On Motility Issues

When we had our consultation with the pediatric surgeon, we brought up the fact that Nolan had shown delayed gastric emptying on an earlier test (called a scintiscan). The surgeon reassured us that the majority of kids with reflux appear to have delayed gastric emptying because the food goes up as well as down, resulting a a delayed time. Once the fundoplication is performed, the food can only go down and motility "improves." This was a reassuring thought and we figured the delayed emptying was simply an artifact on the test because of his reflux.

When Dr. Wonderful called me at home the other night, he told me to forget everything he told me about motility. The aforementioned is true for kids with regular, straightforward severe reflux. It is not true for Nolan.

Motility issues have brought everything to a grinding halt.

From the few tests that have been performed, it is apparent that Nolan has two motility issues (one definite, the other possible):

1) Delayed Gastric Emptying, or Gastroparesis
2) Esophageal Motility Problems

Nolan's scintiscan showed 0 episodes of reflux, but significant delayed gastric emptying. In this case, there was no reflux to create a "false positive" result. He truly has delayed gastric emptying, and it is possibly the underlying cause to the ridiculous levels of reflux.

Nolan's 24 hour impedance probe (from when he was 2 years old) showed a slow "bolus transit time" in his esophagus. This means that food took too long to get from his mouth to his stomach.

So, why are motility problems important?

If Nolan had a Nissen Fundoplication without addressing the motility, several things could go awry. His stomach would not empty fast enough, and with the top of the stomach "tied off," he could get painfully bloated. The potential esophageal motility issues are more worrisome - with a Nissen, a child could actually lose the ability to swallow food if there are esophageal motility problems.

We are going to have a repeat gastric emptying scintiscan done at Golisano Children's Hospital. If he still shows gastroparesis, then a pyloroplasty will be performed alongside the fundoplication. A pyloroplasty is the same surgery as performed on children with pyloric stenosis - it is a cut into the bottom sphincter of the stomach to allow food to empty faster. This comes with its own set of risks and benefits, which is why they don't perform this routinely with Nissen Fundoplications - it is reserved for kids with gastric emptying problems. A g-tube is always used for kids with delayed emptying with a fundoplication, to allow venting of stomach contents or air, in the event that the stomach can't empty itself fast enough.

There isn't much to be done about esophageal motility problems. This is usually an absolute contra-indication to a Nissen fundoplication. There is a test for esophageal motility, but it is quite invasive (the test is called esophageal manometry).

Anyhow, there are a few decent articles on motility problems in children. I'm linking them here for my own reference, but also to help others understand the difference between "typical reflux" and "reflux with motility issues."

http://www.articles.complexchild.com/00005.pdf: A great, plain-English description of various motility problems in children.

http://www.articles.complexchild.com/00028.pdf: A great article about the difference between "regular" reflux and reflux with motility issues.

http://www.nature.com/gimo/contents/pt1/full/gimo46.html: An article about the difference between LPR and GERD types of reflux (Nolan has LPR reflux)

Article on gastroparesis: http://www.usagiedu.com/articles/gastrop/gastrop.pdf

And then there is this little gem, about understanding the surgical treatment of reflux in the presence of delayed gastric emptying: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1422000/

"the existence of delayed gastric emptying should not be a contraindication for a Nissen fundoplication. Second, those who do operations other than a Nissen fundoplication for the treatment of reflux may wish to consider a fundoplication in the group of patients found to have abnormal emptying, as this operation is the only one that clearly improves gastric emptying. Third, in patients with delayed emptying the surgeon should not advocate a pyloroplasty, but simply a fundoplication. This recommendation, while indirectly supported by the results of this study, stems from a number of other observations. First, pyloroplasty increases duodenogastric reflux, which may damage the gastric mucosa or worsen esophagitis if the cardia remains incompetent. Second, pyloroplasty without vagotomy is ineffective in the treatment of idiopathic or diabetic gastroparesis regardless of its effects on gastric emptying. Lastly, this study and the preponderance of evidence from other studies suggest that there is a good chance that gastric emptying will be normalized after a Nissen fundoplication. If that does not occur, there is always time to reassess the situation and devise a new strategy to deal with the problem."

What is a Fundo-Majigger, Anyway?

The surgery recommended to aid in controlling Nolan's acid reflux is called a fundoplication. Essentially, the surgeon takes the top part of the stomach (the fundus) and wraps it around the esophagus. This tightens the lower esophageal sphincter, and helps to prevent reflux. It is a remarkable cure for reflux for some children.

So why would we question its necessity?

For some children, the quality of life is worse after fundoplication. Depending on how tight the wrap is, kids may not be able to burp or vomit. This can lead to a painful condition called gas bloat, particularly for children who do not have a g-tube. Retching is another side effect that can occur - some children get the dry heaves after the procedure, and it is extremely debilitating. Kids have to go on a liquid diet for a period after the surgery, then they are advanced to a pureed diet - after several weeks, carefully chewed solid food is allowed. Because the lower esophageal sphincter is tight, some children may choke or be unable to swallow larger lumps of food.

Most of the kids who have negative side effects are those with pre-existing motility issues: the peristaltic action in the stomach or esophagus is reduced.

We know Nolan has borderline/mild delayed gastric emptying. There is some question as to the accuracy of the test he had performed, since his gastric emptying scintiscan was not performed with one of the foods with a known digestion time. In addition, the definition for pediatric delayed gastric emptying varies widely by clinic. Nolan had about 25% emptying after one hour - in our area, that is considered mild. In others, that would be considered flat-out delayed emptying.

His first impedance probe also showed a slowed bolus transit time for his esophagus. This means that food doesn't pass efficiently from his mouth to his stomach, and a fundoplication could cause this situation to worsen in intensity. Here is a story of a child who had a fundoplication with undiagnosed motility problems, and she had several complications post-fundoplication.

There are very few motility centers in the United States. There is one in Boston, and one in Cincinatti. Prior to consenting to a fundoplication, we must have motility testing performed to verify that Nolan's reflux is "uncomplicated." For many children, the motility disorder is the cause of the reflux - and motility medications can be added to increase the peristaltic contractions. This helps to reduce the amount of reflux occuring.

Kids who have one fundoplication performed are at risk for the wrap slipping and causing a hiatal hernia, which requires another fundoplication - the procedure is irreversible, and the potential for more surgeries down the line remains.

Added to the motility concerns is the possibility that Nolan's hypopneas are caused by his low muscle tone, and not because of the reflux. Children with low muscle tone (hypotonia) are prone to obstructive apnea. In this case, a BiPap would be the best solution for his apnea. This is why we have booked an appointment with his sleep neurologist, to discuss his opinion on Nolan's tone and the potential efficacy of a BiPap machine.

If we finish our investigation and find that 1) Nolan has no underlying condition, 2) there is not a complicating motility problem, and 3) the only way to help the apnea is to do a fundoplication, then we will move forward with the procedure. Until then, we have a lot of research to do to ensure the best decisions are made for Nolan.