What your Insurance Company might ask about R-CPD (Inability to Burp, No-Burp)

The information below is offered to both patients and their physicians. It can be sent to their insurers or may help them explain the proposed procedure to payors during the pre-authorization process.

What is the diagnosis for which this procedure is to be done?

Retrograde cricopharyngeus dysfunction (R-CPD) causing lifelong inability to belch, with severe associated gastrointestinal symptoms like bloating, embarrassing gurgling noises, flatulence, painful hiccups, nausea after eating, and several others.

Can you summarize what R-CPD is?

The cricopharyngeus muscle is the main component of the upper esophageal sphincter. It is located at the junction between the throat and the esophagus, behind the lower part of the voicebox. That sphincter relaxes normally for each swallow of liquid or food (antegrade relaxation). But it refuses to relax in the opposite (retrograde) direction to permit burping (and sometimes vomiting).

Inability to burp when needed is already a significant discomfort by itself. But the follow-on symptoms as additional air is swallowed reflexively, are much worse:

• Gurgling noises as the stomach and esophagus churn air upwards to be burped, to no avail;
• Abdominal bloating, often with distention;
• Sometimes, chest and low-neck pressure caused by ballooning of the esophagus with air that cannot be burped;
• Remarkable flatulence especially after meals and late in the day/through the night.

And there are some common but less universal symptoms:

• Painful hiccups
• A feeling of being so full that it impossible to complete a deep breath
• Nausea after eating
• Hypersalivation
• Constipation
• Rapid heartbeat, flushing, dizziness.

Why doesn’t this diagnosis and procedure show up in billing and coding manuals?

R-CPD has been until recently an orphan disorder. It was first codified as a syndrome, and successfully treated by injecting botulinum toxin into the sphincter in 2015 by Robert W. Bastian, MD. His original series of 51 patients was published in 2019.

He has subsequently coauthored 4 additional articles. He has also shared his information with many other doctors in this country individually and in educational conferences. He has also shared his work out-of-country: Originally in England, then Canada, and now in Europe, Australia, Asia, and Africa.

Others have also published successful series. As of June 2024, Dr. Bastian’s group has successfully treated nearly 1,800 patients from 50 states and 26 countries. And so, billing and coding manuals are simply behind the curve…

What more can you tell me about the literature on the subject?

There are three individual case reports (see references below) partially describing the disorder, though not using the term R-CPD. Each report describes the problem as severe and proposes theoretical treatments (not including botulinum toxin). None, however, reports successful treatment. Here they are:

1. Kahrilas PJ, Dodds WJ, Hogan WJ. Dysfunction of the belch reflex. A cause of incapacitating chest pain. Gastroenterology. 1987 Oct;93(4):818-22. doi: 10.1016/0016-5085(87)90445-8. PMID: 3623025.
2. Waterman DC, Castell DO. Chest pain and inability to belch. Gastroenterology. 1989 Jan;96(1):274-5. doi: 10.1016/0016-5085(89)90822-6. PMID: 2909435.
3. Tomizawa M, Kusano M, Aoki T, Ohashi S, Kawamura O, Sekiguchi T, Mori M. A case of inability to belch. J Gastroenterol Hepatol. 2001 Mar;16(3):349-51. doi: 10.1046/j.1440-1746.2001.02333.x. PMID: 11339431.

Dr. Bastian first codified the diagnostic syndrome in a 2019 manuscript that describes his experience with 51 patients who have already undergone successful botulinum toxin injection. That article was then updated with additional data and information in 2020. He has also put detailed information on his teaching and public service website here on Laryngopedia. Here are those articles:

1. Partial Cricopharyngeal Myotomy for Treatment of Retrograde Cricopharyngeal Dysfunction Read more →
2. The Long-term Efficacy of Botulinum Toxin Injection to Treat Retrograde Cricopharyngeus Dysfunction Read more →
3. Efficacy and Safety of Electromyography-Guided Injection of Botulinum Toxin to Treat Retrograde Cricopharyngeus Dysfunction Read more →
4. Retrograde Cricopharyngeus Dysfunction: An Orphan Disease? Read more →

And finally, publications have also appeared by follow-on authors in other places: Here is a list, possibly not exhaustive:

1. Pavesi L, Balzano C, Mauramati S, Giudice C, Fresia M, Todisco M, Alfonsi E, Cosentino G. Retrograde Cricopharyngeus Dysfunction effectively treated with low dose botulinum toxin. A case report from Italy. Front Neurol. 2023 Aug 9;14:1238304. doi: 10.3389/fneur.2023.1238304. PMID: 37621856; PMCID: PMC10444988.
2. Hoffman MR, Schiffer B, Patel RA, Smith ME. “I’ve never been able to burp”: Preliminary description of retrograde cricopharyngeal dysfunction in children. Int J Pediatr Otorhinolaryngol. 2022 Oct;161:111261. doi: 10.1016/j.ijporl.2022.111261. Epub 2022 Aug 4. PMID: 35939873.
3. Karagama Y. Abelchia: inability to belch/burp-a new disorder? Retrograde cricopharyngeal dysfunction (RCPD). Eur Arch Otorhinolaryngol. 2021 Dec;278(12):5087-5091. doi: 10.1007/s00405-021-06790-w. Epub 2021 Apr 24. PMID: 33893849; PMCID: PMC8553696.
4. Arnaert S, Arts J, Raymenants K, Baert F, Delsupehe K. Retrograde Cricopharyngeus Dysfunction, a New Motility Disorder: Single Center Case Series and Treatment Results. J Neurogastroenterol Motil. 2024 Apr 30;30(2):177-183. doi: 10.5056/jnm23099. PMID: 38576368; PMCID: PMC10999848.
5. Sato H, Ikarashi S, Terai S. A Rare Case Involving the Inability to Belch. Intern Med. 2019 Apr 1;58(7):929-931. doi: 10.2169/internalmedicine.1908-18. Epub 2018 Nov 19. PMID: 30449811; PMCID: PMC6478983.

Why does this syndrome happen?

The underlying cause is not known but is clearly identified from birth in some but not all patients with R-CPD. Their parents describe inability to burp their child after nursing, and often, colic, gassiness, and projectile vomiting. We don’t think the problem is structural or neurological, because the cricopharyngeus muscle functions normally for swallowing (antegrade relaxation). That is, it relaxes momentarily to permit passage of food or liquid from throat to upper esophagus. And the muscle seems “trainable” since approximately 80% of those who are treated continue to burp long after the botulinum toxin has worn off.

Hence we call botulinum toxin injection “training wheels for burping.”

What is the magnitude of effect on quality of life and sense of well-being?

When asked to indicate severity on a 7-point (maximum) scale, virtually every patient (of a total of nearly 1,800 as of June 2024) have chosen either 6 or 7. When we use the descriptor “severe daily misery,” most patients nod their heads in agreement. Another indicator of motivation is the distance patients are willing to travel to get help for this condition. So far, (June 2024) we have had patients from 50 states and 26 foreign countries.

How is the diagnosis proven?

First, we should note that in our series of nearly 1,800 patients (June 2024), only one or two patients have arrived with this diagnosis from another physician. This is in spite of having seen numerous physicians and having undergone numerous diagnostic tests, including esophagoscopy, manometry, barium swallow studies, gastric emptying, colonoscopy, stool cultures, and empiric trials of acid-reducing pills, simethicone, antispasmodics, etc.

One woman had seen 12 different gastroenterologists without being given a clear diagnosis or any efficacious treatment. Contrast that with the fact that match with the syndrome provides as close to 100% diagnostic accuracy as one ever sees in medical practice. Again, the syndrome is: Most or all of the primary symptoms:

1. Inability to belch at all or “enough”;
2. Gurgling noises as the stomach/ esophagus churn attempting to expel air;
3. Abdominal/chest pain and bloating;
4. Excessive flatulence.

And some combination of the other symptoms already mentioned: nausea after eating, hiccups, hypersalivation, inability to breathe deeply when bloated, constipation, etc.

What is the purpose of the botulinum toxin injection?

The medication introduces temporary flaccidity to the upper esophageal sphincter so that it cannot stop air coming upwards from the stomach. This in turn resolves the other symptoms that comprise the syndrome of R-CPD.

Stated more formally, the benefits are three:

1. Botulinum toxin is a diagnostic test. In our series to date, all but one or two (special case) patients diagnosed syndromically with R-CPD have experienced dramatic relief of symptoms after botulinum toxin injection.
2. It provides symptom relief and is therefore therapeutic.
3. The above-mentioned “training wheels” function: a clear majority of patients seem to keep the ability to belch on a permanent basis. The longest so far is the index case who was treated in 2015 continues to burp normally, even though his inability to belch was lifelong, to his earliest memories.

Why must this be done under brief outpatient anesthesia?

It can be done via EMG in an office setting and Dr. Bastian has done many patients in this way—especially those who live within a few hours’ drive from his office. That approach works well routinely. Still, it is uncomfortable; not something some patients are willing to do; and the muscle is not seen directly. The placement is done by using surface landmarks, surgeon “visualization” and with EMG confirmation. Furthermore, side effects on voice and laryngospasm seem slightly higher with this technique. And then remember that most patients travel a long distance for this procedure, because they cannot find a local physician who understands this disorder and is willing to treat it.

Consequently, most prefer the extremely brief esophagoscopy to directly see the muscle bulge prior to injection. That method works “every time.”^††

What is the expected cost if paying out of pocket from another country?

Through Bastian Voice Institute, the total cost (2024) is approximately $4,000. For the EMG method, the cost is approximately half of that. We have learned from our patients that costs elsewhere are inexplicably much higher…as much as $25,000 in one location!

What are the CPT codes associated with this kind of patient and procedure?

• 43192 – Rigid esophagoscopy
• J0585 – Botulinum Toxin (Patients often pay for this out of pocket as insurers often refuse to pay, or they “reimburse” a tiny fraction of the actual cost of this medication.)

What are the diagnosis codes associated with this kind of patient and procedure?

• R14.2 – Eructation disorder
• K22.4 – Dyskinesia of esophagus
• R14.0 – Abdominal distension

†† One patient (curiously) did not burp after injection #1 but has been burping well for several years after his second injection. A couple of others have responded only partially.