Tracheoesophageal Fistula

By Meg Marinis, Director of Medical Research Nov 10, 2011
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So we sort of left you hanging, didn't we? 

Who are you more worried about? Meredith and Alex outside on an extremely dangerous curve of the road? Or the super sick baby that Meredith's holding? Or are you stressing out about the bleeding family that they just found from the accident aftermath? What about the young mother Laura that's in surgery because she suffered a pericardial tear from an orthopedic screw? Or Teddy, while she operates on Laura, having NO idea that her husband has just died in surgery?

Yeah, we left you hanging. Which means… I can tell you NOTHING. Well, no… That's not entirely right. I can tell you that Meredith and Alex need to get that baby to Seattle Grace for surgery as soon as possible.

Meredith and Alex need to transport the baby to Seattle Grace because she suffers from a congenital defect: tracheoesophageal fistula (TEF).

First, let's clarify the difference between the esophagus and the trachea: the esophagus is the tube that leads from the throat to the stomach (feeding tube), and the trachea is the tube that goes from the throat to the windpipe and lungs (breathing tube). During the development of the fetus, the esophagus and trachea begin as one tube. At approximately four to eight weeks after conception, a wall forms, separating the tube into two distinct entities. If that wall never forms, a tracheoesophageal fistula occurs – an abnormal connection between the esophagus and the trachea.

Babies with TEF need prompt intervention because the defect can cause life-threatening problems. For example, when the baby tries to swallow liquid, it will pass into the lungs, causing pneumonia and other breathing issues. And when the baby inhales, the air will instead pass through the esophagus and continue into the stomach.

Symptoms of a TEF usually present shortly after birth. Signs include:

- Coughing or choking while feeding.
- Vomiting.
- Frothy white bubbles around the mouth.
- Difficulty breathing.
- Blue color to the skin, especially while feeding.
- Round and full abdomen.

If doctors suspect TEF, they will perform a physical exam, medical history, and X-rays of the chest and abdomen. Also, a small tube can be placed into the mouth or nose and then guided into the esophagus; if there is a TEF, the tube cannot be inserted very far into the esophagus. The tube will coil in the chest and never reach the stomach.

What did Arizona tell Alex to do while at the other hospital? Why did it help?

Alex inserted a tube with a balloon at the end of it that can be blown up when it's in place. He put it into the esophagus as far as he could, blew up the balloon, and then pulled back until it stopped. This maneuver temporarily closed off the fistula so stomach contents would not get into the lungs, and the baby could breathe better.

These babies need surgery soon after birth so that normal swallowing and digestion of food can be established.

Surgery always depends on the type of fistula and its location, but generally an incision will be made in the right side of the chest, so that the fistula can be resected. Then, the two ends of the connection will be sutured together. Sometimes the surgery needs to be completed over two stages due to the severity of the baby's illness. In these instances, doctors will first place a tube into the baby's stomach (called a gastrostomy tube) to decompress the stomach and prevent fluid from flowing up from the stomach into the esophagus, through the fistula, and into the lungs.

Surgical complications include recurrence of the fistula, leak of contents at the point of anastomosis, or stricture due to gastric acid erosion of a shortened esophagus.

TEF is not thought to be an inherited disorder, but several babies with TEF possess other congenital defects, such as:

- Trisomy 13, 18, or 21.
- Other digestive tract problems (diaphragmatic hernia, duodenal atresia, imperforate anus).
- Heart problems (ventricular septal defect, tetralogy of Fallot, or patent ductus arteriosus).
- Kidney and urinary tract problems (horseshoe or polycystic kidney, absent kidney, hypospadias).
- Muscular or skeletal problems.
- VATERL syndrome (involves vertebral, anal, cardiac, TE fistula, limb abnormalities).

For more information on TEF, please visit the following:

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0004767/