Imaging Pearls ❯ Esophagus ❯ Achalasia
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- Achalasia: Facts
Primary achalasia is due to impaired relaxation of the lower esophageal sphincter, leading to abnormal uncoordinated contractions, esophageal dilation, chronic esophagitis, and possible aspiration. Secondary achalasia or pseudoachalasia is due to distal esophageal obstruction from non-functional causes including mass. - Achalasia: Facts
CT demonstrates dilated thin-walled esophagus with fluid and debris, and primarily used to evaluate complications such as aspiration pneumonitis or malignancy. Fluoroscopic evaluation demonstrates bird beak sign with incomplete lower esophageal sphincter relaxation, dysmotility, and short segment stricture of the distal esophagus. - “Achalasia is a well-recognized oesophageal motility disorder characterized by a combination of absent peristalsis in the thoracic oesophagus and impaired opening of the lower oesophageal sphincter (LES) in response to deglutition. Two major forms of achalasia have been described: primary and secondary. The primary or idiopathic form is caused by degeneration of myenteric plexi in the wall of the oesophagus. The aetiology of this neural degeneration remains unknown but is postulated to be secondary to viral or autoimmune disease.”
Utility of chest CT for differentiating primary and secondary achalasia
M.Y. Licurse et al.
Clinical Radiology, Volume 69, Issue 10, 2014, Pages 1019-1026 - “In contrast, secondary achalasia (also known as pseudo-achalasia) is a less common form caused by extra-oesophageal conditions (especially malignant tumours) that induce an achalasia-like motility disorder by a variety of proposed mechanisms. Adenocarcinoma of the gastric cardia or fundus is by far the most frequent malignant tumour that causes secondary achalasia, but lung, breast, pancreatic, and other remote cancers may also be responsible for this condition.”
Utility of chest CT for differentiating primary and secondary achalasia
M.Y. Licurse et al.
Clinical Radiology, Volume 69, Issue 10, 2014, Pages 1019-1026 - "Differentiation of primary from secondary achalasia has critical implications for patient management because of vastly different approaches for the work-up and treatment of these conditions. Primary achalasia is a benign motility disorder, usually treated by C. botulinum toxin injection or pneumatic dilation of the LES or by laparoscopic myotomy. In contrast, secondary achalasia is a far more serious condition caused by underlying malignant tumours (except in South America, where it may be caused by Chagas' disease), necessitating treatment with surgery, radiation, or chemotherapy, depending on the stage and location of the underlying neoplasm. Therefore, a confident diagnosis of primary or secondary achalasia is essential for instituting appropriate therapy in these patients.”
Utility of chest CT for differentiating primary and secondary achalasia
M.Y. Licurse et al.
Clinical Radiology, Volume 69, Issue 10, 2014, Pages 1019-1026 - "Findings in all nine achalasia patients were similar: moderate to marked esophageal dilatation (mean diameter 4.35 cm at carinal level) with normal wall thickness. Findings are in distinct contrast to the three patients with other diseases, in which the degree of esophageal dilatation and/or wall thickness was atypical. Complications in the patients with proven achalasia included secondary carcinoma (one), iatrogenic esophageal perforation (one), and pulmonary aspiration (three). Computed tomography may not be indicated as a routine study, but in complicated cases CT may be invaluable in confirming the diagnosis or in detecting atypical features that may indicate the presence of other diseases or superimposed benign or malignant processes.”
CT Evaluation of Achalasia.
Rabushka, L. , Fishman, E. , Kuhlman, J.
Journal of Computer Assisted Tomography, 15 (3), 434-439.
- “Achalasia is a well-recognized oesophageal motility disorder characterized by a combination of absent peristalsis in the thoracic oesophagus and impaired opening of the lower oesophageal sphincter (LES) in response to deglutition.1 Two major forms of achalasia have been described: primary and secondary.”
Utility of chest CT for differentiating primary and secondary achalasia M.Y. Licurse et al. Clinical Radiology, Volume 69, Issue 10, October 2014, Pages 1019-1026 - “The primary or idiopathic form is caused by degeneration of myenteric plexi in the wall of the oesophagus. The aetiology of this neural degeneration remains unknown but is postulated to be secondary to viral or autoimmune disease. In contrast, secondary achalasia (also known as pseudo-achalasia) is a less common form caused by extra-oesophageal conditions (especially malignant tumours) that induce an achalasia-like motility disorder by a variety of proposed mechanisms.”
Utility of chest CT for differentiating primary and secondary achalasia M.Y. Licurse et al. Clinical Radiology, Volume 69, Issue 10, October 2014, Pages 1019-1026 - “Adenocarcinoma of the gastric cardia or fundus is by far the most frequent malignant tumour that causes secondary achalasia, but lung, breast, pancreatic, and other remote cancers may also be responsible for this condition.”
Utility of chest CT for differentiating primary and secondary achalasia M.Y. Licurse et al. Clinical Radiology, Volume 69, Issue 10, October 2014, Pages 1019-1026 - “Primary achalasia classically occurs in young or middle-aged individuals presenting with long-standing dysphagia but little or no weight loss, whereas secondary achalasia occurs in patients over 60 years of age presenting with recent onset of progressive dysphagia and weight loss.”
Utility of chest CT for differentiating primary and secondary achalasia M.Y. Licurse et al. Clinical Radiology, Volume 69, Issue 10, October 2014, Pages 1019-1026 - “Achalasia is a primary rare motor disorder of the esophagus, with an incidence of about 1/100,000. Symptoms usually become manifest in early adult age, but even children may be affected. Achalasia is characterized by incomplete relaxation of the lower esophageal sphincter (LES) on swallowing and aperistalsis of the esophageal body. In 1947, Ogilvie recognized the syndrome of neoplastic involvement of the distal esophagus that mimicked idiopathic achalasia, with submucosal infiltration of the lower esophagus and cardia by carcinoma, which is now commonly referred to as pseudoachalasia. Therefore, CT can be helpful in differentiating between achalasia and the pseudoachalasia of malignancy.”
Dedicated multi-detector CT of the esophagus: spectrum of diseases Ahmed Ba-Ssalamah et al. Abdominal Imaging Jan 2009, Volume 34, Issue 1, pp 3–18 - “In advanced cases CT shows uniform dilatation that affects a long segment of the esophagus, with no wall thickening and with normal-appearing boundary surfaces and medistinal fat. The esophagus narrows abruptly at the esophagogastric junction with no evidence of an intramural or extrinsic obstructive lesion. In contrast to a stricture, the esophageal wall is not thinned at the site of the narrowing, and the wall is not thickened as it is with the esophageal tumor or esophagitis. Most pseudoachalasia patients have CT findings of esophageal dilation, more marked and/or asymmetric wall thickening, or mass. In this group, asymmetric or marked thickening (>10 mm) indicates pseudoachalasia.”
Dedicated multi-detector CT of the esophagus: spectrum of diseases Ahmed Ba-Ssalamah et al. Abdominal Imaging Jan 2009, Volume 34, Issue 1, pp 3–18
- Achalasia: Facts
- GE jxn fails to relax due to Wallerian degeneration of Auerbach’s plexus
- Usually patients age 20-40
- Symptoms typically are dysphagia and weight loss - Achalasia: Facts
- Recurrent aspiration pneumonia
- Increased incidence of esophageal cancer