The Esophagus

Scarring may develop in the esophagus as a result of injury and blistering and often results in the formation of esophageal strictures. An esophageal stricture is an abnormal narrowing of the esophagus and has been reported in almost 60 percent of individuals with recessive dystrophic EB.

The first indication that there may be a problem in the esophagus is slow eating, difficulty swallowing (dysphagia) and choking. A preference for soft foods may be noted, as well. If these potential side effects pass within a day or two, a blister most likely was present before it drained and left the individual feeling a bit sore but able to swallow. Cold fluids and foods such as milkshakes or ice cream may be comforting, relieve pain, and decrease swelling and inflammation in the area.

If choking and difficulty swallowing persists or worsens during a period of days and weeks, an esophageal stricture may be present. A barium swallow, ordered by the physician, will confirm the presence of a stricture.

A barium swallow is a test that uses an X-ray to examine the esophagus. Barium is a liquid that shows up on X-ray film. The patient drinks a barium preparation, which looks like a milkshake but has a chalky consistency and taste. The barium temporarily coats the lining of the throat and esophagus, providing an outline that will be visible on X-rays. X-rays are taken as the barium passes down the throat and esophagus. If a stricture is present, it will be visible on the X-rays.

When ordering a barium swallow, the order should specify: “In evaluating an EB patient for stricture, it is important to include the request for an evaluation of the entire esophagus from the oropharynx through the gastroesophageal junction, as significant strictures occur in the proximal cervical esophagus and can be overlooked in a less thorough exam.”

After the test, the person should drink a lot of water to help clear the barium and prevent constipation. Stools will be chalky and light-colored for two or three days after the test. If the person does not pass the barium, it can harden and block the bowel. Retaining barium also may lead to dehydration. To avoid these problems, be certain the barium is passed in the bowel movement. A mild laxative may be needed.

If a stricture is seen on the barium swallow X-rays, an esophageal dilatation may be needed. This procedure usually is done in the operating room under general anesthesia. During the procedure, a balloon catheter is placed into the esophagus and, using X-rays, the physician locates the narrowing and dilates (stretches) the stricture with the inflated balloon catheter. The procedure usually takes less than an hour, and an overnight hospital stay usually is not required.

A clear liquid diet will be offered after the effects of the anesthesia have worn off. Swallowing cold fluids provides comfort and may alleviate swelling. After fluids are tolerated without difficulty, a soft diet, then a regular diet, may be taken, as ordered by the physician.

As with the skin, blisters and erosions most likely will recur in the esophagus, and strictures may develop again. Barium swallows and esophageal dilatations may need to be repeated.