The esophageal pull-down technique improves the outcome of laparoscopic Heller-Dor myotomy in end-stage achalasia.

BACKGROUND: The treatment of patients with end-stage achalasia with a sigmoid-shaped esophagus is particularly challenging. A modified technique (pull-down technique) has been developed to straighten the esophageal axis, but only a limited number of studies on this topic are available in the literature. This study aimed to compare the outcome of patients who underwent the pull-down technique with that of patients who had a classical laparoscopic Heller-Dor (CLHD) myotomy. METHODS: All patients with a radiologic diagnosis of end-stage achalasia who underwent an LHD myotomy between 1995 and 2022 were considered eligible for the study. All patients underwent symptom score, barium swallow, endoscopy, and manometry tests before and after the procedure was performed. Treatment failure was defined as the persistence or reoccurrence of an Eckardt score (ES) of >3 or the need for retreatment. RESULTS: Of the 94 patients who were diagnosed with end-stage achalasia (male-to-female ratio of 52:42), 60 were treated with CLHD myotomy, and 34 were treated with the pull-down technique. Of note, 2 patients (2.1%), both belonging to the CLHD myotomy group, developed a squamous cell carcinoma during the follow-up. The overall success of LHD myotomy was seen in 76 of 92 patients (82.6%). All patients in both groups achieved a lower ES after surgery. The failure rates were 27.6% (16/58) in the CLHD myotomy group and 5.9% (2/34) in the pull-down technique group (P < .01). CONCLUSION: Our findings confirm that LHD myotomy is an effective treatment of end-stage achalasia and that the pull-down technique further improves the outcome in patients with end-stage achalasia who are difficult to treat.

A Rare Case of Achalasia Sigmoid Esophagus Obstructed by Food Bolus.

Achalasia is a primary esophageal motility disorder that involves a failure of the lower esophageal sphincter to relax in response to swallowing. Specifically, the lower esophageal sphincter becomes hypertensive, and there is an absence of peristalsis in the esophagus. The pathophysiology is thought to be due to a loss of inhibitory nerve function from an autoimmune attack that targets the esophageal myenteric nerves. As a result, these abnormalities lead to a functional obstruction at the gastroesophageal junction. In severe cases, achalasia may present as a "sigmoid esophagus," a term used to describe the dilation and distortion of the cervical esophagus. In this case report, we discuss a patient with a known history of achalasia who presented with extra-esophageal symptoms including respiratory distress and tracheal compression from an esophagus dilated with a food impaction. She was found to have a sigmoid esophagus and required direct endoscopy and removal of the food bolus. We will review the pathogenesis of achalasia as well as medical and surgical approaches to treating severe achalasia as presented through other case reports.

Surgical Options for End-Stage Achalasia.

PURPOSE OF REVIEW: Achalasia is one of the most commonly described primary esophageal motility disorders worldwide, but there is significant controversy regarding ideal management of end-stage disease. This article reviews the definition of end-stage achalasia and summarizes past and present surgical treatment. RECENT FINDINGS: Myotomy of the lower esophageal sphincter remains the mainstay of treatment of achalasia, even in advanced disease. Esophagectomy may have benefit as a primary treatment modality in end-stage achalasia with sigmoid esophagus, but international guidelines recommend consideration of laparoscopic or endoscopic approaches initially in most patients. Novel peroral esophageal plication techniques may provide alternative treatment options in patients with significant esophageal dilation that fail myotomy or esophagectomy. SUMMARY: End-stage achalasia is characterized by progressive tortuosity and dilation of the esophagus as a failure of primary peristalsis. Up to 20% of patients with achalasia will progress to end-stage disease. In most cases, laparoscopic or endoscopic myotomy is recommended as initial approach to surgical management.

Laparoscopic Heller-Dor is an effective long-term treatment for end-stage achalasia.

BACKGROUND: The end-stage achalasia is a difficult condition to treat, for the esophageal diameter and conformation of the gullet, that may progress to a sigmoid shape. The aim of this study was to examine the outcome of Laparoscopic Heller-Dor in patients with end-stage achalasia, comparing them with patients who had mega-esophagus without a sigmoid shape. METHODS: From 1992 to 2020, patients with a diagnosis of sigmoid esophagus, or radiological stage IV achalasia (the SE group), and patients with a straight esophagus larger than 6 cm in diameter, or radiological stage III achalasia (the NSE group), were all treated with LHD. The two groups were compared in terms of patients' symptoms, based on the Eckardt score, and on barium swallow, endoscopy and manometry performed before and after the treatment. The failure of the treatment was defined as an Eckardt score > 3, or the need for further treatment. RESULTS: The study involved 164 patients: 73 in the SE group and 91 in the NSE group. No intra- or postoperative mortality was recorded. The median follow-up was 51 months (IQR 25-107). The outcome was satisfactory in 71.2% of patients in the SE group, and in 89% of those in the NSE group (p = 0.005). CONCLUSIONS: SE is certainly the worst condition of the disease and the final outcome of LHD, in term of symptom control, is inferior compared to NSE. Despite this, almost 3/4 of the SE patients experienced a significant relieve in symptoms after LHD, which may therefore still be the first surgical option to offer to these patients, before considering esophagectomy.

Resultado da miotomia endoscópica peroral (POEM) em acalasia avançada em um seguimento mediano de 17 meses / Outcome of per oral endoscopic myotomy (poem) in sigmoid achalasia at a median follow up of 17 months

ABSTRACT Background: Advanced achalasia cardia (AC) represents the end stage in the natural history of AC. Role of per oral endoscopic myotomy (POEM) in this technically difficult subset is emerging. Methods: Retrospective review of the patients who had undergone POEM for advanced AC with sigmoid esophagus. We assessed the technical success, clinical success and adverse event rate. Pre and post POEM Eckardt score (ES), integrated relaxation pressure-4sec (IRP-4), lower oesophageal sphincter pressure (LESP) and height and width of barium column at 5 minutes were noted. Results: Of the 85 patients who underwent POEM for AC, 10 patients had advanced AC with sigmoid esophagus of which eight were sigmoid and two were advanced sigmoid. The clinical and technical success was 100% with significant reduction of ES, IRP-4, LESP and height and width of barium column at 5 minutes. One patient had a minor adverse event in the form of mucosal injury that was closed with hemoclips. At a median follow up of 17 months there was no recurrence. Conclusion: Our study demonstrates POEM to be a safe and effective modality of treatment in this technically difficult subset of AC with sigmoid morphology.

RESUMO Contexto: Acalasia cárdia avançada representa o estágio final na história natural do megaesôfago. Está emergindo o papel da miotomia endoscópica peroral (POEM) neste subconjunto tecnicamente difícil. Métodos: Foi realizada uma revisão retrospectiva dos pacientes submetidos a POEM para tratamento do megaesôfago avançado. Avaliamos o sucesso técnico, o sucesso clínico e a taxa de eventos adversos. O escore de Eckardt, pré e pós POEM, a pressão integral de relaxamento de 4 seg (IRP-4), a pressão do esfíncter inferior do esôfago e a altura e largura da coluna de bário em 5 minutos, foram anotadas. Resultados: Dos 85 pacientes submetidos a POEM para acalasia cárdia, 10 pacientes apresentaram acalasia cárdia avançado com esôfago com aspecto sigmoide, dos quais dois eram muito avançados. O sucesso clínico e técnico foi de 100% com redução significativa do escore de Eckardt, do IRP-4, da pressão do esfíncter inferior do esôfago e da altura e largura da coluna bário em 5 minutos. Um paciente teve um pequeno evento adverso na forma de lesão mucosa que foi fechada com hemoclipes. Em um seguimento mediano de 17 meses não houve recorrência. Conclusão: Nosso estudo demonstra que a POEM é uma modalidade segura e eficaz de tratamento neste subconjunto tecnicamente difícil de megaesôfago com morfologia sigmoide.

Surgical Management of Advanced Achalasia With Sigmoid Esophagus: A Case Report.

The surgical management of achalasia with sigmoid esophagus involves multiple significant challenges due to the difficulty in endoscopic assessment, esophageal motility disorders, and potential complication and recurrence rates. We report a 34-year-old female with worsening dysphagia and malnourishment due to advanced achalasia. An esophagogastroduodenoscopy (EGD) revealed an esophageal dilation, tortuosity, and distal blockage with undigested food. Esophagram demonstrated the typical bird beak appearance with a tortuous dilated esophagus. She underwent a laparoscopic Heller myotomy with Dor fundoplication with no complications. She was discharged on the second postoperative day, tolerating clear liquids, and then a normal diet within six weeks. Several treatment options exist for the surgical management of a sigmoid esophagus with achalasia, but there is no clear gold standard. In our case, Heller myotomy with Dor fundoplication provided favorable results, but treatment should be individualized for each case.

Treatment challenges of sigmoid-shaped esophagus and severe achalasia.

BACKGROUND: Achalasia is a chronic motility disorder which may require surgical interventions to effectively manage patients' symptoms and improve functional status. In late stage achalasia, patients may present with sigmoid-shaped esophagus which complicates traditional treatment approaches for achalasia as the esophagus is massively dilated and dysfunctional with delicate tissue integrity. Severe Achalasia with sigmoid esophagus imposes significant challenge to surgeons and treating physicians. Various assessment modalities and treatment approaches have been tried. Surgical treatment continues to be controversial. Some have argued that a less aggressive approach similar to that in early Achalasia results in satisfactory outcomes. Others have argued a more aggressive approach of esophagectomy is necessary. We present a review of the challenges encountered in each approach with recommendation for selecting the right treatment for the individual cases. CONCLUSIONS: Different treatment options for sigmoid type achalasia are available with ongoing controversy among the options. Heller myotomy with Dor fundoplication can provide satisfactory symptoms improvement and treatment outcomes.

Sigmoid megaesophagus with thoracic pseudotumor appearance.

A 61-year-old man presented with dysphagia, weight loss and shortness of breath. On examination, he had reduced lung sounds on the right, and obvious neck vein distention. Chest X-ray raised a suspicion of lung tumor with possible superior vena cava syndrome. Subsequent CT scan of chest confirmed the presence of markedly dilated and tortuous esophagus (sigmoid megaesophagus) extending to the right hemithorax and pressing on the trachea. The patient was referred for surgery.

POEM for special patient cohorts: A review.

Peroral endoscopic myotomy (POEM) is a natural orifice, translumenal endoscopic surgical procedure that achieves endoscopic myotomy by conducting a submucosal tunnel as an operating space. It is conventionally performed in achalasia. Recently, several centers worldwide have reported the feasibility of this procedure not only in early achalasia but also in other particular patient cohorts. Possible indications for POEM include previously failed Heller myotomy, pneumatic balloon dilatation or POEM, sigmoid achalasia, pediatric patients, spastic esophagus disorders, Zenker's diverticulum and gastroparesis. However, its application in large numbers of special patient groups awaits knowledge of the long-term outcome and greater experience with POEM.

Laparoscopic Esophagogastroplasty in Management of Megaesophagus with Axis Deviation.

The results of cardiomyotomy in patients of achalasic megaesophagus with axis deviation are not satisfactory. Usually, an esophagectomy is advocated. We describe the technical details and outcomes of laparoscopic esophagogastroplasty for end-stage achalasia. The patient had end-stage achalasia, characterized by tortuous megaesophagus with axis deviation. The surgery was performed in supine position using four abdominal ports. The steps included mobilization of the gastroesophageal junction and lower intrathoracic esophagus, straightening the pulled intrathoracic esophagus into the abdomen, and a side-side esophagogastroplasty using purple Endo GIA Articulating Tri-Staple load, two firings. Duration of surgery was 52 min. The patient was ambulated on the first postoperative day. Oral feeding was initiated by the third postoperative day. The patients had significant improvements of dysphagia. At 3 months of follow-up, the patient is euphagic without significant symptoms of gastroesophageal reflux. Laparoscopic esophagogastroplasty is an effective option for relieving dysphagia in megaesophagus due to achalasia with axis deviation. It is a reasonable alternative before subjecting to a major and potentially morbid esophagectomy. It creates a large gastroesophageal (GE) junction, which, with the help of gravity, helps food transit. By dividing the muscles of the GE junction completely, it also achieves a complete cardiomyotomy. Less operative time and blood loss, quicker recovery, and better cosmesis make it an attractive option. While potential reflux is a possibility, the reported case has not shown significant GERD symptoms.