Looking for Zebras

Wednesday morning we did an esophagram. Its a real time x-ray taken while the patient swallows a barium contrast in order to watch the progress of her swallow and see the anatomy of her esophagus and upper GI tract.

One thing to look for was a tracheoesophageal fistula, which is an abnormal connection between the trachea and esophagus.  Presence of milk in the trachea would explain why Hannah's airway closes and she loses consciousness when eating.  The attending doctor said TEF's are extremely rare and unlikely.  He said its like looking for zebras in The United States...you just aren't going to find them.

Sure enough, no fistula. There was however the acid reflux as the first doctor had said the day before.

But Hannah's swallow was far from normal.  I watched the radiology techs literally scratch their heads during the esophagram.  I didn't know what normal looked like, so I had no idea what to expect.

The pediatric ENT surgeon, Dr. Schramm visited our hospital room to tell us what the x-ray had found and give us the next steps. The x-ray showed a cricopharyngeal bar- the sphincter muscle at the top of Hannah's esophagus was abnormally large, and failing to contract and relax properly during the swallowing process. The muscle is in the way, creating a significant narrowing of her esophagus, which was causing milk to pool above the bar rather than drain down at the appropriate rate.  As the milk pools it can spill over and go down the trachea, which tries to stop it by closing or spasming, which stops her breathing. That's why she passed out and turned blue whenever she tried to swallow.

This condition is called cricopharyngeal achalasia.  And guess what? Its way more rare than a fistula. Zebras, indeed!  I like to think of it more as a Space Monkey.  Dr. Schramm had seen this particular condition only a few times, and usually as an acquired condition in elderly people.  He consulted with a colleage at Primary Children's, and he had only seen 5 neonatal cases in his 25+ years of practicing medicine.

The treatment options we were given are 1. Botox 2. Balloon dilation 3. Myotomy

I know what you're thinking: botox!? Isn't that for facelifts?  Botox is used to paralyze a muscle, and in this case would be used on the cricopharyngeus to keep it from spasming and allow it to relax and get out of the way.  A dilation means making something wider. A balloon is inserted into the esophagus and "inflated" to open up the esophageal canal. And a mytomy is a procedure in which the muscle is cut. These options were given in order of least invasive to most invasive.

After getting the results, we were given time to process and consider the options. Surgery was scheduled for 1:00pm the following day, even though we weren't yet sure which procedure we would do.  Hannah was allowed to get some nutrition through an NG tube, but only for a little while, as she had to have an empty stomach for surgery.

At about this point I was feeling relieved to know that I hadn't been imaging that there was a problem, and I was so glad to have a name to go with it!  But it is kind of weird to be in the hospital with a baby who isn't actually sick-she looks and sounds just fine, and as long as she isn't trying to eat, she is. But she really isn't fine.  The advantage to having a rare and bizarre congenital condition (if you can call anything about this advantageous), is that the only hits on google searches are for medical journals rather than buzzfeed or a mommy forum.