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Clinical Challenges - July 2017 What's Your Diagnosis?

The diagnosis

Answer to “What’s your diagnosis?” on page 2: Dysphagia lusoria

Dysphagia may be divided into an oropharyngeal cause or an esophageal cause. Esophageal dysphagia may be due to a luminal narrowing or a motility dysfunction. Causes of luminal narrowing include lesions within the lumen such as a foreign body, lesions within the wall of the esophagus such as a mucosal or submucosal tumor, and extrinsic lesions such as an enlarged mediastinal lymph node or mass. Esophageal dysphagia typically presents with difficulty in swallowing solids compared with liquids.

Dysphagia lusoria is a congenital disorder in which an aberrant right subclavian artery (ARSA) causes extrinsic esophageal compression. This woman’s CTA confirmed the presence of an ARSA arising directly from the aortic arch (Figure D, arrow). The term dysphagia lusoria was coined by Bayford in 1794 from the Latin phrase lusus naturae (meaning “freak of nature”).1 Of all the congenital aortic anomalies, an isolated ARSA is the most common. This occurs in approximately 0.5% of the population. The ARSA assumes a retroesophageal position; it proceeds out of the thorax into the right arm, compressing the esophagus and causing dysphagia.

Evaluation of this condition involves a barium swallow study, CTA, or magnetic resonance angiography.2 Both CTA and MRA have largely supplanted the role of conventional angiography, which is invasive. Both CTA and magnetic resonance angiography may also diagnose any other intrathoracic pathology causing esophageal compression. The management of patients with mild to moderate dysphagia is diet modification (minced feeds to well-chewed food; eating slowly and with liquids). Vascular repair of the aberrant vessel is considered only if the patient has severe symptoms and has failed conservative management.3 Because our subject did not have significant weight loss or regurgitation, only dietary advice was offered. An interval outpatient upper endoscopy was planned upon discharge, for which she defaulted.

 

References

1. Bayford. An account of a singular case of obstructed degluitition. Memoirs of the Medical Society of London. 1794;2:275-86.

2. Alper, F., Akgun, M., Kantarci, M. et al. Demonstration of vascular abnormalities compressing esophagus by MDCT: special focus on dysphagia lusoria. Eur J Radiol. 2006;59:82-7.

3. Levitt, B. and Richter, J.E. Dysphagia lusoria: a comprehensive review. Dis Esophagus. 2007;20:455-60.

 

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The diagnosis

Answer to “What’s your diagnosis?” on page 2: Dysphagia lusoria

Dysphagia may be divided into an oropharyngeal cause or an esophageal cause. Esophageal dysphagia may be due to a luminal narrowing or a motility dysfunction. Causes of luminal narrowing include lesions within the lumen such as a foreign body, lesions within the wall of the esophagus such as a mucosal or submucosal tumor, and extrinsic lesions such as an enlarged mediastinal lymph node or mass. Esophageal dysphagia typically presents with difficulty in swallowing solids compared with liquids.

Dysphagia lusoria is a congenital disorder in which an aberrant right subclavian artery (ARSA) causes extrinsic esophageal compression. This woman’s CTA confirmed the presence of an ARSA arising directly from the aortic arch (Figure D, arrow). The term dysphagia lusoria was coined by Bayford in 1794 from the Latin phrase lusus naturae (meaning “freak of nature”).1 Of all the congenital aortic anomalies, an isolated ARSA is the most common. This occurs in approximately 0.5% of the population. The ARSA assumes a retroesophageal position; it proceeds out of the thorax into the right arm, compressing the esophagus and causing dysphagia.

Evaluation of this condition involves a barium swallow study, CTA, or magnetic resonance angiography.2 Both CTA and MRA have largely supplanted the role of conventional angiography, which is invasive. Both CTA and magnetic resonance angiography may also diagnose any other intrathoracic pathology causing esophageal compression. The management of patients with mild to moderate dysphagia is diet modification (minced feeds to well-chewed food; eating slowly and with liquids). Vascular repair of the aberrant vessel is considered only if the patient has severe symptoms and has failed conservative management.3 Because our subject did not have significant weight loss or regurgitation, only dietary advice was offered. An interval outpatient upper endoscopy was planned upon discharge, for which she defaulted.

 

References

1. Bayford. An account of a singular case of obstructed degluitition. Memoirs of the Medical Society of London. 1794;2:275-86.

2. Alper, F., Akgun, M., Kantarci, M. et al. Demonstration of vascular abnormalities compressing esophagus by MDCT: special focus on dysphagia lusoria. Eur J Radiol. 2006;59:82-7.

3. Levitt, B. and Richter, J.E. Dysphagia lusoria: a comprehensive review. Dis Esophagus. 2007;20:455-60.

 

The diagnosis

Answer to “What’s your diagnosis?” on page 2: Dysphagia lusoria

Dysphagia may be divided into an oropharyngeal cause or an esophageal cause. Esophageal dysphagia may be due to a luminal narrowing or a motility dysfunction. Causes of luminal narrowing include lesions within the lumen such as a foreign body, lesions within the wall of the esophagus such as a mucosal or submucosal tumor, and extrinsic lesions such as an enlarged mediastinal lymph node or mass. Esophageal dysphagia typically presents with difficulty in swallowing solids compared with liquids.

Dysphagia lusoria is a congenital disorder in which an aberrant right subclavian artery (ARSA) causes extrinsic esophageal compression. This woman’s CTA confirmed the presence of an ARSA arising directly from the aortic arch (Figure D, arrow). The term dysphagia lusoria was coined by Bayford in 1794 from the Latin phrase lusus naturae (meaning “freak of nature”).1 Of all the congenital aortic anomalies, an isolated ARSA is the most common. This occurs in approximately 0.5% of the population. The ARSA assumes a retroesophageal position; it proceeds out of the thorax into the right arm, compressing the esophagus and causing dysphagia.

Evaluation of this condition involves a barium swallow study, CTA, or magnetic resonance angiography.2 Both CTA and MRA have largely supplanted the role of conventional angiography, which is invasive. Both CTA and magnetic resonance angiography may also diagnose any other intrathoracic pathology causing esophageal compression. The management of patients with mild to moderate dysphagia is diet modification (minced feeds to well-chewed food; eating slowly and with liquids). Vascular repair of the aberrant vessel is considered only if the patient has severe symptoms and has failed conservative management.3 Because our subject did not have significant weight loss or regurgitation, only dietary advice was offered. An interval outpatient upper endoscopy was planned upon discharge, for which she defaulted.

 

References

1. Bayford. An account of a singular case of obstructed degluitition. Memoirs of the Medical Society of London. 1794;2:275-86.

2. Alper, F., Akgun, M., Kantarci, M. et al. Demonstration of vascular abnormalities compressing esophagus by MDCT: special focus on dysphagia lusoria. Eur J Radiol. 2006;59:82-7.

3. Levitt, B. and Richter, J.E. Dysphagia lusoria: a comprehensive review. Dis Esophagus. 2007;20:455-60.

 

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What’s your diagnosis?

By Eric Wee, MD and Ma Clarissa Buenaseda, MD. Published previously in Gastroenterology (2013;144:273,467,468).

A 51-year-old woman was admitted to our hospital for a myocardial infarction. During the admission, she complained of chronic dysphagia for more than 1 year. Her medical history was that of a stroke with good functional recovery and no documented oropharyngeal dysphagia during that admission. Her current complaint of dysphagia was worse with solid foods and better with liquids. She localized her symptoms to the level of the suprasternal notch. A neurologic examination did not reveal any new focal deficits and there was no alarming feature such as weight loss or anemia.

In view of her myocardial infarction, a barium swallow study was performed, which showed a persistent smooth extrinsic indentation on the posterior aspect of the esophagus at the level of T4–T5. There was no retention of contrast in this area (Figure A, arrow). Incidentally, she had a prior computed tomography four-vessel angiogram (CTA) study of the circle of Willis, performed during her previous admission for a stroke. The CTA showed a vessel compressing on the esophagus posteriorly causing proximal dilatation. This corresponded to the level of indentation noted on the barium swallow (Figures B and C, arrows).

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