Aortoesophageal fistula
Updates to Article Attributes
Aortoesophageal fistulaAorto-oesophageal fistulae is a rare fistulous communication are pathologic communications between the aorta and oesophagus and resultsresult in life threatening -threatening upper gastrointestinal haemorrhage. It isThey are fatal in the absence of prompt management.
Epidemiology
Aortoesophageal fistula has an incidence of 0.5 to 1.7% fistulas are a rare entity that accounts for approximately 10% of all 8. Rupture of aortic aneurysm within the oesophagus is the most common cause and constitutes 54.2casesaortoenteric fistulae or an estimated frequency of less than 0.5% in patients admitted with upper gastrointestinal haemorrhage 31-3. Other causes includeAn incidence of 1.7% to 1.9% has been reported in the setting of thoracic endovascular aortic repair (TEVAR) 1,2,4.
Associations
It has been commonly associated with rupture of the descending thoracic aorta and the following conditions 1-6:
atherosclerotic plaqueaortic aneurysmforeign bodyaortic dissectionmalignancy: especially oesophageal cancer
infectionsinfection: e.g. tuberculosis 1radiotherapy
-
postinterventional or postoperative complications
endovascular stent erosion
aortic graft infection
Diagnosis
CT AngiographyThe diagnosis of aortooesophageal fistulae is used to diagnose aortoesophageal fistula althoughprimarily based on imaging or CT angiography. Although upper gastrointestinal endoscopy may be performed to rule out other possible causes of upper gastrointestinal haemorrhage2,36,7.
Clinical presentation
The patient usually presents as a case of emergency with massive haemorrhage and haematemesis. Clinical presentation is classically described by the Chiari triad consisting of 3,52-6:
mid
Thoracicthoracic painsentinel arterial haemorrhage
exsanguination following a symptom
free-free period (seen in 80% of the patients)
Pathology
The etiopathogenesisBy definition, aortoesophageal fistulae are abnormal connections between the oesophagus and the aorta, usually resulting in the passage of aortoesophageal fistulablood from the aorta to the oesophagus or vice versa, i.e. air and oesophageal contents into the aorta 1. Mechanisms include direct erosion in the case of foreign bodies or implants and/or pressure necrosis of the aortic wall and soft tissues between the oesophagus and aorta 1,2.
Location
The most common location is only known in certain cases. Continuous arterial pulsations and resultant trauma is cited as a causethe descending thoracic aorta, but aortooesophageal fistulae have been also reported for the development of aortoesophageal fistula in patients with aortic stentsarch and ascending aorta 7. Rupture of an abscess following infection also leads to formation of aortoesophageal fistulas in some cases 72.
Radiographic features
CT
CT angiography in an aortoesophageal fistula may show1,83,5:
extravasation of contrast material
gas effusionair bubbles in the aortic wall or around the graftdirect connection between the aorta and oesophagus
oesophageal narrowing
false aneurysm
mediastinal haematoma
teat like outpouching from aorta into oesophagusstent migration
Radiology report
The radiology report should contain the following:
-
diagnosis and location of the fistula with supporting features as
extravasation of contrast material
stentair bubbles in the aortic wall or around the graft after TEVAR
-
possible causes
implants (stent migration)
mural thrombusforeign bodiestrue or false aortic aneurysms
aortic dissection
-
additional features and complications
mediastinal haemorrhage
Treatment and prognosis
AortoesophagealManagement usually includes a combination of bleeding control in the urgent phase and surgical intervention with radical debridement of the fistula hasand the contaminated zone as well as reconstruction of the aorta and the oesophagus later as a mortalitysemi-urgent intervention 1. Initial treatment and bleeding control has been done with a Sengstaken-Blakemore tube in the past but is usually done with TEVAR nowadays 1 followed by a perioperative management period and radical surgery once the patient is in the condition for the latter 1.
Prognosis is not good with an estimated death rate of 77up to 60% within 6 months after symptom onset 1,2.
History and requires urgent management 3.etymology
Haematemesis is managed with the Sengstaken-Blakemore tube, which temporarily stabilises the patient with its tamponade effect 1,3.
Management ofThe first aortoesophageal fistula involves two steps: aortic repair and oesophageal repair. Aortic repair is usually performedwas described in 1818 by a minimally invasive procedure called thoracic endovascular aortic repair. Oesophageal repair involves oesophagectomy and oesophageal stent placementthe French naval surgeon Joseph-MarieDubreiul (1790-1852) 1,3.
Surgical management can also be done by open surgery, which has a higher mortality rate but allows a thorough management of infection in the presence of an infectious cause 38,10.
Differential diagnosis
Aortoesophageal fistula needs to be differentiated from other causes of upper gastrointestinal haemorrhage.
History and etymology
Aortoesophageal fistula was first described in 1818 by a French naval surgeon Dubreiul 6.
See also
-<p><strong>Aortoesophageal fistula</strong> is a rare fistulous communication between the aorta and oesophagus and results in life threatening <a href="/articles/upper-gastrointestinal-bleeding" title="Upper gastrointestinal bleeding">upper gastrointestinal haemorrhage</a>. It is fatal in the absence of prompt management. </p><h4>Epidemiology</h4><p>Aortoesophageal fistula has an incidence of 0.5 to 1.7% <sup>8</sup>. Rupture of <a href="/articles/thoracic-aortic-aneurysm" title="Thoracic aortic aneurysm (TAA)">aortic aneurysm </a>within the oesophagus is the most common cause and constitutes 54.2% of all cases<sup> 3</sup>. Other causes include <sup>1,2,4</sup>:</p><ul>-<li><p>atherosclerotic plaque</p></li>-<li><p>foreign body </p></li>-<li><p><a href="/articles/oesophageal-carcinoma-1" title="Esophageal cancer">oesophageal cancer</a></p></li>-<li><p>infections</p></li>- +<p><strong>Aorto-oesophageal fistulae</strong> are pathologic communications between the aorta and oesophagus and result in life-threatening <a href="/articles/upper-gastrointestinal-bleeding" title="Upper gastrointestinal bleeding">upper gastrointestinal haemorrhage</a>. They are fatal in the absence of prompt management. </p><h4>Epidemiology</h4><p>Aortoesophageal fistulas are a rare entity that accounts for approximately 10% of all <a href="/articles/aortoenteric-fistula" title="Aortoenteric fistula">aortoenteric fistulae</a> or an estimated frequency of less than 0.5% in patients admitted with <a href="/articles/upper-gastrointestinal-bleeding" title="Upper gastrointestinal bleeding">upper gastrointestinal haemorrhage</a> <sup>1-3</sup>. An incidence of 1.7% to 1.9% has been reported in the setting of thoracic endovascular aortic repair (TEVAR) <sup>1</sup>.</p><h5>Associations</h5><p>It has been commonly associated with rupture of the descending thoracic aorta and the following conditions <sup>1-6</sup>:</p><ul>
- +<li><p><a href="/articles/aortic-aneurysm-1" title="Aortic aneurysm">aortic aneurysm</a></p></li>
- +<li><p><a href="/articles/aortic-dissection" title="Aortic dissection">aortic dissection</a></p></li>
- +<li><p><a href="/articles/foreign-body-1" title="Foreign body">foreign body</a></p></li>
- +<li><p><a href="/articles/cancer" title="Malignancy">malignancy</a>: especially <a href="/articles/oesophageal-carcinoma-1" title="Esophageal cancer">oesophageal cancer</a></p></li>
- +<li><p>infection: e.g. tuberculosis <sup>1</sup></p></li>
- +<li>
- +<p>postinterventional or postoperative complications</p>
- +<ul>
-</ul><h4>Diagnosis</h4><p>CT Angiography is used to diagnose aortoesophageal fistula although upper gastrointestinal endoscopy may be performed to rule out other possible causes of upper gastrointestinal haemorrhage <sup>2,3</sup>. </p><h4>Clinical presentation </h4><p>The patient usually presents as a case of emergency with massive haemorrhage and haematemesis. Clinical presentation is classically described by the Chiari triad consisting of <sup>3,5</sup>:</p><ul>-<li><p>mid Thoracic pain </p></li>-<li><p>sentinel haemorrhage </p></li>-<li><p>exsanguination following a symptom free period (seen in 80% of the patients)</p></li>-</ul><h4>Pathology</h4><p>The etiopathogenesis of aortoesophageal fistula is only known in certain cases. Continuous arterial pulsations and resultant trauma is cited as a cause for the development of aortoesophageal fistula in patients with aortic stents <sup>7</sup>. Rupture of an abscess following infection also leads to formation of aortoesophageal fistulas in some cases <sup>7</sup>.</p><h4>Radiographic features</h4><p>CT angiography in an aortoesophageal fistula may show <sup>1,8</sup>: </p><ul>-<li><p>extravasation of contrast material </p></li>-<li><p>gas effusion</p></li>- +</ul>
- +</li>
- +</ul><h4>Diagnosis</h4><p>The diagnosis of aortooesophageal fistulae is primarily based on imaging or CT angiography. Although upper gastrointestinal endoscopy may be performed to rule out other possible causes of upper gastrointestinal haemorrhage <sup>6,7</sup>.</p><h4>Clinical presentation </h4><p>The patient usually presents as a case of emergency with massive haemorrhage and haematemesis. Clinical presentation is classically described by the Chiari triad <sup>2-6</sup>:</p><ul>
- +<li><p>mid thoracic pain </p></li>
- +<li><p>sentinel arterial haemorrhage </p></li>
- +<li><p>exsanguination following a symptom-free period (seen in 80% of the patients)</p></li>
- +</ul><h4>Pathology</h4><p>By definition, aortoesophageal fistulae are abnormal connections between the oesophagus and the aorta, usually resulting in the passage of blood from the aorta to the oesophagus or vice versa, i.e. air and oesophageal contents into the aorta <sup>1</sup>. Mechanisms include direct erosion in the case of foreign bodies or implants and/or pressure necrosis of the aortic wall and soft tissues between the oesophagus and aorta <sup>1,2</sup>.</p><h5>Location</h5><p>The most common location is the descending thoracic aorta, but aortooesophageal fistulae have been also reported for the aortic arch and ascending aorta <sup>2</sup>.</p><h4>Radiographic features</h4><h5>CT</h5><p>CT angiography in an aortoesophageal fistula may show <sup>3,5</sup>: </p><ul>
- +<li><p>extravasation of contrast material</p></li>
- +<li><p>air bubbles in the aortic wall or around the graft</p></li>
- +<li><p>direct connection between the aorta and oesophagus</p></li>
-<li><p>teat like outpouching from aorta into oesophagus </p></li>-<li><p>stent migration </p></li>-<li><p>mural thrombus</p></li>-</ul><h4>Treatment and prognosis </h4><p>Aortoesophageal fistula has a mortality rate of 77% and requires urgent management <sup>3</sup>. </p><p>Haematemesis is managed with the Sengstaken-Blakemore tube, which temporarily stabilises the patient with its tamponade effect<sup> 1,3</sup>. </p><p>Management of aortoesophageal fistula involves two steps: aortic repair and oesophageal repair. Aortic repair is usually performed by a minimally invasive procedure called thoracic endovascular aortic repair. Oesophageal repair involves oesophagectomy and oesophageal stent placement <sup>1,3</sup>. </p><p>Surgical management can also be done by open surgery, which has a higher mortality rate but allows a thorough management of infection in the presence of an infectious cause <sup>3</sup>. </p><h4>Differential diagnosis</h4><p>Aortoesophageal fistula needs to be differentiated from other causes of <a href="/articles/upper-gastrointestinal-bleeding-differential" title="Upper gastrointestinal bleeding (differential)">upper gastrointestinal haemorrhage</a>. </p><h4>History and etymology </h4><p>Aortoesophageal fistula was first described in 1818 by a French naval surgeon <strong>Dubreiul </strong><sup>6</sup>. </p><h4>See also </h4><p><a href="/articles/aortoenteric-fistula" title="Aortoenteric fistula">Aortoenteric fistula</a> </p><p><br></p>- +<li><p>stent migration</p></li>
- +</ul><h4>Radiology report</h4><p>The radiology report should contain the following:</p><ul>
- +<li>
- +<p>diagnosis and location of the fistula with supporting features as</p>
- +<ul>
- +<li><p>extravasation of contrast material </p></li>
- +<li><p>air bubbles in the aortic wall or around the graft after TEVAR</p></li>
- +</ul>
- +</li>
- +<li>
- +<p>possible causes</p>
- +<ul>
- +<li><p>implants (stent migration)</p></li>
- +<li><p>foreign bodies</p></li>
- +<li><p>true or false aortic aneurysms</p></li>
- +<li><p>aortic dissection</p></li>
- +</ul>
- +</li>
- +<li>
- +<p>additional features and complications</p>
- +<ul>
- +<li><p>mediastinal haemorrhage</p></li>
- +<li><p><a href="/articles/haemothorax" title="Haematothorax">haematothorax</a></p></li>
- +</ul>
- +</li>
- +</ul><h4>Treatment and prognosis </h4><p>Management usually includes a combination of bleeding control in the urgent phase and surgical intervention with radical debridement of the fistula and the contaminated zone as well as reconstruction of the aorta and the oesophagus later as a semi-urgent intervention <sup>1</sup>. Initial treatment and bleeding control has been done with a Sengstaken-Blakemore tube in the past but is usually done with TEVAR nowadays <sup>1</sup> followed by a perioperative management period and radical surgery once the patient is in the condition for the latter <sup>1</sup>.</p><p>Prognosis is not good with an estimated death rate of up to 60% within 6 months after symptom onset <sup>1,2</sup>.</p><h4>History and etymology </h4><p>The first aortoesophageal fistula was described in 1818 by the French naval surgeon <strong>Joseph-Marie</strong> <strong>Dubreiul </strong>(1790-1852) <sup>8,10</sup>. </p><h4>Differential diagnosis</h4><p>Aortoesophageal fistula needs to be differentiated from other causes of <a href="/articles/upper-gastrointestinal-bleeding-differential" title="Upper gastrointestinal bleeding (differential)">upper gastrointestinal haemorrhage</a>.<br></p>
References changed:
- 3. Kieffer E, Chiche L, Gomes D. Aortoesophageal Fistula. Ann Surg. 2003;238(2):283-90. <a href="https://doi.org/10.1097/01.sla.0000080828.37493.e0">doi:10.1097/01.sla.0000080828.37493.e0</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12894023">Pubmed</a>
- 6. Zhong X & Li G. Successful Management of Life-Threatening Aortoesophageal Fistula: A Case Report and Review of the Literature. WJCC. 2022;10(12):3814-21. <a href="https://doi.org/10.12998/wjcc.v10.i12.3814">doi:10.12998/wjcc.v10.i12.3814</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/35647167">Pubmed</a>
- 7. Wong A, Chou Y, Goh Z, Chang K, Seak C. Case Report: Aortoesophageal Fistula—an Extremely Rare but Life-Threatening Cardiovascular Cause of Hematemesis. Front Cardiovasc Med. 2023;10:1123305. <a href="https://doi.org/10.3389/fcvm.2023.1123305">doi:10.3389/fcvm.2023.1123305</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/37153464">Pubmed</a>
- 11. Levine M. Miscellaneous Abnormalities of the Esophagus. Textbook of Gastrointestinal Radiology, 2-Volume Set. 2015;:412-37. <a href="https://doi.org/10.1016/b978-1-4557-5117-4.00025-8">doi:10.1016/b978-1-4557-5117-4.00025-8</a>
- 4. Heckstall R & Hollander J. Aortoesophageal Fistula: Recognition and Diagnosis in the Emergency Department. Ann Emerg Med. 1998;32(4):502-5. <a href="https://doi.org/10.1016/s0196-0644(98)70182-9">doi:10.1016/s0196-0644(98)70182-9</a>
- 8. Lui R, Johnson F, Horovitz J, Cunningham J. Aortoesophageal Fistula: Case Report and Literature Review. J Vasc Surg. 1987;6(4):379-82. <a href="https://doi.org/10.1016/0741-5214(87)90009-7">doi:10.1016/0741-5214(87)90009-7</a>
- 9. Al-Thani H, Wahlen B, El-Menyar A et al. Presentation, Management and Outcome of Aorto-Esophageal Fistula in Young Patients: Two Case-Reports and Literature Review. Journal of Surgical Case Reports. 2021;2021(6):rjab213. <a href="https://doi.org/10.1093/jscr/rjab213">doi:10.1093/jscr/rjab213</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/34211691">Pubmed</a>
- 5. Gulati A, Kapoor H, Donuru A, Gala K, Parekh M. Aortic Fistulas: Pathophysiologic Features, Imaging Findings, and Diagnostic Pitfalls. Radiographics. 2021;41(5):1335-51. <a href="https://doi.org/10.1148/rg.2021210004">doi:10.1148/rg.2021210004</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/34328814">Pubmed</a>
- 1. Uno K, Koike T, Takahashi S, Komazawa D, Shimosegawa T. Management of Aorto-Esophageal Fistula Secondary After Thoracic Endovascular Aortic Repair: A Review of Literature. Clin J Gastroenterol. 2017;10(5):393-402. <a href="https://doi.org/10.1007/s12328-017-0762-z">doi:10.1007/s12328-017-0762-z</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28766283">Pubmed</a>
- 2. Hollander J & Quick G. Aortoesophageal Fistula: A Comprehensive Review of the Literature. Am J Med. 1991;91(3):279-87. <a href="https://doi.org/10.1016/0002-9343(91)90129-l">doi:10.1016/0002-9343(91)90129-l</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/1892150">Pubmed</a>
- 1.Kieffer E, Chiche L, Gomes D. Aortoesophageal Fistula. Ann Surg. 2003;238(2):283-90. <a href="https://doi.org/10.1097/01.sla.0000080828.37493.e0">doi:10.1097/01.sla.0000080828.37493.e0</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12894023">Pubmed</a>
- 2.Zhong X & Li G. Successful Management of Life-Threatening Aortoesophageal Fistula: A Case Report and Review of the Literature. WJCC. 2022;10(12):3814-21. <a href="https://doi.org/10.12998/wjcc.v10.i12.3814">doi:10.12998/wjcc.v10.i12.3814</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/35647167">Pubmed</a>
- 3.Wong A, Chou Y, Goh Z, Chang K, Seak C. Case Report: Aortoesophageal Fistula—an Extremely Rare but Life-Threatening Cardiovascular Cause of Hematemesis. Front Cardiovasc Med. 2023;10:1123305. <a href="https://doi.org/10.3389/fcvm.2023.1123305">doi:10.3389/fcvm.2023.1123305</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/37153464">Pubmed</a>
- 4.Levine M. Miscellaneous Abnormalities of the Esophagus. Textbook of Gastrointestinal Radiology, 2-Volume Set. 2015;:412-37. <a href="https://doi.org/10.1016/b978-1-4557-5117-4.00025-8">doi:10.1016/b978-1-4557-5117-4.00025-8</a>
- 5.Heckstall R & Hollander J. Aortoesophageal Fistula: Recognition and Diagnosis in the Emergency Department. Ann Emerg Med. 1998;32(4):502-5. <a href="https://doi.org/10.1016/s0196-0644(98)70182-9">doi:10.1016/s0196-0644(98)70182-9</a>
- 6.Lui R, Johnson F, Horovitz J, Cunningham J. Aortoesophageal Fistula: Case Report and Literature Review. J Vasc Surg. 1987;6(4):379-82. <a href="https://doi.org/10.1016/0741-5214(87)90009-7">doi:10.1016/0741-5214(87)90009-7</a>
- 7.Al-Thani H, Wahlen B, El-Menyar A et al. Presentation, Management and Outcome of Aorto-Esophageal Fistula in Young Patients: Two Case-Reports and Literature Review. Journal of Surgical Case Reports. 2021;2021(6):rjab213. <a href="https://doi.org/10.1093/jscr/rjab213">doi:10.1093/jscr/rjab213</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/34211691">Pubmed</a>
- 8.Gulati A, Kapoor H, Donuru A, Gala K, Parekh M. Aortic Fistulas: Pathophysiologic Features, Imaging Findings, and Diagnostic Pitfalls. Radiographics. 2021;41(5):1335-51. <a href="https://doi.org/10.1148/rg.2021210004">doi:10.1148/rg.2021210004</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/34328814">Pubmed</a>
Systems changed:
- Gastrointestinal