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ACG Clinical Guideline: Diagnosis and Management of Achalasia
Abstract
Achalasia is a primary motor disorder of the esophagus characterized by insufficient lower esophageal sphincter relaxation and loss of esophageal peristalsis. This results in patients' complaints of dysphagia to solids and liquids, regurgitation, and occasional chest pain with or without weight loss. Endoscopic finding of retained saliva with puckered gastroesophageal junction or barium swallow showing dilated esophagus with birds beaking in a symptomatic patient should prompt appropriate diagnostic and therapeutic strategies. In this ACG guideline the authors present an evidence-based approach in patients with achalasia based on a comprehensive review of the pertinent evidence and examination of relevant published data.Am J Gastroenterol advance online publication, 23 July 2013; doi:10.1038/ajg.2013.196.
... While these symptoms are often attributed to benign, self-limiting conditions such as viral gastroenteritis, clinicians must remain alert to more insidious and diagnostically challenging causes. Structural anomalies like vascular rings [1][2][3] and functional disorders such as achalasia [4][5][6][7] can closely mimic more common gastrointestinal conditions, frequently leading to significant diagnostic delays. ...
... A barium swallow study showed a characteristic smooth tapering at the gastroesophageal junction, with delayed contrast transit that improved with the administration of water. These radiological findings were consistent with primary achalasia (Figure 2) [4][5][6][7]. ...
... Both double aortic arch and achalasia are rare but significant causes of esophageal obstruction in children [1][2][3][4][5][6][7]. Although they stem from fundamentally different pathologies -one congenital and structural, the other functional and neuromuscular -their overlapping symptoms can complicate the diagnosis. ...
... Based on these radiographic findings, Dr. Hertz and his colleague, Sir Cooper Perry, coined the term "achalasia" (in Greek: a, not; χαλάω, I relax) to describe the failure of the LES to deglutitively relax resulting in a functional obstruction to bolus passage at the esophagogastric junction, EG-J [2,3]. In addition, there is also loss of the esophageal peristaltic function. ...
... Type I (classic achalasia) has absent contractility in the esophageal body with an elevated median IRP, type II has ≥ 20% of swallows of panesophageal pressurization and an elevated median IRP, and type III (spastic/vigorous) has shortened distal latency (< 4.5 s), DCI > 450 mmHg cm s and an elevated IRP. Figure 2 shows representative swallow examples of the three subtypes of achalasia. Subtyping achalasia is of clinical importance as the three subtypes clinically present similarly, but treatment response varies considerably between the three subtypes [3,11,14,15]. ...
... Upright images should be obtained in the left posterior oblique (LPO) position to position the esophagus off the vertebral bodies. This can show smooth tapering or "bird's beak" at the lower esophageal sphincter (LES), a standing column of contrast, and esophageal dilation [3,[18][19][20][21]. Esophageal motility should be assessed in the prone right anterior oblique (RAO) position. ...
Achalasia is a rare esophageal motility disorder characterized by lack of primary peristalsis and a poorly relaxing lower esophageal sphincter. This disease process can be examined several ways and these evaluations can offer complementary information. There are three manometric subtypes of achalasia, with differing appearances on esophagram. Differentiating them is clinically important, because treatment for the subtypes varies. Timed barium esophagram (TBE) is a simple test to quantitatively evaluate esophageal emptying. TBE can be used to diagnose achalasia and assess treatment response. Considerable variation in the TBE protocol exist in the literature. We propose a standardized approach for TBE to allow for comparison across institutions.
... Patients can present with dysphagia to solids and liquids, chest pain, weight loss, and regurgitation. 2 There are multiple treatment modalities available, ranging from botulinum toxin injections, pneumatic dilation (PD), peroral endoscopic myotomy (POEM), to esophagectomy for certain cases. 3 AXIOS stents create an anastomotic conduit between 2 lumens, allowing the bypass of blockages and strictures while facilitating the drainage of large fluid collections. ...
... Achalasia is characterized by the failure of LES relaxation, leading to impaired esophageal function and consequent symptoms such as dysphagia to solids and liquids, chest pain, weight loss, and regurgitation. 2,5 Patients suspected to have achalasia should first undergo an EGD and/or esophageal barium swallow; regardless of initial imaging findings, esophageal manometry has been the standard for diagnosis and classification. Current management options are aimed toward alleviating symptoms, enhancing esophageal emptying, and preventing further esophageal dilation as there is no intervention that can significantly improve esophageal peristalsis; repeat interventions are often required as LES hypertonicity seen in achalasia rebounds over time. 2 Management is determined based on the degree of the patient's surgical risk. ...
... Current management options are aimed toward alleviating symptoms, enhancing esophageal emptying, and preventing further esophageal dilation as there is no intervention that can significantly improve esophageal peristalsis; repeat interventions are often required as LES hypertonicity seen in achalasia rebounds over time. 2 Management is determined based on the degree of the patient's surgical risk. For those with low risk, PD is often attempted before performing LES myotomy because it is less invasive and has a faster recovery period. ...
AXIOS stents create an anastomotic connection between 2 lumens, facilitating bypass of blockages and strictures as well as drainage of large fluid collections. Historically, AXIOS stents have primarily been used for draining pancreatic fluid collections, with no documented cases of their use within the esophagus until now. In this article, we present a case of a 65-year-old man with cirrhosis admitted for dysphagia. On evaluation, he was diagnosed with type 1 achalasia and concurrent esophageal varices. A novel approach was used, utilizing an AXIOS stent, to provide both symptomatic relief and targeted treatment for his varices.
... 14 The most effective therapies are currently considered to be pneumatic dilation and surgical myotomy. 3,5,14 Pneumatic dilation involves employing air pressures to disrupt the LES circular muscle fibers, while surgical myotomy involves the division of the circular muscle fibers of the LES, via open thoracotomy, laparotomy, or laparoscopy. ...
... Our patient was treated with Heller cardiomyotomy, a form of surgical myotomy, which is the recommended treatment modality in adolescents. 5,14 An anti-reflux procedure, fundoplication is usually done to minimize the risk of gastroesophageal reflux after the procedure, which is one of the complications of surgical myotomy. 14,15 In our patient, a Belsey Mark IV fundoplication as an anti-reflux closure technique was done. ...
... 5,14 An anti-reflux procedure, fundoplication is usually done to minimize the risk of gastroesophageal reflux after the procedure, which is one of the complications of surgical myotomy. 14,15 In our patient, a Belsey Mark IV fundoplication as an anti-reflux closure technique was done. ...
... Traditionally, treatment options have included endoscopic intervention via endoscopic botulinum toxin injection to LES or pneumatic dilation [8]. If unsuccessful, medical management with calcium channel blockers or long-acting nitrates is considered [8]. ...
... Traditionally, treatment options have included endoscopic intervention via endoscopic botulinum toxin injection to LES or pneumatic dilation [8]. If unsuccessful, medical management with calcium channel blockers or long-acting nitrates is considered [8]. Finally, surgical intervention via surgical myotomy or esophagectomy may be required, especially in end-stage disease (those who have developed a megaesophagus) [8]. ...
... If unsuccessful, medical management with calcium channel blockers or long-acting nitrates is considered [8]. Finally, surgical intervention via surgical myotomy or esophagectomy may be required, especially in end-stage disease (those who have developed a megaesophagus) [8]. ...
Achalasia is a motility disorder of the esophagus in which the lower esophageal sphincter fails to relax. Megaesophagus is a rare complication of achalasia characterized by severe dilatation of the esophagus, often indicative of end-stage achalasia. Typical presenting symptoms include dysphagia, nausea, vomiting, weight loss, and chest pain. The majority of patients with achalasia typically have excellent outcomes after surgical intervention with Heller myotomy. We discuss an interesting case of unsuccessful surgical intervention and hypothesize the reason for its failure in our patient.
... Chẩn đoán CTTV thường dựa vào các triệu chứng lâm sàng (sử dụng bộ câu hỏi Eckardt) và các phương pháp thăm dò bao gồm chụp baryt thực quản, nội soi đường tiêu hóa trên và đo áp lực và nhu động thực quản độ phân giải cao (high resolution manometry, HRM) [1,2].Trong đó chụp baryt thực quản là phương pháp không xâm lấn, dễ thực hiện, phổ biến, phù hợp cho những đơn vị chưa triển khai được kĩ thuật HRM. Nội soi đường tiêu hóa trên được khuyến cáo ở hầu hết các bệnh nhân có triệu chứng gợi ý CTTV để loại trừ các các bệnh lý khác, đặc biệt là ung thư thực quản hoặc các tổn thương đè đẩy gây hẹp. ...
... Nội soi đường tiêu hóa trên được khuyến cáo ở hầu hết các bệnh nhân có triệu chứng gợi ý CTTV để loại trừ các các bệnh lý khác, đặc biệt là ung thư thực quản hoặc các tổn thương đè đẩy gây hẹp. HRM được coi là tiêu chuẩn vàng trong chẩn đoán CTTV với đặc trưng là giảm khả năng giãn cơ thắt thực quản dưới thể hiện qua tăng chỉ số áp lực tích hợp khi nghỉ của LES trong 4 giây (IRP4s) [1,2]. Phân loại Chicago 3.0 chia CTTV thành ba type trên HRM dựa trên hình thái nhu động thực quản, từ đó giúp xác định phương pháp điều trị và tiên lượng đáp ứng điều trị. ...
... -Lâm sàng: điểm Eckardt ≥6 -Trên nội soi: dây soi khó qua tâm vị, thực quản giãn rộng, ứ đọng thức ăn, nước [1,2]. ...
Nghiên cứu cắt ngang thực hiện trên 148 bệnh nhân nghi ngờ co thắt tâm vị (CTTV) từ 9/2020 đến 2/2023 tại Viện nghiên cứu và đào tạo tiêu hóa gan mật. Tuổi trung bình là 41,5 ± 14,8 với 37,9% là nam giới. Triệu chứng lâm sàng thường gặp nhất là nuốt khó (85,1%) và trào ngược (75,7%), tỷ lệ bệnh nhân có điểm Eckardt ≥6 là 60,0%. Trong 148 bệnh nhân, 140 bệnh nhân được chẩn đoán xác định bởi bác sĩ lâm sàng dựa vào triệu chứng kết hợp các phương pháp thăm dò hình ảnh (nội soi/chụp baryt thực quản/ đo áp lực và nhu động thực quản độ phân giải cao (HRM)). 81,8% bệnh nhân có hình ảnh CTTV trên nội soi với độ nhạy 82%, nhưng độ đặc hiệu chưa cao (57%). Kỹ thuật HRM cho thấy độ nhạy tốt hơn trong chẩn đoán CTTV so với các phương pháp truyền thống như chụp baryt thực quản và nội soi đường tiêu hóa (độ nhạy 87%, độ đặc hiệu 100%). Kết hợp các phương pháp chẩn đoán CTTV với triệu chứng lâm sàng sẽ giúp cải thiện độ đặc hiệu trong chẩn đoán.
... A large multicenter randomized clinical trial compared treatment naïve achalasia patients who were randomized to either POEM or pneumatic dilation, and it was found that patients undergoing POEM had higher reported treatment success at 2 years (92% vs. 54%, based on Eckhardt scores ≤ 3) with fewer adverse outcomes [32]. However, pneumatic dilations remain the most effective non-surgical treatment option for achalasia [33]. Due to the 1.9% risk of perforation associated with pneumatic dilations, these procedures should only be performed among patients who are surgical candidates [33]. ...
... However, pneumatic dilations remain the most effective non-surgical treatment option for achalasia [33]. Due to the 1.9% risk of perforation associated with pneumatic dilations, these procedures should only be performed among patients who are surgical candidates [33]. ...
Achalasia is a chronic esophageal motility disorder comprised of ineffective esophageal peristalsis and incomplete relaxation of the lower esophageal sphincter. This disease had historically been managed through medical means as well as endoscopic dilations. However, surgical interventions are now considered standard of care, including minimally invasive Heller myotomy, which was popularized in 1990s, followed by per oral endoscopic myotomy in the 2010s. Both surgical approaches provide acceptable resolution of dysphagia symptoms. Classification of the achalasia as well as other patient-level factors may drive the clinical decision-making between the two approaches, as well as surgical training and surgeon preference.
... They are usually reserved for elderly patients who cannot undergo more definitive treatments like pneumatic dilation, or surgical myotomy as well as for those who have not responded to botulinum toxin injections. 25 Endoscopic treatment involves injecting botulinum toxin into the LES to block acetylcholine release and restore the balance between excitatory and inhibitory neurotransmitters. However, this treatment has limited value, as only about 30% of patients experience relief of dysphagia 1 year after treatment, and most patients require repeated botulinum toxin injections. ...
... 16,19 Both PBD and LHM exhibit comparable success rates and post-treatment quality of life. 20,25 However, PBD is the most common first-line treatment for achalasia, targeting the circular muscle fibers of the LES to relieve obstruction, because of its cost-effectiveness, and less invasive nature. 16,19 Post-intervention, achalasia patients usually experience significant relief from dysphagia, regurgitation, and chest pain, leading to improved quality of life. ...
Key Clinical Message
This case emphasizes the need for early recognition and accurate diagnosis of achalasia in young adults to avoid exacerbation of the condition and misdiagnosis as GERD. Patient outcomes and quality of life are greatly enhanced by suitable diagnostic techniques, appropriate therapy, interdisciplinary care, and comprehensive patient education along with frequent follow‐ups.
Abstract
Achalasia results from the degeneration of inhibitory ganglion cells within the esophageal myenteric plexus and the lower esophageal sphincter (LES), leading to a loss of inhibitory neurons and resulting in the absence of peristalsis with failure of LES relaxation. Its origins are multifactorial, potentially involving infections, autoimmune responses, and genetics, with equal incidence in males and females. The hallmark symptoms include progressive dysphagia for solids and liquids, along with regurgitation, heartburn, and non‐cardiac chest pain. A 22‐year‐old female patient initially diagnosed with gastroesophageal reflux disease (GERD) received proton pump inhibitors and antacid gel for persistent dysphagia and regurgitation. Subsequent tests including barium esophagogram and manometry indicated Type II Achalasia Cardia. The patient showed clinical improvement with relief of dysphagia, regurgitation, and heartburn symptoms after pneumatic balloon dilatation (PBD). She was advised to follow up after 6 months with upper gastrointestinal (UGI) endoscopy and manometry in the outpatient clinic for regular endoscopic surveillance as there is a risk of transformation to esophageal carcinoma. Diagnosing achalasia in young adults poses challenges due to its diverse presentation and resemblance to other esophageal disorders like GERD. Diagnosis relies on clinical symptoms and imaging studies such as barium esophagogram revealing a bird's beak appearance and esophageal manometry showing absent peristalsis. UGI endoscopy is needed to rule out malignancy. Treatment options include non‐surgical approaches like medication and Botox injections, as well as surgical methods such as pneumatic balloon dilation, laparoscopic Heller myotomy, and per‐oral endoscopic myotomy (POEM). The treatment options depend upon the patient's condition at presentation and their individual choices. This case report emphasizes that it is crucial to consider achalasia as a potential differential diagnosis in young adults with dysphagia, especially if conventional treatments for acid peptic disorder do not alleviate symptoms. Prompt diagnosis and appropriate management can lead to significant clinical improvement and better patient outcomes.
... The incident rate escalates as age increases, with the mean age of diagnosis surpassing 50 years. Additionally, it is indifferent to ethnicity and affects both sexes [3]. ...
... All therapeutic alternatives aim to alleviate the patient's symptoms by decreasing the tone of the lower esophageal sphincter and promoting esophageal emptying. Furthermore, it is imperative that the treatments halt the disease's progression and, as a result, avert its later complications [3]. ...
There are various therapeutic options for achalasia. Nevertheless, peroral endoscopic myotomy (POEM) and laparoscopic Heller myotomy with fundoplication (LHM) are distinguished by their efficacy and low incidence of complications. Compare POEM and LHM regarding several outcomes in patients with achalasia. This systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. An exhaustive literature search was performed using PubMed, Web of Science, and Cochrane Library databases. Studies comparing several outcomes between POEM and LHM in patients with achalasia were included. Data on clinical success, operative time, intraoperative complications, length of stay, reintervention rates, postoperative pain, overall complications, occurrence of GERD symptoms, use of proton bomb inhibitors and esophagitis were extracted. Quality assessment of the included studies was performed using the MINORS scale. We included 20 retrospective observational studies with a combined total of 5139 participants. The results demonstrated that there was no statistically significant difference in terms of intraoperative complications, postoperative complications, reintervention rate, occurrence of GERD symptoms, GERD HRQL, use of proton pump inhibitors, and esophagitis between POEM and LHM groups. Conversely, POEM was associated with higher clinical success and shorter operative time, length of stay, and postoperative pain. This meta-analysis concludes that both POEM and LHM, are effective and safe treatments for achalasia. However, POEM demonstrates better results regarding clinical success, operative time, length of stay, postoperative pain, and a tendency towards lower recurrence.
... Most of the cases are idiopathic; however, cases secondary to Chagas disease and herpes virus are also reported. 5,6 Pharmacological treatment with agents like calcium channel blockers and botulinum toxin injection has been used for the treatment but they have low long-term success rates and few poorly tolerated side effects. 6,7 Surgical treatment of choice is LHM with partial fundoplication. ...
... 5,6 Pharmacological treatment with agents like calcium channel blockers and botulinum toxin injection has been used for the treatment but they have low long-term success rates and few poorly tolerated side effects. 6,7 Surgical treatment of choice is LHM with partial fundoplication. Endoscopic treatment with PBD is less durable than LHM and these patients often require reintervention. ...
Introduction In the last one decade, per oral endoscopic myotomy (POEM) has been found to be an effective minimally invasive approach for the treatment of esophageal achalasia cardia and was lately shown to be noninferior to laparoscopic Heller's myotomy in a randomized controlled trial.
Here we describe a new method of using sublingual nitroglycerine spray during submucosal tunneling to facilitate the procedure.
Materials and Methods This study includes a cohort of 50 patients who were diagnosed with achalasia cardia on high-resolution esophageal manometry. Out of the 50 patients, 27 patients were administered two metered doses of sublingual nitroglycerine spray during submucosal tunneling across the gastroesophageal junction (GEJ), and 23 patients were not administered nitroglycerine. This process was nonrandomized; patients were assigned sublingual nitroglycerine using alternating sequence enrollment number.
Results All the procedures were technically feasible and successful without any major complications. Eckhardt's scores of all patients normalized after the procedure. The mean submucosal tunneling time and mean time across GEJ were 36.8 ± 7.9, 14.2 ± 2.9 in intervention group (sublingual nitroglycerine), and 50.2 ± 8.9 and 23.0 ± 3.6 minutes in nonintervention group, respectively. Time for each procedure was significantly less (p < 0.05) in patients who were given sublingual nitroglycerine. Mucosal injury and bleeding during procedure were not significantly different in the two groups.
Conclusions This POEM technique with the use of sublingual nitroglycerine spray is simple and renders POEM easier and less time consuming.
... Achalasia is characterized by the degeneration of the myenteric plexus ganglion cells, leading to loss of peristalsis in the lower esophagus and failure of lower esophageal sphincter relaxation [1]. Patients may experience dysphagia, chest discomfort, and regurgitation of food particles [2]. Treatment of symptomatic achalasia includes medical therapy with botulinum toxin injection and oral nitrates or surgical methods that lead to symptomatic resolution, including pneumatic dilation, laparoscopic heller myotomy (LHM), and its endoscopic equivalent, POEM [2]. ...
... Patients may experience dysphagia, chest discomfort, and regurgitation of food particles [2]. Treatment of symptomatic achalasia includes medical therapy with botulinum toxin injection and oral nitrates or surgical methods that lead to symptomatic resolution, including pneumatic dilation, laparoscopic heller myotomy (LHM), and its endoscopic equivalent, POEM [2]. POEM and LHM are utilized for treatment-resistant achalasia, and although both are comparably effective in the resolution of symptoms with a success rate of 90%, studies show that POEM is more cost-effective and associated with less postoperative pain than its laparoscopic counterpart [3,4]. ...
Per-oral endoscopic myotomy (POEM) is a minimally invasive procedure that is very effective in the treatment of achalasia, a rare esophageal motility disorder. POEM has become the first-line treatment for achalasia, with high success rates reported in the literature. However, a known complication of POEM is gastroesophageal reflux disease (GERD). The exact cause and risk factors of post-POEM GERD are not fully understood; however, a number of factors have played a role in its development. The management of post-POEM GERD is mainly by conservative measures, such as lifestyle changes and medications, like proton pump inhibitors (PPI), which are often the first-line method of treatment. However, surgical procedures, such as fundoplication, may be necessary in some patients. This literature review will discuss the effectiveness of the use of PPIs as a management strategy for post-POEM GERD, the factors that lead to PPI-resistant GERD, and other management strategies utilized in these cases.
... The current treatment modalities for achalasia cardia include nonsurgical options such as oral pharmacologic therapy, endoscopic pharmacologic therapy such as Botox injections, and pneumatic dilatation. The surgical options include laparoscopic Heller's cardiomyotomy and endoscopic options like peroral endoscopic myotomy (POEM) [4]. ...
Achalasia cardia is a primary motility disorder of the esophagus marked by the absence of peristalsis and the failure of the lower esophageal sphincter (LES) to relax during swallowing. The preferred surgical approach is laparoscopic Heller’s cardiomyotomy with Dor’s fundoplication. Given the significant risks of mucosal perforation and the possibility of incomplete myotomy, which can lead to symptom recurrence, it is essential to ensure both the completeness of the myotomy and the preservation of the mucosal integrity. In this study, we present a case series of 15 patients diagnosed with achalasia cardia who underwent laparoscopic Heller’s cardiomyotomy with Dor’s fundoplication. Intraoperatively, we utilized intraluminal administration of indocyanine green (ICG) dye as an alternative to endoscopy to assess the completeness of the myotomy and to check for any mucosal perforations.
... The current treatment modalities for achalasia cardia include nonsurgical options such as oral pharmacologic therapy, endoscopic pharmacologic therapy such as Botox injections, and pneumatic dilatation. The surgical options include laparoscopic Heller's cardiomyotomy and endoscopic options like peroral endoscopic myotomy (POEM) [4]. ...
... The role of botulinum toxin in the treatment of esophageal motility disorders has been repeatedly discussed [1], although without any encouraging recommendation for its routine use [2][3][4]. The most researched role was in patients with achalasia [5], where the botulinum toxin is applied into the lower esophageal sphincter (LES). ...
The use of botulinum toxin in the therapy of esophageal motility disorders is reserved for elderly and comorbid patients considered risky for endoscopic or surgical treatment. However, there is a lack of data on the treatment of motility disorders outside the Chicago classification.
We present the case of a 56-year-old patient with dysphagia and non-cardial chest pain (Eckardt 8). High resolution manometry ruled out achalasia or other motility disorder, but confirmed a localized 7-cm-long spastic segment in the upper to middle third of esophagus. We considered endoscopic or surgical therapy in this location too risky, therefore we decided to apply botulinum toxin into this segment. The spasm on high resolution manometry correlated with the thickened muscularis propria layer according to the endoscopic ultrasound. We used endoscopic ultrasound for the navigation of botulinum toxin application into the muscularis propria layer. We applied 100 IU of botulinum toxin into four quadrants, 20 and 24 cm from front teeth (12.5 IU for 1 application).
The therapy led to improvement of symptoms (Eckardt 3) and to restitution of propulsive peristalsis with complete elimination of spastic segment. The worsening of symptoms appeared after 2 years, with subsequent recurrence of motility disorder fulfilling criteria of type II achalasia.
Presenting this case, we wanted to point at the unique use of botulinum toxin as useful treatment in selected cases of unclassified esophageal motility disorder as a bridge therapy. Moreover, endoscopic ultrasound could be used to guide precise application of botulinum toxin.
... Management of achalasia is aimed at reducing the pressure of the LES by using botulinum toxin injection or myotomy of the LES muscle [13][14][15][16]. Laparoscopic Heller Myotomy (LHM) has been the gold standard therapy [2,[17][18][19]. The technique consists of an 8cm myotomy extending for 2.5cm onto the gastric wall and a Dor fundoplication [2]. ...
... The 40 mm balloon can be reserved for those who remain unresponsive with an ESS >3. [52][53][54] EsoFLIP (Medtronic, Minneapolis, Minnesota) incorporates impedance planimetry with real-time objective visualisation and monitoring capable of dilating diameters between 10 mm and 30 mm via controlled volumetric distension with an external pressure sensor, thus facilitating graduated dilation during the same session. 55 A recent systematic review of eight ...
Achalasia is a rare oesophageal disease characterised by an unrelaxing lower oesophageal sphincter and abnormal peristalsis of the oesophageal body. Achalasia symptoms include dysphagia to solid and liquid, chest pain, regurgitation and weight loss. Achalasia diagnosis might be delayed for many years when atypical symptoms dominate. Significant progress has been made over the last two decades regarding our understanding of pathophysiology, methods of evaluation and management. The development of high-resolution manometry in particular has improved the diagnosis of achalasia, as well as other major motility disorders. Subtyping achalasia into manometric patterns has pathophysiological and therapeutic implications. Furthermore, complementary tests such as timed barium swallow and the functional lumen imaging probe (EndoFLIP; Crospon Medical Devices, Galway, Ireland) have significantly added to achalasia diagnosis algorithm and management decision-making. Progression in management has helped optimise established therapy (pneumatic dilatation and Heller myotomy) and introduced new endoscopic treatments (peroral endoscopic myotomy) and surgical techniques (surgical robotic Heller myotomy). This review will aim to shed light on the most recent advances in achalasia diagnosis, classification and management.
... Through endoscopy is insensitive for diagnosing primary motility disorders but is necessary to exclude inflammatory and neoplastic conditions of the esophagus that can produce patterns of achalasia or diffuse oesophagal spasm [14]. Upper GI endoscopy is an important mean to follow up the patients with structural lesions of the esophagus [15]. ...
Background: Dysphagia is defined as difficulty in deglutition. It can be either structural or motility abnormality in the passage of food from the oral cavity to the stomach. Upper GI endoscopy is the most important tool to diagnose dysphagia and rule out premalignant and malignant lesions. The purpose of the study was to classify various causes of dysphagia. Methods: This prospective observational study was conducted on 206 patients with complaints of dysphagia. Detailed history, physical examination was done. Upper GI endoscopy was done in all cases, and biopsies were taken if required. Oropharyngeal and neurological dysphagia were excluded from the study. The statistical analysis was performed using Microsoft Excel. The mean, percentage and proportions were calculated. Results: Two hundred and six patients were included in the study. Out of 206 patients, 127 were females, and 79 were males. The mean age was 43.62 years. The commonest age group was 21-40 years contributing 41.7% cases, followed by 41-60 years contributing to 30.8% cases. Benign etiology (n= 141) was more common than that of malignant (n= 65). The commonest benign etiology was reflux esophagitis (n =54) followed by esophageal candidiasis (n= 38). The commonest malignant etiology was adenocarcinoma of the esophagus (n= 38), followed by squamous cell carcinoma (n= 24). Conclusions: The upper GI endoscopy is effective and safe modality to diagnose dysphagia. Benign etiologies were more common among females, but malignant causes were more common among males. The incidence of esophageal malignancy increases with advanced age.
... Achalasia is an infrequent disorder of esophageal motility, known by the lower esophageal sphincter's (LES) inability to relax, coupled with the loss of peristaltic movements within the esophagus. [1,2] This disorder manifests through symptoms including dysphagia (difficulty swallowing), chest pain, regurgitation of undigested food, and weight loss, substantially affecting the life quality of those afflicted. [3,4] The etiology and pathophysiology of achalasia are attributed to the degeneration of esophageal inhibitory neurons, resulting in the inability of the lower esophageal sphincter to relax properly and abnormal motility. ...
Achalasia, a rare esophageal motility disorder characterized by the inability of the lower esophageal sphincter to relax and loss of esophageal peristalsis, significantly impacts pediatric patient quality of life through symptoms like dysphagia, chest pain, and weight loss. This nationwide retrospective cohort study evaluates the efficacy and safety of peroral endoscopic myotomy (POEM) for pediatric achalasia in Mongolia, contributing to the limited global data on this minimally invasive treatment in children. Conducted between February 2020 and March 2022 at 2 tertiary centers, the study included symptomatic achalasia patients, treatment-naive or those with unsatisfactory outcomes from previous esophageal dilations. The POEM procedure was assessed for its impact on esophageal structure and function, symptom severity via the Eckardt score, and procedure-related safety, with outcomes measured at baseline, 3 days, and 12 months post-procedure. The study demonstrated notable post-procedure improvements across all measured outcomes: abnormal contraction length and esophageal width significantly reduced, underscoring the procedure’s effectiveness. More precisely, the integrated relaxation pressure showed a significant improvement from a mean of 26.8 mm Hg (standard deviation [SD], 5.4 mm Hg) pre-procedure to 10.8 mm Hg (SD, 1.1 mm Hg) 12 months ( P < .001). Similarly, Eckardt scores, which assess symptom severity, improved significantly from a pre-procedure mean of 7.0 (SD, 1.0) to a substantially lower score post-procedure ( P < .001), reflecting enhanced patient quality of life and symptom alleviation. This study underscores POEM’s role as an effective, minimally invasive option for pediatric achalasia management within the Mongolian population, offering significant symptomatic relief and improved esophageal function.
... Esophageal achalasia is a rare disorder, and the patients present with a variety of symptoms due to impaired relaxation of the lower esophageal sphincter and lack of esophageal motility. Although rarely lifethreatening, it decreases the quality of life, and cases of malnutrition and sarcopenia have been reported [1][2][3]. The treatment of esophageal achalasia is aimed at symptomatic improvement. ...
Esophageal achalasia is a disease characterized by esophageal motor dysfunction, leading to various symptoms, including vomiting and chest pain. There is no curative treatment for this disease, and the consensus on nutritional therapy or rehabilitation is unclear. Herein, we present the case of a 90-year-old woman with symptoms of esophageal achalasia, exacerbated by secondary sarcopenia and sarcopenic dysphagia after coronavirus disease 2019 (COVID-19) pneumonia. The patient presented with chest pain and vomiting while on a soft diet, and esophagography revealed typical esophageal achalasia. Her esophageal achalasia symptoms resolved, with improvements in nutritional status, skeletal muscle mass, and physical capacity, when a combination of nutritional and comprehensive rehabilitation therapies was adopted. This case highlights that oral dysphagia is associated with worsening esophageal achalasia symptoms and that nutritional and rehabilitative interventions are effective in relieving the symptoms of achalasia in patients with esophageal achalasia and sarcopenia.
... For achalasia, the injection of botulinum toxin has been approved as a temporary solution due to its frequent relapse and the need for further interventions [7]. Standard procedures like pneumatic dilation or laparoscopic heller myotomy (LHM) are techniques that offer symptomatic relief in 80% and 85% of patients, respectively [8,9]. Originally performed in 2008 by Professor Inoue, E-POEM offers various advantages [10]. ...
Background: Intramural surgery techniques, particularly esophageal peroral endoscopic myotomy (E-POEM), gastric peroral endoscopic myotomy (G-POEM), and peroral endoscopic myotomy for Zenker’s (Z-POEM), have emerged as forefront minimally invasive endoscopic procedures. While several studies have reported on the outcomes in North America and Asia, evidence in the Middle East and North Africa remains limited. This study aims to evaluate the feasibility and safety of intramural surgery techniques within this region. Methods: This retrospective cohort study was conducted with approval from the institutional review board. All patients who underwent esophageal peroral endoscopic myotomy, gastric peroral endoscopic myotomy, and peroral endoscopic myotomy for Zenker’s from January 2016 to August 2023 were included. Results: In total, 119 patients underwent intramural surgery procedures during this period. The esophageal peroral endoscopic myotomy group had 81 (68%) patients, the gastric peroral endoscopic myotomy had 34 (28.6%) patients, and the peroral endoscopic myotomy for Zenker’s had 4 (3.4%) patients. The full cohort was 48.7% female, with a mean overall age of 40.5 years. The mean overall body mass index was 27.5 kg/m². The chief complaint was dysphagia (n = 80, 67.2%). All cases were successfully completed endoscopically. During the first 30 days, the most common complications were nausea/vomiting requiring admission (n = 4, 4.76%) and pneumomediastinum (n = 2, 2.38%). At a follow-up of 19 months, there were four mortalities; the causes of death were cardiac arrest (three cases) and end-stage prostate cancer (one case). Conclusions: Intramural surgery techniques are safe and technically feasible with low complication rates. Our study suggests that clinical success in the Middle East and Northern Africa population is comparable to larger international series.
... Keywords Achalasia · POEM · Peroral endoscopic myotomy · GERD · Efficacy Achalasia (AC) is a rare motility disorder [1] with four main clinical manifestations including dysphagia, regurgitation, chest pain, and weight loss [2]. The diagnosis of AC requires and Other Interventional Techniques Content courtesy of Springer Nature, terms of use apply. ...
Background
Peroral endoscopic myotomy (POEM) has emerged as a widely accepted treatment for achalasia, with limited studies for over 2 years. Additionally, traditional measurements of achalasia after POEM have deficiencies. The study aimed to analyze the long-term outcomes of POEM under different criteria.
Methods
Patients with achalasia who received POEM between November 2012 and March 2021 were recruited. Patients and characteristics were shown, and risk factors related to two novel definitions of recurrence, symptomatic reflux, and reflux esophagitis were analyzed.
Results
Three hundred and twenty-one patients were included. At a median follow-up of 52 months, twenty-three failures happened (7.17%) under the modified criterion, and forty-seven failures occurred (14.64%) under the normal standard. Hospitalization (P = 0.027) and esophageal myotomy length (P = 0.039) were significantly associated with long-term efficacy under the modified and normal criteria, respectively. Fifty-two patients (16.20%) reported reflux symptoms and endoscopy performed in 88 patients revealed reflux esophagitis in 22 cases (25.00%). There were no predictors in the analysis of symptomatic reflux and gender (P = 0.010), LESP (P = 0.013), IRP (P = 0.015), and the esophageal myotomy length (P = 0.032) were statistically related to reflux esophagitis.
Conclusion
POEM is an extremely safe and effective treatment for achalasia with long-term follow-up. Shorter hospitalization and shorter esophageal myotomy length may decrease the incidence of recurrence under the modified and normal criteria, respectively. Long-term outcomes of POEM are unpredictable. No risk factors were related to symptomatic reflux, and male patients with low preoperative LESP and IRP needed relatively shorter esophageal myotomy to prevent reflux esophagitis.
... 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). ...
... It is characterized by loss of esophageal peristalsis and impaired lower esophageal sphincter (LES) relaxation. 1 Achalasia is associated with an increased risk of esophageal cancer, as first noted by Fagge 2 in 1872. The incidence of esophageal cancer in patients with achalasia is higher than that in the general population, even though the absolute risk is low. ...
Achalasia, a rare motility disorder of the esophagus, is generally accepted as a premalignant disorder. This paper presents the case of a 72-year-old male with achalasia and synchronous superficial esophageal cancer who experienced dysphagia symptoms for five years. As achalasia is associated with an increased risk of esophageal cancer, both can be treated simultaneously if detected at the time of diagnosis. Achalasia and synchronous esophageal cancer are rarely detected and treated endoscopically. This paper reports a case of concurrent successful treatment.
... It is characterized by esophageal smooth muscle motility disorder and impaired lower esophageal sphincter (LES) relaxation in response to swallowing [1]. The common symptoms of achalasia include dysphagia, regurgitation, heartburn, chest pain, weight loss, and respiratory complications such as nocturnal cough, aspiration, and pneumonia [2][3][4][5][6]. ...
Background
Video-assisted laparoscopic Heller myotomy (LHM) has become the standard treatment option for achalasia. While robotic surgery offering some specific advantages such as better three-dimensional (3D) stereoscopic vision, hand-eye consistency, and flexibility and stability with the endowrist is expected to be shorter in learning curve than that of LHM for surgeons who are proficient in LHM. The aim of this study was to describe a single surgeon’s experience related to the transition from video-assisted laparoscopic to robotic Heller myotomy with Dor fundoplication.
Methods
We conducted a retrospective observational study based on the recorded data of the first 66 Heller myotomy performed with laparoscopic Heller myotomy with Dor fundoplication (LHMD, 26 cases) and with the robotic Heller myotomy with Dor fundoplication (RHMD, 40 cases) by the same surgeon in Department of Thoracic Surgery of The First Affiliated Hospital of Nanchang University in China. The operation time and intraoperative blood loss were analyzed using the cumulative sum (CUSUM) method. Corresponding statistical tests were used to compare outcomes of both serials of cases.
Results
The median operation time was shorter in the RHMD group compared to the LHMD group (130 [IQR 123–141] minutes vs. 163 [IQR 153–169]) minutes, p < 0.001). In the RHMD group, one patient (2.5%) experienced mucosal perforation, whereas, in the LHMD group, the incidence of this complication was significantly higher at 19.2% (5 patients) (p = 0.031). Based on cumulative sum analyses, operation time decreased starting with case 20 in the LHMD group and with case 18 in the RHMD group. Intraoperative blood loss tended to decline starting with case 19 in the LHMD group and with case 16 in the RHMD group.
Conclusions
Both RHMD and LHMD are effective surgical procedures for symptom relief of achalasia patients. RHMD demonstrates superior outcomes in terms of operation time and mucosal perforation during surgery compared to LHMD. Proficiency with RHMD can be achieved after approximately 16–18 cases, while that of LHMD can be obtained after around 19–20 cases.
... Es una entidad poco frecuente que se caracteriza por presentar disfagia para sólidos y líquidos en un 90% de los pacientes, regurgitación de comida no digerida en el 75%, dolor torácico en el 50% y pirosis en el 40% (2) . Como consecuencia de esta sintomatología, los pacientes podrían presentar pérdida de peso, bronquitis y neumonías a repetición (3) . ...
Introducción: existe información limitada sobre las características clínicas y manométricas de los diferentes subtipos de acalasia. Este estudio describe dichas características
en pacientes manejados en un hospital de referencia en Colombia. Método: estudio
descriptivo observacional que incluye a pacientes con diagnóstico de acalasia por manometría esofágica de alta resolución manejados en el Hospital Universitario San Ignacio
de Bogotá, Colombia, entre 2016 y 2020. Se describen las manifestaciones clínicas,
hallazgos manométricos, tratamiento utilizado y respuesta al mismo según el subtipo de
acalasia. Resultados: se incluyeron a 87 pacientes (mediana de edad: 51 años, 56,4%
mujeres). La mayoría de tipo II (78,1%), seguido por tipo I (16%) y tipo III (5,7%). Todos
presentaron disfagia, 40,2% dolor torácico y 27,6% reflujo gastroesofágico. La clínica y
los valores del integral de presión de relajación (IRP; mediana: 24 mm Hg, rango intercuartílico [RIC]: 19-33), presión del esfínter esofágico superior (EES; mediana: 63 mm
Hg, RIC: 46-98) y presión del esfínter esofágico inferior (EEI; mediana: 34 mm Hg, RIC:
26-45) fueron similares en los diferentes subtipos. El aclaramiento esofágico fue incompleto en todos los pacientes. Entre 35 pacientes que recibieron manejo intervencionista,
la miotomía de Heller fue la intervención más utilizada (68,5%), seguido por la dilatación
esofágica (28,6%). La totalidad de estos pacientes presentó una mejoría sintomática con
la mediana de Eckardt pretratamiento de 5 (RIC: 5-6) y postratamiento de 1 (RIC: 1-2).
Conclusiones: la acalasia tipo II es la más común. La clínica y los hallazgos manométricos y respuesta a tratamiento son similares entre los subtipos de acalasia. En Colombia,
esta entidad se comporta de forma similar a lo reportado en otras partes del mundo.
... 4 High-resolution manometry (HRM) is the gold standard diagnostic method in adults. 5 Diagnostic criteria in adults are also used in children, but some normal HRM values may change depending on age. 4 The youngest peroral endoscopic myotomy (POEM) procedure was performed on an 11-month-old boy in 2015. 6 Our case is one of the patients who underwent POEM at the youngest age reported in the literature. ...
Introduction Primary achalasia is an idiopathic motility disorder of the esophagus characterized by esophageal aperistalsis and incomplete relaxation of the lower esophageal sphincter (LES) in response to swallowing. The gold standard diagnostic method in adults is high-resolution manometry (HRM). Diagnostic criteria in adults are also used in children, but some HRM normal values may change depending on age.
Case Report A 15-month-old girl was admitted to the hospital for evaluation due to persistent vomiting since birth. Vomiting included what she ate regardless of the amount of food she consumed. Barium esophagography revealed barium retention, esophageal dilatation, and a “bird's beak appearance” in the distal esophagus. Esophagogastroduodenoscopy revealed stenosis in the lower esophagus and bubbles at the esophagogastric junction.
In HRM, the resting LES pressure was 43.4 mm Hg, there was pan-esophageal pressurization with 60% of swallows and no normal peristalsis. The patient was diagnosed with type II achalasia based on the Chicago 3.0 classification.
First, the tube was inserted to ensure adequate nutrition of the patient, and approximately 4 months later, when the patient was 10 kg, the peroral endoscopic myotomy (POEM) procedure was performed.
No complications developed during and after the procedure. At the 6th month after treatment, the patient was completely asymptomatic and her weight was within normal limits for her age.
Conclusion POEM is an effective and safe method in the treatment of pediatric patients with idiopathic achalasia.
... Patients with achalasia may present with a wide variety of clinical symptoms, progressive dysphagia being the most prominent, which leads to significant impairment in functional status and overall decreased quality of life [5,6]. Management is primarily focused on reducing the LES hypertonicity by pharmacological, endoscopic, or surgical interventions [7]. ...
Background:
Per-oral endoscopic myotomy (POEM) is a rapidly emerging minimally invasive procedure for management of achalasia. Same-day discharge after POEM is safe and feasible; however, some patients may need hospitalization. We aimed to identify characteristics and outcomes for achalasia patients requiring hospitalizations after POEM in the United States (US).
Methods:
The US National Inpatient Sample was utilized to identify all adult achalasia patients who were admitted after POEM from 2016 to 2019. Hospitalization characteristics and clinical outcomes were highlighted.
Results:
From 2016 to 2019, we found that 1,885 achalasia patients were admitted after POEM. There was an increase in the total number of hospitalizations after POEM from 380 in 2016 to 490 in 2019. The mean age increased from 54.2 years in 2016 to 59.3 years in 2019. Most POEM-related hospitalizations were for the 65 - 79 age group (31.8%), females (50.4%), and Whites (68.4%). A majority (56.2%) of the study population had a Charlson Comorbidity Index of 0. The Northeast hospital region had the highest number of POEM-related hospitalizations. Most of these patients (88.3%) were eventually discharged home. There was no inpatient mortality. The mean length of stay decreased from 4 days in 2016 to 3.2 days in 2019, while the mean total healthcare charge increased from 65,109 in 2019. Esophageal perforation was the most common complication seen in 1.3% of patients.
Conclusion:
The number of achalasia patients needing hospitalization after POEM increased. There was no inpatient mortality conferring an excellent safety profile of this procedure.
Background: Our case highlights the challenges in diagnosing and managing achalasia cardia, particularly in resource-limited settings and more so in adolescents who fall outside of the typical age range.
Case Presentation: We present a case of an 18-year-old female from Uganda who was admitted with a 6-month history of progressive dysphagia, weight loss, and postprandial vomiting. Diagnosis of achalasia cardia was confirmed via endoscopy and barium swallow. Heller cardiomyotomy via open transthoracic approach was performed, but she developed an esophageal perforation, which was successfully managed with repeat thoracotomy and esophageal repair. Complete resolution of achalasia symptoms was achieved at a 5-month follow-up.
Conclusion: This case highlights the importance of maintaining a high index of clinical suspicion, especially in young patients, and the significance of informed consent prior to initiating treatment. Additionally, it emphasizes the importance of early recognition of treatment-related complications, such as esophageal perforation, as key to prompt management and improved patient outcomes.
Main Recommendations
Peroral endoscopic myotomy (POEM) is an advanced endoscopic procedure that has become a first-line treatment for esophageal achalasia and other esophageal spastic disorders. Structured training is essential to optimize the outcomes of this technique. The European Society of Gastrointestinal Endoscopy (ESGE) has recognized the need to formalize and enhance training in POEM. This Position Statement presents the results of a systematic review of the literature and a formal Delphi process, providing recommendations for an optimal training program in POEM that aims to produce endoscopists competent in this procedure. In a separate document (POEM curriculum Part II), we provide technical guidance on how to perform the POEM procedure based on the best available evidence.
1 POEM trainees should acquire a comprehensive theoretical knowledge of achalasia and other esophageal motility disorders that encompasses pathophysiology, diagnostic tool proficiency, clinical outcome assessment, potential adverse events, and periprocedural management.
2 Experience in advanced endoscopic procedures (endoscopic mucosal resection and/or endoscopic submucosal dissection [ESD]) is encouraged as a beneficial prerequisite for POEM training.
3 ESGE suggests that POEM trainees without ESD experience should perform an indicative minimum number of 20 cases on ex vivo or animal models before advancing to human POEM cases with an experienced trainer.
4 ESGE recommends that the trainee should observe an indicative minimum number of 20 live cases at expert centers before starting to perform POEM in humans.
5 The trainee should undertake an indicative minimum number of 10 cases under expert supervision for the initial human POEM procedures, ensuring that trainees can complete all POEM steps independently.
6 ESGE recommends avoiding complex POEM cases during the early training phase.
7 POEM competence should reflect the technical success rate, both the short- and long-term clinical success rates, and the rate of true adverse events.
8 A POEM center should maintain a prospective registry of all procedures performed, including patient work-up and outcomes, procedural techniques, and adverse events.
Achalasia is a rare esophageal motility disorder that is mostly manifested by the basic triad of symptoms: dysphagia, regurgitation, and retrosternal pain. Patients often disregard the symptoms for years or are treated for overlapping conditions with similar symptoms, such as GERD, gastritis, or various lung diseases from asthma to obstructive pulmonary disease. A common complication is aspiration leading to pneumonia, and these patients often go from pulmonologists to gastroenterologists in search of a diagnosis and cure. This study presents the case of a patient treated for severe, rare complication of achalasia, megaesophagus and esophagopleural fis-tula. The case study highlights the importance of a multidisciplinary approach, choosing the right therapeutic approach, and objective assessment of the situation, when we decide on one option while another option arises as the only one that is sustainable. In this case it is a complex intensive care approach.
Achalasia is a rare esophageal motility disorder characterized by impaired relaxation of the lower esophageal sphincter and loss of peristalsis. Laparoscopic Heller myotomy (LHM) combined with anti-reflux surgery is considered the gold standard for long-term treatment. However, there is ongoing debate regarding the optimal myotomy length and choice of anti-reflux procedure. This retrospective study analyzed 49 achalasia patients treated between 2016 and 2023 using two Heller myotomy techniques (standard vs. extended) combined with two anti-reflux procedures (Nissen vs. Dor fundoplication). Data on demographic characteristics, surgical details, complications, and postoperative outcomes were collected. Patients were followed up to assess dysphagia, Gastroesophageal reflux disease (GERD) symptoms, recurrence rates, and satisfaction levels. Extended myotomy significantly reduced recurrence rates compared to standard myotomy (0% vs. 22.2%, p = 0.014). Nissen fundoplication was associated with fewer GERD symptoms than Dor fundoplication (p = 0.01). Patient satisfaction was highest in the extended myotomy with the Nissen fundoplication group (p = 0.009). No mortality occurred, and complications were minimal. Postoperative timed water-soluble contrast esophagograms showed effective esophageal emptying in 91.8% of patients. Relapse cases were successfully managed with additional interventions. Extended myotomy with Nissen fundoplication provides superior outcomes in achalasia treatment, including lower recurrence rates, fewer GERD symptoms, and higher patient satisfaction. These findings highlight the importance of myotomy length and anti-reflux procedure selection for optimizing treatment outcomes. Further studies are needed to validate these results and establish standardized protocols.
Objectives
Achalasia is an esophageal motility disorder of unknown etiology. However, no studies have determined the populations in which sleep problems occur and whether they are improved by peroral endoscopic myotomy (POEM). We investigated the rate of sleep problems assessed by GERD‐Q (AGQ) in achalasia patients, evaluated whether POEM improves these issues, and identified factors associated with sleep improvement after POEM.
Methods
We retrospectively analyzed the data of patients who were diagnosed with achalasia and who underwent POEM at a single institution between March 2016 and December 2020. We examined the Eckardt symptom score and the GERD‐Q before and 3 months after POEM to assess the presence of sleep problems (AGQ) and other symptoms. The univariate logistic regression analysis was performed to identify factors associated with sleep problem (AGQ) improvement after POEM.
Results
A total of 177 patients were included. The average age was 52.6 ± 17.2 years. Preoperatively, dysphagia (172 [97.2%]), regurgitation (123 [69.5%]), sleep problems (AGQ; 110 [62.1%]), chest pain (102 [57.6%]), and weight loss (83 [46.9%]) were observed. Before POEM, 62.1% of patients experienced sleep problems (AGQ) compared with 9.6% after POEM (p < 0.0001). Postoperative dysphagia and regurgitation were significant factors determining whether patients continued to experience sleep problems (AGQ) after POEM.
Conclusions
Sleep problems (AGQ) were the third most common symptom in > 60% of patients with achalasia. Improving dysphagia and regurgitation using the POEM procedure improved sleep problems (AGQ).
Gastroesophageal reflux disease (GERD) after peroral endoscopic myotomy (POEM) has been a limiting factor with POEM. Sling-fiber preservation during POEM was reported to reduce postoperative GERD in Japan. This study investigates the efficacy of this technique in a western population. This is a retrospective, single-center study of patients undergoing POEM from October 2017 to January 2023. The initial cohort of patients were treated by conventional POEM, after which a second cohort underwent POEM with sling-fiber preservation. The primary outcome was the incidence of postoperative reflux symptoms. The secondary outcomes were clinical success rate (Eckardt score ≤ 3), procedure time, and adverse events rate. Multivariate regression was then performed to identify factors associated with the incidence of postoperative reflux symptoms. One hundred and forty eight POEM cases (52.5 ± 15.6 y/o, female: 61[43%]) were included. The mean procedure time (108.6 ± 34.5 vs. 109.1 ± 45.7 min, P = 0.93) was similar between the groups. In the sling-fiber preservation group, gastric myotomy length was significantly longer (2.2 ± 0.7 vs. 1.6 ± 0.8 cm, P < 0.05), yet the incidence rate of postoperative reflux symptoms at follow-up was significantly lower (22.4% vs. 42.3%, P < 0.05). The clinical success rate was similar between groups (89.5% vs. 83.1%, P = 0.32), and the rate of adverse events requiring intervention (13.5% vs. 12.2%, P = 0.36) was similar. Regression analysis indicated that, after adjusting for other risk factors of postoperative GERD, sling-fiber preservation during POEM had an odds ratio of 0.24 (95% CI: 0.07–0.85, P < 0.05) for the incidence of reflux symptoms. Sling-fiber preservation during POEM reduces the incidence of postoperative reflux symptoms. As such, sling-fiber preservation may be a useful solution to reduce post-POEM GERD in western populations.
Objectives
This retrospective study aimed to compare treatment outcomes and postoperative courses, including the incidence of esophageal cancer (EC), according to disease types, in 450 achalasia patients who underwent peroral endoscopic myotomy (POEM).
Methods
Data from consecutive POEM procedures performed from September 2011 to January 2023 at a single institution were reviewed. Achalasia was classified into straight (St), sigmoid (S1), and advanced sigmoid (S2) types using esophagography findings. Regarding efficacy, POEM was considered successful if the Eckardt score was ≤3. A statistical examination of the incidence and trend of EC occurrence across the disease type of achalasia was conducted using propensity score matching.
Results
Of the 450 patients, 349 were diagnosed with St, 80 with S1, and 21 with S2. POEM efficacy was 97.9% at 1 year and 94.2% at 2 years postprocedure, with no statistical difference between disease types. Using propensity score matching, the incidence of EC in each disease type was as follows: St, 1% (1/98); S1, 2.5% (2/77); S2, 10% (2/18). While no statistical significance was observed between St (1.0%: 1/98) and all sigmoid types (4.0%, 4/95; P = 0.3686). However, a trend test revealed a tendency for EC to occur more frequently in the order of S2, S1, and St type with a statistically significant difference ( P = 0.0413).
Conclusions
Outcomes of POEM are favorable for all disease types. After POEM, it is important not only to monitor the improvement of achalasia symptoms but also to pay attention to the occurrence of EC, especially in patients with sigmoid‐type achalasia.
A acalasia cricofaríngea é uma enfermidade que gera diminuição do relaxamento da musculatura esofágica, provocando bloqueio da passagem de alimentos através do esfíncter cranial do esôfago. A causa deste distúrbio é desconhecida, contudo sabe-se que geralmente é de origem congênita. É considerada uma doença rara em caninos e em outras espécies, afetando principalmente cães jovens com menos de um ano de idade. Este trabalho objetivou relatar um caso de uma cadela, fêmea, com dois meses de idade, que apresentava sintomatologia de regurgitação, anorexia, tosse, engasgos, pneumonia por aspiração e baixo escore corporal. Exames hematológicos e radiográficos foram solicitados para melhor avaliação do caso, tendo como diagnóstico a acalasia cricofaríngea. O tratamento foi cirúrgico, através de técnica de miectomia cricofaríngea, onde referido músculo foi removido. Após a realização do procedimento, a paciente parou de apresentar sinais clínicos, mesmo após dois meses pós-cirúrgico, obtendo significativo ganho de peso. Conclui-se que a miectomia do músculo cricofaríngeo para correção da acalasia cricofaríngea é uma terapêutica eficiente, uma vez que a paciente apresentou melhora clínica em pouco tempo após a cirurgia.
Achalasia is a rare disease of esophageal motility marked by absent peristalsis and insufficient relaxation of the lower esophageal sphincter. Treatment is palliative and aimed at improving symptoms. The management ranges from medical therapy to endoscopic and surgical interventions, with refractory end stage disease necessitating permanent enteral feeding tube or an esophagectomy. A Heller myotomy has been the main stay of treatment for over a century; a minimally invasive approach is the current standard of care because of its low risk and durable improvement in quality of life for these patients. For the past decade, an endoscopic option known as per oral endoscopic myotomy (POEM) has been increasingly performed and shows comparative symptom relief, offers an incisionless approach, and is the preferred procedure for a certain subtype of achalasia. Here we will detail the diagnosis, workup, and treatment options for this disease.
Endoluminal Functional Lumen Imaging Probe (EndoFLIP) is a device that measures gastro-esophageal junction (GEJ) distensibility. However, it is not demonstrated that GEJ distensibility increases proportionally with varying gastric myotomy length in peroral endoscopic myotomy (POEM). This study aimed to investigate the association between gastric myotomy length in POEM and intraoperative EndoFLIP findings.
This single-center, retrospective cohort study included patients who underwent POEM with intraoperative EndoFLIP from December 2019 to January 2023. Using EndoFLIP, minimal balloon diameter and its distensibility index (DI) were measured pre- and post-myotomy. Primary and secondary outcomes were the post-myotomy EndoFLIP findings at 30 ml and 40 ml volume fills.
The study included 44 patients (mean age 53.1 years, 50% female). Chicago classification included achalasia type I (39%), II (41%), III (9%), hypercontractile esophagus (2%), and EGJOO (9%). The mean esophageal myotomy length was 7.5 ± 2.2 cm and gastric myotomy was 2.1 ± 0.6 cm. Simple linear regression analyses indicated that for each 1 cm increase in gastric myotomy length, the DI at 30 ml volume fill was estimated to increase by 2.0 mm2/mmHg (p < 0.05, R2 = 0.41), the minimal diameter at 30 ml volume fill was estimated to increase by 2.4 mm (p < 0.05, R2 = 0.48), and the minimal diameter at 40 ml volume fill was estimated to increase by 1.3 mm (p < 0.05, R2 = 0.09).
This study demonstrates a significant linear relationship between gastric myotomy length and GEJ distensibility measured by EndoFLIP during POEM. These findings may be useful in clinical practice by enabling EndoFLIP to help calibrate a desired gastric myotomy length to achieve optimal DI and minimal diameter.
Objectives
The prevalence of dysphagia is 16–22%. Achalasia is characterised by impaired relaxation of the lower oesophageal sphincter (LES) and absent oesophageal peristalsis. Minimally invasive procedures, endoscopic pneumatic balloon dilatation (PD) and peroral endoscopic myotomy (POEM) are treatments of choice. We aimed to assess the manometric profile of patients presented with dysphagia and pre- and post-LES pressure and integrated relaxation pressure (IRP) of patients who underwent PD and POEM.
Materials and Methods
One hundred and twenty-nine patients presented with dysphagia underwent high resolution oesophageal manometry (HREM). Manometric parameters: IRP, LES and pattern of motility were noted. According to Chicago classification 4.0, those who are diagnosed with achalasia underwent PD (17) and POEM (15). Statistical analysis was performed using the Statistical Package for the Social Sciences version 28. Pre- and post-procedure values (IRP and basal LES) were compared using the Wilcoxon signed-rank test.
Results
Out of 129 patients with dysphagia, 57 had increased IRP and 75 had simultaneous motility. The mean LES pressure and IRP were found to be 28 ± 19.82 and 17 ± 14.04 mmHg, respectively. On analysis, of patients who underwent procedures PD (17) and POEM (15), a statistically significant decrease in IRP was observed in patients with post POEM compared to post PD status ( P = 0.005 and 0.017, respectively). Reduced basal LES pressure was observed in patients with post-POEM status compared to post-PD status, which was not statistically significant.
Conclusion
About 44% of patients presented with dysphagia were diagnosed with achalasia cardia. POEM procedure was found to be more effective compared to pneumatic dilatation.
Achalasia is characterised by incomplete relaxation of the lower oesophageal sphincter and aberrant oesophageal peristaltic activity resulting in impaired oesophageal emptying. This rare condition in pregnancy is unique as both the disease and its treatment are associated with fetomaternal risks and complications. A woman in her early 30s, gravida 3 para 2 at 35 weeks’ pregnancy with suspected oesophageal achalasia, presented with shortness of breath, cough and fever following frequent bouts of vomiting and fluid regurgitation. She was diagnosed with aspiration pneumonia complicated by severe metabolic acidosis, malnutrition syndrome and fetal growth restriction. Following stabilisation of the acute clinical problems, delivery was expedited via caesarean section. Postpartum endoscopy confirmed the diagnosis of achalasia as per initial suspicion. Definitive surgery was performed several months later after optimisation of the patient’s nutritional status. This case illustrates the life-threatening complications of achalasia in pregnancy.
Achalasia is defined as dysfunction of the esophageal wall myenteric plexus, which causes symptoms of dysphagia. While manometry is typically regarded as the gold standard for diagnosing and confirming achalasia, other imaging modalities such as barium swallow and upper endoscopy are often obtained initially. The barium swallow study can be a supportive or confirmatory test, whereas the upper endoscopy is typically used to rule out pseudo-achalasia. Additionally, barium swallow is an imaging modality of choice in resource-limited settings. A standardized approach for categorizing motility disorders is the Chicago Classification. This is a newer classification of achalasia based on high-resolution manometry. However, the role of barium contrast studies does not seem to have been evaluated in the context of the Chicago Classification of the three achalasia subtypes, suggesting an underappreciated role of fluoroscopy in the diagnostic evaluation of achalasia subtypes.
Introducción. La miotomía de Heller como tratamiento quirúrgico de elección para la acalasia, ofrece de buenos a excelentes resultados a corto y largo plazo, pero, incluso en manos de cirujanos experimentados, pueden presentarse complicaciones. Objetivos. Los objetivos del estudio fueron evaluar la frecuencia de perforaciones de la mucosa esofágica cuando se practica la miotomía de Heller para tratar la acalasia, determinar las características preoperatorias de los pacientes que presentan este tipo de complicación y evaluar su evolución posoperatoria. Materiales y métodos. Se hizo una revisión retrospectiva de una base de datos. Se evaluó la incidencia de las perforaciones de la mucosa esofágica durante la miotomía de Heller por vía laparoscópica en 116 pacientes con diagnóstico de acalasia, en el Center for Esophageal Diseases de la University of Chicago. Además, se describe el tratamiento de este tipo de lesiones descubiertas durante el procedimiento. Resultados. De 116 pacientes sometidos a miotomía de Heller por laparoscopia, en 4 se presentó perforación de la mucosa esofágica durante el procedimiento, con una incidencia de 3,4 %. En ellos la perforación se reparó en el mismo procedimiento. La estancia hospitalaria media fue de dos días y el puntaje posoperatorio de Eckardt fue de 0 en todos los pacientes. Conclusiones. En las manos adecuadas, la miotomía de Heller es un procedimiento seguro y efectivo para el tratamiento de la acalasia, con una baja tasa de complicaciones intraoperatorias. La detección y el tratamiento de las perforaciones esofágicas durante el procedimiento quirúrgico, ofrecen excelentes resultados.
Background
Laparoscopic surgery remains the mainstay of treating foregut pathologies. Several studies have shown improved outcomes with the robotic approach. A systematic review and meta-analysis comparing outcomes of robotic and laparoscopic hiatal hernia repairs (HHR) and Heller myotomy (HM) repairs is needed.
Study Design
PubMed, Embase and Scopus databases were searched for studies published between January 2010 and November 2022. The risk of bias was assessed using the Cochrane ROBINS-I tool. Assessed outcomes included intra- and post-operative outcomes. We pooled the dichotomous data using the Mantel-Haenszel random effects model to report odds ratio (OR) and 95% confidence intervals (95% CIs) and continuous data to report mean difference (MD) and 95% CIs.
Results
Twenty-two comparative studies enrolling 196,339 patients were included. Thirteen (13,426 robotic, 168,335 laparoscopic patients) studies assessed HHR outcomes, while nine (2,384 robotic, 12,225 laparoscopic patients) assessed HM outcomes. Robotic HHR had a non-significantly shorter length of hospital stay (LOS) [MD -0.41 (95% CI -0.87, -0.05)], fewer conversions to open [OR 0.22 (95% CI 0.03, 1.49)], and lower morbidity rates [OR 0.76 (95% CI 0.47, 1.23)]. Robotic HM led to significantly fewer esophageal perforations [OR 0.36 (95% CI 0.15, 0.83)], reinterventions [OR 0.18 (95% CI 0.07, 0.47)] a non-significantly shorter LOS [MD -0.31 (95% CI -0.62, 0.00)]. Both robotic HM and HHR had significantly longer operative times.
Conclusions
Laparoscopic and robotic HHR and HM repairs have similar safety profiles and perioperative outcomes. Randomized controlled trials are warranted to compare the two methods, given the low to moderate quality of included studies.
Gastrointestinal motility disorders are caused by primary or secondary abnormalities of the neuromuscular system of the oropharynx, esophagus, stomach, and intestine. These are divided into two broad categories: oropharyngeal and upper esophageal motility disorders and lower esophageal motility disorders. In the last category, the most representative pathologies are achalasia and diffuse esophageal spasm. The most affected population by these diseases are young/middle-aged people. For diagnosis are recommended radiological, endoscopic, or manometric methods. Treatment of patients with lower esophageal motility disorders such as achalasia or DES includes medical, endoscopic, or surgical therapies, with individual indication and treatment response.
Background/purpose:
Peroral endoscopic myotomy (POEM), a novel minimally invasive treatment for esophageal achalasia, has been shown to be effective and safe for both adult and pediatric patients. However, studies on its application in children in Taiwan and its impact on growth and esophageal motility are lacking.
Methods:
We conducted a retrospective study on consecutive pediatric patients who were diagnosed with esophageal achalasia at National Taiwan University Hospital and underwent POEM during 2015-2022. Disease characteristics and treatment outcomes were analyzed.
Results:
Ten patients (age 16.9 ± 3.1 years), nine newly diagnosed and one previously treated with pneumatic dilatation, underwent POEM for achalasia (type I/II/III: 3/7/0). Average symptom duration before diagnosis was 19.4 ± 19.9 months, mean POEM procedure time was 83.6 ± 30.7 min, and clinical success (Eckardt score ≤3) was achieved in all patients. Eight patients experienced mild adverse events during POEM, but none required further endoscopic or surgical intervention. Over a mean follow-up period of 3.7 ± 1.6 years, mean Eckardt score decreased significantly from 5.7 ± 2.4 to 1.1 ± 0.7 (p = 0.0001). The BMI z-score also increased significantly after POEM (p = 0.023). Five patients received follow-up high-resolution impedance manometry (HRIM), and all had improved lower esophageal sphincter resting pressures (p = 0.011), body contractility, and bolus transit (p = 0.019).
Conclusion:
POEM is an effective and safe treatment for pediatric achalasia in Taiwan. Early diagnosis and treatment with POEM may help to restore esophageal function and nutrition status in children.
Background—Intrasphincteric injection of botulinum toxin (Botx) has been pro- posed as treatment for oesophageal acha- lasia. However, the predictors of response and optimal dose remain unclear. Aims—To compare the eVect of diVerent doses of Botx and to identify predictors of response. Patients/methods—A total of 118 achalasic patients were randomised to receive one of three doses of Botx in a single injection: 50 U (n=40), 100 U (n=38), and 200 U (n=40). Of those who received 100 U, responsive patients were reinjected with an identical dose after 30 days. Clinical and manomet- ric assessments were performed at base- line, 30 days after the initial injection of botulinum toxin, and at the end of follow up (mean 12 months; range 7-24 months). Results—Thirty days after the initial in- jection, 82% of patients were considered responders without a clear dose related eVect. At the end of follow up however, relapse of symptoms was evident in 19% of patients who received two injections of 100 U compared with 47% and 43% in the 50 U and 200 U groups, respectively. Using Kaplan-Meier analysis, patients in the 100×2 U group were more likely to remain in remission at any time (p
Background: Peroral endoscopic myotomy (POEM) was developed by our group to provide a less invasive permanent treatment for esophageal achalasia.
Patients and methods: POEM was performed in 17 consecutive patients with achalasia (10 men, 7 women; mean age 41.4 years). A long submucosal tunnel was created (mean length 12.4 cm), followed by endoscopic myotomy of circular muscle bundles of a mean total length of 8.1 cm (6.1 cm in distal esophagus and 2.0 cm in cardia). Smooth passage of an endoscope through the gastroesophageal junction was confirmed at the end of the procedure.
Results: In all cases POEM significantly reduced the dysphagia symptom score (from mean 10 to 1.3; P = 0.0003) and the resting lower esophageal sphincter (LES) pressure (from mean 52.4 mmHg to 19.9 mmHg; P = 0.0001). No serious complications related to POEM were encountered. During follow-up (mean 5 months), additional treatment or medication was necessary in only one patient (case 17) who developed reflux esophagitis (Los Angeles classification B); this was well controlled with regular intake of protein pump inhibitors (PPIs).
Conclusions: The short-term outcome of POEM for achalasia was excellent; further studies on long-term efficacy and on comparison of POEM with other interventional therapies are awaited.
Objectives:
Pneumatic dilation (PD) and laparoscopic Heller myotomy (LHM) can be definitive therapies for achalasia; recent data suggest comparable efficacy. However, risk must also be considered. We reviewed the major complication rate of PD and LHM in a high-volume center and reviewed the corresponding literature.
Methods:
We reviewed 12 years of our institution's achalasia treatment experience. During this interval, a consistent technique of PD was used utilizing Rigiflex dilators. Medical records were reviewed for post-procedure complications. We administered a telephone survey and examined medical records to assess efficacy of treatment. We also performed a systematic review of the literature for comparable clinical data and examined 80 reports encompassing 12,494 LHM and PD procedures.
Results:
At our center, 463 achalasia patients underwent 567 PD or LHM procedures. In all, 78% of the PDs used a 30-mm Rigiflex dilator. In all, 157/184 (85%) patients underwent 1 or 2 PD without any subsequent treatment. There were seven clinically significant perforations; one from PD and six from LHM. There were no resultant deaths from these perforations; two deaths occurred within 30 days of LHM from unrelated causes. Complications and deaths post-PD were significantly fewer than those post-LHM (P=0.02).
Conclusions:
Esophageal perforation from PD at our high-volume center was less common than often reported and lower than that associated with LHM. We conclude that, in the hands of experienced operators using conservative technique, PD has fewer major complications and deaths than LHM.
Background:
The type of fundoplication that should be performed in conjunction with Heller myotomy for esophageal achalasia is controversial. We prospectively compared anterior fundoplication (Dor) with partial posterior fundoplication (Toupet) in patients undergoing laparoscopic Heller myotomy.
Methods:
A multicenter, prospective, randomized-controlled trial was initiated to compare Dor versus Toupet fundoplication after laparoscopic Heller myotomy. Outcome measures were symptomatic GERD scores (0-4, five-point Likert scale questionnaire) and 24-h pH testing at 6-12 months after surgery. Data are mean ± SD. Statistical analysis was by Mann-Whitney U test, Wilcoxon signed rank test, and Freidman's test.
Results:
Sixty of 85 originally enrolled and randomized patients who underwent 36 Dor and 24 Toupet fundoplications had follow-up data per protocol for analysis. Dor and Toupet groups were similar in age (46.8 vs. 51.7 years) and gender (52.8 vs. 62.5% male). pH studies at 6-12 months in 43 patients (72%: Dor n = 24 and Toupet n = 19) showed total DeMeester scores and % time pH < 4 were not significant between the two groups. Abnormal acid reflux was present in 10 of 24 Dor group patients (41.7%) and in 4 of 19 Toupet patients (21.0%) (p = 0.152). Dysphagia and regurgitation symptom scores improved significantly in both groups compared to preoperative scores. No significant differences in any esophageal symptoms were noted between the two groups preoperatively or at follow-up. SF-36 quality-of-life measures changed significantly from pre- to postoperative for five of ten domains in the Dor group and seven of ten in the Toupet patients (not significant between groups).
Conclusion:
Laparoscopic Heller myotomy provides significant improvement in dysphagia and regurgitation symptoms in achalasia patients regardless of the type of partial fundoplication. Although a higher percentage of patients in the Dor group had abnormal 24-h pH test results compared to those of patients who underwent Toupet, the differences were not statistically significant.
TO THE EDITOR: We would like to appreciate the interest shown in our article by Dr. Joo. Our response to his queries is as follows:
(1) The risk factors for prediction of recurrence after endoscopic pneumatic dilatation are young age, male sex, shorter duration of symptoms, low pre-treatment lower esophageal sphincter (LES) pressure and post therapy LES pressure above 10 mmHg. In the present study, the response to pneumatic dilatation was not influenced by sex and age.1 In a retrospective study of 52 patients by Mehta et al2 regarding the response of achalasia to pneumatic dilatation, there was no significant difference with respect to gender but younger age was associated with poor response in the Indian subpopulation. The reason why pneumatic dilatation is less effective in male is not known while Ghoshal et al3 have hypothesized that this might be due to the stronger LES musculature in men.
The previous studies have shown that younger patients had a poor outcome. The Indian sub-population studies by Ghoshal et al4 have shown that age did not affect the outcome of pneumatic dilatation which is similar to our study. The explanation for the worse outcome with young age is unknown.
Post pneumatic dilatation manometry was not done in the present study. In the present study, the statistically insignificant association between age and gender with the outcome may be due to the smaller number of patients in each of the subtypes.1
(2) In our study, the failure of therapy was defined as the persistence of symptoms for 4 weeks after pneumatic dilatation and need for further dilatations. The definition of successful treatment has been described in the methodology of the article.
(3) The major finding in our study was that the typing of achalasia predicted the response to pneumatic dilatation. We agree that the number of patients in our study was small. A study by Pandolfino et al5 has shown similar results. A larger prospective study with a large number of patients in each subtype and long-term follow-up is needed to further validate the findings of our study.
Many experts consider laparoscopic Heller's myotomy (LHM) to be superior to pneumatic dilation for the treatment of achalasia, and LHM is increasingly considered to be the treatment of choice for this disorder.
We randomly assigned patients with newly diagnosed achalasia to pneumatic dilation or LHM with Dor's fundoplication. Symptoms, including weight loss, dysphagia, retrosternal pain, and regurgitation, were assessed with the use of the Eckardt score (which ranges from 0 to 12, with higher scores indicating more pronounced symptoms). The primary outcome was therapeutic success (a drop in the Eckardt score to ≤3) at the yearly follow-up assessment. The secondary outcomes included the need for retreatment, pressure at the lower esophageal sphincter, esophageal emptying on a timed barium esophagogram, quality of life, and the rate of complications.
A total of 201 patients were randomly assigned to pneumatic dilation (95 patients) or LHM (106). The mean follow-up time was 43 months (95% confidence interval [CI], 40 to 47). In an intention-to-treat analysis, there was no significant difference between the two groups in the primary outcome; the rate of therapeutic success with pneumatic dilation was 90% after 1 year of follow-up and 86% after 2 years, as compared with a rate with LHM of 93% after 1 year and 90% after 2 years (P=0.46). After 2 years of follow-up, there was no significant between-group difference in the pressure at the lower esophageal sphincter (LHM, 10 mm Hg [95% CI, 8.7 to 12]; pneumatic dilation, 12 mm Hg [95% CI, 9.7 to 14]; P=0.27); esophageal emptying, as assessed by the height of barium-contrast column (LHM, 1.9 cm [95% CI, 0 to 6.8]; pneumatic dilation, 3.7 cm [95% CI, 0 to 8.8]; P=0.21); or quality of life. Similar results were obtained in the per-protocol analysis. Perforation of the esophagus occurred in 4% of the patients during pneumatic dilation, whereas mucosal tears occurred in 12% during LHM. Abnormal exposure to esophageal acid was observed in 15% and 23% of the patients in the pneumatic-dilation and LHM groups, respectively (P=0.28).
After 2 years of follow-up, LHM, as compared with pneumatic dilation, was not associated with superior rates of therapeutic success. (European Achalasia Trial Netherlands Trial Register number, NTR37, and Current Controlled Trials number, ISRCTN56304564.).
High-resolution manometry (HRM) with pressure topography is used to subtype achalasia cardia, which has therapeutic implications. The aim of this study was to compare the clinical characteristics, manometric variables and treatment outcomes among the achalasia subtypes based on the HRM findings.
The patients who underwent HRM at the Asian Institute of Gastroenterology, Hyderabad between January 2008 and January 2009 were enrolled. The patients with achalasia were categorized into 3 subtypes: type I - achalasia with minimum esophageal pressurization, type II - achalasia with esophageal compression and type III - achalasia with spasm. The clinical and manometric variables and treatment outcomes were compared.
Eighty-nine out of the 900 patients who underwent HRM were diagnosed as achalasia cardia. Fifty-one patients with a minimum follow-up period of 6 months were included. Types I and II achalasia were diagnosed in 24 patients each and 3 patients were diagnosed as type III achalasia. Dysphagia and regurgitation were the main presenting symptoms in patients with types I and II achalasia. Patients with type III achalasia had high basal lower esophageal sphincter pressure and maximal esophageal pressurization when compared to types I and II. Most patients underwent pneumatic dilatation (type I, 22/24; type II, 20/24; type III, 3/3). Patients with type II had the best response to pneumatic dilatation (18/20, 90.0%) compared to types I (14/22, 63.3%) and III (1/3, 33.3%).
The type II achalasia cardia showed the best response to pneumatic dilatation.
Achalasia is an esophageal motility disorder of unknown cause, characterised by aperistalsis of the esophageal body and impaired lower esophageal sphincter relaxation. Patients present at all ages, primarily with dysphagia for solids/liquids and bland regurgitation. The diagnosis is suggested by barium esophagram or endoscopy and confirmed by esophageal manometry. Achalasia cannot be cured. Instead, our goal is to relieve symptoms, improve esophageal emptying and prevent the development of megaesophagus. The most successful therapies are pneumatic dilation and surgical myotomy. The advantages of pneumatic dilation include an outpatient procedure, minimal pain, return to work the next day, mild if any GERD, and can be performed in any age group and even during pregnancy. Pneumatic dilation does not hinder future myotomy, and all cost analyses find it less expensive than Heller myotomy. Laparoscopic myotomy with a partial fundoplication has the advantage of being a single procedure, dysphagia relief is longer at the cost of more troubling heartburn, and a myotomy may be more effective treatment in adolescents and younger adults, especially men. Over a two year horizon, the clinical success of pneumatic dilation and laparoscopic myotomy are comparable in a recent large European randomised trial. The prognosis for achalasia patients to return to near-normal swallowing and good quality of life are excellent, but few are "cured" with a single treatment and intermittent "touch up" procedures may be required.
A new manometric classification of esophageal achalasia has recently been proposed that also suggests a correlation with the final outcome of treatment. The aim of this study was to investigate this hypothesis in a large group of achalasia patients undergoing laparoscopic Heller-Dor myotomy.
We evaluated 246 consecutive achalasia patients who underwent surgery as their first treatment from 2001 to 2009. Patients with sigmoid-shaped esophagus were excluded. Symptoms were scored and barium swallow X-ray, endoscopy, and esophageal manometry were performed before and again at 6 months after surgery. Patients were divided into three groups: (I) no distal esophageal pressurization (contraction wave amplitude <30 mmHg); (II) rapidly propagating compartmentalized pressurization (panesophageal pressurization >30 mmHg); and (III) rapidly propagating pressurization attributable to spastic contractions. Treatment failure was defined as a postoperative symptom score greater than the 10th percentile of the preoperative score (i.e., >7).
Type III achalasia coincided with a longer overall lower esophageal sphincter (LES) length, a lower symptom score, and a smaller esophageal diameter. Treatment failure rates differed significantly in the three groups: I = 14.6% (14/96), II = 4.7% (6/127), and III = 30.4% (7/23; p = 0.0007). At univariate analysis, the manometric pattern, a low LES resting pressure, and a high chest pain score were the only factors predicting treatment failure. At multivariate analysis, the manometric pattern and a LES resting pressure <30 mmHg predicted a negative outcome.
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.
Achalasia patients are considered at increased risk for esophageal cancer, but the reported relative risks vary. Identification of this risk is relevant for patient management. We performed a prospective evaluation of the esophageal cancer risk in a large cohort of achalasia patients with long-term follow-up.
Between 1975 and 2006, all patients diagnosed with primary achalasia in our hospital were treated and followed by the same protocol. After graded pneumatic dilatation, all patients were offered a fixed surveillance protocol including gastrointestinal endoscopy with esophageal biopsy sampling.
We surveyed a cohort of 448 achalasia patients (218 men, mean age 51 years at diagnosis, range 4-92 years) for a mean follow-up of 9.6 years (range 0.1-32). Overall, 15 (3.3%) patients (10 men) developed esophageal cancer (annual incidence 0.34 (95% confidence interval 0.20-0.56)). The mean age at cancer diagnosis was 71 years (range 36-90) after a mean of 11 years (range 2-23) following initial presentation, and a mean of 24 years (range 10-43) after symptom onset. The relative hazard rate of esophageal cancer was 28 (confidence interval 17-46) compared with an age- and sex-identical population in the same timeframe. Five patients received a potential curative treatment.
Although the gastro-esophageal cancer risk in patients with longstanding achalasia is much higher than in the general population, the absolute risk is rather low. Despite structured endoscopical surveillance, most neoplastic lesions remain undetected until an advanced stage. Efforts should be made to identify high-risk groups and develop adequate surveillance strategies.
Some patients with suspected achalasia are found on manometry to have preserved peristalsis, thereby excluding that diagnosis. This study evaluated a series of such patients with functional esophagogastric junction (EGJ) obstruction.
Among 1,000 consecutive high-resolution manometry studies, 16 patients had functional EGJ obstruction characterized by impaired EGJ relaxation and intact peristalsis. Eight patients with post-fundoplication dysphagia and similarly impaired EGJ relaxation were studied as a comparator group with mechanical obstruction. Intrabolus pressure (IBP) was measured 1 cm proximal to the EGJ. Sixty-eight normal controls were used to define normal IBP. Patients' clinical features were evaluated.
Functional EGJ obstruction patients presented with dysphagia (96%) and/or chest pain (42%). IBP was significantly elevated in idiopathic and post-fundoplication dysphagia patients versus controls. Among the idiopathic EGJ obstruction group treated with pneumatic dilation, BoTox(TM), or Heller myotomy, only the three treated with Heller myotomy responded well. Among the post-fundoplication dysphagia patients, three of four responded well to redo operations.
Functional EGJ obstruction is characterized by pressure topography metrics demonstrating EGJ outflow obstruction of magnitude comparable to that seen with post-fundoplication dysphagia. Affected patients experience dysphagia and/or chest pain. In some cases, functional EGJ obstruction may represent an incomplete achalasia syndrome.
With the increasing availability of manometry, patients with achalasia are often referred at an early stage when they lack the classic features of established disease. A prospective five year study of the presenting features of untreated achalasia referred to our department was undertaken. Twenty men and 18 women presented throughout adult life, with a mean age at the time of diagnosis of 44 years (range 17 to 76 years). The presenting symptoms were dysphagia: for solids (100%) and for liquids (97%), chest pain (74%), and weight loss (60%). Endoscopy was reported as normal in 15 patients and achalasia was suggested in only 21 of 33 barium examinations. Fourteen had been treated for gastrooesophageal reflux but none had been misdiagnosed as having cardiac or psychiatric disease. The annual incidence of achalasia in the Lothian region is 0.8/100,000 of population. Persistent dysphagia is the cardinal symptom of achalasia which presents throughout adult life. Nevertheless, recent onset achalasia is often misdiagnosed as gastrooesophageal reflux disease. Because endoscopy is frequently normal and the diagnosis is often not made by radiology, manometric investigation is necessary if the condition is to be recognised and treated at an early stage.
The use of a constantly perfused side hole sensor (CPSH) for continuous measurement of maximal lower esophageal sphincter pressure (LESP) is associated with important but variable undermeasurement as a result of side hole displacement. A 5 cm long pressure sensor has been developed which measures maximal LESP continuously in the face of movement of the sphincter within the sensor length. This sensor, the perfused sleeve, is described, and validated by comparison with CPSH in man, the dog, and a model esophageal sphincter. The sleeve detects maximal LESP accurately, regardless of sphincter length, over the pressure range encountered in the lower esophageal sphincter. Continuous recording of LESP with the sleeve is unaffected by displacement that causes serious undermeasurement with CPSH.
Objective.
—To determine more precise and accurate cancer risk estimates for achalasia that could be used to plan surveillance.
Design.
—Cohort.
Setting.
—Swedish population.
Participants.
—All patients with achalasia listed in the population-based Swedish Inpatient Register from 1964 through 1989.
Main Outcome Measures.
—The observed number of cancers in the cohort was compared with expected numbers of cancers (standardized incidence ratio [SIR]) for each 5-year age group and calendar year of observation, calculated using data from the Swedish Cancer Registry.
Results.
—A total of 1062 patients with achalasia accumulated 9864 years of follow-up. The mean age at entry was 57.2 years, and the mean age at cancer diagnosis was 71.0 years. Esophageal cancer occurred in 24 patients. The risk of esophageal cancer in the first year after achalasia diagnosis was extremely high (SIR, 126.3; 95% confidence interval [Cl], 63.0 to 226.1) as a consequence of prevalent cancers leading to distal esophageal obstruction simulating achalasia. During years 1 to 24, the risk was increased more than 16-fold (SIR, 16.6; 95% CI, 8.8 to 28.3). Annual surveillance after the first year would require 406 endoscopic examinations in men and 2220 in women to detect one cancer.
Conclusions.
—Patients with achalasia are at markedly increased risk of developing esophageal cancer. A substantial number of surveillance examinations might be required to screen for cancers, especially in women. It is not known whether surveillance will result in improved survival.(JAMA. 1995;274:1359-1362)
Patients with diffuse esophageal spasm (DES) and nutcracker esophagus/high amplitude esophageal contraction (HAEC) have a thicker esophageal muscularis propria than do healthy subjects. The goals of this study were to determine the esophageal muscle cross-sectional area (MCSA), a measure of muscle mass, in patients with achalasia of the esophagus; and to compare it with that in patients with DES, patients with HAEC, and normal subjects.Methods
Using a high-frequency ultrasound probe catheter, concurrent manometry and ultrasound images of the esophagus were recorded in four subject groups: normal volunteers, patients with HAEC, patients with DES, and patients with achalasia of the esophagus. Recordings were obtained from the lower esophageal sphincter (LES) and multiple sites in the esophagus 2, 4, 6, 8, and 10 cm above the LES.ResultsThe LES and esophageal muscle thickness as well as esophageal MCSA were greater in all three patient groups than in the normal subject group. Muscle thickness and MCSA were observed to be greatest in patients with achalasia, which were greater than in patients with DES, which were greater than in those with HAEC, which in turn were greater than in normal subjects.Conclusions
We propose that an increase in the MCSA is an important feature of patients with primary motility disorders of the esophagus. The degree of increase in muscle mass may be an important determinant of the type and the severity of esophageal motor dysfunction.
The American Journal of Gastroenterology is published by Nature Publishing Group (NPG) on behalf of the American College of Gastroenterology (ACG). Ranked the #1 clinical journal covering gastroenterology and hepatology*, The American Journal of Gastroenterology (AJG) provides practical and professional support for clinicians dealing with the gastroenterological disorders seen most often in patients. Published with practicing clinicians in mind, the journal aims to be easily accessible, organizing its content by topic, both online and in print. www.amjgastro.com, *2007 Journal Citation Report (Thomson Reuters, 2008)
The American Journal of Gastroenterology is published by Nature Publishing Group (NPG) on behalf of the American College of Gastroenterology (ACG). Ranked the #1 clinical journal covering gastroenterology and hepatology*, The American Journal of Gastroenterology (AJG) provides practical and professional support for clinicians dealing with the gastroenterological disorders seen most often in patients. Published with practicing clinicians in mind, the journal aims to be easily accessible, organizing its content by topic, both online and in print. www.amjgastro.com, *2007 Journal Citation Report (Thomson Reuters, 2008)
Background Achalasia is an oesophageal motility disorder, of unknown cause, which results in increased lower oesophageal sphincter (LOS) tone and symptoms of difficulty swallowing. Treatments are aimed at reducing the LOS tone. Current endoscopic therapeutic options include pneumatic dilation (PD) or botulinum toxin injection (BTX). Objectives The objective of this review was to compare the efficacy and safety of two endoscopic treatments, pneumatic dilatation and intrasphincteric botulinum toxin injection, in the treatment of oesophageal achalasia. Search strategy We searched the Cochrane Upper Gastrointestinal and Pancreatic Diseases Group trials register, the Cochrane Central Register of Controlled Trials, MEDLINE (1966 to Oct 2005), EMBASE (1980 to Oct 2005), BIOSIS (1969 to Oct 2005) and Web of Science (1955 to October 2005). We also searched abstracts from significant Gastroenterology meetings (DDW, UEGW) and reference lists of articles. Selection criteria Randomised controlled trials comparing PD to BTX injection in patients with primary achalasia. Data collection and analysis Two review authors independently performed quality assessment and data extraction. Main results Six studies involving 178 participants were included. Two studies were excluded from the meta-analysis of remission rates on the basis of clinical heterogeneity of the initial endoscopic protocols. There was no significant difference in remission between PD or BTX treatment within four weeks of the initial intervention, with a relative risk of remission of 1.15 ( 95% CI 0.95 to 1.38, P = 0.39) for PD compared to BTX. There was also no significant difference in the mean oesophageal pressures between the treatment groups; weighted mean difference for PD of -0.77 ( 95% CI -2.44 to 0.91, P = 0.37). Data on remission rates following the initial endoscopic treatment was available for two studies at six months and three studies at 12 months. At six months 22 of 29 PD participants were in remission compared to 7 of 27 in the BTX group, giving a relative risk of 2.90 ( 95% CI 1.48 to 5.67, P = 0.002); whilst at 12 months 33 of 47 PD participants were in remission compared to 11 of 43 BTX participants, relative risk of 2.67 ( 95% CI 1.58 to 4.52, P = 0.0002). No serious adverse outcomes occurred in participants receiving BTX, whilst PD was complicated by perforation in three cases. Authors' conclusions The results of this meta-analysis would suggest that PD is the more effective endoscopic treatment in the long term (greater than six months) for patients with achalasia.
Three clinical criteria have been reported to distinguish patients with primary achalasia from patients with achalasia secondary to tumor invasion of the gastroesophageal junction. These criteria (age greater than 50 years, duration of symptoms less than one year, and weight loss greater than 15 pounds) are important because of their potential use for deciding between pneumatic dilation and exploratory surgery. In the present investigation we assessed the frequency of these criteria alone and in combination in 79 patients with primary and in two patients with secondary achalasia seen at our institution over a 91/2-year period. Our results indicate that while these criteria are highly sensitive and moderately specific, their predictive value for distinguishing secondary achalasia from primary achalasia is exceedingly low. For this reason, early exploratory surgery is not indicated in patients with newly diagnosed achalasia who meet these criteria unless there is prior radiologic or endoscopic evidence for tumor.
Esophageal achalasia is a chronic and progressive motility disorder that leads to massive esophageal dilation when left untreated. Treatment for achalasia is palliative and aimed to relieve the outflow obstruction at the level of the lower esophageal sphincter, yet protecting the esophageal mucosa from refluxing gastric acids. The best way to accomplish this goal is through an esophageal myotomy and partial fundoplication, with a success rate >90%. Progression of disease, treatment failure, and complications from gastroesophageal reflux disease cause progressive deterioration of the esophageal function to an end stage in about 5% of patients. The only chance to improve symptoms in this small group of patients is through an esophageal resection. This article will review the indications for esophagectomy in end-stage achalasia, present the different types of surgical approach and possibilities for reconstruction of the alimentary tract, and summarize the short-term and long-term postoperative results.
OBJECTIVES: The reported success rate of pneumatic dilation in patients with achalasia varies from 50% to 93%. This wide variability may be due to using symptom relief postdilation as the only assessment of success. There are no studies comparing subjective symptom improvements to objective improvement in esophageal emptying after pneumatic dilation.METHODS: Patients with achalasia undergoing pneumatic dilation from 1995 to 1997 were evaluated. Pre- and postdilation symptoms were recorded using a standardized scoring system. Barium column height was measured 1 min and 5 min after upright ingestion to assess esophageal emptying. Based on percentage of total symptom and barium height improvement post-dilation, patients were grouped according to one of nine outcomes; the association between subjective and objective parameters of improvement was tested.RESULTS: A total of 37 patients underwent 53 pneumatic dilations. There was a significant association (p < 0.001) between improvement in patient symptoms and barium height. In 38 of 53 (72%) pneumatic dilations, the degree of symptom and barium height improvement was similar. Near complete symptom resolution was reported after 26 dilations. In eight of 26 (31%) patients however, there was <50% improvement in barium height (group A). Compared with the 16 patients with 91–100% improvement in both symptoms and barium height (group B), forward stepwise regression identified age as the only difference between the two groups, with group A patients being significantly (p = 0.04) older.CONCLUSIONS: Objective assessment of esophageal emptying pre- and post-dilation identifies an important subset of patients with poor esophageal emptying who report near complete symptom resolution. This group may benefit from any early repeat pneumatic dilation.
Background:
This study examined esophageal resection as treatment for recurrence or treatment complications of achalasia.
Methods:
From 1976 through 1992, 37 patients (20 men and 17 women) underwent esophageal resection after initial surgical treatment for achalasia. The median age was 56 years (range, 19 to 84 years). Initial surgical treatment consisted of modified Heller myotomy in 28 patients, combined myotomy and antireflux procedure in 6, and antireflux procedure alone in 3. Twenty-six patients required an additional surgical procedure before esophageal resection (70.3%). Indication for esophageal resection was obstructive symptoms in 30 patients, cancer in 3, bleeding in 2, and perforation during dilation in 2. Reconstruction was established with the stomach in 26 patients, colon in 6, and small bowel in 5. Anastomosis was at the cervical level in 20 patients (54.1%) and intrathoracic in 17 (45.9%).
Results:
There were two operative deaths (5.4%), both caused by intraoperative hemorrhage during transhiatal resection. Twelve patients (32.4%) had complications, which included cardiac dysrhythmia in 3, cervical anastomotic leak in 2, transient vocal cord paralysis in 2, pneumonia in 2, pulmonary embolus in 2, and reexploration for bleeding in 1. Follow-up was complete in all patients and ranged from 1.4 to 16 years (median, 6.3 years). Excellent or good long-term functional results were present in 32 patients (91.4%).
Conclusions:
Esophageal resection provides reasonable long-term functional results in patients with recurrence or treatment complications of achalasia. In our experience, transhiatal resection is associated with increased morbidity and mortality.
Endoscopic balloon dilatation and laparoscopic myotomy are established treatments for achalasia. Recently, a new endoscopic technique for complete myotomy was described. Herein, we report the results of the first prospective trial of peroral endoscopic myotomy (POEM) in Europe.
POEM was performed under general anesthesia in 16 patients (male:female (12:4), mean age 45 years, range 26-76). The primary outcome was symptom relief at 3 months, defined as an Eckhard score ≤3. Secondary outcomes were procedure-related adverse events, lower esophageal sphincter (LES) pressure on manometry, reflux symptoms, and medication use before and after POEM.
A 3-month follow-up was completed for all patients. Treatment success (Eckhard score ≤3) was achieved in 94% of cases (mean score pre- vs. post-treatment (8.8 vs. 1.4); P<0.001). Mean LES pressure was 27.2 mm Hg pre-treatment and 11.8 mm Hg post-treatment (P<0.001). No patient developed symptoms of gastro-esophageal reflux after treatment, but one patient was found to have an erosive lesion (LA grade A) on follow-up esophagogastroduodenoscopy. No patient required medication with proton pump inhibitors or antacids after POEM.
POEM is a promising new treatment for achalasia resulting in short-term symptom relief in >90% of cases. Studies evaluating long-term efficacy and comparing POEM with established treatments have been initiated.
Peroral endoscopic myotomy (POEM) has recently been described in humans as a treatment for achalasia. This concept has evolved from developments in natural orifice translumenal endoscopic surgery (NOTES) and has the potential to become an important therapeutic option. We describe our approach as well as our initial clinical experience as part of an ongoing study treating achalasia patients with POEM.
Five patients (mean age 64 ± 11 years) with esophageal motility disorders were enrolled in an IRB-approved study and underwent POEM. This completely endoscopic procedure involved a midesophageal mucosal incision, a submucosal tunnel onto the gastric cardia, and selective division of the circular and sling fibers at the lower esophageal sphincter. The mucosal entry was closed by conventional hemostatic clips. All patients had postoperative esophagograms before discharge and initial clinical follow-up 2 weeks postoperatively.
All (5 of 5) patients successfully underwent POEM treatment, and the myotomy had a median length of 7 cm (range 6 to 12 cm). After the procedure, smooth passage of the endoscope through the gastroesophageal junction was observed in all patients. Operative time ranged from 120 to 240 minutes. No leaks were detected in the swallow studies and mean length of stay was 1.2 ± 0.4 days. No clinical complications were observed, and at the initial follow-up, all patients reported dysphagia relief without reflux symptoms.
Our initial experience with the POEM procedure demonstrates its operative safety, and early clinical results have shown good results. Although further evaluation and long-term data are mandatory, POEM could become the treatment of choice for symptomatic achalasia.
A patient presented with chronic substernal discomfort and intermittent dysphagia for solids. High-resolution impedance manometry (HRIM) of the esophagus showed that there was no peristalsis in the esophageal body but incomplete relaxation of the lower esophageal sphincter and incomplete bolus transit, so the patient was diagnosed with achalasia. Moreover, probably because of esophageal stasis, eosinophilic infiltration that mimicked a pattern of eosinophilic esophagitis was observed, on the basis of multiple biopsies of the esophagus. The patient was given 50 mg prednisolone once daily; the symptoms improved dramatically, and HRIM showed complete recovery of esophageal peristalsis, deeper relaxation of the lower esophageal sphincter, and complete bolus transit profile. HRIM can therefore be used to assess dysmotility abnormalities in patients with achalasia and eosinophilic-like esophagitis, and steroids relieve these symptoms. Treatment with a high dose of prednisolone resulted in a complete disappearance of dysphagia because of improved esophageal motility and reduced eosinophilic infiltrate. It is therefore important to control the inflammatory process in patients with idiopathic achalasia, which is likely to result from an autoimmune reaction.
Achalasia is a rare esophageal motor disorder in children. The manometric hallmarks are esophageal body aperistalsis, lack of lower esophageal sphincter (LES) relaxation, and high LES pressure. However, LES relaxation and function may be heterogeneous, making the diagnosis difficult at times. The aim of the study was to describe LES function in children with achalasia.
The present study is a retrospective review of manometric tracings of pediatric patients with achalasia and controls. LES response after each swallow was evaluated. LES parameters were defined as homogeneous (normal or abnormal) if the same response was observed in >75% of swallows. Otherwise, the LES parameters were considered heterogeneous.
Twenty-nine patients with achalasia and 16 control patients were included. All of the controls had normal LES pressure and relaxation in all of the wet swallows. In the achalasia group, some LES relaxation was present in 74% of swallows and 25% of swallows had normal relaxation. Depending on the parameter, a heterogeneous LES (resting pressure, relaxation, residual pressure, and duration of relaxation) was observed in 27.6% to 34.5% of patients with achalasia versus 0% of the controls (P < 0.001). The rest of the patients with achalasia had homogeneous abnormal responses versus homogeneous normal responses in 100% of control patients (P < 0.001).
The LES function in pediatric patients with achalasia is heterogeneous. The classic description of a nonrelaxing high-pressure LES in patients with achalasia is rarely found in children. Partial relaxations are common, and normal relaxations may be present.
Most experienced gastroenterologists have seen one or several cases of achalasia patients who have been erroneously diagnosed with gastroesophageal reflux disease (GERD) or even underwent antireflux surgery. We aim to describe the current knowledge about the diagnostic features of achalasia and their overlap with GERD. Furthermore, we present 3 cases in which achalasia was mistaken for GERD.
Search of the literature published in English using the PubMed database and relevant abstracts presented at international conventions.
Typical features of GERD such as heartburn, retrosternal pain, esophagitis, and pathologic acid exposure can be observed in achalasia patients. Diagnostic tests such as endoscopy and radiography lack sensitivity and specificity for achalasia. Current diagnostic guidelines for antireflux surgery do not stipulate that achalasia should be ruled out preoperatively.
Clinical presentation as well as the diagnostic work-up of achalasia patients can show overlap with GERD. Mistaking achalasia for GERD can be avoided by esophageal manometry and this should therefore be performed in all patients undergoing surgical fundoplication.
Controversy exists with regard to the optimal treatment for achalasia and whether surveillance for early recognition of late complications is indicated. 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. Clinical predictors of outcome, patient preferences and local expertise should be considered when making a decision on the most appropriate treatment option. Owing to a lack of long-term benefit, endoscopic botulinum toxin injection and medical therapies are reserved for patients of advanced age and those with clinically significant comorbidites. The value of new endoscopic, radiologic or surgical treatments, such as peroral endoscopic myotomy, esophageal stenting and robotic-assisted myotomy has not been fully established. Finally, long-term follow-up data in patients with achalasia support the notion that surveillance strategies might be beneficial after a disease duration of more than 10-15 years.
Advances in esophageal manometry have facilitated identification of variants of achalasia, suggesting they are more common than previously thought. This study assesses the frequency and clinical characteristics of patients with motility abnormalities similar to, but not meeting criteria for, classic achalasia.
Records of patients undergoing high-resolution esophageal manometry between January 2008 and January 2010 were screened for diagnosis of achalasia, impaired lower esophageal sphincter (LES) relaxation, or severe peristaltic dysfunction of the esophageal body. Forty-four patients with classic achalasia and 31 with variant characteristics were identified. Clinical and manometric characteristics were recorded and compared.
Variant achalasia was almost as common as the classic type (31 versus 44 patients). Eighty-two percent (36 of 44) of those with classic and 48% (15 of 31) of those with variant characteristics had dysphagia. Classic achalasia patients' mean age was 62 years (SD 19 years) versus 53 years (SD 14 years) in the variant group. The classic achalasia group had 26 male patients and 18 female patients and the variant group had 9 male patients and 22 female patients. Two thirds (21 of 31) of the variant group had impaired LES relaxation. Three variant patterns emerged: impaired LES relaxation with normal/hypertensive peristalsis (n = 10), impaired/borderline LES relaxation with mixed peristalsis/simultaneous contractions (n = 14), and impaired/normal LES and aperistalsis with occasional short segment peristalsis (n = 7). Mean intrabolus pressure was 16.3 mmHg in variant patients with normal LES relaxation and 23.1 mmHg in those with impaired relaxation.
Variants of achalasia are more common than previously recognized. LES dysfunction defined by high relaxation pressure occurs in two-thirds of variant achalasia patients and might be a hallmark that could direct therapy. The notion that esophageal body dysfunction/aperistalsis in achalasia is all or none should be reconsidered.
Despite symptom improvement offered to achalasia patients by either pneumatic dilation or surgical myotomy, 10% to 15% of those so treated will present progressive deterioration of their esophageal function and up to 5% may eventually require an esophagectomy.
The natural evolution of achalasia to its end stage as well as the timing of esophagectomy in these patients form the basis of this review. The optimal reconstruction for the decompensated resected esophagus will also be explored: gastric interposition, colon interposition, and jejunal interposition all have their respective advantages and disadvantages. Their use is examined in the exclusive context of resection for achalasia.
Patients with achalasia have an increased risk for the development of esophageal squamous cell cancer. Endoscopic surveillance in long-standing achalasia has been advocated by some, but the most recent American Society of Gastrointesinal Endoscopy guidelines regard current data as insufficient to support such an approach. In this issue of the American Journal of Gastroenterology, Leeuwenburgh and colleagues report on the results of a long-term prospective study with fixed surveillance intervals. The authors confirm earlier observations of an increased cancer risk after 10 years of symptomatic achalasia. Despite some limitations, this study and earlier data suggest that it might be worthwhile to consider endoscopic surveillance in patients with long-standing achalasia.
To establish less invasive permanent treatment for esophageal achalasia, per-oral endoscopic myotomy (POEM) was addressed.
POEM was performed in 43 consecutive cases of achalasia. Among them, nine cases of sigmoidal achalasia were involved. POEM got IRB approval from our hospital. Written informed consent was given to all patients.
After creating submucosal tunnel, endoscopic myotomy of circular muscle bundles was carried out at approximately 12cm in total length (10 cm in distal esophagus and 2 cm cardia). Smooth passing of endoscope through GE junction was confirmed at the end of the procedure.
In all cases symptoms of achalasia were dramaticaly reduced or disappeared. Resting pressure was 52.1 mmHg before POEM, and reduced to 18.8 mmHg after procedure. No specific complications related to POEM were experienced. During follow-up period, no additional treatment and no medication were necessary.
Short-term outcome of POEM was excellent with no serious complications. Long-term follow up is scheduled.
The present study compares the efficacy of a self-expanding metallic stent (SEMS, diameter of 30 mm) and pneumatic dilation for the long-term clinical treatment of achalasia. A total of 155 patients diagnosed with achalasia were allocated for pneumatic dilation (n= 80, group A) or a temporary, 30-mm diameter SEMS (n= 75, group B). The SEMSs were placed under fluoroscopic guidance and removed by gastroscopy 4-5 days after placement. Data on clinical symptoms, complications, and long-term clinical outcomes were collected, and follow-up observations were performed at 6 months and at 1, 3-5, 5-8, 8-10, and >10 years, postoperatively. Pneumatic dilation and stent placement were technically successful in all of the patients. There were no significant differences in technique success, 30-day mortality, or complications between the two groups. The clinical remission rate in group A was significantly lower than that in group B at 1, 1-3, 3-5, 5-8 and, >10 years (P < 0.05), while the cumulative clinical failure rate in group A (66%, 53/80) was higher than that in group B (92%, 6/75). The mean primary patency in group B was significantly longer than that in group A (4.2 vs 2.1 years, respectively; P < 0.001). A temporary, 30-mm diameter SEMS was associated with a better long-term clinical efficacy in the treatment of patients with achalasia as compared with treatment with pneumatic dilation.
Achalasia is treated with pneumatic dilation or Heller myotomy, but studies suggest poor long-term outcomes. We analyzed long-term outcomes after initial pneumatic dilation and studied factors associated with failure.
A total of 209 patients (111 men; mean age, 51.2 +/- 1.4 years) with achalasia who were treated with pneumatic dilation between 1992 and 2002 were followed. Outcomes were correlated with demographics, presenting symptoms, manometric features, and treatment variables by using chi(2) and Student t tests.
All patients were initially treated with consecutive esophageal dilations up to balloon diameters of 3.0 (26%), 3.5 (41%), or 4.0 cm (33%). After dilations, mean lower esophageal sphincter (LES) pressure had decreased from 31.3 +/- 1.3 to 14.0 +/- 0.7 mm Hg (P < .0001); dysphagia decreased from 96% to 26%; and 49% had gained an average of 4.6 +/- 0.5 kg (weight loss at presentation was 10.6 +/- 0.7 kg in 39%). During follow-up, 66% required no additional treatment, whereas 23% underwent repeat dilations after 79 +/- 8 months. Patients without recurrence were older (41.2 +/- 2.1 vs 56.6 +/- 1.6 years; P < .0001) and had lower post-treatment LES pressure (17.8 +/- 1.2 vs 12.9 +/- 0.6 mm Hg; P < .005). After 70-month follow-up, balloon dilation yielded good or excellent outcomes in 72% of patients. In nonresponders, rescue surgery yielded higher success rates than botulinum toxin therapy (84% vs 44%). Patient satisfaction ranged from good to excellent in 81% of patients.
Treating achalasia with initial dilation and then surgery for short-term failures yielded good long-term results in more than 70% and treatment satisfaction in more than 80% of patients. Management of dilation failures is more problematic.
To choose which treatment would be most effective for the individual patient with newly diagnosed achalasia is difficult for the tending physician. A diagnostic tool that would allow prediction of the symptomatic and functional response after treatment for achalasia is therefore needed. The timed barium esophagogram (TBE) is a method that allows objective assessment of esophageal emptying, but the value of TBE in the clinical management of achalasia remains to be clarified. The aim of this study was first, to assess the ability of TBE to predict symptoms and treatment failure during post-treatment follow-up. Second, to determine whether esophageal emptying as assessed by TBE differs after treatment with pneumatic dilatation or laparoscopic myotomy. Fifty-one patients with newly diagnosed achalasia were prospectively randomized to pneumatic dilatation (n = 26) or laparoscopic myotomy (n = 25). Evaluation with TBE was performed before (n = 46) and after treatment (n = 43). The median interval between treatment and post-treatment TBE was 6 months, and the median follow-up time after the post-treatment TBE was 18 months. Following therapeutic intervention, TBE parameters did not differ significantly between treatment groups. However, significant correlations were found between the height of the barium column at 1 min and the symptom scores at the end of follow up for 'dysphagia for liquids' (P < 0.05, rho = 0.47), 'chest pain' (P < 0.05, rho = 0.42), and the 'Watson dysphagia score' (P < 0.05, rho = 0.46). Patients with less than 50% improvement in this TBE-parameter (height at 1 min) post-treatment had a 40% risk of treatment failure during follow-up. In summary, pneumatic balloon dilatation and laparoscopic myotomy similarly affected esophageal function as assessed by TBE-emptying. Lack of improvement in barium-column height post-treatment was associated with an increased risk of treatment failure which should motivate close surveillance in order to detect symptomatic recurrence at an early stage.
Although rare, esophageal achalasia is the best described primary esophageal motility disorder. Commonly used treatments are endoscopic botulin toxin injection (EBTI), endoscopic balloon dilation (EBD), and surgical myotomy with or without a fundoplication; however, reported outcomes mostly come from cohort studies.
To summarize and compare the safety and efficacy of endoscopic and surgical treatments for esophageal achalasia.
A systematic electronic Medline literature search of articles on esophageal achalasia. Treatment options reviewed included EBTI, EBD, and surgical myotomy using open and minimally invasive techniques. Main outcome measures were frequency of symptom relief, prevalence of post-treatment gastroesophageal reflux (GER), and complications. Outcome probability was estimated using weighted averages of the sample prevalence in each study, with weights equal to the number of patients. Outcomes, within or across studies, were compared using meta-analysis and meta-regression, respectively.
A total of 105 articles reporting on 7855 patients were selected, tabulated and reviewed. Symptom relief after EBD was better than after EBTI (68.2% vs. 40.6%; OR 3.4; 95% CI, 1.2-9.8; P = 0.02), and the need for additional therapy was greater for patients receiving EBTI (46.6% vs. 25%; OR, 2.6; 95% CI, 1.05-6.5; P = 0.04). Laparoscopic myotomy, when combined with an antireflux procedure, provided better symptom relief (90%) than all endoscopic and other surgical approaches and a low complication rate (6.3%). The incidence of postoperative GER was lower when a fundoplication was added to a laparoscopic myotomy (31.5% without a fundoplication vs. 8.8% with; OR, 6.3; 95% CI, 2.0-19.4; P = 0.003).
EBD is superior to EBTI. Laparoscopic myotomy with fundoplication was the most effective surgical technique and can be considered the operative procedure of choice.
Achalasia cannot be cured. Instead, our goal is to relieve symptoms of dysphagia and regurgitation, improve esophageal emptying and prevent the development of megaesophagus. The most definitive therapies are pneumatic dilation and surgical myotomy. The overall success of grade pneumatic dilation is 78%, with women and older patients performing best. Laparoscopic myotomy has an overall success rate of 85%, but can be complicated by the sequelae of severe acid reflux disease. Young patients, especially men, are the best candidates for surgical myotomy. There are no prospective, randomized studies comparing these two procedures. Botulinum toxin injections into the esophagus and smooth muscle relaxants are reserved for older patients or those with major comorbid illnesses. Some patients with end-stage achalasia will require esophagectomy.
Patients with achalasia require repeated invasive therapies and may experience multiple complications. The objectives of this study were to determine the incidence of such complications, causes of death, and life expectancy in 253 patients.
Patients consisted of two groups: group A comprised 177 patients with newly diagnosed achalasia; group B consisted of 76 patients in whom the diagnosis had been established in external institutions. All patients regularly underwent structured interviews and were reinvestigated if changes in health status occurred. Survival rates were determined by Kaplan-Meier estimates and were compared with those of an average German population. Causes of death were determined from hospital records, information supplied by private physicians, and from death certificates.
Complete follow-up was obtained in 98.9% (group A) and in 100% (group B) of all patients. The observation period for group A ranged from 2 to 33 years and for group B from 2 to 26 years (disease duration: 4-68 years). The most frequent complications were reflux esophagitis (group A: 6.2%, group B: 19.7%) and megaesophagus (group A: 6.2%, group B: 21.0%). Thirty-six patients had died during follow-up. Five of these deaths were related to achalasia. In group A, the estimated 20-year survival rates in patients with achalasia [76% (95% confidence interval (CI): 66-85%)] did not significantly differ from those in controls 80% (95% CI: 71-89%). In group B, 25-year survival rates were also similar in patients [87% (95% CI: 78-97%)] and controls [86% (95% CI: 76-97%)].
Patients with achalasia experience a significant number of complications. Causes of death and life expectancy, however, do not differ from those of the average population.
Although the diagnosis of achalasia hinges on demonstrating impaired esophagogastric junction (EGJ) relaxation and aperistalsis, 3 distinct patterns of aperistalsis are discernable with high-resolution manometry (HRM). This study aimed to compare the clinical characteristics and treatment response of these 3 subtypes.
One thousand clinical HRM studies were reviewed, and 213 patients with impaired EGJ relaxation were identified. These were categorized into 4 groups: achalasia with minimal esophageal pressurization (type I, classic), achalasia with esophageal compression (type II), achalasia with spasm (type III), and functional obstruction with some preserved peristalsis. Clinical and manometric variables including treatment response were compared among the 3 achalasia subtypes. Logistic regression analysis was performed using treatment success as the dichotomous dependent variable controlling for independent manometric and clinical variables.
Ninety-nine patients were newly diagnosed with achalasia (21 type I, 49 type II, 29 type III), and 83 of these had sufficient follow-up to analyze treatment response. Type II patients were significantly more likely to respond to any therapy (BoTox [71%], pneumatic dilation [91%], or Heller myotomy [100%]) than type I (56% overall) or type III (29% overall) patients. Logistic regression analysis found type II to be a predictor of positive treatment response, whereas type III and pretreatment esophageal dilatation were predictive of negative treatment response.
Achalasia can be categorized into 3 subtypes that are distinct in terms of their responsiveness to medical or surgical therapies. Utilizing these subclassifications would likely strengthen future prospective studies of treatment efficacy in achalasia.
The clinical and diagnostic features of a secondary type of achalasia of the esophagus are described in seven patients with various types of malignancies. Patients with secondary achalasia presented with dysphagia of short duration and marked weight loss; mean age was 64 years. Esophageal manometry showed features identical to those of idiopathic primary achalasia: aperistalsis, poor lower esophageal sphincter relaxation, and elevated sphincter pressure. Endoscopy and barium swallow showed evidence of a tumor in only two cases. Various types of malignancies may produce a secondary form of achalasia that has diagnostic features identical to those of primary achalasia and is best identified by its clinical presentation.
The use of a constantly perfused side hole sensor (CPSH) for continuous measurement of maximal lower esophageal sphincter pressure (LESP) is associated with important but variable undermeasurement as a result of side hole displacement. A 5 cm long pressure sensor has been developed which measures maximal LESP continuously in the face of movement of the sphincter within the sensor length. This sensor, the perfused sleeve, is described, and validated by comparison with CPSH in man, the dog, and a model esophageal sphincter. The sleeve detects maximal LESP accurately, regardless of sphincter length, over the pressure range encountered in the lower esophageal sphincter. Continuous recording of LESP with the sleeve is unaffected by displacement that causes serious undermeasurement with CPSH.
This prospective study investigates whether the effect of pneumatic dilation in patients with achalasia can be predicted on the basis of patient characteristics, type of treatment, or results of postdilation investigations. Over a period of 10 years, 54 consecutive patients with newly diagnosed achalasia were treated with pneumatic dilation and underwent pretreatment and posttreatment manometric, radiographic, and scintigraphic investigations. They were followed up every 2 years until the fall of 1991. Among the factors evaluated in the initial examination, only young age adversely affected outcome (P < 0.05). With the exception of the diameter of the dilating balloon, the treatment characteristics had a low predictive value. Postdilation lower esophageal sphincter pressure was the single most valuable factor for predicting the long-term clinical response (P < 0.0005). However, patients with high sphincter pressures and poor treatment results benefited from repeated dilations by having progressively longer remissions. It is concluded that young patients are poor candidates for pneumatic dilation, that treatment should be aimed at near complete inflation of the dilating bag, and that postdilation sphincter pressure may guide further treatment.
Topographic plots constructed from conventional esophageal manometric waves were examined in 12 healthy volunteers to better understand spatial relationships of peristaltic contractions. Typical postdeglutitive waves were spatially interconnected using a computerized gridding and plotting system. Contour plots of the resultant peristaltic contraction revealed a consistent separation of a proximal pressure segment from the prominent distal contraction at 21.7 +/- 1.3% of the esophageal length. A second trough in the contraction sequence was found in 11 (91.7%) of the subjects at 64.0 +/- 2.7% of esophageal length separating the distal (smooth muscle) esophagus into two contraction segments. Similar topographic studies were also performed in eight symptomatic patients with frequent double-peaked waves. Poor alignment of the two distal contraction segments was observed in six (75%) of the eight patients. These topographic plots demonstrate previously unrecognized wave relationships and suggest that two neuromuscular contraction units can be detected in the distal smooth muscle esophagus. This technique should be useful in understanding altered relationships of these units under differing physiological conditions or with disease.
Eighteen patients with cancer-induced or secondary achalasia (SA) were compared to 421 patients with idiopathic or primary achalasia (PA). The aim of the study was to detect any differences in clinical presentation between the two groups. Mean age of patients with SA was 57.1 (range 15-78) and 47.1 (range 1-90) in patients with PA (p = 0.02). Three patients with SA were 15, 24, and 36 yr old, respectively. Symptom frequency was comparable in SA versus PA. Mean duration of symptoms in SA was 4.5 months, with 15 of the 18 patients experiencing symptoms for six months or less. Weight loss occurred in 88.2% of patients with SA and 57.3% of patients with PA (p less than 0.05). Cancer was at the gastroesophageal junction in 16 patients, duodenum in one, and breast in one. Endoscopy showed tumor in 12 (67%). The esophagram was suspicious for tumor in only 25%. We conclude that patients with SA are older, more likely to lose weight, and have a short duration of symptoms. However, SA may occur in younger patients, and endoscopy with biopsy is necessary in any newly diagnosed case of achalasia.
Primary motility disorders of the esophagus require the exclusion of intramural tumors. The procedures currently used for the differential diagnosis of achalasia such as endoscopy with biopsy, esophageal and gastric radiography, abdomino-thoracic computed tomography and intraluminal esophageal manometry, are unsatisfactory when a tumor growing intramurally is suspected. A more recent method of studying the integrity of the gastrointestinal wall and its surrounding tissue is endoscopic ultrasonography. In 16 patients suspected of having achalasia, endosonography was performed in addition to the above-mentioned conventional examinations. Fifteen of them showed a normal ultrasonic structure of the wall of the gastro-esophageal junction, with no sign of hypertrophy of the smooth muscle layer. In the remaining case endoscopic ultrasonography was able to detect an intramural tumor, as evidenced by the inhomogeneous ultrasonic structure of the esophageal wall. Computed tomography and all the other conventional diagnostic procedures used failed to demonstrate this tumor. In conclusion, the findings presented strongly suggest that endosonography can contribute to the differential diagnosis of achalasia and intramural tumors.