Endoscopic Diagnosis of a Clinically SilentAortoesophageal Fistula: Case Report andReview 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 duringelective upper endoscopy. The patient had previously undergone repair of a descending thoracicaortic aneurysm with a Dacron interposition graft. Esophagoscopy 2 months after the aneurysmrepair demonstrated a large mid-esophageal erosion with visualization of the aortic graft at thebase. The aortoesophageal fistula had been clinically silent to this point. During preparation forsurgery the patient developed large-volume esophageal hemorrhage and died following at-tempted endovascular repair of the fistula. A review of the literature on the diagnosis andsurgical 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 ero-

sion of a prosthetic graft or suture line.1 Aort-

oesophageal fistula (AEF) is a rare variant of this

disease process that most commonly presents with

exsanguinating upper gastrointestinal (GI) hemor-

rhage. 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 sac-

cular aneurysm of the descending thoracic aorta with

compression of adjacent mediastinal structures (Fig. 1).

Esophagoscopy and bronchoscopy demonstrated extrin-

sic compression of the mid-esophagus and trachea but no

luminal lesions. She underwent repair of the thoracic

aneurysm under hypothermic circulatory arrest, with

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 hospitali-

zation. 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 ero-

sive 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), confirm-

ing 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-vol-

ume hematemesis and was prepared for immediate op-

erative repair.

Considering the patient’s debilitated condition and

previous thoracic surgery, it was decided to attempt

endovascular occlusion of the fistulous connection, fol-

lowed 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 interpo-

sition 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

Department of Surgery, Madigan Army Medical Center, Fort Lewis,WA, USA.

Presented at the 30th Annual Meeting of the Military Society forVascular Surgery, Bethesda, MD, December 5, 2002.

Correspondence to: Philip Mullenix, MD, Department of Surgery,Madigan Army Medical Center, Attn: MCHJ-SGY, Tacoma, WA 98431-1100, USA, E-mail: philip.mullenix@nw.amedd.army.mil

Ann Vasc Surg 2004; 18: 352-356DOI: 10.1007/s10016-004-0027-4� Annals of Vascular Surgery Inc.Published online: 21 April 2004

352

unsuccessful because of dense adhesions from the prior

thoracic surgery and mediastinitis.

DISCUSSION

Aortoenteric fistula is a pathologic connection be-

tween the aorta and GI tract which can occur an-

ywhere from the esophagus to the colon. The most

commonly involved area of the GI tract is the du-

odenum, 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 aneu-

rysm, resulting in pressure necrosis and fistuliza-

tion 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 Fis-

tulae involving the suture lines may be caused by

low-grade prosthetic infection with resultant

pseudoaneurysm and fistula formation.4 Alterna-

tively, 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 demon-

strated hemorrhage into the esophagus in up to

22% of ruptured thoracic aneurysms.7 Other pri-

mary causes include malignancy, congenital

Fig. 1. Contrast-enhanced chest CT demonstrates a sac-

cular aneurysm of the descending aorta with contained

rupture compressing the esophagus (arrow).

Fig. 2. Esophageal endoscopy shows a large esophageal

ulceration with the prosthetic aortic graft visible at the

base (arrow).

Vol. 18, No. 3, 2004 Case reports 353

anomalies, and reflux esophagitis.8,9 Secondary

causes include foreign body ingestion, trauma, in-

strumentation 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 de-

scribed the classic clinical triad of thoracic pain and

initial sentinel hemorrhage, followed by exsangu-

ination. Most patients with both primary and sec-

ondary 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 ab-

normal 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 fist-

ulous 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 esopha-

gram can be as useful as aortography in the diag-

nostic 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 endo-

scopic 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 overly-

ing clot, or active arterial bleeding.19 This case

represents the first report of endoscopic visualiza-

tion of the actual aortic graft through the esopha-

geal wall. Biopsy of the esophageal lesion in these

situations is contraindicated and has been associ-

ated 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.

AEF is a surgical disease and there have been no

reported survivors of nonoperative management.

Preoperative preparation should focus on correct-

ing any coagulopathy, administration of broad-

spectrum antibiotics, and blood pressure control,

but should not delay surgery. An esophageal bal-

loon 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 contami-

nation, 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 compro-

mised mediastinal tissue should be performed and

wide mediastinal drainage employed.

A variety of techniques have been used to ad-

dress 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 vascu-

larized tissue such as pleura, intercostal muscle, or

omentum. Esophageal resection is the most defin-

itive procedure, and reconstruction can be per-

formed immediately or in a delayed fashion.

Esophageal exclusion with distal transection or li-

gation and proximal diversion (cervical esopha-

gostomy) is another alternative that can provide

rapid control of the esophageal perforation and

resultant mediastinitis.

Oliva et al.21 reported the first successful endo-

vascular repair of a primary AEF in 1997. Aortog-

raphy 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 intrave-

nous 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 re-

quires the availability of highly specialized equip-

ment 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-

354 Martin et al. Annals of Vascular Surgery

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 intraop-

erative 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 resec-

tion, mortality was 25% with immediate esophageal

reconstruction, compared to 13% if reconstruction

was delayed.

AEF remains a diagnostic and therapeutic chal-

lenge. 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 resec-

tion has been associated with the lowest reported

mortality rates among the ‘‘traditional’’ repair op-

tions. Primary repair of the aorta and/or esophagus

may be attempted in select cases with minimal

contamination but is associated with a higher

overall mortality and morbidity. Placing a vascu-

larized flap between the aortic graft and esophagus

should be considered and could possibly have pre-

vented 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 under-

scores the concept that the only chance for survival

from AEF is prompt diagnosis and immediate sur-

gical repair.

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356 Martin et al. Annals of Vascular Surgery