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Endoscopic Diagnosis of a Clinically Silent Aortoesophageal Fistula: Case Report and Review of the Literature
Endoscopic Diagnosis of a Clinically Silent Aortoesophageal Fistula: Case Report and Review of the Literature Matthew Martin, MD, Scott Steele, MD, Philip Mullenix, MD, Mohamad Haque, MD, and Charles Andersen, MD, FACS, Fort Lewis, Washington
We report a rare case of a secondary aortoesophageal fistula discovered incidentally during elective upper endoscopy. The patient had previously undergone repair of a descending thoracic aortic aneurysm with a Dacron interposition graft. Esophagoscopy 2 months after the aneurysm repair demonstrated a large mid-esophageal erosion with visualization of the aortic graft at the base. The aortoesophageal fistula had been clinically silent to this point. During preparation for surgery the patient developed large-volume esophageal hemorrhage and died following attempted endovascular repair of the fistula. A review of the literature on the diagnosis and surgical management of aortoesophageal fistula is presented.
Aortoenteric fistulae are uncommon and often fatal complications of aneurysmal disease and aneurysm repair. They most commonly result from aneurysm rupture into an adjacent viscus or progressive erosion of a prosthetic graft or suture line.1 Aortoesophageal fistula (AEF) is a rare variant of this disease process that most commonly presents with exsanguinating upper gastrointestinal (GI) hemorrhage. We present a case of a clinically silent AEF diagnosed by upper endoscopy.
CASE REPORT A 71-year-old female presented with acute onset of chest pain, nausea, and emesis. A chest film demonstrated widening of the mediastinum with aortic calcifications. Chest computed tomography (CT) revealed a large saccular aneurysm of the descending thoracic aorta with compression of adjacent mediastinal structures (Fig. 1). Esophagoscopy and bronchoscopy demonstrated extrinsic compression of the mid-esophagus and trachea but no luminal lesions. She underwent repair of the thoracic aneurysm under hypothermic circulatory arrest, with
Department of Surgery, Madigan Army Medical Center, Fort Lewis, WA, USA. Presented at the 30th Annual Meeting of the Military Society for Vascular Surgery, Bethesda, MD, December 5, 2002. Correspondence to: Philip Mullenix, MD, Department of Surgery, Madigan Army Medical Center, Attn: MCHJ-SGY, Tacoma, WA 984311100, USA, E-mail: [email protected] Ann Vasc Surg 2004; 18: 352-356 DOI: 10.1007/s10016-004-0027-4 Ó Annals of Vascular Surgery Inc. Published online: 21 April 2004 352
placement of an in situ prosthetic interposition graft. Her postoperative course was complicated by respiratory failure, pneumonia, and diverticulitis, but she recovered well and was discharged home after a 3-week hospitalization. One month after discharge the patient was seen in the gastroenterology clinic for complaints of persistent dysphagia and atypical chest pain. An elective upper endoscopy was performed, which demonstrated an erosive lesion in the mid-esophagus with a white base (Fig. 2). This was initially interpreted by the endoscopist as a healing esophageal ulcer with fibrinous deposition. On closer inspection of the esophageal lesion, there was white material at the base which was noted to be the wall of the aortic interposition graft (Fig. 2, arrow), confirming the diagnosis of AEF. Prolene suture material was visualized at the base of the lesion, confirming that the fistula originated from the distal aortic graft suture line. The patient subsequently had an episode of small-volume hematemesis and was prepared for immediate operative repair. Considering the patient’s debilitated condition and previous thoracic surgery, it was decided to attempt endovascular occlusion of the fistulous connection, followed by esophageal exclusion. While being transported to the operating room, the patient had several additional episodes of self-limited hematemesis. In the operating room, vascular access was obtained through exposure of the left common femoral artery while a second team constructed a covered stent graft. The thoracic interposition graft was visualized on aortography but there was no evidence of contrast extravasation into the esophagus. The patient then developed large-volume, bright red hematemesis and ventricular fibrillation before the stent graft could be deployed. Placement of an esophageal balloon catheter failed to tamponade the hemorrhage and the patient died of exsanguination. Rapid control of the aorta was attempted by median sternotomy but was
Vol. 18, No. 3, 2004
Case reports 353
Fig. 2. Esophageal endoscopy shows a large esophageal ulceration with the prosthetic aortic graft visible at the base (arrow).
Fig. 1. Contrast-enhanced chest CT demonstrates a saccular aneurysm of the descending aorta with contained rupture compressing the esophagus (arrow).
unsuccessful because of dense adhesions from the prior thoracic surgery and mediastinitis.
DISCUSSION Aortoenteric fistula is a pathologic connection between the aorta and GI tract which can occur anywhere from the esophagus to the colon. The most
commonly involved area of the GI tract is the duodenum, with a reported incidence of 0.9 to 4% following abdominal aortic reconstruction.2,3 The exact pathogenesis of these lesions is unknown, but there are several proposed mechanisms. Primary fistulae are thought to be secondary to progressive enlargement and inflammation of an aortic aneurysm, resulting in pressure necrosis and fistulization to the GI tract. Secondary fistulae usually occur following aortic repair with a prosthetic graft and subsequent erosion of the body of the graft or a suture line into the GI tract. Chronic pulsation of the prosthetic graft against an adherent viscus is felt to be the cause of graft body-enteric fistulae.1 Fistulae involving the suture lines may be caused by low-grade prosthetic infection with resultant pseudoaneurysm and fistula formation.4 Alternatively, DeWeese and Fry5 have proposed that GI tract erosion is the primary event leading to abscess formation and fistulization. AEF is a rare and usually fatal source of upper GI bleeding. They can be categorized as primary or secondary according to their underlying etiology. The most common cause of primary AEF is rupture of a thoracic aortic aneurysm, which accounts for 75% of all cases.6 Autopsy series have demonstrated hemorrhage into the esophagus in up to 22% of ruptured thoracic aneurysms.7 Other primary causes include malignancy, congenital
354 Martin et al.
anomalies, and reflux esophagitis.8,9 Secondary causes include foreign body ingestion, trauma, instrumentation and, as in this case, graft-esophageal fistula following thoracic aneurysm repair.10 The presentation of both primary and secondary AEF can be highly variable. In 1914, Chiari11 described the classic clinical triad of thoracic pain and initial sentinel hemorrhage, followed by exsanguination. Most patients with both primary and secondary fistulae present with GI bleeding (96%) and 76% experience a herald or sentinel bleed. Other less common signs and symptoms reported with primary AEF include chest pain (45%), dysphagia (41%), sepsis (21%), and back pain (18%).12 The time interval from sentinel bleed to exsanguination can vary from hours to days.13 The need for a high index of suspicion coupled with a rapid evaluation is underscored by the fact that the large majority of AEF are diagnosed postmortem. Timely diagnosis of AEF usually involves one or a combination of imaging studies. In a series of 78 primary AEF, the chest X-ray was reported as abnormal in only 32%.12 CT is rarely diagnostic but can easily demonstrate the aortic aneurysm and any adjacent abscess or inflammatory process.14 Aortography can delineate the vascular anatomy but visualization of the fistulous connection may be limited by low flow or clot formation.15 If the fistulous connection is identified during aortography, embolization has been reported to temporarily tamponade the hemorrhage and may serve as a bridge to the operating room.16 Barium esophagram can be as useful as aortography in the diagnostic evaluation. Findings on an esophagram suggestive of AEF include flow of contrast into the aorta, extrinsic esophageal compression, and/or deviation of the esophagus anteriorly and to the right.17 The most sensitive and specific diagnostic study appears to be esophageal endoscopy. Early endoscopic findings in both primary and secondary AEF include normal esophageal mucosa with external compression, a traction diverticulum, or small mucosal erosions.18 More commonly, they present with late endoscopic findings that include mucosal necrosis, a pulsatile submucosal mass with overlying clot, or active arterial bleeding.19 This case represents the first report of endoscopic visualization of the actual aortic graft through the esophageal wall. Biopsy of the esophageal lesion in these situations is contraindicated and has been associated with rupture of the AEF.17 If the diagnosis is made endoscopically, the procedure should be terminated immediately and the patient should be prepared for the operating room.
Annals of Vascular Surgery
AEF is a surgical disease and there have been no reported survivors of nonoperative management. Preoperative preparation should focus on correcting any coagulopathy, administration of broadspectrum antibiotics, and blood pressure control, but should not delay surgery. An esophageal balloon catheter (Sengstaken-Blakemore) should be kept immediately available and may tamponade the fistula if bleeding begins before surgery.20 Surgical repair is most commonly done via a left thoracotomy and must address both the aortic rupture and the esophageal perforation. Options for the aortic repair include primary suture repair if the defect is small and there is minimal contamination, in situ aortic replacement with prosthetic graft, or extraanatomic bypass. If a prosthetic graft is being used to repair an aortic aneurysm, the aneurysm wall should be considered contaminated by the fistula and not used to wrap the graft. In every case a thorough debridement of all compromised mediastinal tissue should be performed and wide mediastinal drainage employed. A variety of techniques have been used to address the esophageal perforation and should be tailored to the findings at thoracotomy. Primary repair of the esophagus may be attempted if there is a small defect with minimal contamination, but this procedure carries the highest risk of continued leak. The repair should be reinforced with vascularized tissue such as pleura, intercostal muscle, or omentum. Esophageal resection is the most definitive procedure, and reconstruction can be performed immediately or in a delayed fashion. Esophageal exclusion with distal transection or ligation and proximal diversion (cervical esophagostomy) is another alternative that can provide rapid control of the esophageal perforation and resultant mediastinitis. Oliva et al.21 reported the first successful endovascular repair of a primary AEF in 1997. Aortography and intravascular ultrasound were used to guide the deployment of a covered stent graft in the thoracic aorta. The esophageal perforation was managed nonoperatively with prolonged intravenous antibiotics and the patient was alive and well at 13-month follow-up. There is little experience to date with the endovascular approach, and it requires the availability of highly specialized equipment and technical expertise. However, the almost universal presence of severe comorbid disease and critical illness in this patient population makes a minimally invasive approach an attractive option. A review of the English-language literature on both primary and secondary aortic fistulae yielded 44 cases of initially successful AEF re-
Vol. 18, No. 3, 2004
Case reports 355
Table I. AEF mortality by type of repair Procedure
Aorta In situ Primary EAB Endovascular Esophagus Primary Resection Reconstruction Immediate Delayed
N
90-day mortality [n (%)]
10 8 4 6
2 5 3 1
10 12
6 (60) 2 (17)
4 8
1 (25) 1 (13)
(20) (63) (75) (17)
may be attempted in select cases with minimal contamination but is associated with a higher overall mortality and morbidity. Placing a vascularized flap between the aortic graft and esophagus should be considered and could possibly have prevented the fistula formation in this patient. There is increasing experience being reported with the endovascular approach to AEF and it appears to offer an effective and less invasive approach to this difficult problem. The rapid onset of hemorrhage and clinical deterioration seen in this case underscores the concept that the only chance for survival from AEF is prompt diagnosis and immediate surgical repair.
EAB, extraanatomic bypass.
REFERENCES
pair.6,7,10,12,13,15,19,21-41 Adequate survival data were available for 35 patients. Eighteen patients died within 6 months, with a mean survival of 46 days (range 1-182). Seventeen patients survived greater than 6 months, with a mean survival of 15 months (range 9-30). The most common causes of intraoperative and immediate perioperative mortality were massive hemorrhage and sepsis with multiple organ failure. Late (>6 months) mortalities were due to coexisting pulmonary and/or cardiac disease. We then analyzed 90-day mortality independently by type of aortic repair and type of esophageal repair, if reported (Table I). An in situ aortic repair with prosthetic graft was associated with a 20% mortality rate, compared to 63% for primary repair and 75% for extraanatomic bypass. Of note, all three deaths in the extraanatomic bypass group were secondary to proximal anastamotic failure. Endovascular repair was associated with a 17% mortality rate among the six reported cases to date, all of which were primary fistulae.21,22,38-42 Management of the esophageal lesion with primary repair was associated with a 60% mortality, compared to 17% with esophageal resection or exclusion. Following esophageal resection, mortality was 25% with immediate esophageal reconstruction, compared to 13% if reconstruction was delayed. AEF remains a diagnostic and therapeutic challenge. Esophageal endoscopy is the diagnostic study of choice but should be performed carefully and the procedure terminated if a fistula is identified. To our knowledge, this case represents the first published endoscopic images of an intact graft-to-esophageal fistula. There are a variety of repair options, but in situ aortic graft placement with esophageal resection has been associated with the lowest reported mortality rates among the ‘‘traditional’’ repair options. Primary repair of the aorta and/or esophagus
1. Montgomery RS, Wilson SE. The surgical management of aortoenteric fistulas. Surg. Clin. 1996;76:1147-1157. 2. Elliott JP, Smith RF, Szilagyi DE. Aortoenteric and paraprosthetic-enteric fistulas. Am. J. Surg. 1974;108:479490. 3. Donovan TJ, Bucknam CA. Aortoenteric fistula. Arch. Surg. 1967;95:810-820. 4. Busuttil RW, Rees W, Wilson SE, et al. Pathogenesis of aortoduodenal fistula: experimental and clinical correlates. Surgery 1979;85:1-12. 5. DeWeese MS, Fry WJ. Small bowel erosion following aortic resection. J.A.M.A. 1962;179:882-886. 6. Heckstall RL, Hollander JE. Aortoesophageal fistula: recognition and diagnosis in the emergency department. Ann. Emerg. Med. 1998;32:502-505. 7. da Silva ES, Tozzi FL, Otochi JP, et al. Aortoesophageal fistula caused aneurysm of the thoracic aorta: successful surgical treatment, case report, and literature review. J. Vasc. Surg. 1999;30:1150-1157. 8. Sigalet DL, Laberge JM, DiLorenzo M, et al. Aortoesophageal fistula: congenital and acquired causes. J. Pediatr. Surg. 1994;29:1212-1214. 9. Singh AK, Kothawla LK, Karlson KE. Tracheoesophageal and aortoesophageal fistulae complicating corrosive esophagitis. Chest 1976;70:549-551. 10. Pipinos II, Reddy DJ. Secondary aortoesophageal fistulae. Ann. Vasc. Surg. 1999;13:649-652. 11. Chiari H. Ueber Fremdkorpeverletzung des Oesophagus mit Aortenperforation. Berlin Klin. Wochenschr. 1914;51: 7-9. 12. Dossa CD, Pipinos II, Shepard AD, et al. Primary aortoenteric fistula: Part II. Ann. Vasc. Surg. 1994;8:207-211. 13. Amin S, Luketich J, Wald A. Aortoesophageal fistula: case report and review of the literature. Dig. Dis. Sci. 1998; 43:1665-1671. 14. Tierney LM, Wall SD, Jacobs RA. Aortoesophageal fistula after perigraft abscess with characteristic CT findings. J. Clin. Gastroenterol. 1984;6:535-537. 15. Bogey WM, Thomas JH, Hermreck AS. Aortoesophageal fistula: report of a successfully managed case and review of the literature. J. Vasc. Surg. 1992;16:90-95. 16. Reedy FM. Emoblization of aortoesophageal fistula: a new therapeutic approach. J. Vasc. Surg. 1988;8:349-350. 17. Baron RL, Koehler RE, Gutierrez FR, et al. Clinical and radiographic manifestations of aortoesophageal fistulas. Radiology 1981;141:599-605.
356 Martin et al.
18. Sosnowik D, Greenberg R, Bank S, et al. Aortoesophageal fistula: early and late endoscopic features. Am. J. Gastroenterol. 1988;83:1401-1404. 19. Khawaja FI, Varindani MK. Aortoesophageal fistula: review of clinical, radiographic, and endoscopic features. J. Clin. Gastroenterol. 1987;9:342-344. 20. Baker MS, Baker BH. Aortoesophageal fistula. South. Med. J. 1982;75:770-771. 21. Oliva VL, Bui BT, Leclerc G, et al. Aortoesophageal fistula: repair with transluminal placement of a thoracic aortic stent-graft. J. Vasc. Interv. Radiol. 1997;8:35-38. 22. Maruyama K, Ishiguchi T, Kato K, et al. Stent-graft placement for pseudoaneurysm of the aorta. Radiation Med. 2000;18:177-185. 23. Taniguchi I, Takemoto N, Yamaga T, et al. Primary aortoesophageal fistula secondary to thoracic aneurysm. Jpn. J. Thorac. Cardiovasc. Surg. 2002;50:263-267. 24. Lee OJ, Kim SH. Aortoesophageal fistula associated with tuberculous medistinitis, mimicking esophageal dieulafoy’s disease. J. Korean Med. Sci. 2002;17:266-269. 25. Luketich JD, Sommers E, Griffith BP. Successful management of secondary aortoesophageal fistula. Ann. Thorac. Surg. 1996;62:1852-1854. 26. Madan AK, Santora TA, DiSesa VJ. Extra-anatomic bypass grafting for aortoesophageal fistula: a logical operation. J. Vasc. Surg. 2000;32:1030-1033. 27. Coselli JS, Crawford ES. Primary aortoesophageal fistula from aortic aneurysm: successful surgical treatment by use of pedicle omental graft. J. Vasc. Surg. 1990;12:269-277. 28. Lui RC, Johnson FE, Horovitz JH, et al. Aortoesohageal fistula: case report and literature review. J. Vasc. Surg. 1987;6:379-382. 29. Debras B, Enon B, Kanane O, et al. Successful management of an aortoesophageal fistula using a cryopreserved arterial allograft. Ann. Vasc. Surg. 1996;10:292-296. 30. Wickstrom PH, Streitz JM, Erickson RV, et al. Repair of aortoesophageal fistula after aortic grafting. Ann. Thorac. Surg. 1997;64:253-255.
Annals of Vascular Surgery
31. von Oppell UO, de Groot M, Thierfelder C, et al. Successful management of aortoesophageal fistula due to thoracic aortic aneurysm. Ann. Thorac. Surg. 1991;52: 1168-1170. 32. Cronen P, Snow N, Nightingale D. Aortoesophageal fistula secondary to reflux esophagitis. Ann. Thorac. Surg. 1982; 33:78-80. 33. Reardon MJ, Brewer RJ, Le Maire SA, et al. Surgical management of primary aortoesophageal fistula secondary to thoracic aneurysm. Ann. Thorac. Surg. 2000;69:967970. 34. Wu MH, Lai WW. Aortoesophageal fistula induced by foreign bodies. Ann. Thorac. Surg. 1992;54:155-156. 35. 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. 36. Wong RS, Champlin A, Temes T, et al. Aortoesophageal fistula after repair of descending aortic dissection. Ann. Thorac. Surg. 1996;62:588-590. 37. Chughtai TS, Sheiner NM. Successful repair of aortoesophageal fistula secondary to traumatic pseudoaneurysm. Ann. Thorac. Surg. 1998;66:936-938. 38. Leobon B, Roux D, Rousseau H, et al. Endovascular treatment of thoracic aortic fistulas. Ann. Thorac. Surg. 2002; 74:247-249. 39. Van Doorn RC, Reekers J, de Mol BA, et al. Aortoesophageal fistula secondary to mycotic thoracic aortic aneurysm: endovascular repair and transhiatal esophagectomy. J. Endovasc. Ther. 2002;9:212-217. 40. Bond SE, McGuinness CL, Reidy JF, et al. Repair of secondary aortoesophageal fistula by endoluminal stent-grafting. J. Endovasc. Ther. 2001;8:597-601. 41. Kato N, Tadanori H, Tanaka K, et al. Aortoesophageal fistula-relief of massive hematemesis with an endovascular stent-graft. Eur. J. Radiol. 2000;34:6366. 42. Carter R, Mulder GA, Snyder EN, et al. Aortoesophageal fistula. Am. J. Surg. 1978;136:26-30.
We report a rare case of a secondary aortoesophageal fistula discovered incidentally during elective upper endoscopy. The patient had previously undergone repair of a descending thoracic aortic aneurysm with a Dacron interposition graft. Esophagoscopy 2 months after the aneurysm repair demonstrated a large mid-esophageal erosion with visualization of the aortic graft at the base. The aortoesophageal fistula had been clinically silent to this point. During preparation for surgery the patient developed large-volume esophageal hemorrhage and died following attempted endovascular repair of the fistula. A review of the literature on the diagnosis and surgical management of aortoesophageal fistula is presented.
Aortoenteric fistulae are uncommon and often fatal complications of aneurysmal disease and aneurysm repair. They most commonly result from aneurysm rupture into an adjacent viscus or progressive erosion of a prosthetic graft or suture line.1 Aortoesophageal fistula (AEF) is a rare variant of this disease process that most commonly presents with exsanguinating upper gastrointestinal (GI) hemorrhage. We present a case of a clinically silent AEF diagnosed by upper endoscopy.
CASE REPORT A 71-year-old female presented with acute onset of chest pain, nausea, and emesis. A chest film demonstrated widening of the mediastinum with aortic calcifications. Chest computed tomography (CT) revealed a large saccular aneurysm of the descending thoracic aorta with compression of adjacent mediastinal structures (Fig. 1). Esophagoscopy and bronchoscopy demonstrated extrinsic compression of the mid-esophagus and trachea but no luminal lesions. She underwent repair of the thoracic aneurysm under hypothermic circulatory arrest, with
Department of Surgery, Madigan Army Medical Center, Fort Lewis, WA, USA. Presented at the 30th Annual Meeting of the Military Society for Vascular Surgery, Bethesda, MD, December 5, 2002. Correspondence to: Philip Mullenix, MD, Department of Surgery, Madigan Army Medical Center, Attn: MCHJ-SGY, Tacoma, WA 984311100, USA, E-mail: [email protected] Ann Vasc Surg 2004; 18: 352-356 DOI: 10.1007/s10016-004-0027-4 Ó Annals of Vascular Surgery Inc. Published online: 21 April 2004 352
placement of an in situ prosthetic interposition graft. Her postoperative course was complicated by respiratory failure, pneumonia, and diverticulitis, but she recovered well and was discharged home after a 3-week hospitalization. One month after discharge the patient was seen in the gastroenterology clinic for complaints of persistent dysphagia and atypical chest pain. An elective upper endoscopy was performed, which demonstrated an erosive lesion in the mid-esophagus with a white base (Fig. 2). This was initially interpreted by the endoscopist as a healing esophageal ulcer with fibrinous deposition. On closer inspection of the esophageal lesion, there was white material at the base which was noted to be the wall of the aortic interposition graft (Fig. 2, arrow), confirming the diagnosis of AEF. Prolene suture material was visualized at the base of the lesion, confirming that the fistula originated from the distal aortic graft suture line. The patient subsequently had an episode of small-volume hematemesis and was prepared for immediate operative repair. Considering the patient’s debilitated condition and previous thoracic surgery, it was decided to attempt endovascular occlusion of the fistulous connection, followed by esophageal exclusion. While being transported to the operating room, the patient had several additional episodes of self-limited hematemesis. In the operating room, vascular access was obtained through exposure of the left common femoral artery while a second team constructed a covered stent graft. The thoracic interposition graft was visualized on aortography but there was no evidence of contrast extravasation into the esophagus. The patient then developed large-volume, bright red hematemesis and ventricular fibrillation before the stent graft could be deployed. Placement of an esophageal balloon catheter failed to tamponade the hemorrhage and the patient died of exsanguination. Rapid control of the aorta was attempted by median sternotomy but was
Vol. 18, No. 3, 2004
Case reports 353
Fig. 2. Esophageal endoscopy shows a large esophageal ulceration with the prosthetic aortic graft visible at the base (arrow).
Fig. 1. Contrast-enhanced chest CT demonstrates a saccular aneurysm of the descending aorta with contained rupture compressing the esophagus (arrow).
unsuccessful because of dense adhesions from the prior thoracic surgery and mediastinitis.
DISCUSSION Aortoenteric fistula is a pathologic connection between the aorta and GI tract which can occur anywhere from the esophagus to the colon. The most
commonly involved area of the GI tract is the duodenum, with a reported incidence of 0.9 to 4% following abdominal aortic reconstruction.2,3 The exact pathogenesis of these lesions is unknown, but there are several proposed mechanisms. Primary fistulae are thought to be secondary to progressive enlargement and inflammation of an aortic aneurysm, resulting in pressure necrosis and fistulization to the GI tract. Secondary fistulae usually occur following aortic repair with a prosthetic graft and subsequent erosion of the body of the graft or a suture line into the GI tract. Chronic pulsation of the prosthetic graft against an adherent viscus is felt to be the cause of graft body-enteric fistulae.1 Fistulae involving the suture lines may be caused by low-grade prosthetic infection with resultant pseudoaneurysm and fistula formation.4 Alternatively, DeWeese and Fry5 have proposed that GI tract erosion is the primary event leading to abscess formation and fistulization. AEF is a rare and usually fatal source of upper GI bleeding. They can be categorized as primary or secondary according to their underlying etiology. The most common cause of primary AEF is rupture of a thoracic aortic aneurysm, which accounts for 75% of all cases.6 Autopsy series have demonstrated hemorrhage into the esophagus in up to 22% of ruptured thoracic aneurysms.7 Other primary causes include malignancy, congenital
354 Martin et al.
anomalies, and reflux esophagitis.8,9 Secondary causes include foreign body ingestion, trauma, instrumentation and, as in this case, graft-esophageal fistula following thoracic aneurysm repair.10 The presentation of both primary and secondary AEF can be highly variable. In 1914, Chiari11 described the classic clinical triad of thoracic pain and initial sentinel hemorrhage, followed by exsanguination. Most patients with both primary and secondary fistulae present with GI bleeding (96%) and 76% experience a herald or sentinel bleed. Other less common signs and symptoms reported with primary AEF include chest pain (45%), dysphagia (41%), sepsis (21%), and back pain (18%).12 The time interval from sentinel bleed to exsanguination can vary from hours to days.13 The need for a high index of suspicion coupled with a rapid evaluation is underscored by the fact that the large majority of AEF are diagnosed postmortem. Timely diagnosis of AEF usually involves one or a combination of imaging studies. In a series of 78 primary AEF, the chest X-ray was reported as abnormal in only 32%.12 CT is rarely diagnostic but can easily demonstrate the aortic aneurysm and any adjacent abscess or inflammatory process.14 Aortography can delineate the vascular anatomy but visualization of the fistulous connection may be limited by low flow or clot formation.15 If the fistulous connection is identified during aortography, embolization has been reported to temporarily tamponade the hemorrhage and may serve as a bridge to the operating room.16 Barium esophagram can be as useful as aortography in the diagnostic evaluation. Findings on an esophagram suggestive of AEF include flow of contrast into the aorta, extrinsic esophageal compression, and/or deviation of the esophagus anteriorly and to the right.17 The most sensitive and specific diagnostic study appears to be esophageal endoscopy. Early endoscopic findings in both primary and secondary AEF include normal esophageal mucosa with external compression, a traction diverticulum, or small mucosal erosions.18 More commonly, they present with late endoscopic findings that include mucosal necrosis, a pulsatile submucosal mass with overlying clot, or active arterial bleeding.19 This case represents the first report of endoscopic visualization of the actual aortic graft through the esophageal wall. Biopsy of the esophageal lesion in these situations is contraindicated and has been associated with rupture of the AEF.17 If the diagnosis is made endoscopically, the procedure should be terminated immediately and the patient should be prepared for the operating room.
Annals of Vascular Surgery
AEF is a surgical disease and there have been no reported survivors of nonoperative management. Preoperative preparation should focus on correcting any coagulopathy, administration of broadspectrum antibiotics, and blood pressure control, but should not delay surgery. An esophageal balloon catheter (Sengstaken-Blakemore) should be kept immediately available and may tamponade the fistula if bleeding begins before surgery.20 Surgical repair is most commonly done via a left thoracotomy and must address both the aortic rupture and the esophageal perforation. Options for the aortic repair include primary suture repair if the defect is small and there is minimal contamination, in situ aortic replacement with prosthetic graft, or extraanatomic bypass. If a prosthetic graft is being used to repair an aortic aneurysm, the aneurysm wall should be considered contaminated by the fistula and not used to wrap the graft. In every case a thorough debridement of all compromised mediastinal tissue should be performed and wide mediastinal drainage employed. A variety of techniques have been used to address the esophageal perforation and should be tailored to the findings at thoracotomy. Primary repair of the esophagus may be attempted if there is a small defect with minimal contamination, but this procedure carries the highest risk of continued leak. The repair should be reinforced with vascularized tissue such as pleura, intercostal muscle, or omentum. Esophageal resection is the most definitive procedure, and reconstruction can be performed immediately or in a delayed fashion. Esophageal exclusion with distal transection or ligation and proximal diversion (cervical esophagostomy) is another alternative that can provide rapid control of the esophageal perforation and resultant mediastinitis. Oliva et al.21 reported the first successful endovascular repair of a primary AEF in 1997. Aortography and intravascular ultrasound were used to guide the deployment of a covered stent graft in the thoracic aorta. The esophageal perforation was managed nonoperatively with prolonged intravenous antibiotics and the patient was alive and well at 13-month follow-up. There is little experience to date with the endovascular approach, and it requires the availability of highly specialized equipment and technical expertise. However, the almost universal presence of severe comorbid disease and critical illness in this patient population makes a minimally invasive approach an attractive option. A review of the English-language literature on both primary and secondary aortic fistulae yielded 44 cases of initially successful AEF re-
Vol. 18, No. 3, 2004
Case reports 355
Table I. AEF mortality by type of repair Procedure
Aorta In situ Primary EAB Endovascular Esophagus Primary Resection Reconstruction Immediate Delayed
N
90-day mortality [n (%)]
10 8 4 6
2 5 3 1
10 12
6 (60) 2 (17)
4 8
1 (25) 1 (13)
(20) (63) (75) (17)
may be attempted in select cases with minimal contamination but is associated with a higher overall mortality and morbidity. Placing a vascularized flap between the aortic graft and esophagus should be considered and could possibly have prevented the fistula formation in this patient. There is increasing experience being reported with the endovascular approach to AEF and it appears to offer an effective and less invasive approach to this difficult problem. The rapid onset of hemorrhage and clinical deterioration seen in this case underscores the concept that the only chance for survival from AEF is prompt diagnosis and immediate surgical repair.
EAB, extraanatomic bypass.
REFERENCES
pair.6,7,10,12,13,15,19,21-41 Adequate survival data were available for 35 patients. Eighteen patients died within 6 months, with a mean survival of 46 days (range 1-182). Seventeen patients survived greater than 6 months, with a mean survival of 15 months (range 9-30). The most common causes of intraoperative and immediate perioperative mortality were massive hemorrhage and sepsis with multiple organ failure. Late (>6 months) mortalities were due to coexisting pulmonary and/or cardiac disease. We then analyzed 90-day mortality independently by type of aortic repair and type of esophageal repair, if reported (Table I). An in situ aortic repair with prosthetic graft was associated with a 20% mortality rate, compared to 63% for primary repair and 75% for extraanatomic bypass. Of note, all three deaths in the extraanatomic bypass group were secondary to proximal anastamotic failure. Endovascular repair was associated with a 17% mortality rate among the six reported cases to date, all of which were primary fistulae.21,22,38-42 Management of the esophageal lesion with primary repair was associated with a 60% mortality, compared to 17% with esophageal resection or exclusion. Following esophageal resection, mortality was 25% with immediate esophageal reconstruction, compared to 13% if reconstruction was delayed. AEF remains a diagnostic and therapeutic challenge. Esophageal endoscopy is the diagnostic study of choice but should be performed carefully and the procedure terminated if a fistula is identified. To our knowledge, this case represents the first published endoscopic images of an intact graft-to-esophageal fistula. There are a variety of repair options, but in situ aortic graft placement with esophageal resection has been associated with the lowest reported mortality rates among the ‘‘traditional’’ repair options. Primary repair of the aorta and/or esophagus
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