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Achalasia subtypes according to Chicago classifi cation. (A) Type I (classic achalasia) refers to patients with absence of peristalsis, no pressurization within the esophageal body, high integrated relaxation pressure (IRP). (B) Type II (achalasia with compression) refers to patients with absence of peristalsis, and contractile activity, panesophageal pressurization >30 mmHg, and high IRP. (C) Type III patient (spastic achalasia), refers to patients with absence of peristalsis, and two or more spastic contractions with or without periods of compartmentalized pressurization and a high IRP
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Esophageal achalasia is a primary motility disorder characterized by impaired lower esophageal sphinc ter relaxation and absence of esophageal peristalsis leading to impa ired bolus transit, manifested with symptoms such as dysphagia, regurgitation, retrosternal pain, and weight loss. Th e standard diagnostic tool is esophageal manometry which demo...
Context in source publication
Context 1
... is a rare primary esophageal motor disorder characterized by the absence of peristalsis and a defective relaxation of the lower esophageal sphincter ( LES) resulting in impaired bolus transport and food stasis in the esophagus [1]. Achalasia occurs equally in men and women with an incidence of 1 in 100,000 individuals and a prevalence of 10 in 100,000. Th e peak incidence occurs between 30 and 60 years of age [2,3]. Th e most frequent symptoms of achalas ia are dysphagia for both solids and liquids, regurgitation of saliva and undigested food, resp iratory complications (nocturnal cough and aspiration), chest pain, heartburn, and weight loss [4]. Heartburn can mimic gastroesophageal refl ux disease (GERD). Dysphagia and regurgitation usually respond to t reatment, but chest pain is much more diffi cult to treat [5]. Th e Eckardt symptom score is the grading system most frequen tly used for the evaluation of symptoms, stages and effi cacy of achalasia treatment. A symptom score of 0-1 corresponds to clini cal stage 0, a score of 2-3 to stage I, a score of 4-6 to stage II, and a score >6 to stage III. Stages 0 and I indicate remission of the disease. On the other hand, stages II a nd III represent failure of treatment (Table 1) [6,7]. Th e pathogenesis of achalasia is not well understood but it is believed to be due to an infl ammatory neurodegenerative process with possible viral involvement. Measl es and herpes viruses have been suggested as causal candidates. However, molecular techniques have failed to confi rm these claims and the causative agent remains undiscovered [8]. It has been hypothesized that an autoimmune pr ocess triggered by a still unidentifi ed cause results, in a genetically predisposed subject, in chronic infl ammatory process leading to neuronal damage [9]. Th is chronic infl ammation within the esophagus leads to the loss of postganglionic inhibitory neurons in the myenteric plexus and a consequent reduction in the inhibitory transmitters, nitric oxide and vasoactive intestinal pept ide. The excitatory neurons remain unaffected; this causes an imbalance between excitatory and inhibitory neurons that prevents LES relaxation [10,11]. The first diag nostic step is to exclude a benign or malignant obstruction using endoscopy or radiology. In early stages, both tests may be completely normal. In advanced cases, the esophagus will be dilated with retained food and saliva; endosc opically, the esophagogastric junction will have a rosette appearance sometimes with increased resistance to scope passage into the stomach. Barium studies show a "bird beak" appearance from t he non-relaxing LES, varying degrees of esophageal dilation up to sigmoid esophagus, aperistalsis and sometimes an air-fluid level and absence of the gastric air bubble. To assess esophageal emptying, a timed barium swallo w can be done, in which the height of the barium column at 5 min after ingesting 8 oz (236 mL) of barium is a good measure of esophageal emptying [5]. Manometry is still the gold standard di agnostic test for achalasia [12]. On conventional manometry, the main features of achalasia are: absence of peristalsis, sometimes with increased intraesophageal pressure, and incomplete relaxation of the LES on deglutition (residual pressure >10 mmHg ) [13]. An increase in the resting tone of the LES is often observed [4]. However, the accuracy of these traditional studies has been challenged by the recent emergence of advanced techniques for the diagnosis of esophageal achalasia such as high- resolution manometry (HRM) and the addition of pressure topography plotting [12]. The use of multiple high-sensitivity sensors to capture manometric data as a spatial continuum allows a detailed pressure recording from the pharynx to the stom ach and is regarded as the gold standard for diagnosis of achalasia [4,14]. Diagnostic algorithms for defining conventional manometric diagnoses of achalasia are improved with HRM, primarily due to the objectivity and accuracy with which it identifies impaired esophagogastric junction relaxation and the metric of peristaltic contraction [14]. The use of HRM has led to the subclassification of achalasia (Chicago classification) into three clinically relevant groups based on the contractility pattern in the esophageal body (Table 2, Fig. ...
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Achalasia is the most common primary motility disorder of the esophagus and presents as dysphagia to solids and liquids. It is characterized by impaired deglutitive relaxation of the lower esophageal sphincter. Highresolution manometry allows for definitive diagnosis and classification of achalasia, with type II being the most responsive to therapy...
Background:
The Chicago Classification describes three distinct subtypes of achalasia and it appears to be a promising tool in predicting results of treatment with standard Heller Myotomy. The aim of this study is to analyze the outcomes of surgical treatment for achalasia using an extended Heller myotomy for each subtype and to identify additiona...
Purpose of Review
Esophagogastric junction outflow obstruction (EGJOO), defined manometrically by impaired esophagogastric junction relaxation (EGJ) with preserved peristalsis, can be artifactual, due to secondary etiologies (mechanical, medication-induced), or a true motility disorder. The purpose of this review is to go over the evolving approach...
Background:
Symptomatic evaluation using Eckardt score and achalasia-specific health-related quality-of-life and physiological evaluation using timed barium examination (TBE) and high-resolution manometry is integral to the diagnosis of achalasia. However, the correlation of physiological outcomes with symptomatic outcomes and their role in routin...
Background and aims:
The clinical efficacy of POEM has been commonly established by the reduction in the Eckardt score (<3) after the procedure. However, achalasia can lead to significant impairment in the patient's quality of life that may go beyond the 4 classical achalasia symptoms as measured by the Eckardt score. The aims of our study were (1...
Citations
... Esophageal achalasia is a motility disorder defined by impaired lower esophageal sphincter (LES) relaxation and absence of esophageal peristalsis, clinically characterized by dysphagia, regurgitation, retrosternal pain, and weight loss. Pathogenetic hypothesis of an autoimmune process triggered by an unidentified cause resulting, in a genetically predisposed subject, in chronic inflammatory process leading to neuronal damage is the most widely accepted [1]. The first diagnostic goal is to exclude, endoscopically or radiologically, a benign or malignant obstruction. ...
Herein, we present a 57-year-old man presented with dysphagia in solids and liquids deteriorating the last months and weight loss of 3 kg. A thoracic CT revealed a limit dilatation of the lower esophagus with food residue. Upper endoscopy was performed revealing bubble content and contraction of the LES. A barium esophagogram demonstrated deceleration of esophageal emptying and a bird beak sign indicative of esophageal achalasia. HRM was performed to evaluate the subtype of achalasia. The catheter could not be intubated after many clinical and technical maneuvers (change patient position, overhead hand raise, clockwise rotation along the long axis of the catheter and it reposition), into the stomach because of LES spasticity. It folded back cephalad at this level, producing a mirror image, the characteristic “butterfly wings” appearance of a folded manometry catheter
... Achalasia is an uncommon but quintessential esophageal motility disorder that occurs equally in men and women (1). Achalasia characterized by reduced relaxation of the lower esophageal sphincter (LES) and absence of esophageal peristalsis resulted in impaired bolus transit, demonstrated with symptoms including dysphagia, retrosternal pain, regurgitation, and weight loss (2). ...
... In the end stage of achalasia, patients present dilation of the esophagus with a sigmoid shape (7). Unfortunately, there is no promising treatment for achalasia due to its unknown pathogenesis, and standard treatment options include pharmacological therapy (nitrates and calcium channel blockers), pneumatic dilation, endoscopic myotomy (2,3), Botulinum toxin (Botox) (8), surgical myotomy, and esophagectomy (2,3). ...
... Achalasia is an esophageal motility disorder characterized by esophageal aperistalsis and impaired relaxation of the lower esophageal sphincter. Since the introduction of high-resolution manometry (HRM) in 2008, achalasia has been classified into clinically relevant subtypes [2,3]. The updated Chicago classification (version 4.0) categorizes achalasia into 3 subtypes [4], that have important implications for management outcomes [5]. ...
Background: Pneumatic dilation (PD) is often presented today as a “short term” treatment for achalasia but, in truth, it can last for years. our study aims to determine the short and long-term efficacy of pneumatic dilatation on clinical remission in a treatment-naïve achalasia patient. Materials and Methods: A single center retrospective, descriptive and analytical study conducted between January 2007 and September 2022. Treatment naïve patients with manometric diagnosis of primary achalasia (subtype I and II) were included. We evaluated the clinical outcomes; single vs multiple PD, complications, and estimated duration of effect. Results: 121 patients were included, (mean age 42,2±14,5; 53,8% male), 88,4% (n=104) underwent 2 PD and 11,6% (n=17) underwent 3 PD, 23,1% (n=28) needed repeated PD at one year, The PD procedure was completed without major complications in all patients. Patients with subtype II had a better response to PD than subtype I(p=0,003). In the long-term follow up (median time: 50 months), the mean Eckardt score was 2.2. A subsequent 35 mm dilatation was associated with long-term clinical remission than an initial dilatation with 35 mm.(p=0.003). On univariate analysis, the non-responders more often was male gender, had age< 40 years (p=0.0006), and had high baseline LESP (lower esophageal sphincter pressure>50 mmHg), p=0.004). On multivariate analysis only age<or= 40 years (p=0.02) was associated with poor outcome. Conclusion: PD is an effective, durable and safe treatment for achalasia. A graded-protocol PD starting with a 30 mm dilation applied in the appropriate achalasia subtypes was shown to be a highly effective approach, in both the short- and long-term. Age<40 years, male patients and hight LESP have poorer outcomes following PD.
... Current therapeutic methodologies for primary idiopathic achalasia include nonsurgical or surgical interventions. Nonsurgical alternatives encompass pharmacotherapy, endoscopic botulinum toxin injection, or pneumatic dilatation [2]. Conversely, interventional alternatives include laparoscopic or robot-assisted Heller myotomy (LHM or RAHM) and peroral endoscopic myotomy [2,3]. ...
... Nonsurgical alternatives encompass pharmacotherapy, endoscopic botulinum toxin injection, or pneumatic dilatation [2]. Conversely, interventional alternatives include laparoscopic or robot-assisted Heller myotomy (LHM or RAHM) and peroral endoscopic myotomy [2,3]. ...
Robot-assisted Heller myotomy (RAHM) is an increasingly popular alternative to the traditional laparoscopic Heller myotomy (LHM) in the surgical management of achalasia, with similar outcomes and potentially lower complication rates. We aimed to systematically review the literature by comparing the technical success, outcomes, and complications of RAHM and LHM.
We searched PubMed, Medline, and Cochrane Central Register for articles published between 2001 and 2023. Data on technical success, clinical outcomes, length of hospital stay, esophageal perforation rate, and overall mortality were extracted.
A total of 11 articles were included in the study, comparing a total of 3,543 RAHM and 15,434 LHM cases. The mean operative time was significantly higher in the RAHM procedure with a total mean difference of 23.95 (95% confidence interval (Cl) 17.09, 30.81; p < 0.00001; I² = 99%). However, the RAHM was associated with a significantly shorter hospital stay, with a total mean difference of -0.24 (95% Cl = -0.40, -0.08; p < 0.00001; I² = 81%). The volume of blood loss was significantly smaller in RAHM with a total mean difference of -61.11 (95% CI = -150.31, 28.09; p < 0.00001; I² = 99%). Esophageal mucosal perforation was significantly lower in RAHM with an odds ratio of 0.36 (95% CI = 0.16, 0.82; p = 0.02; I² = 22%). Both procedures were associated with similar rates of symptom relief. Although no mortality was recorded in patients who underwent RAHM as opposed to 16 cases in patients who underwent LHM, no statistically significant difference could be reached.
Our results demonstrate that while both procedures yield comparable clinical outcomes, RAHM is associated with a lower overall complication rate, particularly a lower rate of esophageal mucosal perforation, shorter hospital stay, and possibly a lower mortality rate. This confirms that RAHM is a viable and justifiable alternative to the conventional LHM in the surgical management of achalasia.
... The severity of symptoms was classified according to a fivegrade scale ranging from 0 to 4 (absent, mild, moderate, severe, and very severe, respectively) [15]. Also, the frequency of symptoms was graded according to the Eckardt score, and the total score was calculated [16]. ...
Background
Many surgeons believe that pre-operative balloon dilatation makes laparoscopic myotomy more difficult in achalasia patients. Herein, we wanted to see if prior pneumatic balloon dilatation led to worse outcomes after laparoscopic myotomy. We also assessed if the frequency of dilatations and the time interval between the last one and the surgical myotomy could affect these outcomes.
Methods
The data of 460 patients was reviewed. They were divided into two groups: the balloon dilation (BD) group (102 patients) and the non-balloon dilatation (non-BD) group (358 patients).
Results
Although pre-operative parameters and surgical experience were comparable between the two groups, the incidence of mucosal perforation, operative time, and intraoperative blood loss significantly increased in the BD group. The same group also showed a significant delay in oral intake and an increased hospitalization period. At a median follow-up of 4 years, the incidence of post-operative reflux increased in the BD group, while patient satisfaction decreased. Patients with multiple previous dilatations showed a significant increase in operative time, blood loss, perforation incidence, hospitalization period, delayed oral intake, and reflux esophogitis compared to single-dilatation patients. When compared to long-interval cases, patients with short intervals had a higher incidence of mucosal perforation and a longer hospitalization period.
Conclusion
Pre-operative balloon dilatation has a significant negative impact on laparoscopic myotomy short and long term outcomes. It is associated with a significant increase in operative time, blood loss, mucosal injury, hospitalization period, and incidence of reflux symptoms. More poor outcomes are encountered in patients with multiple previous dilatations and who have a short time interval between the last dilatation and the myotomy.
... Upper endoscopy is recommended to exclude malignancy. Esophageal manometry is the most sensitive test and remains the gold standard, with high-resolution manometry being the preferred method [13]. Regarding treatment, different management options are available to ease symptoms by decreasing outflow resistance. ...
Background: Achalasia is a medical ailment characterized by irregular contractions of the esophagus and incomplete relaxation of the lower esophageal sphincter. This condition results in difficulty swallowing food and liquids, and often leads to regurgitation and heartburn. Case presentation: This case report describes a 36-year-old female patient who presented with difficulty swallowing, heartburn, chest pain, and coughing while eating or drinking. The patient had lost significant weight despite a good appetite, and a physical examination revealed poor nutrition. Esophageal manometry confirmed the diagnosis of Achalasia Type 1, and endoscopy showed severe Candida esophagitis and a very dilated esophagus with diverticula. Due to the patient's condition and endoscopic finding of diverticula, surgery was considered, as she did not qualify for the Peroral Endoscopic Myomectomy (POEM) procedure. The patient was treated with fluconazole to address the esophageal candidiasis, and NG feeding was initiated to improve weight and nutrition. After recovering, the patient underwent a laparoscopic Heller myotomy with Dor fundoplication using a video laryngoscope to facilitate intubation. Anesthesia was maintained with O2/air with Sevoflurane, and the patient was extubated and transferred to the post-anesthesia care unit. Conclusion: Achalasia is a frequently researched esophageal motility disorder that is distinguished by inadequate relaxation of the LES and absent or irregular peristalsis in the esophagus. Common symptoms in most patients include difficulty swallowing solids and liquids, regurgitation, and varying degrees of weight loss. This case highlights the importance of prompt diagnosis and treatment of Achalasia Type 1, as well as the use of rapid sequence induction and proper anesthesia techniques during surgery.
... 21 Manometry will demonstrate incomplete LES relaxation in response to swallowing, sometimes a lack of peristalsis in the lower esophagus, and a pressure increase in the lower esophageal sphincter. 22 This case did not undergo EGD since we did not suspect any premalignant or malignant esophagus lesions. Esophageal tumor is rare in children and causes dysphagia for solids more than liquids. ...
... 23 These treatments are less effective, provide only short-term symptom relief, and are primarily reserved for patients who are waiting for or who refused more definitive therapy, such as pneumatic dilatation or surgery. 1,22 For most achalasia patients, treatment with pneumatic dilation, surgical myotomy, or peroral endoscopic myotomy (POEM) is more recommended rather than botulinum toxin injection. Pneumatic balloon dilation and surgical myotomy have comparable high success rates. ...
... 27 Disease progression after five years subsequently reduces the success rate. 22 An open approach (laparotomy) to the Heller myotomy is rarely performed as the initial treatment and is reserved for patients who have had multiple prior abdominal operations or who cannot tolerate a pneumoperitoneum because of cardiac or pulmonary disease. 25 In our patient, we performed an open (laparotomy) surgical myotomy with fundoplication. ...
Achalasia is an esophageal smooth muscle motility disorder due to relaxation failure of the lower esophageal sphincter. This condition is rarely found in children; 2-5% cases occur in less than 16-year old. Case: A 16-year-old girl presented with chief complaint of regurgitation. She also complained of dysphagia, weight loss, and general weakness. Her general appearance was weak, underweight, and decreased skin turgor. Barium esophagogram demonstrated a narrowing of esophagus lumen distal to the esophagogastric junction with a “bird’s beak” appearance or rat tail sign. Esophageal achalasia was diagnosed and managed with open (laparotomy) surgical myotomy and fundoplication. Post-operative diet arrangement was carried out gradually and the patient was discharged 4 days post-surgery. Akalasia merupakan gangguan motilitas otot polos esofagus yang terjadi akibat kegagalan relaksasi lower esophageal sphincter (LES). Kondisi ini jarang ditemukan pada anak-anak; 2-5% kasus pada anak berusia di bawah 16 tahun. Kasus: Seorang remaja perempuan 16 tahun datang ke poliklinik bedah anak dengan keluhan utama regurgitasi. Anak juga mengeluh disfagia, penurunan berat badan, dan kelemahan umum. Keadaan umum anak lemah, berat badan rendah, dan penurunan turgor kulit. Esofagogram barium menunjukkan penyempitan lumen esofagus distal terhadap esophagogastric junction dengan tampakan “bird’s beak” atau tanda rat tail. Akalasia esofagus didiagnosis dan ditatalaksana dengan pembedahan laparotomi miotomi dan fundoplikasi. Pemulihan diet pasca-operasi secara perlahan dan pasien dipulangkan 4 hari pasca pembedahan
... In contrast, stages II and III indicate a failed treatment (11). ...
... El proceso inflamatorio se asocia con un infiltrado de linfocitos T que causan la destrucción ganglionar, como ocurre en la enfermedad de Chagas [3][4][5] . La inflamación del plexo mioentérico produce una disfunción de las neuronas posganglionares inhibidoras en el esófago distal, las cuales liberan óxido nítrico y péptido intestinal vasoactivo como neurotransmisores, por lo que su disfunción lleva a un desequilibrio en el control de excitación e inhibición del esfínter esofágico [4][5][6] . ...
Introducción. La acalasia es un trastorno motor del esófago poco común, de etiología no clara, caracterizado por la pérdida de relajación del esfínter esofágico inferior, pérdida del peristaltismo normal, regurgitación y disfagia.
Métodos. Se realizó una revisión narrativa de la literatura en revistas científicas y bases de datos en español e inglés, con el fin de presentar información actualizada en lo referente al diagnóstico y tratamiento de esta patología.
Resultado. Se presenta la actualización de los criterios de los trastornos motores esofágicos según la clasificación de Chicago (CCv4.0) para el diagnóstico de acalasia y sus subtipos de acuerdo con los nuevos criterios, así como los tratamientos actuales.
Conclusión. La acalasia es un trastorno esofágico multimodal, con manifestaciones de predominio gastrointestinal, por lo que su diagnóstico y abordaje terapéutico oportuno es esencial para mejorar la calidad de vida de los pacientes.
... patients, particularly with robust data supporting the broad adoption of more novel endoscopic techniques such as peroral endoscopic myotomy (POEM) [2,3]. There is now recognition that specific achalasia subtypes may respond better to certain treatments [4][5][6], allowing for a personalized approach to management [7][8][9]. ...
... As a result, and because of the increasing evidence base for tailored therapy leading to optimal outcomes, it is important to establish quality metrics for achalasia management. Based on recently published achalasia management guidelines from several societies [6][7][8][9] as well as expert opinion, we previously developed 12 QIs for achalasia (Table 1), which cover the domains of patient education, diagnostic management, and treatment approach to highlight best practice concepts [12]. These QIs provide an opportunity to measure variations in achalasia care against quality metrics. ...
Background
Quality indicators (QIs) are formal ways to track health care performance and outcomes, guide quality improvement, and identify gaps in care delivery. We developed twelve quality indicators for achalasia management which cover the domains of patient education, diagnosis, and treatment of achalasia.AimTo determine adherence to established quality indicators for achalasia management.Methods
We performed a retrospective, multicenter evaluation of care patterns for adult patients greater than 18 years old with newly diagnosed achalasia from January 2018 to May 2020. A balanced random patient sample was obtained at four large academic medical centers. Independent electronic health record chart abstraction was performed using a standardized form to determine adherence to applicable QIs. Pooled and de-identified data were analyzed to identify gaps in care.ResultsA total of 120 patients were included and the overall adherence to applicable quality indicators across all centers was 86%. The median follow-up for all patients from time of diagnosis to end of study was 511 days. Clinicians adhered to all applicable quality indicators in 49 patients (39%). The quality indicator domain with the poorest adherence was patient education (67%), with 50% of patients having had a documented discussion of the risks of gastroesophageal reflux disease following surgical or endoscopic myotomy.Conclusions
Gaps in the quality of achalasia care delivery were identified, the largest of which relates to patient education about treatment risks. These findings highlight a potential area for future quality improvement studies and form the basis for developing fully specified quality measures.
