Guest Editor: H. Ralph Schumacher, Jr, MD
Professor of Medicine
University of Pennsylvania School of Medicine, Philadelphia

Dysphagia in the elderly can arise from functional causes, neuromuscular disorders, or mechanical causes. A rare cause of mechanical dysphagia is an extrinsic compression of the esophagus by a thoracic aortic aneurysm. Patients are generally elderly (>65 years) and have progressive intolerance to solid foods and, consequently, concomitant progressive weight loss. Management is generally conservative, because many of the patients with this condition are elderly and are not good candidates for surgery. We report the case of an elderly patient with dysphagia, weight loss, and an aortic aneurysm who was diagnosed with dysphagia aortica.

Case Presentation
An 84-year-old woman was admitted to the hospital for the evaluation of dysphagia, epigastric discomfort of 1 month’s duration, and weight loss of 14.5 kg (32 lb) over a 2-month period. She reported progressive dysphagia to solids only as well as a reduction in her oral intake, because of her fear of eliciting the epigastric discomfort. Before this hospitalization she had scheduled an appointment with a gastroenterologist, which she did not keep.

Her medical history included hypertension, hypothyroidism, hyperlipidemia, osteoarthritis, macular degeneration, a coronary artery bypass graft, and a thoracoabdominal aneurysm occurring 10 years ago. She denied any history of nausea, vomiting, halitosis, diarrhea, constipation, chest pain, or dyspnea.

The physical examination at admission was unremarkable, except for a murmur of aortic stenosis. The patient weighed 79 kg (174 lb) and was 1.68 m tall. Laboratory studies included electrolytes and a complete blood cell count, which were normal. Because of her history of thoracoabdominal aneurysm, chest radio­graphs were ordered to rule out aortic rupture or dissection. The radiographs revealed a thoracic aortic aneurysm, with prominent aortic ectasia. The maximum diameter of the aorta was approximately 7 cm at the level below the aortic arch. A comparison with radiographs taken 4.5 years earlier showed that the aneurysm size increased significantly (Figure 1).

An upper gastrointestinal (GI) endoscopy revealed extrinsic compression along the length of the esophagus by a pulsatile mass consistent with the thoracic aorta, which created a concave, slitlike opening in that portion of the esophagus (Figure 2). The endoscope was passed with ease to the distal duodenum, revealing that the rest of the esophagus was normal. A contrast-enhanced computed tomography (CT) scan of the chest confirmed the finding of thoracic aortic aneurysm and also showed an extensive mural thrombus (Figure 3). A 3-dimensional reconstruction of the thoracic aortic aneurysm is shown in Figure 4.

The patient’s symptoms and the radiographic findings supported the diagnosis of dysphagia aortica. Following the patient’s wishes, and, because of her advanced age, a conservative approach to management was opted. She was placed on a pureed diet and liquid supplements. Eighteen months later, she is still doing well with conservative therapy.

Discussion
Dysphagia, defined as difficulty in swallowing, can be classified based on its etiology as oropharyngeal (neuromuscular), mechanical, or functional. Mechanical causes may be intrinsic or extrinsic and usually result in a progressive inability to swallow solid boluses and concomitant weight loss.

Dysphagia aortica
A rare mechanical cause of dysphagia is the extrinsic compression of the esophagus by the thoracic aorta as illustrated in this case. Dysphagia aortica, which was first described by Pape in 1932,¹ is a rare condition usually associated with hypertension and old age and is classically seen in elderly women of short stature.²

Diagnosis
The differential diagnosis of dysphagia includes several causes of mechanical esophageal obstruction and is limited largely to those causes that present as dysphagia to solids only (Table). The diagnosis of dysphagia aortica should be considered in any patient with a history of aortic aneurysm who develops dysphagia.

The following imaging modalities are used for the diagnosis of dysphagia aortica, each capable of revealing specific findings:

• Upper GI endoscopy shows a pulsatile extrinsic compression or stenosis of the lower esophagus with proximal dilatation.

• Barium swallow may show a tortuous and enlarged esophagus, and a dilated atonic esophagus with proximal dilatation is occasionally observed.

• Manometry shows low-amplitude propagated peristaltic waves in the proximal part of the esophagus and a high-pressure band at the site of compression. Pulsations coinciding with the cardiac cycle may be seen. The manometric findings, however, have no clear relationship with the barium swallow findings.^2-4 Manometric findings of vascular compression do not necessarily correlate with symptomatic dysphagia.³

Other diagnostic modalities that aid the diagnosis of dysphagia aortica include chest radiographs, CT, and magnetic resonance imaging.

Treatment
The treatment of dysphagia aortica usually takes a conservative route, because the majority of patients with this condition are elderly, and many of them have multiple comorbidities. Therefore, they usually are not candidates for surgery. The management consists of control of the associated hypertension and the institution of a semisolid or liquid diet. In severe cases (ie, total dysphagia to solids and liquids with a high risk for aspiration and malnutrition), when the patient is not a candidate for surgery, a percutaneous endoscopic gastrostomy tube may be inserted for feeding.²

Conclusion
Dysphagia aortica is a rare cause of mechanical esophageal obstruction that results from extrinsic compression of the esophagus by an enlarging aortic aneurysm. The condition usually occurs in an elderly patient who has several comorbidities. Conservative treatment is often preferred because of the patient’s age and the associated comorbidities.

References
1. Pape R. Über einen abnormen verlauf (“tiefe Rechtslage”) der mesa aortitischen descendens [in German]. Fortschr Roentgenstr. 1932; 46:257-269.

2. Wilkinson JM, Euinton HA, Smith LF, et al. Diagnostic dilemmas in dysphagia aortica. Eur J Cardiothorac Surg. 1997;11:222-227.

3. Stagias JG, Ciarolla D, Campo S, et al. Vascular compression of the esophagus: a manometric and radiologic study. Dig Dis Sci. 1994;39: 782-786.

4. Sundaram U, Traube M. Radiologic and manometric study of the gastroesophageal junction in dysphagia aortica. J Clin Gastroenterol. 1995;21:275-278.

Commentary

Dysphagia in the elderly population has an extremely broad differential diagnosis, including achalasia, esophageal webs, peptic strictures, neoplasms, and vascular aberrations. As with most conditions, a careful history and physical examination are key to the diagnosis. Untreated gastroesophageal reflux disease or a history of excessive tobacco or alcohol use may prompt an investigation for esophageal cancer. Vascular abnormalities resulting in esophageal compression, such as dysphagia aortica, are relatively rare conditions.

Other causes of dysphagia in the elderly, such as esophageal cancer, are far more common and should always be at the top of the differential diagnosis list. A history of scleroderma may lead the physician to believe the patient is suffering from the associated esophageal complications of this condition.

Diagnosis can be confirmed only with the use of medical technology, such as computed tomography, magnetic resonance imaging, or endoscopic ultrasonography. Still an extremely important test, barium studies can demonstrate achalasia, webs, and strictures. Intrinsic or extrinsic esophageal lesions can give rise to luminal irregularities, which may be found by use of barium studies. In many patients with dysphagia, it is also extremely important to assess the adequacy of the esophageal motor function.

Manometric studies can clearly show the mechanical swallowing abnormalities that may be producing symptoms. Endoscopy now allows the physician to directly visualize the esophageal mucosa and potentially to biopsy any intraluminal abnormality.

The authors present the case of an elderly woman complaining of dysphagia that resulted from thoracic aortic aneurysm. Also known as dysphagia aortica, this condition is an uncommon cause of dysphagia. Dysphagia aortica occurs when an enlarging thoracic or abdominal aorta causes extrinsic compression of the esophagus. This enlarging aneurysm classically results in progressive dysphagia to solid foods over a period of several months. Because of its association with an aortic aneurysm, it is not surprising that dysphagia aortica is typically seen in elderly patients who have a long-standing history of hypertension.

In patients with dysphagia aortica, an extrinsic pulsatile esophageal mass may be observed on endoscopy, which was an important clue to the diagnosis in this patient. If manometric studies were performed, low-amplitude propagated peristaltic waves with an area of high pressure at the site of aortic compression might have been seen.

As demonstrated in this case, conservative management with pureed or liquid diets can produce adequate results, and surgical correction should be reserved only for those who have a low surgical risk.

In addition to dysphagia aortica, more common vascular anomalies resulting in dysphagia have been described, including double aortic arch and atrial dilatation.^1-3 Among these, the best described is dysphagia lusoria, which occurs when an aberrant right subclavian artery causes extrinsic compression of the esophagus. Presenting symptoms are similar to those of dysphagia aortica; however, patients with dysphagia lusoria generally present at a younger age than those with dysphagia aortica.

The earlier onset of dysphagia lusoria is likely the result of the congenital nature of the condition, in contrast to the acquired anomaly seen in dysphagia aortica. In the younger population, symptoms associated with dysphagia lusoria are usually best treated by surgical correction.

Given the increasing age of the American population and the increased risk of vascular disease associated with aging, it is possible that dysphagia aortica may become more frequent in the next several decades. A careful history and a thorough investigation are crucial for the diagnosis.

References
1. Shanmugam G, Macarthur K, Pollock J. Surgical repair of double aortic arch: 16-year experience. Asian Cardiovasc Thorac Ann. 2005; 13:4-10.

2. Kress S, Martin WR, Benz C, Riemann JF. Dysphagia secondary to left atrial dilatation. Z Gastroenterol. 1997;35:1007-1011.

3. van Son JA, Julsrud PR, Hagler DJ, et al. Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc. 1993;68: 1056-1063.