Pseudoachalasia: A case series and analysis of the literature

Objective Pseudoachalasia frequently cannot be distinguished from idiopathic achalasia by manometry, radiologic examination or endoscopy. Mechanisms proposed to explain the clinical features of pseudoachalasia include a circumferential mechanical obstruction of the distal esophagus or a malignant infiltration of inhibitory neurons within the myenteric plexus. Material and methods Between January 1980 and December 2002, the clinical features of 5 patients with pseudoachalasia and 174 patients with primary achalasia, diagnosed in a single center, were compared. A literature analysis of the etiology of pseudoachalasia for the time period 1968 to December 2002 was performed. The search concentrated on the databases and online catalogues PubMed, Web of Science, Cochrane Library and Current Contents Connect. Results In our case series, patients with pseudoachalasia reported a shorter duration of symptoms and tended to be older than patients with primary achalasia. Conventional manometry, endoscopy and radiologic examination of the esophagus proved to be of little value in distinguishing between the diseases. In the majority of cases only surgical exploration revealed the underlying cause. A coincidence of primary achalasia and disorders of the gastroesophageal junction was excluded by showing return of peristalsis following treatment. The analysis of the literature showed a total of 264 cases of pseudoachalasia in 122 publications. Most cases of were due to malignant disease (53.9% primary and 14.9% secondary malignancy), followed by benign lesions (12.6%) and sequelae of surgical procedures at the distal esophagus or proximal stomach (11.9%). In rare instances, the disease was an expression of a paraneoplastic process due to distant neuronal involvement rather than to local invasion with destruction of the myenteric plexus (2.6%). Conclusions The diagnosis of pseudoachalasia is difficult to establish by conventional diagnostic measures. The main distinguishing feature of secondary versus primary achalasia is the complete reversal of pathologic motor phenomena following successful therapy of the underlying disorder.     

Original article: Outcomes in achalasia from a surgical unit where pneumatic dilatation is first‐line therapy

The management of achalasia remains controversial, with little consensus on the optimal patient treatment pathway. In our own esophageal unit, we offer pneumatic dilatation as the initial therapy in most patients as first‐line therapy. In this study, we aimed to examine the safety and efficacy of our own approach to the management of patients with a diagnosis of achalasia, examining symptomatic outcomes, patient satisfaction, and need for further intervention, as well as examining patient factors associated with treatment failure. Sixty‐seven consecutive patients underwent pneumatic dilatation as first‐line therapy (53% male, mean age 46 years). All attended regular outpatient follow‐up (mean 37, range 3–132 months). Twenty‐five percent of patients required a second intervention because of symptom recurrence, at a median period of 4.5 months. Symptomatic outcomes were excellent or good in 80%. Significant predictors of treatment failure and poor symptom score included a younger age at the time of diagnosis and increased esophageal diameter on barium swallow. This study suggests that pneumatic dilatation is a safe and effective approach as first‐line therapy in patients with newly diagnosed achalasia.     

Peroral endoscopic myotomy as salvation technique post‐Heller: International experience

Background

Treatment for achalasia has traditionally been Heller myotomy (HM). Despite its excellent efficacy rate, a number of patients remain symptomatic post‐procedure. Limited data exist as to the best management for recurrence of symptoms post‐HM. We present an international, multicenter experience evaluating the efficacy and safety of post‐HM peroral endoscopic myotomy (POEM).

Methods

Patients who underwent POEM post‐HM from 13 centers from January 2012 to January 2017 were included as part of a prospective registry. Technical success was defined as successful completion of the myotomy. Clinical success was defined as an Eckardt score of ≤3 on 12‐month follow up. Adverse events (AE) including anesthesia‐related, operative, and postoperative complications were recorded.

Results

Fifty‐one patients were included in the study (mean age 54.2, 47% male). Technical success was achieved in 100% of patients. Clinical success on long‐term follow up was achieved in 48 patients (94%), with a mean change in Eckardt score of 6.25. Seven patients (13%) had AE: six experienced periprocedural mucosal defect treated endoscopically and two patients developed mediastinitis treated conservatively.

Conclusion

For patients with persistent symptoms after HM, POEM is a safe salvation technique with good short‐term efficacy. As a result of the challenge associated with repeat HM, POEM might become the preferred technique in this patient population. Further studies with longer follow up are needed.     

Management of achalasia with transabdominal esophagocardiomyotomy and partial posterior fundoplication

In this article we present our experience in the management of achalasia. From May 1988 through August 2005, 71 patients with achalasia underwent transabdominal esophagocardiomyotomy and partial posterior fundoplication. Barium swallow, manometry, and 24-h pH studies were performed in all patients preoperatively. Manometry and 24-h pH monitoring were only carried out in 58 patients at the third post-operative week and in 43 patients during follow-up, even though 52 patients were included in the follow-up. There were no operative deaths or complications. All the 71 patients were able to eat semifluid or solid food without dysphagia and heartburn at discharge. Esophageal barium studies showed that the maximum esophageal diameter decreased 2.2 cm and the minimum gastroesophageal junction diameter increased 8.4 mm after operation. Manometry examination in 58 patients revealed that the lower esophageal sphincter resting pressure decreased 15.0 mmHg in the wake of the procedure. Twenty-four hour pH monitoring demonstrated that reflux events were within the normal post-operative range. Fifty-five of the 58 patients had normal DeMeester scores. Among the patients with a mean 90-month follow-up, 49 patients had normal intake of food without reflux, the remaining three had mild dysphagia without requiring treatment. All the patients resumed their preoperative work and social activities. The manometry and 24-h pH studies in the 43 patients showed there were no significant changes between the third post-operative week and during follow-up. Transabdominal esophagocardiomyotomy and posterior partial fundoplication are able to relieve the functional outflow obstruction of the lower esophageal sphincter, obviate the rehealing of the myotomy edge and prevent gastroesophageal reflux in patients who have undergone myotomy alone.     

Pseudoachalasia secondary to bariatric surgery

Secondary achalasia may result from diseases that either infiltrate or compress the lower esophageal sphincter to create an increased high‐pressure zone and subsequent esophageal body changes. With bariatric surgery, a potential high‐pressure zone is created by a sleeve, band, or bypass just distal to the esophagus. We report four patients who years after a bariatric procedure developed dysphagia followed by esophageal body radiographic and/or manometric features of achalasia. In addition, each of these patients responded symptomatically to endoscopic or surgical therapy either which lowered this high‐pressure zone. Furthermore, review of prior cases in the literature suggests this has occurred before. Physicians should be aware of this association but further study is needed on its true prevalence.     

Peroral endoscopic myotomy for the treatment of esophageal achalasia: systematic review and pooled analysis

Peroral endoscopic myotomy (POEM) is a novel approach to performing esophageal myotomy for the treatment of achalasia. This review aims to assess subjective and objective metrics of achalasia treatment efficacy, perioperative adverse events and the incidence of postoperative gastroesophageal reflux disease in patients treated with POEM. Secondary aims include a pooled analysis comparison of the clinical outcomes and procedural safety of POEM versus laparoscopic Heller's myotomy (LHM). A systematic review of the literature, up to and including January 15, 2015, was conducted for studies reporting POEM outcomes. Studies comparing POEM to LHM were also included for the purpose of pooled analysis. Outcomes from 1122 POEM patients, from 22 studies, are reported in this systematic review. Minor operative adverse events included capno/pneumo‐peritoneum (30.6%), capno/pneumo‐thorax (11.0%) and subcutaneous emphysema (31.6%). Major operative adverse events included mediastinal leak (0.3%), postoperative bleeding (1.1%) and a single mortality (0.09%). There was an improvement in lower esophageal sphincter pressure and timed barium esophagram column height of 66% and 80% post‐POEM, respectively. Symptom improvement was demonstrated with a pre‐ and post‐POEM Eckardt score ± standard deviation of 6.8 ± 1.0 and 1.2 ± 0.6, respectively. Pre‐ and post‐POEM endoscopy showed esophagitis in 0% and 19% of patients, respectively. The median (interquartile range) points scored for study quality was 15 (14–16) out of total of 32. Pooled analysis of three comparative studies between LHM and POEM showed similar results for adverse events, perforation rate, operative time and a nonsignificant trend toward a reduced length of hospital stay in the POEM group. In conclusion, POEM is a safe and effective treatment for achalasia, showing significant improvements in objective metrics and achalasia‐related symptoms. Randomized comparative studies of LHM and POEM are required to determine the most effective treatment modality for achalasia.     

经口内镜下括约肌切开术对贲门失驰缓症疗效分析

[目的]分析经口内镜下括约肌切开术(POEM术)对贲门失驰缓症患者的治疗效果。[方法]以42例贲门失驰缓症患者为研究对象,随机分为试验组与对照组,每组21例。试验组患者使用POEM术治疗,对照组患者使用传统开胸Heller手术,观察比较2组患者的治疗效果、手术时间等相关指标。[结果]与对照组比较,试验组患者手术时间短、手术中出血量少、患者住院时间短,2组比较差异有统计学意义(P0.05)。[结论]POEM术治疗贲门失驰缓症的效果较好。     

Safety and efficacy of the modified peroral endoscopic myotomy with shorter myotomy for achalasia patients: a prospective study

Peroral endoscopic myotomy (POEM) has been developed as a minimally invasive endoscopic treatment for achalasia for years. However, the optimal length of submucosal tunnel and myotomy of muscle bundles during procedure of POEM has not yet been determined, so we aim to assess safety and efficacy of modified POEM with shorter myotomy of muscle bundles in achalasia patients. Consecutive achalasia patients had been performed modified POEM with shorter myotomy, and assessed by symptoms, high‐resolution manometry, and barium swallow examinations before and 3 months after POEM for safety and efficacy evaluation. Modified POEM with shorter submucosal tunnel (mean length 6.8 cm) and endoscopic myotomy of muscle bundles (total mean length 5.4 cm) were completed in 46 consecutive achalasia patients. During the 3‐month follow up in all cases, significant improvement of symptoms (a significant drop in the Eckardt score 8.4 ± 3.2 vs. 2.7 ± 1.9; P < 0.001), decreased lower esophageal sphincter pressure (39.4 ± 10.1 vs. 24.4 ± 9.1 mmHg; P < 0.001) and integrated relaxation pressure (38.6 ± 10.4 vs. 25.7 ± 9.6 mmHg; P < 0.01), and a drop in height of esophagus barium‐contrast column (5.4 ± 3.1 vs. 2.6 ± 1.8 cm; P < 0.001) were observed. The frequencies of adverse events were lower in those under endotracheal anesthesia and CO₂ insufflations compared with intravenous anesthesia and air insufflations. Only three patients were found to have gastroesophageal reflux disease on follow up. Modified POEM with shorter myotomy under endotracheal anesthesia and CO₂ insufflations shows its good safety and excellent short‐term efficacy in the treatment of achalasia. But further studies are warranted to assess the long‐term efficacy.     

Prospective evaluation of the efficacy of peroral endoscopic myotomy in patients with achalasia

Peroral endoscopic myotomy (POEM) is an endoscopic alternative to surgical myotomy in patients with achalasia. This study aimed to evaluate the efficacy and clinical outcomes of POEM.A total of 20 patients with achalasia who underwent POEM between October 2016 and November 2017 were prospectively recruited. The intraoperative esophagogastric junction distensibility index (mm²/mm Hg) was measured pre- and post-myotomy using an endoluminal functional lumen imaging probe. Clinical response was defined as Eckardt score ≤3. Health-related quality of life was measured by the 36-item short-form health survey score.POEM was successfully completed in all cases. The median procedure time was 68.5 minutes (range 50.0–120.0), and the median myotomy length was 13 cm (range 11–18). Major adverse events were encountered in 2 cases. Overall, clinical responses were observed in all patients during a median follow-up of 11.9 months (range 1.2–26.2). Postoperative esophagogastric junction distensibility index was significantly higher than baseline (from 1.3 [range 0.8–6.9] to 6.3 [range 25–19.2], P < .001). The median Eckardt scores were decreased after POEM (5 [range 2–11] to 1 [range 0–3], P < .001), and the 36-item short-form health survey score was also improved significantly after POEM (67.5 [range 34.5–93.9] to 85.7 [range 53.4–93.3], P = .004).POEM is an effective treatment for achalasia, based on the improvement of both symptoms and objective measures.Clinicaltrial.gov {"type":"clinical-trial","attrs":{"text":"NCT 02989883","term_id":"NCT02989883"}}NCT 02989883     

Recurrent achalasia treated with Heller myotomy： A review of the literature

AIM： To evaluate the efficacy and safety of Heller myotomy （HM） for recurrent achlasia, performed after different methods of first-line treatment. METHODS： We searched for studies published in PubMed from 1966 to March 2008 on treatment of recurrent achalasia with HM after failure with different methods of first-line treatment. The efficacy of HM was assessed by a pooled estimate of response rate with individual studies weighted proportionally to sample size. RESULTS： Sixteen studies were eligible and included in the review, The results showed that HM has a better remission rate for recurrent achalasia after failure of HM [weighted mean （SD）] of 86.9% （21,8%） compared with 81.6% （23.8%） for pneumatic dilatation （PD）. One study evaluated the efficacy of HM after failure of PD combined with botulinum toxin injection （83%）, The most common complications were perforation and gastroesophageal reflux, CONCLUSION： HM has the best efficacy in patients with recurrent achiasia who were treated with HM as first-line treatment, Future studies should focus on how to increase the success rate and decrease the complications of HM.     

Achalasia – advances in treatment

Achalasia is a primary esophageal motility disorder, which shows distinct clinical, manometric, radiologic, and pathologic features. Available treatment strategies are pharmacological, endoscopic or surgical. In the past decades preferred treatment has alternated between surgical myotomy (presently Laparoscopic Heller Myotomy [LHM]) and endoscopic balloon dilation (EBD). While surgical myotomy promises superior long-term results and significantly less retreatment, endoscopic balloon dilation is initially far less invasive and yields comparable results after redilation. Peroral Endoscopic Myotomy (POEM) aims to combine the minimal invasive approach through the mouth with the better long-term results after LHM. Initial findings in the literature point to comparable success-rates after POEM and LHM. In the literature complication rates are similar to those obtained after surgery. This new interventional technique should be subject to randomized controlled trials and compared to EBD and LHM.     

Achalasia familiar: report of a family with an autosomal dominant pattern of inherence

Esophageal achalasia is a well‐known pathology with an autosomal recessive pattern of inherence described in the familiar cases. Its principal symptom is dysphagia, secondary to the poor relaxation of the lower esophageal sphincter. Chagas disease is one of the many causes involved in the development of this disease, being of great importance in our country because of the high prevalence of the vector. Various syndromes include achalasia in their symptomatology, such as the triple A syndrome or Allgrove syndrome (Addisonianism, achalasia, and alacrimia). We reported a family with a classical autosomal pattern of inherence with six affected members, four men and two women, with achalasia diagnosis as well as esophagus cancer in two of them, secondary to the disease, and no other findings.     

慢传输型便秘结肠肌间神经丛超微结构改变

目的　慢传输型便秘（ＳＴＣ） 病因不清、症状顽固、临床处理较困难． 进一步探讨其发生和发展的病理学基础．方法　应用电镜技术对１４ 例ＳＴＣ 患者和１１ 例非梗阻性直肠癌患者经手术切除的乙状结肠标本进行了对照研究．结果　与对照组相比,ＳＴＣ 患者结肠肌间神经丛的超微病理改变表现为：神经元及其突起有十分明显的退行性变,轴突及树突空化呈网格状结构,胞质内出现空泡及脂褐素等;轴突末端膨体及突触前区内突触小泡含量明显减少,大部分突触小泡出现空化．结论　ＳＴＣ 患者结肠肌间神经丛有明显的神经病理学改变,存在递质耗竭或递质合成、传输障碍等现象,它是ＳＴＣ 患者肠道传输减慢的超微病理基础． 这种改变可能与ＳＴＣ 患者长期滥用接触性泻剂有关．     

Update on therapeutic interventions for the management of achalasia

Achalasia is a primary esophageal motility disorder. It is the absence of peristalsis in the esophageal body and inability of the lower esophageal sphincter to relax, which characterizes this rare condition. Its features typically include dysphagia, regurgitation, chest pain, and weight loss. The ultimate goal in treating achalasia is to relieve the patient's symptoms, improve esophageal emptying, and prevent further dilatation of the esophagus. Current treatment modalities targeted at achalasia include pharmacological therapy, endoscopic therapy, and surgery. This review focuses on the current therapeutic options and explores the role of peroral endoscopic myotomy in the management armamentarium.     

Functional analysis of long‐term outcome after Heller's myotomy for achalasia

Treatment of achalasia aims at reducing the pressure of the lower esophageal sphincter (LES) and palliate symptoms. Our objective in this study was to investigate functional changes of the esophagus after Heller myotomy and evaluate their influence on postoperative gastroesophageal reflux and esophageal morphologic changes. Between 1980 and 2003, 216 patients with achalasia underwent Heller myotomy, associated with anterior partial fundoplication (Dor fundoplication). Preoperative and long‐term outcome data were collected from these patients at our hospital. The objective was to analyze esophageal functional results after Heller myotomy in the long term. Results were classified as excellent, good, fair, or poor, according to Vantrappen and Hellemans’ modified classification. One‐year, 2‐year, 5‐year, 10‐year, and 20‐year postoperative follow‐up information was available in 100% of all patients, 91.7%, 85.1%, 60%, 52.6%, and 45.9%, respectively. There were no perioperative deaths. One year after the surgery, all patients had a significant reduction in symptoms of dysphagia and regurgitation. Five years, 10 years, 15 years, and 20 years after surgery, there were 77.2% of patients (142 in 184), 68.1%, 57.1%, and 54.5%, respectively, who were satisfied (excellent to good) with surgery. No esophageal peristalsis was demonstrated in patients during follow‐up. Contractile waves in the body of the esophagus were simultaneous. The difference in the distal esophageal amplitude, the LES relaxation rate, and LES pressures in the anterior wall and/ or two sides was significant (P < 0.05) when compared before and after operation. However, there was no significant difference in the LES length and LES pressure in the posterior side. The change of direction of the LES pressure and the relaxation of LES correlate with long‐term outcomes. Postoperative gastroesophageal reflux rates, including nocturnal reflux, increased with time. The percentage of patients whose esophageal diameter became normal or remained mildly increased with time in the first 10 years after surgery changed significantly. Myotomy is an effective way to palliate symptoms in patients with achalasia. Adequate myotomy can lead to reduction of LES pressure in two or three directions, which may facilitate esophageal emptying by gravity. Surgical intervention does not lead to the return of esophageal peristalsis. Functional damage of LES in patients with achalasia is irreversible.     

The prognostic impact of occult lymph node metastasis in cancer of the esophagus or esophago-gastric junction: systematic review and meta-analysis

SUMMARY.  Attempts to define the clinical significance of occult lymph node metastasis have yielded mixed results. We set out to quantify the influence on disease-free survival of occult lymph node metastasis in cases of esophageal or gastro-esophageal cancer previously staged as lymph node-negative by conventional H&E staining. We performed a systematic review and meta-analysis of observational studies published between 1966 and 2006 (identified through Medline and Embase). Twelve suitable cohort studies were identified. These studies suggest there is a significant ( P < 0.001) association between occult lymph node metastasis and prognosis in cancer of the esophagus or esophago-gastric junction (pooled hazard ratio 3.16 with 95% confidence intervals of 2.25–4.42). We did not demonstrate study quality, number of nodes examined or number of lymph node sections examined to be significant sources of intertrial heterogeneity. Data from observational studies suggest that occult lymph node metastasis is an important prognostic factor in cancer of the esophagus or gastro-esophagus. Meta-analysis using individual patient data can now be justified.     

Esophageal achalasia secondary to mesothelioma

Summary Achalasia secondary to malignancy is rare, with most cases associated with gastric adenocarcinoma of the gastroesophageal junction. This report describes the clinicopathologic features of a 64-year-old man found to have mesothelioma as the cause of secondary achalasia. To our knowledge, this is the first case of secondary achalasia produced by a mesothelioma. We reviewed the English literature in regard to achalasia induced by tumors.This work was supported by the Veterans Administration.     

经口内镜下环形肌切开术治疗老年贲门失弛缓症的疗效

目的 探索经口内镜下环形肌切开术（POEM）治疗老年贲门失弛缓症（AC）的安全性、可行性、围手术期及远期疗效。方法 回顾性分析2010年8月至2014年12月期间在复旦大学附属中山医院确诊为AC并接受POEM治疗、可获得完整随访资料的老年（≥65岁）患者共41例,观察手术相关并发症、治疗前后Eckardt评分及食管下段括约肌压力变化,并分析远期食管反流及治疗失败的发生情况。结果 41例患者均成功完成POEM手术,中位手术时间42 min,中位住院天数为3 d,共4例（975%）患者出现主要围手术期不良事件,均为因围手术期不良事件导致住院时间长于5 d。中位随访时间40个月（四分位数区间 24~57个月）,Eckardt评分中位数从术前8分下降为术后1分（P<0001）,食管下段括约肌压力中位数从术前2385 mmHg (1 mmHg=0133 kPa) 下降为术后905 mmHg (P=0005)。术后随访12例（2927%）患者出现临床反流,5年临床治疗成功率8780%(36/41)。结论 POEM治疗老年AC患者安全可靠,近期和远期疗效均较好。