Retrograde cricopharyngeal dysfunction
Retrograde cricopharyngeus dysfunction (R-CPD; also known as the inability to belch syndrome) is a medical condition first identified by gastroenterologist, Dr Peter Kahrilas, in 1987.[1] However, the condition only began to become more well-recognized amongst patients and some medical specialists following a 2019 report by Dr Robert Bastian which described a very high rate of symptomatic relief from injection of botulinum toxin into the cricopharyngeus muscle.[2] Uniquely, awareness of the condition has spread predominantly through patients themselves rather than the medical community via numerous social media forums, the most prominent being the "No Burp" subreddit.[3][4] Many patients report poor awareness of the condition amongst primary care physicians and specialists and needing to seek out specialists who can treat the condition through online research.[5][6]
Symptoms
[edit]The condition is characterised by chronic gastrointestinal symptoms relating to excessive gas retention including abdominal bloating with distension, flatulence, audible gurgling sounds, and chest and abdominal discomfort.[2] Some with the condition are also unable to vomit, or can only do so with great difficulty. In most cases, inability to belch is a lifelong problem.[7] R-CPD has a significant detrimental impact on patients' quality of life.[6][8]
Anatomy and Pathophysiology
[edit]The cricopharyngeus muscle is the major muscle comprising the upper esophageal sphincter. It is a strap-like, C-shaped muscle at the base of the throat, behind the larynx. The upper esophageal sphincter encircles the proximal (upper) opening of the oesophagus.[9] The upper esophageal sphincter has a residual tone which is in fact augmented as it is stretched open.[10]
Swallowing of air during eating and drinking is normal. However, as the air cannot be absorbed by the gastrointestinal tract, it is mostly vented via burping. A transient relaxation of the lower esophageal sphincter allows swallowed air in the stomach to rise into the esophagus where it triggers a reflex relaxation and opening of the UES. In R-CPD, all steps of this 'belch reflex' occur normally up until the last one; despite gaseous distension of the esophagus, the UES fails to open. It is believed that all the symptoms of R-CPD occur due to failed expulsion of swallowed air, and thereby buildup of gas throughout the entire gastrointestinal tract.[11]
Diagnosis
[edit]Many patients have R-CPD suffered from years of delayed diagnosis and misdiagnosis, and unsuccessful empiric treatment of gastrointestinal disorders including GERD, aerophagia, gallbladder dysfunction, and IBS.[6] Patients with R-CPD often undergo a battery of tests including nasendoscopy, gastrointestinal endoscopy, and barium swallow; which are almost always unrevealing.
In case where symptoms are classical, some specialists choose to treat the condition without formal diagnostic testing. However, physicians from the Netherlands and Australia have validated a specific diagnostic protocol using esophageal high-resolution manometry which is highly accurate in obtaining a conclusive diagnosis of R-CPD.[12][7]
Treatments
[edit]The treatment of choice is injection of Botulinum toxin into the cricopharyngeus muscle.[2] The effect of Botulinum toxin is to temporarily weaken or paralyze the muscle. Reported success rates for inducing the ability to belch are extremely high, ranging from 88-92%.[13] The effect of botox is delayed for three days on average and most patients with successful results report the ability to burp by the fifth day after the procedure. For most, the effect will last beyond the first three to six months of direct Botox effect and is most frequently a lifelong cure.[13] A small percentage of patients will require a subsequent injection of Botox for lasting results.[14] An alternative if the injection is unsuccessful is to undergo partial cricopharyngeal myotomy.[15]
Method of Injection
[edit]Most commonly, injection is performed by direct (rigid) laryngoscope, under general anesthesia. An injection technique via flexible gastrointestinal endoscopy has also been described, which appears to have equivalent high success rates.[7] Injection can also be performed by a percutaneous (directly through the skin) approach, though success rates are lower.[16]
Side effects of Treatment
[edit]Being related to the temporary effect of the Botulinum toxin, side effects are also temporary and typically resolve without treatment. The most common side effect is difficulty swallowing which often requires temporary diet modification and altered eating style. Less common side effects include sore throat, difficulty breathing on exertion, voice change and reflux/regurgitation.
References
[edit]- ^ Kahrilas, P.J.; Dodds, W.J.; Hogan, W.J. (October 1987). "Dysfunction of the belch reflex". Gastroenterology. 93 (4): 818–822. doi:10.1016/0016-5085(87)90445-8. PMID 3623025.
- ^ a b c Bastian, R. W.; Smithson, M. L. (2019). "Inability to Belch and Associated Symptoms Due to Retrograde Cricopharyngeus Dysfunction: Diagnosis and Treatment". Oto Open. 3 (1): 2473974X19834553. doi:10.1177/2473974X19834553. PMC 6572913. PMID 31236539.
- ^ "Reddit - noburp". www.reddit.com. Retrieved 2025-01-13.
- ^ "'Fifty years of people telling me I'm crazy': But not only does Amy's medical condition have a name, it can be treated". ABC News. 2023-02-27. Retrieved 2025-01-13.
- ^ Sangastiano, Brittany; McCafferty, Elizabeth (2023-03-10). "Experience: I didn't burp for more than 20 years". The Guardian. ISSN 0261-3077. Retrieved 2025-01-13.
- ^ a b c Chen, Jason N.; Evans, Jacob; Fakhreddine, Aya Bou; Stroever, Stephanie; Islam, Ebtesam; Islam, Sameer (February 2024). "Retrograde cricopharyngeus dysfunction: How does the inability to burp affect daily life?". Neurogastroenterology and Motility. 36 (2): e14721. doi:10.1111/nmo.14721. ISSN 1365-2982. PMID 38115814.
- ^ a b c Sanagapalli, Santosh; Eid, Mary; Kim, Matthew Bong-Sik; Tudehope, Fiona (2024-11-26). "Prospective Controlled Study of Endoscopic Botulinum Toxin Injection for Retrograde Cricopharyngeus Dysfunction: The Inability to Belch Syndrome". American Journal of Gastroenterology. doi:10.14309/ajg.0000000000003242. ISSN 0002-9270. PMID 39589004.
- ^ "Non-burping can damage quality of life, researchers say". 2023-12-21. Retrieved 2025-01-13.
- ^ Heyd, Cameron; Yellon, Robert (2025), "Anatomy, Head and Neck, Pharynx Muscles", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 30969574, retrieved 2025-01-13
- ^ Lang, Ivan M; Shaker, Reza (November 1997). "Anatomy and Physiology of the Upper Esophageal Sphincter". The American Journal of Medicine. 103 (5): 50S – 55S. doi:10.1016/s0002-9343(97)00323-9. ISSN 0002-9343. PMID 9422624.
- ^ Kahrilas, Peter J. (2022-02-04). "Retrograde upper esophageal sphincter function… and dysfunction". Neurogastroenterology & Motility. 34 (5): e14328. doi:10.1111/nmo.14328. ISSN 1350-1925. PMC 9007908. PMID 35122356.
- ^ Oude Nijhuis, Renske A.B.; Snelleman, Jurjaan A.; Oors, Jac M.; Kessing, Boudewijn F.; Heuveling, Derrek A.; Schuitenmaker, Jeroen M.; ten Cate, Liesbeth; Smout, Andreas J.P.M.; Bredenoord, Albert J. (2021-08-26). "The inability to belch syndrome: A study using concurrent high-resolution manometry and impedance monitoring". Neurogastroenterology & Motility. 34 (5): e14250. doi:10.1111/nmo.14250. ISSN 1350-1925. PMC 9285907. PMID 34435723.
- ^ a b Miller, Mattea E.; Lina, Ioan; Akst, Lee M. (2024-01-11). "Retrograde Cricopharyngeal Dysfunction: A Review". Journal of Clinical Medicine. 13 (2): 413. doi:10.3390/jcm13020413. ISSN 2077-0383. PMC 10817096. PMID 38256547.
- ^ "Retrograde Cricopharyngeus Dysfunction (R-CPD)". 26 September 2016.
- ^ Bastian, R. W.; Hoesli, R. C. (2020). "Partial Cricopharyngeal Myotomy for Treatment of Retrograde Cricopharyngeal Dysfunction". Oto Open. 4 (2): 2473974X20917644. doi:10.1177/2473974X20917644. PMC 7163242. PMID 32328538.
- ^ Doruk, Can; Pitman, Michael J. (2023-07-08). "Lateral Transcervical In-office Botulinum Toxin Injection for Retrograde Cricopharyngeal Dysfunction". The Laryngoscope. 134 (1): 283–286. doi:10.1002/lary.30871. ISSN 0023-852X.