Esophageal Achalasia (Megaesophagus)

A motility disorder of the ESOPHAGUS in which the LOWER ESOPHAGEAL SPHINCTER (near the CARDIA) fails to relax resulting in functional obstruction of the esophagus, and DYSPHAGIA. Achalasia is characterized by a grossly contorted and dilated esophagus (megaesophagus).

Also Known As:

Networked: 3450 relevant articles (457 outcomes, 422 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Deglutition Disorders (Dysphagia)
2.	Gastroesophageal Reflux (GERD)
3.	Esophageal Motility Disorders (Nutcracker Esophagus)
4.	Esophageal Achalasia (Megaesophagus)
5.	Chest Pain (Chest Pains)

Experts

1.	Inoue, Haruhiro: 79 articles (11/2022 - 09/2010)
2.	Patti, Marco G: 44 articles (01/2022 - 05/2003)
3.	Pandolfino, John E: 43 articles (01/2022 - 05/2004)
4.	Kahrilas, Peter J: 36 articles (02/2022 - 05/2004)
5.	Onimaru, Manabu: 32 articles (11/2022 - 11/2011)
6.	Khashab, Mouen A: 32 articles (01/2022 - 01/2013)
7.	Nabi, Zaheer: 29 articles (12/2022 - 10/2016)
8.	Ikeda, Haruo: 25 articles (04/2022 - 11/2011)
9.	Roman, Sabine: 25 articles (02/2022 - 02/2013)
10.	Hungness, Eric S: 25 articles (01/2022 - 02/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Esophageal Achalasia:

1.	Botulinum Toxins (Botulinum Toxin)IBA 07/01/2003 - "Botulinum toxin is the best treatment option for achalasia in patients whose condition makes them unfit for pneumatic dilation or surgery. " 03/01/2002 - "Achalasia treatment in the elderly: is botulinum toxin injection the best option?" 07/01/2015 - "Endoscopic botulinum toxin injection to the lower esophageal sphincter is an effective and safe option in the treatment algorithm of achalasia, particularly in high-surgical-risk patients. " 10/01/1999 - "Intrasphincteric injection of botulinum toxin has been reported as a safe and effective alternative treatment in oesophageal achalasia, especially in high-risk and elderly patients. " 01/01/1999 - "Recently, botulinum toxin injection was suggested as a therapeutic option in achalasia patients with good results on lower esophageal sphincter pressure (LESP) and symptom scores that were similar to the results achieved by pneumatic balloon dilation. "
2.	BariumIBA 05/01/2010 - "The diagnosis of primary cricopharyngeal achalasia is difficult, and we must apply exclusive diagnosis according to the examinations of fibrolaryngoscopy, esophagoscopy and barium meal et al. Surgical treatment is the best option. " 09/01/2011 - "Esophageal emptying assessed at the 'timed barium' esophagogram correlates well with symptomatic outcomes after pneumatic dilation for esophageal achalasia, although 30% of patients with satisfactory outcome exhibit partial improvement in emptying. " 01/01/2022 - "The purpose of our study was to evaluate the role of timed barium esophagogram (TBE) in quantitative measurement of improved esophageal emptying in primary achalasia patients treated with POEM. " 12/01/2017 - "Achalasia was also strongly suspected due to the enlargement of the esophagus to the maximum transverse diameter of 55 mm by esophagography along with delayed clearance of barium. " 09/01/2017 - "Eight hundred ninety two adult achalasia patients of both genders were treated from January 1988 till December 2011, with pneumatic balloon (Rigiflex Microvasive®) dilatation, under fluoroscopy Barium swallow was obtained prior to and five minutes after dilatation to evaluate for efficacy of dilatation as well as for complications. "
3.	Type A Botulinum Toxins (Botox)FDA Link 07/01/2002 - "Over a five-year time horizon, pneumatic dilation was the most cost-effective treatment strategy for achalasia, with an incremental cost-effectiveness ratio of $1348 per quality-adjusted life-year compared to Botox. " 04/01/2002 - "Multiple controlled studies have shown that Botox is effective in short-term management of achalasia. " 01/01/2023 - "Intrasphincteric Botox injection proved to be feasible, safe and reasonably effective in children with HSCR and postoperative anal achalasia. " 01/01/1996 - "Endoscopic intrasphincteric BoTox injection is safe, simple and effective in the short term treatment for achalasia. " 01/01/1996 - "In this study we evaluated the efficacy of transendoscopic injection of BoTox in 13 symptomatic patients with achalasia G II (Siewert classification) without previous treatment of an ongoing randomized-controlled trial. "
4.	Nifedipine (Adalat)FDA LinkGeneric 06/16/2022 - "In a rat model of achalasia, nifedipine-loaded BNPs significantly enhanced esophageal drug exposure, increased therapeutic efficacy, and reduced systemic drug exposure compared to NNPs and free drug. " 06/01/1991 - "It is concluded that longterm treatment with sublingual nifedipine and pneumatic dilatation are equally effective in the treatment of oesophageal achalasia of mild or moderate degree." 01/01/1990 - "In the 3 cases of achalasia, treatment with nifedipine led to clinical improvement with relaxation of inferior esophageal contraction on radiologic examination. " 01/01/1988 - "The only case of vigorous achalasia studied showed an adynamic pattern with activity in peaks which improved after the administration of nifedipine. " 03/01/1982 - "Nifedipine improved symptoms of achalasia, but did not improve the degree of esophageal dilatation. "
5.	AcidsIBA 06/01/1971 - "Although the nadir of pressure attained with acid suppression was the same, the per cent inhibition was significantly greater in patients with achalasia. " 06/01/2012 - "Among these, 48 (14%) had macro/microscopic oesophagitis on endoscopy, 7 (2%) had achalasia and 96 (28%) had excessive acid reflux (pH study). " 02/01/2006 - "Very late results of esophagomyotomy for patients with achalasia: clinical, endoscopic, histologic, manometric, and acid reflux studies in 67 patients for a mean follow-up of 190 months." 01/01/1995 - "We made a prospective assessment of acid exposure in the distal esophagus in 48 consecutive untreated patients with achalasia using 24-h ambulatory esophageal pH studies. " 08/18/2023 - "A manual review of pH tracings helps identifying "true acid reflux" in post-POEM achalasia patients. "
6.	Calcium Channel Blockers (Blockers, Calcium Channel)IBA 03/01/1991 - "Calcium channel blockers have been previously shown to decrease lower esophageal sphincter (LES) pressure and improve symptoms in achalasia. " 02/01/1992 - "Calcium channel blockers may be the initial choice for high or moderate risk patients with achalasia prior to proceeding with pneumatic dilatation or surgical myotomy. " 02/01/1992 - "Early studies confirmed the beneficial effects of calcium channel blockers on the normal oesophagus, which included a decrease in lower oesophageal sphincter tone in achalasia and a decrease in oesophageal contractions and amplitude in diffuse oesophageal spasm. " 09/01/1990 - "Although calcium channel blockers, like nifedipine, lower LOS basal pressure, they are not very useful on long term relief of symptoms of achalasia. " 02/01/1996 - "Based on the rationale that the calcium channel blocker, nifedipine, decreases lower esophageal sphincter pressure in achalasia, a prospective controlled trial was performed to evaluate the effect of sublingual nifedipine on the anal sphincter of controls and patients with high anal resting pressures. "
7.	Opioid Analgesics (Opioids)IBA 01/01/2021 - "Endoscopic ultrasound is an essential tool that has improved our treatment algorithm for suspected achalasia in patients with chronic opioid usage. " 01/01/2023 - "In this single-center study of 89 patients with type III achalasia, EGJOO, DES, or HE treated with POEM, daily opioid use was present in 20%. " 12/01/2023 - "Our results revealed that opioid use is significantly associated with type III achalasia but not with type I and II achalasia. " 01/01/2021 - "Chronic opioid use can induce esophageal dysfunction with symptoms resembling achalasia and a manometric pattern of esophagogastric junction-outflow obstruction (EGJ-OO). " 01/01/2021 - "Mean BMI in the opioid-induced achalasia group was 30.2 kg/m2, mean BMI in the primary achalasia group 26.8 kg/m2 (p = 0.11). "
8.	Neurotransmitter Agents (Neurotransmitter)IBA 03/10/1995 - "Different neurotransmitter systems are involved in the development of esophageal achalasia." 10/30/2021 - "Neuromodulators Are Effective for Treatment of Spastic Chest Pain After Heller Myotomy for Achalasia." 06/01/1999 - "Identification of nitric oxide as the primary inhibitory neurotransmitter of the gastrointestinal tract has improved our understanding of the pathophysiology of primary achalasia. " 06/28/2012 - "In the initial stage, degeneration of inhibitory nerves in the esophagus results in unopposed action of excitatory neurotransmitters such as acetylcholine, resulting in high amplitude non-peristaltic contractions (vigorous achalasia); progressive loss of cholinergic neurons over time results in dilation and low amplitude simultaneous contractions in the esophageal body (classic achalasia). " 02/01/2010 - "The pathophysiology of achalasia is still incompletely understood, but recent evidence suggests that degeneration of the postganglionic inhibitory nerves of the myenteric plexus could be due to an infectious or autoimmune mechanism, and nitric oxide is the neurotransmitter affected. "
9.	Radioisotopes (Radionuclides)IBA 11/01/2010 - "Serendipitous diagnosis of achalasia cardia on a radionuclide study." 07/01/2001 - "The results suggest that radionuclide esophageal emptying test using a solid meal remains a useful objective study of esophageal function and may have an important future role in the follow-up evaluation of treatment for achalasia." 05/01/1999 - "The aim of this study was to assess the value of radionuclide esophageal transit time (RET) in prediction of the results of pneumatic dilation in patients with achalasia. " 09/01/1994 - "Radionuclide and videofluoroscopic studies represent valuable and complementary, although relatively insensitive, procedures to detect achalasia." 09/01/1994 - "Radionuclide and videofluoroscopic studies confirmed 25 and 15, respectively, of 37 manometrically diagnosed achalasias and provided indications for the disorder in another 8 and 16 patients. "
10.	Proton Pump InhibitorsIBA 10/01/2017 - "The aim of our study was to evaluate the yield of prolonged wireless pH monitoring in patients with successfully treated achalasia and its influence on proton pump inhibitor (PPI) use. " 12/13/2021 - "Patients with untreated achalasia and those with refractory reflux symptoms despite ≥8 weeks of proton-pump inhibitor treatment were enrolled prospectively. " 09/21/2021 - "Achalasia should therefore be considered in patients with an initial diagnosis of gastro-oesophageal reflux disease who do not respond to proton pump inhibitors." 10/01/2019 - "Key strong recommendations are made regarding the benefit of: (i) HRM over standard manometry in the investigation of dysphagia and, in particular, in characterising achalasia, (ii) adjunctive testing with larger volumes of water or solids during HRM, (iii) oesophageal manometry prior to antireflux surgery, (iv) pH/impedance monitoring in patients with reflux symptoms not responding to high dose proton pump inhibitors and (v) pH monitoring in all patients with reflux symptoms responsive to proton pump inhibitors in whom surgery is planned, but combined pH/impedance monitoring in those not responsive to proton pump inhibitors in whom surgery is planned. " 01/01/2018 - "This trial was designed to assess the prevalence and characteristics of Jackhammer esophagus (JE), a novel hypercontractile disorder associated with progression to achalasia and limited outcomes following anti-reflux surgery in patients with typical symptoms of GERD and responsiveness to proton pump inhibitor (PPI) therapy. "

Therapies and Procedures

1.	Myotomy 06/01/2011 - "Currently, surgical myotomy and pneumatic dilation are the most effective treatments for patients with idiopathic achalasia, and a multicenter, randomized, international trial has confirmed similar efficacy of these treatments, at least in the short term. " 11/01/2010 - "This is the first study by a surgical group to assess the outcome of surgery in 3 manometric achalasia subtypes: patients with panesophageal pressurization have the best outcome after laparoscopic Heller-Dor myotomy." 10/01/2021 - "Although peroral endoscopic myotomy (POEM) is highly effective for the management of achalasia, clinical failures may occur. " 01/01/2021 - "Peroral Endoscopic Myotomy Is Safe and Highly Effective Treatment for Advanced Achalasia With Sigmoid Esophagus." 01/01/2021 - "Peroral endoscopic myotomy is highly effective for achalasia patients with recurrent symptoms after pneumatic dilatation."
2.	Heller Myotomy 05/01/2020 - "In paediatric achalasia, the laparoscopic Heller myotomy seems to be the best treatment because of its multiple advantages offered by the minimally invasive technique. " 01/01/2020 - "Laparoscopic Heller Myotomy is the most effective treatment of achalasia. " 04/01/2019 - "Laparoscopic Heller myotomy (LHM) is the most effective treatment for achalasia. " 06/01/2018 - "Heller myotomy (HM) is widely recognized as the most effective treatment of achalasia. " 04/01/2018 - "Heller's myotomy (HM) is one of the most effective treatments for esophageal achalasia. "
3.	Fundoplication 04/01/2005 - "Surgery actually achieves the best results in the treatment of esophageal achalasia; the options vary from a short extramucosal esophagomyotomy to an extended esophagogastromyotomy with an associated partial fundoplication to restore the main antireflux barrier. " 05/01/2007 - "Laparoscopic cardiomyotomy with anterior partial fundoplication achieves excellent symptomatic relief for patients with achalasia, and it can be performed with minimal morbidity." 07/01/2001 - "Laparoscopic cardiomyotomy with anterior partial fundoplication achieves excellent symptomatic relief for patients with achalasia, and it can be performed with minimal morbidity." 04/01/2019 - "CONCLUSION LHM with anterior or posterior partial fundoplication is safe and achieves a good outcome in the treatment of achalasia, especially in patients who have not responded to other methods while no serious complication was reported despite several prior endoscopic interventions." 04/01/2000 - "Based on the obtained results it could be concluded that cardiomyotomy with Dor fundoplication through a laparoscopic approach leeds to good functional results and seems effective and safe procedure in the treatment of esophageal achalasia."
4.	Therapeutics 10/14/2015 - "Various studies have suggested that the most effective treatment of esophageal achalasia is surgical therapy. " 01/01/2023 - "This RCT will be the first one to evaluate which endoscopic therapy is most effective and safe for treatment of naïve pediatric patients with achalasia." 01/01/2018 - "Type II achalasia patients respond best to all treatment options compared to those with types I and III. Pneumatic dilation (PD) or Heller miotomy (LHM) can be both chosen as initial therapy in type I and II as they have good outcome , while type III achalasia patients respond better to LHM as a first therapeutic option. " 10/01/1991 - "Surgical therapy for achalasia of the esophagus gives good results in only 80-90% of cases. " 05/18/2022 - "The aim of this study is to find a more suitable therapy for achalasia by comparing the efficacy of FCARMS implantation and POEM. "
5.	Esophagectomy 09/01/2012 - "The laparoscopic esophagectomy shown to be effective in the treatment of advanced achalasia. " 10/01/2008 - "Subtotal esophagectomy and gastric pull-up with cervical anastomosis is the main treatment for advanced achalasia. " 04/01/2018 - "Esophagectomy for end-stage achalasia is safe and effective in tertiary referral centers. " 01/01/2018 - "Esophagectomy for end-stage achalasia is safe and effective. " 08/16/2018 - "MINIMALLY INVASIVE LAPAROSCOPIC ESOPHAGECTOMY VS. TRANSHIATAL OPEN ESOPHAGECTOMY IN ACHALASIA: A RANDOMIZED STUDY."