but this was due to pushing the diaphragm into the chest; the actual distal esophagus was only a 5-6 millimeter nubbin.