CASE REPORT
Aortoesophageal Fistula: Recognition and Diagnosis in the Emergency Department From the Departments of Emergency Medicine, University Medical Center, State University of New York, Stony Brook, NY,* and Hospital of University of Pennsylvania, University of Pennsylvania School of Medicine, Philadelphia, PA.‡ Received for publication October 27, 1997. Revision received February 27, 1998. Accepted for publication May 27, 1998. Copyright © 1998 by the American College of Emergency Physicians. 0196-0644/98/$5.00 + 0 47/1/92995
Robert L Heckstall, MD* Judd E Hollander, MD‡
An aortoesophageal fistula is a life-threatening cause of gastrointestinal bleeding where an abnormal communication between the esophagus and the aorta may result from a thoracic aortic aneurysm, foreign body ingestion, esophageal malignancy, or postoperative complications. The diagnosis can be made on the basis of clinical findings alone. Classic patients present with the triad of midthoracic pain, sentinel arterial hemorrhage, and exsanguination after a symptom-free interval (Chiari’s triad). The identification of massive upper gastrointestinal hemorrhage that is bright red and arterial in nature is characteristic. Most diagnostic tests have significant individual limitations. Endoscopy of the upper gastrointestinal tract should exclude alternative bleeding sources and may show a submucosal hematoma. Aortography may be useful during active hemorrhage to demonstrate the fistula, but results of aortography may be negative during the symptom-free interval. Dynamic computed tomography may be a more rapid alternative. For patients who are in stable condition after the sentinel hemorrhage, a confirmatory test is reasonable. Patients in unstable condition should undergo immediate surgery. Survival is now possible with rapid surgical intervention. [Heckstall RL, Hollander JE: Aortoesophageal fistula: Recognition and diagnosis in the emergency department. Ann Emerg Med October 1998;32:502-505.]
INTRODUCTION
Approximately 150 patients per 100,000 population are hospitalized annually for bleeding in the upper gastrointestinal tract.1 Duodenal and gastric ulcers, gastritis, esophageal varices, and Mallory-Weiss tears are the most common sources of gastrointestinal bleeding. Emergency physicians are well trained in the management of these entities. Other less common causes of gastrointestinal bleeding have received less attention in the emergency medicine literature. One such example is an aortoesophageal fistula. Aortoesophageal fistulas are life threatening. Diagnostic
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and treatment protocols differ from those of other sources of gastrointestinal bleeding. As a result, emergency physicians should be aware of the presentation and management of patients with aortoesophageal fistula. We report a case of a massive hemorrhage in the upper gastrointestinal tract resulting from an aortoesophageal fistula. We discuss methods to rapidly diagnose and treat patients in whom aortoesophageal fistula is suspected. CASE REPORT
A 31-year-old Hispanic man with a history of a gunshot wound to the abdomen 6 years earlier presented to the emergency department complaining of right upper quadrant abdominal pain and vomiting blood. The patient had been in his usual state of health until approximately 24 hours before admission when right-sided abdominal pain developed a half hour after he ate Chinese food. He described the pain as sharp, constant, and nonradiating. The pain was not relieved by movement or position. There was no accompanying diarrhea, fever, chills, or recent trauma. He vomited a slight amount of blood the evening before arrival. There was no further vomiting until approximately 1 hour before ED arrival at which time he vomited bright red blood. The patient became lightheaded and diaphoretic. He called EMS and was transported to the ED. On ED arrival the patient was diaphoretic. He had dried blood on his face and clothing. His blood pressure was 130/70 mm Hg, pulse was 130 beats/minute, and respirations were 20 breaths per minute. He was afebrile. Pulse oximetry was 100%. The examination of head and neck was remarkable for blood on his face. Results of the cardiopulmonary examination were normal except for the heart rate. The abdomen had 4 surgical incision sites from previous laparotomy after the gunshot wound. There was no tenderness, guarding, or rebound. Rectal examination was remarkable for melenic stool that was strongly guaiac positive. Neurologically the patient was alert, oriented, and did not have focal abnormalities. The patient had 2 large-bore intravenous lines placed with infusion of normal saline solution. Samples for a CBC, determination of serum electrolytes, coagulation profile, and a type and crossmatch were sent to the laboratory. A bedside hematocrit measurement was 39%. Approximately 45 minutes after arrival, the patient began to continuously vomit copious amounts of bright red blood. A nasogastric tube was placed without difficulty and 150 mL of bright red blood was aspirated. Despite nasogastric suction the patient continued to have emesis of large amounts of bright red blood. He received transfusion with 2 units of O-nega-
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tive blood through a rapid infuser. Despite blood product infusion the patient became unresponsive. The systolic blood pressure dropped to 70 mm Hg and pulse increased to more than 150 beats/minute. Because of the copious amount of emesis, hypotension, and an inability to protect his airway, the patient was intubated with a 7.5-mm endotracheal tube after rapid sequence induction with a defasciculating dose of 1 mg of vecuronium, 20 mg of etomidate, and 120 mg of succinylcholine. A presumptive diagnosis of aortoesophageal fistula was made, and gastrointestinal and surgery consultations were called. Bedside endoscopy was performed by the gastroenterology consultant who was unable to identify a bleeding source because of the large amount of blood in the gastrointestinal tract. The patient continued to have a blood pressure that hovered between 70 and 110 mm Hg despite infusion of crystalloid and blood products. After several minutes of hypotension, the bleeding slowed, the stomach was visualized and did not have signs of active bleeding. While the endoscopist was evaluating the stomach, the patient was noted to have large amounts of bright red blood draining through the nares. Because the stomach did not appear to be actively bleeding, the consultant considered the possibility of a posterior nasal hemorrhage and bilateral posterior packs were placed. The patient’s systolic blood pressure remained labile between 70 and 100 mm Hg despite the infusion of 4 units of packed red cells, 2 units of fresh frozen plasma, 10 units of platelets, and 5 L normal saline solution. The patient continued to bleed through his mouth despite no obvious bleeding in the stomach and bilateral posterior nasal packs. To better evaluate the esophagus, the patient was positioned at a 45-degree angle. After the change in position, blood was noted in the stomach. On slow withdrawal of the endoscope into the esophagus, an area of pulsatile blood clot was noted in the posterior distal esophagus. The clot sloughed inferiorly, and massive bleeding was seen to occur through a small hole in the posterior esophagus. While the patient was being prepared for the operating room, injection with epinephrine was attempted without success. In the operating room an abdominal incision was made, the stomach was mobilized with dissection, and the communication between the aorta and the esophagus was noted to occur superior to the exposed field. A cardiothoracic surgeon performed a thoracotomy to expose the communication but was unable to suture the communication closed. The patient died. Postmortem examination revealed a fistulous tract between the aorta and esophagus at the distal portion of the esophagus. Sutures with surrounding granulation tissue were present in the aorta. The cause of death was a
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fistula resulting from sutures placed during operative repair of the gunshot wound 6 years previously. DISCUSSION
An aortoesophageal fistula is an abnormal communication between the esophagus and the aorta that allows the highpressure aortic blood to enter the esophagus. The most common causes of aortoesophageal fistulas are thoracic aortic aneurysms, foreign body ingestions, esophageal malignancy, and postoperative complications.2 Although postoperative complications have been reported to cause aortoesophageal fistulas in more than 20 patients, the majority of these patients have had prior esophagogastrectomies or prosthetic grafts of the aorta.2 Rare reports have documented aortoesophageal fistulas at sutures lines not involving prosthetic grafts. These patients usually had congenital anomalies of the great vessels such as coarctation of the aorta or aortic ring anomalies.3,4 Trauma infrequently results in aortoesophageal fistulas.2 Two cases related to gunshot wounds occurred within weeks of the injury.5 Our case is unique in that the patient presented 6 years after aortic injury from a gunshot wound with a fistulous connection at a suture site. Chiari described the aortoesophageal syndrome as a painful esophageal injury followed by an asymptomatic interval, then a signal hemorrhage followed by exsanguination hours to days later.6 The triad of midthoracic pain, sentinel arterial hemorrhage, and exsanguination after a symptomfree interval has been termed Chiari’s triad.7 One review of all cases of aortoesophageal fistulas reported in a 30-year period found that midthoracic pain is present in 59% of patients, dysphagia in 45%, and a sentinel hemorrhage in 65%.2 Overall 45% of patients met all the criteria in Chiari’s triad. Our patient had upper abdominal pain, a sentinel hemorrhage, and exsanguination after a symptom-free interval. Aortoesophageal fistulas typically are fatal. Rapid identification and definitive treatment are necessary to increase the likelihood of survival. The bright red bleeding of the oxygenated arterial blood characteristically distinguishes the bleeding of aortoesophageal fistulas from that of esophageal varices, the most common cause of massive upper gastrointestinal bleeding. Other causes of upper gastrointestinal tract bleeding do not produce the volume of bleeding commonly seen with aortoesophageal fistulas. As a result, diagnosis often can be made on clinical grounds alone, with diagnostic testing performed only if time allows. Diagnostic tests used to confirm the presence of an aortoesophageal fistula have limitations. Endoscopy of the upper gastrointestinal tract may reveal the fistula, but is better able to exclude the other more common causes of bleed-
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ing.2,8 Classic endoscopic findings include direct visualization of pulsatile blood, as was observed in our patient, or a pulsatile submucosal mass with adherent clot. 9-12 The esophageal mucosa may appear blue-gray as a result of submucosal hematoma. 10,11,13,14 In 1 case, the aortic graft could be visualized through the esophageal lumen.15 Aortography is believed by some to be the best procedure to demonstrate an aortoesophageal fistula; however, failure to diagnose the condition may occur during the symptom-free interval because of transient clot formation.9,10,13,16 Barium esophagrams have a low sensitivity, but may be of limited use in patients with thoracic aneurysms.2,17,18 Ultrasonography may reveal a false aneurysm. Computed tomography has been useful in patients with graft infections as multiple gas collections throughout an area of heterogeneous soft tissue density may be demonstrated surrounding the graft or within the aorta.19,20 Dynamic computed tomography or magnetic resonance imaging may also reveal the leak or a dissection.21-23 Operative repair of an aortoesophageal fistula is necessary as no survivors have been reported with nonsurgical management. Circulatory support with volume replacement and transfusion of blood products is essential in patients with active hemorrhage. Correction of coagulopathic and electrolyte abnormalities is indicated. Direct pressure on fistulas from Sengstaken-Blakemore tubes has provided temporary control of the exsanguinating hemorrhage.24,25 Other measures such as radiographic embolization may be useful as a temporizing measure; however, delayed exsanguination after embolization can occur.26 While awaiting operative intervention, broad-spectrum antibiotics should be administered as the esophageal flora may invade the mediastinum or the aorta.21 With rapid diagnosis and surgical treatment, longterm survival is possible.2,5,14,22,27 REFERENCES 1. Cutler JA, Mendeloff AI: Upper gastrointestinal bleeding, nature and magnitude of the problem in the US. Dig Dis Sci 1981;26 suppl:90-96. 2. Hollander JE, Quick G: A comprehensive review of the literature. Am J Med 1991;91:279286. 3. Bigge T, Rothnie NG: Aorto-oesophageal fistula: A late complication of a resected coarctation. Br J Surg 1974;61:545-546. 4. Yonago RH, Iben AB, Mark JB: Aortic bypass in the management of aortoesophageal fistula. Ann Thorac Surg 1969;7:235-237. 5. Kennedy FR, Cornwell ED, Camel J, et al: Aortoesophageal fistula due to gunshot wounds: Report of two cases with one survivor. J Trauma 1995;38:971-974. 6. Sloop RD, Thompson JC: Aorto-esophageal fistula: Report of a case and review of the literature. Gastroenterology 1967;53:768-777. 7. Carter R, Mulder GA, Snyder EN Jr, et al: Aortoesophageal fistula. Am J Surg 1978;136:2630. 8. Jones AW, Kirk RS, Bloor K: The association between aneurysm of the abdominal aorta and peptic ulceration. Gut 1970;11;679-689.
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9. Han SY, Jander HP, Ho KJ: Aortoesophageal fistula. South Med J 1981;74:1260-1262. 10. Khawaja FI, Varindani MK: Aortoesophageal fistula: Review of clinical radiographic, and endoscopic features. J Clin Gastroenterol 1987;9:342-344. 11. Baker BH, Baker MS, Van der Reies L, et al: Endoscopy in the diagnosis of aortoduodenal fistula. Gastrointest Endosc 1977;24:35-37. 12. Kirchgatterer A, Punzengruber C, et al: A rare case of gastrointestinal hemorrhage: Aortoesophageal fistula following repair of aortic dissection. Endoscopy 1997;29:137-138. 13. Sinar DR, DeMaria A, Kataria YP, et al: Aortic aneurysm eroding the esophagus: Case report and review. Am J Dig Dis 1977;22:252-254. 14. Pipinos II, Reddy DJ: Secondary aortoesophageal fistula. J Vasc Surg 1997;26:144-149.
Reprint no. 47/1/92995 Address for reprints: Judd E Hollander, MD Department of Emergency Medicine Ground Floor, Silverstein Hospital of the University of Pennsylvania 3400 Spruce Street Philadelphia, PA 19104-4283 215-662-2767
15. Wickstrom PH, Streitz JM, Erickson RV, et al: Repair of aortoesophageal fistula after aortic grafting. Ann Thorac Surg 1997;64:253-255. 16. Baker MS, Baker BH: Aortoesophageal fistula. South Med J 1982;75:770-771. 17. Naschitz JE, Bassan H, Lazarov N, et al: Upper gastrointestinal bleeding, aneurismatic dilatation of the thoracic aorta and filling defect on the esophagram: A diagnostic clinical triad suggesting aortoesophageal fistula. Radiologe 1982;22:283-285. 18. Baron RL, Koehler RE, Gutierrez FR, et al: Clinical and radiographic manifestations of aortoesophageal fistulas. Radiology 1981;141:599-605. 19. Tierney LM Jr, Wall SD, Jacobs RA: Aortoesophageal fistula after perigraft abscess with characteristic CT findings. J Clin Gastroenterol 1984;6:535-537. 20. Kay D, Kalmar JA: Computerized tomographic evaluation of aortic prosthetic graft complications. South Med J 1985;78:296-298. 21. Sosnowik D, Greenberg R, Bank S, et al: Aortoesophageal fistula: Early and late endoscopic features. Am J Gastroenterol 1988;83:1401-1404. 22. Wang N, Sparks SR, Bailey LL: Staged repair using omentum for posttraumatic aortoesophageal fistula. Ann Thorac Surg 1994;58:557-559. 23. Molina PL, Strobl PW, Burstain JM: Aortoesophageal fistula secondary to mycotic aneurym of the descending thoracic aorta: CT demonstration. J Comput Assist Tomogr 1995;19:309-311. 24. Magnussen I, Notander A, Rieger A, et al: Massive hematemesis due to an aortoesophageal fistula [case report]. Acta Chir Scand 1987;153:317-319. 25. Yamada T, Sato H, Seki M, et al: Successful salvage of aortoesophageal fistula caused by a fish bone. Ann Thorac Surg 1996;61:1843-1845. 26. Reedy FM: Embolization of aortoesophageal fistula: A new therapeutic approach [letter]. J Vasc Surg 1988;8:349-350. 27. Luketich JD, Sommers KE, Griffith BP, et al: Successful management of secondary aortoesophageal fistula. Ann Thorac Surg 1996;62:1852-1854.
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