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Zanini LYK, Herbella FAM, Velanovich V, Patti MG. Modern insights into the pathophysiology and treatment of pseudoachalasia. Langenbecks Arch Surg 2024; 409:65. [PMID: 38367052 DOI: 10.1007/s00423-024-03259-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/06/2023] [Accepted: 02/12/2024] [Indexed: 02/19/2024]
Abstract
BACKGROUND Secondary achalasia or pseudoachalasia is a clinical presentation undistinguishable from achalasia in terms of symptoms, manometric, and radiographic findings, but associated with different and identifiable underlying causes. METHODS A literature review was conducted on the PubMed database restricting results to the English language. Key terms used were "achalasia-like" with 63 results, "secondary achalasia" with 69 results, and "pseudoachalasia" with 141 results. References of the retrieved papers were also manually reviewed. RESULTS Etiology, diagnosis, and treatment were reviewed. CONCLUSIONS Pseudoachalasia is a rare disease. Most available evidence regarding this condition is based on case reports or small retrospective series. There are different causes but all culminating in outflow obstruction. Clinical presentation and image and functional tests overlap with primary achalasia or are inaccurate, thus the identification of secondary achalasia can be delayed. Inadequate diagnosis leads to futile therapies and could worsen prognosis, especially in neoplastic disease. Routine screening is not justifiable; good clinical judgment still remains the best tool. Therapy should be aimed at etiology. Even though Heller's myotomy brings the best results in non-malignant cases, good clinical judgment still remains the best tool as well.
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Affiliation(s)
- Leonardo Yuri Kasputis Zanini
- Department of Surgery, Escola Paulista de Medicina, Federal University of São Paulo, Rua Diogo de Faria 1087 Cj 301, São Paulo, 04037-003, Brazil
| | - Fernando A M Herbella
- Department of Surgery, Escola Paulista de Medicina, Federal University of São Paulo, Rua Diogo de Faria 1087 Cj 301, São Paulo, 04037-003, Brazil.
| | - Vic Velanovich
- Department of Surgery, University of South Florida, Tampa, USA
| | - Marco G Patti
- Department of Surgery, University of Virginia, Charlottesville, USA
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Tustumi F, de Sousa JHB, Dornelas NM, Rosa GM, Steinman M, Bianchi ET. The Mechanisms for the Association of Cancer and Esophageal Dysmotility Disorders. Med Sci (Basel) 2021; 9:32. [PMID: 34064058 PMCID: PMC8163009 DOI: 10.3390/medsci9020032] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2021] [Revised: 05/12/2021] [Accepted: 05/21/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Achalasia and other esophageal dysmotility disorders mimicking achalasia can be associated with cancer. This study aimed to review the main mechanisms for which cancer may develop in esophageal dysmotility disorder patients. METHODS A narrative review was performed. RESULTS The mechanism for developing squamous cell carcinoma and adenocarcinoma are discussed. Besides, achalasia-like syndromes related to familial KIT-gene mutation and pseudoachalasia are discussed. CONCLUSIONS Knowing the main mechanism for which achalasia can be related to cancer is essential for clinicians to conduct the proper investigation, surveillance, and treatment.
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Affiliation(s)
- Francisco Tustumi
- Hospital Israelita Albert Einstein, Sao Paulo 05652-900, Brazil; (J.H.B.d.S.); (N.M.D.); (G.M.R.); (M.S.); (E.T.B.)
- Department of Gastroenterology, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo 05403-000, Brazil
| | | | - Nicolas Medeiros Dornelas
- Hospital Israelita Albert Einstein, Sao Paulo 05652-900, Brazil; (J.H.B.d.S.); (N.M.D.); (G.M.R.); (M.S.); (E.T.B.)
| | - Guilherme Maganha Rosa
- Hospital Israelita Albert Einstein, Sao Paulo 05652-900, Brazil; (J.H.B.d.S.); (N.M.D.); (G.M.R.); (M.S.); (E.T.B.)
| | - Milton Steinman
- Hospital Israelita Albert Einstein, Sao Paulo 05652-900, Brazil; (J.H.B.d.S.); (N.M.D.); (G.M.R.); (M.S.); (E.T.B.)
| | - Edno Tales Bianchi
- Hospital Israelita Albert Einstein, Sao Paulo 05652-900, Brazil; (J.H.B.d.S.); (N.M.D.); (G.M.R.); (M.S.); (E.T.B.)
- Department of Gastroenterology, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo 05403-000, Brazil
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Bredenoord AJ, Rösch T, Fockens P. Peroral endoscopic myotomy for achalasia. Neurogastroenterol Motil 2014; 26:3-12. [PMID: 24304406 DOI: 10.1111/nmo.12257] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/03/2013] [Accepted: 10/06/2013] [Indexed: 12/21/2022]
Abstract
BACKGROUND Treatment of achalasia is complicated by symptom recurrence and a significant risk for severe complications. Endoscopic myotomy was developed in the search for a highly efficacious treatment with lower risks. Since its introduction in 2010, several centers have adopted the technique and published excellent short-term results of open label series. Randomized trials with long-term endpoint comparing per-oral endoscopic myotomy (POEM) with the established treatments such as balloon dilation and surgical myotomy are now warranted, before POEM can be regarded as the routine clinical care for achalasia patients. PURPOSE This review describes the development, technical aspects, efficacy, and complications of POEM.
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Affiliation(s)
- A J Bredenoord
- Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands
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Campo SMA, Zullo A, Scandavini CM, Frezza B, Cerro P, Balducci G. Pseudoachalasia: A peculiar case report and review of the literature. World J Gastrointest Endosc 2013; 5:450-454. [PMID: 24044045 PMCID: PMC3773858 DOI: 10.4253/wjge.v5.i9.450] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2013] [Revised: 06/24/2013] [Accepted: 08/09/2013] [Indexed: 02/05/2023] Open
Abstract
Pseudoachalasia is a rare secondary achalasia, which accounts for only a small subgroup of patients. We describe a 77-year-old woman with recent onset of dysphagia and typical esophageal manometric findings of achalasia. Moreover, esophageal manometric findings of vascular compression at 36 cm from the nose were associated with dysphagia. An upper endoscopy showed the absence of lesions both in the esophagus and gastro-esophageal junction, whilst a 15-mm ulcer on the gastric angulus was detected. The gastric ulcer resulted in being a diffuse signet ring cell carcinoma at histology, suggesting pseudoachalasia. An abdominal computed tomography scan showed an irregular concentric thickening of the gastro-esophageal junction wall extending for 7 cm and a dilated ascending thoracic aorta with no presence of the inferior vena cava, with an enlarged azygos as the source of vascular compression of esophagus. Moreover, cardia involvement from diffuse signet ring cell carcinoma of the gastric angulus was also recognized as the cause of dysphagia. The cancer was not suitable for a surgical approach in an old patient with cardiovascular comorbidities and support therapy was started. In our ambulatory series, pseudoachalasia was eventually diagnosed in 4.7% of 234 consecutive patients with esophageal manometric finding suggestive of achalasia. We also reviewed cases in the literature and aimed to evaluate the reported causes of pseudoachalasia.
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Revisional surgery after Heller myotomy for esophageal achalasia. Surg Laparosc Endosc Percutan Tech 2011; 20:321-5. [PMID: 20975503 DOI: 10.1097/sle.0b013e3181f39fdb] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Revisional surgery for persistent or recurrent dysphagia following Heller myotomy is rare and should become even more rare if an extended myotomy has been carried out. It is important to work-up patients who experience persistent or recurrent dysphagia in a systematic fashion that includes the determination of the diagnosis of achalasia, the type of operation performed, the results obtained with the primary operation, and to further classify the type of recurrence. Use of adjunctive studies, including upper gastrointestinal study, endoscopy, manometry, and pH monitoring is critical to guiding clinical decision making. This article will review the differential diagnosis, diagnostic workup, and available treatment options for patients with achalasia who present with persistent or recurrent dysphagia following Heller myotomy.
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Campo SMA, Lorenzetti R, de Matthaeis M, Hassan C, Zullo A, Cerro P, Morini S. Palliation with oesophageal metal stent of pseudoachalasia from gastric carcinoma at the cardia: a case report. DIAGNOSTIC AND THERAPEUTIC ENDOSCOPY 2009; 2009:791627. [PMID: 19746178 PMCID: PMC2738867 DOI: 10.1155/2009/791627] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/12/2009] [Revised: 05/06/2009] [Accepted: 05/29/2009] [Indexed: 12/11/2022]
Abstract
We present an 82-year-old woman with a 3-month history of progressive dysphagia and a normal initial upper gastrointestinal endoscopy. The diagnosis of pseudoachalasia was suspected by oesophageal manometric and barium swallow studies, and confirmed by biopsies revealing an intestinal type carcinoma of the stomach at a repeated endoscopy. In view of the history of heart disease, diabetes, and old age, this patient was treated by a partially covered Ultraflex self-expanding metal stent (Boston Scientific, Natick, MA, USA) placed into the oesophageal body with no direct complications and obtaining the relief from dysphagia. During the 11-month follow-up she was treated for an iron deficiency anaemia due to reflux oesophagitis with ulcerations in the oesophageal body and died from myocardial infarction. According to the localization of the cancer, the old age, and the presence of comorbidities, we should recommend the insertion of a partially covered self-expanding metal stent as a reasonable palliative treatment in selected subjects with pseudoachalasia.
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Affiliation(s)
| | - Roberto Lorenzetti
- Gastroenterology Unit, Nuovo Regina Margherita Hospital, Via Morosini 30, 00153 Rome, Italy
| | - Marina de Matthaeis
- Gastroenterology Unit, Nuovo Regina Margherita Hospital, Via Morosini 30, 00153 Rome, Italy
| | - Cesare Hassan
- Gastroenterology Unit, Nuovo Regina Margherita Hospital, Via Morosini 30, 00153 Rome, Italy
| | - Angelo Zullo
- Gastroenterology Unit, Nuovo Regina Margherita Hospital, Via Morosini 30, 00153 Rome, Italy
| | - Paola Cerro
- Radiology Unit, Nuovo Regina Margherita Hospital, Via Morosini 30, 00153 Rome, Italy
| | - Sergio Morini
- Gastroenterology Unit, Nuovo Regina Margherita Hospital, Via Morosini 30, 00153 Rome, Italy
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Abstract
Esophageal achalasia is the most commonly diagnosed primary esophageal motor disorder and the second most common functional esophageal disorder. Current therapy of achalasia is directed toward elimination of the outflow resistance caused by failure of the lower esophageal sphincter to relax completely upon swallowing. The advent of minimally invasive surgery has nearly replaced endoscopic pneumatic dilation as the first-line therapeutic approach. In this editorial, the rationale and the evidence supporting the use of laparoscopic Heller myotomy combined with fundoplication as a primary treatment of achalasia are reviewed.
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Gockel I, Eckardt VF, Schmitt T, Junginger T. Pseudoachalasia: a case series and analysis of the literature. Scand J Gastroenterol 2005; 40:378-85. [PMID: 16028431 DOI: 10.1080/00365520510012118] [Citation(s) in RCA: 72] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
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.
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Affiliation(s)
- Ines Gockel
- Department of General and Abdominal Surgery, Johannes Gutenberg-University Mainz, Germany.
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Abstract
Achalasia is a rare neurologic deficit of the esophagus, producing a syndrome of impaired relaxation of the lower esophageal sphincter and decreased motility of the esophageal body for which the cause is unknown. The resultant chronic esophageal stasis produces discomforting symptoms that can be managed with medication, chemical paralysis of the lower esophageal sphincter, mechanical dilation, or surgical esophagomyotomy. Chemical paralysis by injection of the esophagus with botulinum toxin and dilation with an inflatable balloon offers good short-term relief of symptoms; however, the best long-term results are produced by surgery, and advancing minimally invasive techniques continually reduce the morbidity of these operations. The type of surgical procedure, the necessity for fundoplication, and the order of treatment continue to be unresolved issues, but prospective evaluation with objective followup should allow us to provide the optimal treatment regimen to our patients.
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Affiliation(s)
- Shawn D St Peter
- Department of General Surgery, Mayo Clinic Scottsdale, Arizona, USA
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Affiliation(s)
- E J Simchuk
- University Department of Surgery, Bristol Royal Infirmary, Bristol BS2 8HW, UK
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Malhi-Chowla N, Wolfsen HC, DeVault KR. Esophageal dysmotility in patients undergoing photodynamic therapy. Mayo Clin Proc 2001; 76:987-9. [PMID: 11605700 DOI: 10.4065/76.10.987] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
OBJECTIVE To study the esophageal motility of patients with esophageal adenocarcinoma or Barrett esophagus with high-grade dysplasia before and after photodynamic therapy. PATIENTS AND METHODS In this prospective study conducted between January 1998 and October 1999, esophageal motility testing of the lower esophageal sphincter and esophageal body was performed with a water-perfused catheter, 2 days before and at least 3 weeks after patients underwent photodynamic therapy for esophageal adenocarcinoma or Barrett esophagus. Results were classified as normal motility, ineffective esophageal motility, or aperistalsis. RESULTS Twenty-three patients were studied, 13 with carcinoma and 10 with Barrett esophagus. Overall, 11 patients (48%) had normal motility, 6 (26%) had ineffective esophageal motility, and 6 (26%) had aperistalsis. Five patients with aperistalsis had carcinoma. Follow-up tracings after photodynamic therapy found that 6 patients (26%) had normal motility, 7 (30%) had ineffective esophageal motility, and 10 (43%) had aperistalsis. CONCLUSIONS Esophageal dysmotility is common in patients with esophageal adenocarcinoma or Barrett esophagus. Photodynamic therapy may worsen esophageal motility in some patients. Dysphagia after photodynamic therapy therefore may be related to underlying esophageal dysmotility and may not always be caused by stricture or underlying carcinoma.
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Affiliation(s)
- N Malhi-Chowla
- Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, Fla 32224, USA
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Bustamante M, Devesa F, Ferrando MJ, Borghol A. [Difficulty in the early diagnosis of pseudoachalasia of tumoral origin]. GASTROENTEROLOGIA Y HEPATOLOGIA 2001; 24:144-5. [PMID: 11261227 DOI: 10.1016/s0210-5705(01)70143-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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