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Retrograde Crico-Pharyngeal
Dysfunction R-CPD
No-Burb Syndrome

R-CPD is a relatively recently defined syndrome where the upper muscle in the throat (the cricopharyngeus) does not relax when there is excessive pressure in the upper GI tract from gas. In other words, the “release valve” fails to operate and unwanted gas causes many of the commonly reported symptoms of nausea, bloating, chest / abdominal pressure and discomfort with a distinct inability to burb or belch. Some individuals also have problems vomiting while others only get relief once doing so. Patients can also have lower GI symptoms, including those mistaken for IBS (Irritable Bowel Syndrome).  Frequently, patients with R-CPD are misdiagnosed with IBS or told that they have a food allergy (lactose intolerance), heartburn (GERD) or Celiac Disease. Many doctors, including gastroenterologists are not yet familiar with the condition.

Carbonated beverages tend to be the most consistent and severe symptom trigger, but there are many others. Some R-CPD patients have discomfort when pressing over the front of the neck 3-4 inches below the Adam’s Apple where the cricopharyngeus muscle is located. Discomfort, however, neither confirms nor excludes a diagnosis of R-CPD.

What makes it retrograde?
A: The cricopharyngeus is also known as the upper esophageal sphincter. The muscle is essentially circular. Its primary job it to stay closed until you swallow and then relax to let the food pass down. If you had non-retrograde crico-pharyngeal dysfunction, then you would experience swallowing difficulties, especially with solid foods. This is something not usually seen in individuals under 50-60 years of age. So, it is a retrograde dysfunction because it occurs only when going in the opposite direction (up, rather than down), when the muscle is acting as a release valve for gas in the upper GI tract.

How is the diagnosis confirmed?
A: Currently, there is no confirmatory test, x-ray or examination for R-CPD. It is considered a clinical diagnosis of exclusion, which means that you would need to have someone knowledgeable and experienced with R-CPD to exclude other possible diagnoses. If your symptoms match those usually seen in R-CPD, and other potential causes for the symptoms are excluded and you respond to appropriate therapy, then you are assigned a diagnosis of R-CPD. Beforehand, it is only suspected clinically. R-CPD can impact both men and women, with an average age at diagnosis of 20 to 30, although all persons affected have known that they lacked the ability to burp their entire lives.

How is R-CPD treated?
A: BoTox! Patients with clinically suspected R-CPD are taken to the operating room for an examination under general anesthesia. A scope is placed in the mouth and the cricopharyngeus muscle is isolated from an endoscopic approach. A microscope is used to carefully inspect the anatomy and if everything looks appropriate, 50-60 units of BoTox are injected into the muscle at three locations. The circular muscle is also dilated to help the BoTox diffuse and stretch the cricopharyngeus. The entire procedure, including anesthesia time, is less than 60 minutes. Patients typically go home 1-2 hours later.

BoTox actually takes 3-7 days before it starts to work, however, some patients begin feeling “micro-burps” in the recovery room, later the same day or the following morning! This occurs because the dilation has a similar impact on the circular muscle as the BoTox by weakening its grip, only quicker and more temporarily. Having micro-burps is a very good sign, but not everyone gets them, so not to worry. Having micro-burps generally means that you are expected to be a full or partial responder to the BoTox (described in a later section).

What happens after the procedure?
A: Once you get home, most individuals will experience a mild sore throat. The tongue may be temporarily numb and / or the sense of taste could be off for awhile. This is from the pressure of the scope and breathing tube on the taste and sensation nerves in your tongue. You will not be placed on any new dietary restrictions, however, avoiding the things like carbonated beverages that gave you problems with the R-CPD beforehand should stay in place until the BoTox kicks in about a week afterwards. Once the BoTox starts to kick in, you will be instructed to test the things that previously gave you problems. At your two-week follow-up appointment, you will be asked whether there is anything that you can not consume, and if so, what symptoms do consumption cause.

What is a full or partial responder to BoTox mean?
A: Almost all R-CPD patients who have post-procedure micro-bumping in the first 24 hours have some degree of ultimate response to the BoTox. If your symptoms improve after the BoTox kicks in, and never return, you are a full-responder. If your symptoms improve after the BoTox kicks in but then some or all of them return 3-12 months later, you are a partial-responder. If you had no micro-burbs after the procedure and no positive response to the BoTox by 2 weeks, you may be a BoTox non-responder, although this determination is made on a case-by-case basis.

If Botox is a temporary medication, how can it permanently cure R-CPD in some patients?
A: The effects of BoTox last only 2-6 months (3 months, on average) and then disappear. The theory is that R-CPD is a neuromuscular dysfunction, which means that the release valve reflex is not responding to distention in the upper GI tract. BoTox is thought (still just a theory) to reset that neuromuscular reflex, and once doing so, is no longer needed for the reflex to function properly moving forward. Think of it as a “hard reset” of the R-CPD reflex. In partial-responders, it is thought that the BoTox only does a “soft reset” of the R-CPD reflex. In these cases, a second procedure is done with a higher BoTox dose, 3-12 months after the first procedure, depending on how quickly or slowly R-CPD symptoms return. Non-responders, as mentioned, are considered on a case-by-case basis.

What has your experience been with R-CPD BoTox injections?
A: Approximately 60-65% of patients are one-and-done full responders if R-CPD is highly suspected. Around 20-30% end up being partial responders and require a second BoTox procedure. Less than 5-10% are ultimately determined to be non-responders or the injection could not be safely completed in the operating room for technical reasons, primarily due to patient anatomy (small mouth, short or thick neck with limited mobility, poor dentition, etcetera). There are other options for these people.

What are the risks involved with the procedure?
A: The procedure is performed through the mouth, so there is a risk for a chipped or broken tooth or teeth. There is a very, very small risk of causing a perforation of the swallowing tube. Sore throat, mouth and TMJ pain are not uncommon and usually temporary. Taste may also be altered for several weeks afterwards. The most common risk is that the clinical diagnosis of R-CPD is incorrect and you may not respond to the BoTox at all. These risks are not a comprehensive list. A careful discussion of the pros and cons, risks and benefits of the procedure occur during the initial consultation.

Is the procedure experimental? Does insurance cover the procedure?
A: No, it is not experimental. Yes, your insurance will cover the procedure minus your co-pay and / or deductible for an operation. To determine your personal out-of pocket cost, telephone your insurance company and inquire. They will ask you for the “CPT Codes” for the R-CPD BoTox procedure, which are: 31571, 43200 and 43450. There may also be a charge for the BoTox medication, depending on your personal insurance coverage. We recommend asking if the BoTox (code J0585) will be covered or not.

How many of these procedures does Dr. Haben perform in a year?
A: A lot.

Who is Dr. Haben?
A: The Center is dedicated to laryngeal surgery. Dr. Haben is Board Certified in otolaryngology (Ears, Nose and Throat) and fellowship trained in laryngology (throat, specifically). He has also earned a master's degree from McGill University for his research in laryngology. Dr. Haben routinely lectures and publishes on laryngeal surgery and related topics. His complete CV may be found elsewhere on the website.