Brief Clinical Observation
Dysphagia aortica: A neglected symptom of aortoesophageal fistula

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Abstract

Aortoesophageal fistula, secondary to thoracic aortic aneurysm, is an uncommon cause of gastrointestinal bleeding that is uniformly fatal without surgical intervention. Typical symptoms are midthoracic pain and/or dysphagia followed by a usually short, albeit unpredictable, symptom-free interval and by a ‘herald’ haemorrhage, which is observed in 80% of patients before fatal exsanguinations. Dysphagia is present in 45% of patients, sometimes for several weeks, before the first bleeding occurs. However, dysphagia aortica is rarely considered in the differential diagnosis of dysphagia and lack of awareness, as well as symptom's underevaluation, both contribute to a significant diagnostic and therapeutic delay. We present a case of a 77-year-old woman who died for a bleeding AEF consequent to a thoracic aortic aneurysm and whose main symptom during the past 2 months was dysphagia, which was not taken seriously into consideration by her general practitioner. This case report emphasises that primary care physicians should be alerted to evaluate carefully the alarming symptoms like dysphagia – especially in elderly patients – before life threatening complications occur, as they are the ones who could suspect early the diagnosis and make a proper referral.

Introduction

Aortoesophageal fistula (AEF) is an uncommon but often catastrophic consequence arising from a number of oesophageal or aortic diseases. AEFs constitute approximately 10% of all aortoenteric fistulas [1] and, since it is suspected very rarely, the vast majority of patients die before a definite diagnosis can be made.

Thoracic aortic aneurysms are the most common cause of AEFs [2]. According to Laplace's equation, as the diameter of the aneurysm increases, wall tension at any given intraluminal pressure increases and causes damage to the vasa vasorum of the aorta; this slowly weakens the aortic wall and may result in gradual erosion of the oesophagus [3].

Chiari [4] first described the typical syndrome of AEF as a triad of midthoracic pain or dysphagia, a short symptom-free interval, followed by a ‘herald’ haemorrage, and fatal exsanguination following a symptom-free interval of hours or days, with very few patients having recurrent, self-limiting haemorrages. Midthoracic pain may be caused by distension, erosion and localised dissection of the aortic wall or oesophageal perforation with mediastinitis [3]. Dysphagia occurs because of compression of the oesophagus or the vagus nerve and usually precedes the herald haemorrage [5]. Spontaneous cessation of bleeding may be caused by temporary occlusion of the fistula due to the spasm of the arterial wall, intravascular hypotension from the initial haemorrhage, and/or occlusion of the fistula by periaortic haematoma, which is later digested by infection or gastrointestinal contents [3].

Once AEF is identified or strongly suspected, immediate surgery with aneurysm and fistula repair is mandatory since it represents the only definitive treatment for AEF [5]. Use of a Sengstaken–Blakemore tube [6], [7] or embolisation of the fistula prior to surgery [8] represent only temporary measures and should be considered only if the patient is exsanguinating. Although surgical repairs have high morbidity and mortality, surgery should be undertaken, as untreated cases of AEF are uniformly fatal.

We present a case of a patient who died due to a bleeding AEF caused by a thoracic aortic aneurysm. Dysphagia was the main symptom, which the patient complained of 2 months previously, whose underevaluation contributed to delay a correct diagnosis of thoracic aneurysm.

Section snippets

Case report

A 77-year-old woman was admitted to our University Hospital with chest pain and haematemesis of bright red blood. Past medical history reported previous surgery for an abdominal aortic aneurysm and an aorto–coronaric bypass. Dysphagia was the main disturbing symptom for more than 2 months before the admission, but was not taken seriously into consideration by her general practitioner. Vital signs (BP, 90/60 mmHg; HR, 110 beats/min) at admission were improved rapidly by intravenous fluid

Discussion

This report concerns an infrequent, though not exceptional, complication of a thoracic aortic aneurysm. Moreover, it highlights how precocious symptoms of this life threatening condition may be overlooked. Approximately 10% of ruptured thoracic aorta aneurysms penetrate the oesophagus, and this is regarded as the commonest case of AEF [1], [9]. Typical symptoms are midthoracic pain and/or dysphagia followed by a usually short, albeit unpredictable, symptom-free interval and by a ‘herald’

Conflict of interest statement

None declared.

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