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Primary aortoenteric fistula in the Emergency Department
The Journal of Emergency Medicine, Vol. 20, No. 1, pp. 25–27, 2001 Copyright © 2001 Elsevier Science Inc. Printed in the USA. All rights reserved 0736-4679/01 $–see front matter
PII S0736-4679(00)00288-2
Clinical Communications
PRIMARY AORTOENTERIC FISTULA IN THE EMERGENCY DEPARTMENT Ferdinando L. Mirarchi,
DO,*
Mark D. Scheatzle,
MD,*
and Ricardo J. Mitre,
MD†
*Department of Emergency Medicine and †Department of Gastroenterology, West Penn Allegheny Health System, Allegheny General Hospital, Pittsburgh, Pennsylvania Reprint Address: Ferdinando L. Mirarchi, DO, Department of Emergency Medicine, Residency Office, Allegheny General Hospital, 320 East North Ave, Pittsburgh, PA 15212-4772
e Abstract—A primary aortoenteric fistula is a rare, lifethreatening cause of gastrointestinal bleeding. Primary aortoenteric fistula results most commonly from an abdominal aortic aneurysm, with the fistula forming most often between the aorta and the third portion of the duodenum. Often, the classic triad of abdominal pain, gastrointestinal bleeding, and pulsatile mass is absent. A heraldic bleed frequently precedes lethal exsanguination from a primary aortoenteric fistula. Patient survival is dependent on prompt diagnosis and emergent therapeutic laparotomy. © 2001 Elsevier Science Inc.
CASE PRESENTATION A 75-year-old man presented to the Emergency Department (ED) complaining of back and right hip pain as well as melena. The past medical history was pertinent for a history of arteriovenous malformations of the small bowel and colon for which he had undergone a right hemicolectomy and oversewing of ileal and jejunal malformations. The patient was discharged from the hospital 7 days previously after an exacerbation of lower GI bleeding. At that time, the patient required multiple transfusions and underwent colonoscopic evaluation and a nuclear scan for bleeding, both of which failed to visualize an active source of bleeding. At the time of his evaluation, the patient complained only of right hip pain. He denied current abdominal or low back pain. Initial vital signs were as follows: temperature of 36.6°C, pulse of 92 beats/min, and blood pressure of 160/90 mmHg. Physical examination demonstrated an elderly man resting comfortably, and in no acute distress. The skin was warm and dry with normal color. The abdomen was soft, nontender, with normal bowel sounds, and without organomegaly or pulsatile mass. The extremities, including the right hip, and the femoral pulses, were normal. The rectal examination revealed dark, heme-positive stool. Immediately following the physical examination, the patient suddenly clutched his abdomen complaining of pain, developed myoclonic activity, and became asystolic. One liter of normal saline by rapid infusion, as well as chest compressions, resulted in a return to a sinus
e Keywords—primary aortoenteric fistula; gastrointestinal bleeding; herald bleed; abdominal aortic aneurysm; lethal exsanguination
INTRODUCTION A primary aortoenteric fistula (PAEF) is a rare, lifethreatening cause of gastrointestinal (GI) bleeding. The classic triad of abdominal pain, GI bleeding, and pulsatile abdominal mass is often absent at the time of presentation, which obscures the diagnosis. Therefore, one must consider the possibility and obtain emergent surgical intervention for survival. We present the case of a 75-year-old man with a history of arteriovenous malformations and prior episodes of GI bleeding, who was found to have a PAEF secondary to an abdominal aortic aneurysm (AAA).
RECEIVED: 27 October 1999; FINAL ACCEPTED: 26 July 2000
SUBMISSION RECEIVED:
13 July 2000; 25
26
F. L. Mirarchi et al.
Figure 1. Noncontrast CT scan of the abdomen remarkable for a hyperdense crescent (white arrows) extending from the aorta. The crescent signifies hemorrhage into the vessel wall but without rupture. A ⴝ aorta, D ⴝ duodenum, I ⴝ inferior vena cava.
Figure 2. Contrast-enhanced CT scan of the abdomen remarkable for a 4.0 cm high infrarenal aortic aneurysm with a bulge to the right but without rupture. A ⴝ aorta, D ⴝ duodenum, I ⴝ inferior vena cava.
tachycardia at a rate of 115 beats/min and a blood pressure of 50 systolic. The patient underwent rapid sequence intubation with etomidate and succinylcholine. A nasogastric tube was placed with aspiration producing 30 cc of bright red blood. After 1.5 L of normal saline, the patient’s blood pressure stabilized at a systolic of 100 mmHg. Diagnostics including a chest X-ray study, electrocardiogram (EKG) and emergent spiral computed tomography (CT) scan of the abdomen were performed. The chest X-ray series was normal and demonstrated endotracheal and nasogastric tubes in adequate position. The electrocardiogram revealed a normal sinus rhythm, nonspecific ST changes, and normal intervals. Because of the sudden onset of profound hypotension with associated pain, which is unusual for a GI bleed, a CT scan of the abdomen (with and without i.v. contrast) was performed to evaluate the patient’s aorta. The CT scan without contrast revealed a dilated infra-renal aorta with a hyperdense crescent to the right. The CT scan with i.v. contrast revealed a 4.0 cm infra-renal AAA with a bulge to the right, but without rupture, and with a question of a small amount of contrast identified in the duodenum (see Figures 1 and 2). On return from CT scan, the patient’s nasogastric tube was reconnected and returned 400 cc of bright red blood.
Additional resuscitation involved the transfusion of 2 units of packed red blood cells. A vascular surgery consult was immediately obtained to further evaluate and treat the AAA with possible fistula. The vascular surgeon elected to obtain an angiogram to differentiate upper GI bleeding versus PAEF. The angiogram revealed a 4.0-cm infrarenal AAA with a penetrating ulcer and a questionable fistula tract. Thus, an emergent exploratory laparotomy was performed confirming an aortoduodenal fistula, which required graft and repair of the duodenal fistula. The patient’s remaining hospital course was uncomplicated, and he was discharged home on the sixth postoperative day.
DISCUSSION A PAEF occurs when a direct communication exists between the aorta and the GI tract. It was first described in 1829 by Sir Astley Cooper (1). In contrast, a secondary aortoenteric fistula arises between a suture line of a vascular graft and the GI tract (4,5). A PAEF is rare, with fewer than 250 cases reported in the literature worldwide as of 1996 (1,3,4). It is often fatal, with a mortality rate of 80 –100% (2,3). These fistula most commonly originate from an AAA, of which 85% are atherosclerotic,
Primary Aortoenteric Fistula in the Emergency Department
and 15% are infectious (Tuberculosis, Syphilis, Salmonella, Staphylococcus, and Mycoses) (2,5,7–9). Rarely, a PAEF is a result of cervical cancer, colon cancer, radiation therapy, diverticulitis, appendicitis, duodenal ulcers, cholecystitis, or foreign body (2,3). The third portion of the duodenum is the most frequent site of fistulazation, followed by the jejunum, ileum, colon, esophagus, and stomach (2,3,5,9,10). Men are at greatest risk (75%) with a mean age of 60 years old (2). In patients with known AAA, the incidence of PAEF is 0.69 –2.36% (3,4,7). Culprit aneurysms can range in size from 3.0 –15 cm (mean 7.0 cm) (2,11). Classically, a triad of symptoms including abdominal pain, GI bleeding, and abdominal mass has been described; however, this is present in only the minority of cases (1,2). Back pain is also present in 50% of presentations (2). Fever has been described with PAEF and should raise concern for its presence in a patient with a known AAA (4). Finally, a “heraldic bleed,” which occurred in our patient, is a well-known event; however, this incidence has not been defined. In this case, the key to the diagnosis was the sudden onset of abdominal pain and profound hypotension, which is a feature that is not typical of GI bleeding. Additionally, this patient had recently been discharged from the hospital after a colonoscopy, and nuclear bleeding scan failed to visualize an active source of bleeding. The Emergency Department work-up and management are critical. They begin with rapid assessment and supportive care as well as considering the possibility of the entity. It is imperative to obtain diagnostic studies promptly as well as to involve the Vascular Surgeon early in the decision and management process. The majority of the literature supports that an abdominal CT scan with i.v. contrast is the diagnostic procedure of choice to diagnose PAEF (2,5,6). Angiography, which is useful in planning the operative approach, rarely demonstrates the actual fistula tract (2,3,5). Esophagogastroduodenoscopy rarely identifies the actual fistula, but can supplement other diagnostic modalities by ruling out other causes of GI bleeding (2– 4). Treatment is surgical and involves emergent exploratory laparotomy with graft repair of the aorta and closure of the fistula tract (3– 6). CONCLUSION Although a PAEF is rare, it should be included in the differential diagnosis of GI bleeding, especially when
27
such bleeding is recurrent. With mortality rates of 80 – 100%, the key to its diagnosis and successful management is to consider the diagnosis, rapid assessment to confirm its presence, and immediate surgery. PAEF should be considered in the differential diagnosis of any patient with recurrent GI bleeding, massive exsanguination, known AAA and GI bleeding or known AAA and unexplained fever. CT scan is the diagnostic procedure of choice and treatment is immediate surgery.
Acknowledgments—We would like to formally acknowledge Dennis P. Hanlon, MD, from the Department of Emergency Medicine for his clinical support, and Anthony R. Lupetin, MD, from the Department of Radiological Services, for his technical support.
REFERENCES 1. Kaushik SP, Cowlishaw JL. Primary aortoenteric fistula. Aust NZ J Med 1998;28:471–2. 2. Berry SM, Fisher JE. Classification and pathophysiology of enterocutaneous fistulas. Surg Clin North Am 1996;76:1009 –18. 3. Brown PW, Sailors DM, Headrick JR, et al. Primary aortoduodenal fistula: a case report. Am Surg 1999;65:139 – 41. 4. Voorhoeve R, Moll FL, de Letter JAM, et al. Primary aortoenteric fistula: report of eight new cases and review of the literature. Ann Vasc Surg 1996;10:40 – 8. 5. Bernhard V. Aortoenteric fistulae. In: Rutherford RS., ed. Vascular surgery, 4th edn. Philadelphia: WB Saunders Company; 1995: 611–18. 6. Voorhoeve R, Moll FL, Bast TJ. The primary aortoenteric fistula in the Netherlands—the unpublished cases. Eur J Vasc EndoVasc Surg 1996;11:429 –31. 7. Hickey NC, Downing R, Hammer JD, et al. Abdominal aortic aneurysms complicated by spontaneous ileocaval or duodenal fistulae. J Cardiovasc Surg 1991;32:181–5. 8. Nagy SW, Marshall JB. Aortoenteric fistula. Postgrad Med 1993; 93:211–22. 9. Peck JJ, Eidemiller LR. Aortoenteric fistulas. Arch Surg 1992;127: 1191– 4. 10. Rhim JA, Rubin RA, Katzka DA, et al. Primary aortoduodenal fistula: a unique presentation of a pseudoaneurysm associated with cystic medial necrosis. Am J Gastroenterol 1993;88:447–50. 11. Calligaro KD, Bergen WS, Saverese RP, et al. Primary aortoduodenal fistula due to septic aortitis. J Cardivasc Surg 1992;33: 192– 8. 12. Vlassopoulos D, Noussias C, Dagounaki P, et al. Aortoenteric fistula: an unusual cause of gastointestinal bleeding in a hemodialysis patient. Nephrol Dial Transplant 1997;12:2171–3. 13. Antinori CH, Andrew CT, John S, et al. The many faces of aortoenteric fistulas. Am Surg 1996;62:344 –9.
PII S0736-4679(00)00288-2
Clinical Communications
PRIMARY AORTOENTERIC FISTULA IN THE EMERGENCY DEPARTMENT Ferdinando L. Mirarchi,
DO,*
Mark D. Scheatzle,
MD,*
and Ricardo J. Mitre,
MD†
*Department of Emergency Medicine and †Department of Gastroenterology, West Penn Allegheny Health System, Allegheny General Hospital, Pittsburgh, Pennsylvania Reprint Address: Ferdinando L. Mirarchi, DO, Department of Emergency Medicine, Residency Office, Allegheny General Hospital, 320 East North Ave, Pittsburgh, PA 15212-4772
e Abstract—A primary aortoenteric fistula is a rare, lifethreatening cause of gastrointestinal bleeding. Primary aortoenteric fistula results most commonly from an abdominal aortic aneurysm, with the fistula forming most often between the aorta and the third portion of the duodenum. Often, the classic triad of abdominal pain, gastrointestinal bleeding, and pulsatile mass is absent. A heraldic bleed frequently precedes lethal exsanguination from a primary aortoenteric fistula. Patient survival is dependent on prompt diagnosis and emergent therapeutic laparotomy. © 2001 Elsevier Science Inc.
CASE PRESENTATION A 75-year-old man presented to the Emergency Department (ED) complaining of back and right hip pain as well as melena. The past medical history was pertinent for a history of arteriovenous malformations of the small bowel and colon for which he had undergone a right hemicolectomy and oversewing of ileal and jejunal malformations. The patient was discharged from the hospital 7 days previously after an exacerbation of lower GI bleeding. At that time, the patient required multiple transfusions and underwent colonoscopic evaluation and a nuclear scan for bleeding, both of which failed to visualize an active source of bleeding. At the time of his evaluation, the patient complained only of right hip pain. He denied current abdominal or low back pain. Initial vital signs were as follows: temperature of 36.6°C, pulse of 92 beats/min, and blood pressure of 160/90 mmHg. Physical examination demonstrated an elderly man resting comfortably, and in no acute distress. The skin was warm and dry with normal color. The abdomen was soft, nontender, with normal bowel sounds, and without organomegaly or pulsatile mass. The extremities, including the right hip, and the femoral pulses, were normal. The rectal examination revealed dark, heme-positive stool. Immediately following the physical examination, the patient suddenly clutched his abdomen complaining of pain, developed myoclonic activity, and became asystolic. One liter of normal saline by rapid infusion, as well as chest compressions, resulted in a return to a sinus
e Keywords—primary aortoenteric fistula; gastrointestinal bleeding; herald bleed; abdominal aortic aneurysm; lethal exsanguination
INTRODUCTION A primary aortoenteric fistula (PAEF) is a rare, lifethreatening cause of gastrointestinal (GI) bleeding. The classic triad of abdominal pain, GI bleeding, and pulsatile abdominal mass is often absent at the time of presentation, which obscures the diagnosis. Therefore, one must consider the possibility and obtain emergent surgical intervention for survival. We present the case of a 75-year-old man with a history of arteriovenous malformations and prior episodes of GI bleeding, who was found to have a PAEF secondary to an abdominal aortic aneurysm (AAA).
RECEIVED: 27 October 1999; FINAL ACCEPTED: 26 July 2000
SUBMISSION RECEIVED:
13 July 2000; 25
26
F. L. Mirarchi et al.
Figure 1. Noncontrast CT scan of the abdomen remarkable for a hyperdense crescent (white arrows) extending from the aorta. The crescent signifies hemorrhage into the vessel wall but without rupture. A ⴝ aorta, D ⴝ duodenum, I ⴝ inferior vena cava.
Figure 2. Contrast-enhanced CT scan of the abdomen remarkable for a 4.0 cm high infrarenal aortic aneurysm with a bulge to the right but without rupture. A ⴝ aorta, D ⴝ duodenum, I ⴝ inferior vena cava.
tachycardia at a rate of 115 beats/min and a blood pressure of 50 systolic. The patient underwent rapid sequence intubation with etomidate and succinylcholine. A nasogastric tube was placed with aspiration producing 30 cc of bright red blood. After 1.5 L of normal saline, the patient’s blood pressure stabilized at a systolic of 100 mmHg. Diagnostics including a chest X-ray study, electrocardiogram (EKG) and emergent spiral computed tomography (CT) scan of the abdomen were performed. The chest X-ray series was normal and demonstrated endotracheal and nasogastric tubes in adequate position. The electrocardiogram revealed a normal sinus rhythm, nonspecific ST changes, and normal intervals. Because of the sudden onset of profound hypotension with associated pain, which is unusual for a GI bleed, a CT scan of the abdomen (with and without i.v. contrast) was performed to evaluate the patient’s aorta. The CT scan without contrast revealed a dilated infra-renal aorta with a hyperdense crescent to the right. The CT scan with i.v. contrast revealed a 4.0 cm infra-renal AAA with a bulge to the right, but without rupture, and with a question of a small amount of contrast identified in the duodenum (see Figures 1 and 2). On return from CT scan, the patient’s nasogastric tube was reconnected and returned 400 cc of bright red blood.
Additional resuscitation involved the transfusion of 2 units of packed red blood cells. A vascular surgery consult was immediately obtained to further evaluate and treat the AAA with possible fistula. The vascular surgeon elected to obtain an angiogram to differentiate upper GI bleeding versus PAEF. The angiogram revealed a 4.0-cm infrarenal AAA with a penetrating ulcer and a questionable fistula tract. Thus, an emergent exploratory laparotomy was performed confirming an aortoduodenal fistula, which required graft and repair of the duodenal fistula. The patient’s remaining hospital course was uncomplicated, and he was discharged home on the sixth postoperative day.
DISCUSSION A PAEF occurs when a direct communication exists between the aorta and the GI tract. It was first described in 1829 by Sir Astley Cooper (1). In contrast, a secondary aortoenteric fistula arises between a suture line of a vascular graft and the GI tract (4,5). A PAEF is rare, with fewer than 250 cases reported in the literature worldwide as of 1996 (1,3,4). It is often fatal, with a mortality rate of 80 –100% (2,3). These fistula most commonly originate from an AAA, of which 85% are atherosclerotic,
Primary Aortoenteric Fistula in the Emergency Department
and 15% are infectious (Tuberculosis, Syphilis, Salmonella, Staphylococcus, and Mycoses) (2,5,7–9). Rarely, a PAEF is a result of cervical cancer, colon cancer, radiation therapy, diverticulitis, appendicitis, duodenal ulcers, cholecystitis, or foreign body (2,3). The third portion of the duodenum is the most frequent site of fistulazation, followed by the jejunum, ileum, colon, esophagus, and stomach (2,3,5,9,10). Men are at greatest risk (75%) with a mean age of 60 years old (2). In patients with known AAA, the incidence of PAEF is 0.69 –2.36% (3,4,7). Culprit aneurysms can range in size from 3.0 –15 cm (mean 7.0 cm) (2,11). Classically, a triad of symptoms including abdominal pain, GI bleeding, and abdominal mass has been described; however, this is present in only the minority of cases (1,2). Back pain is also present in 50% of presentations (2). Fever has been described with PAEF and should raise concern for its presence in a patient with a known AAA (4). Finally, a “heraldic bleed,” which occurred in our patient, is a well-known event; however, this incidence has not been defined. In this case, the key to the diagnosis was the sudden onset of abdominal pain and profound hypotension, which is a feature that is not typical of GI bleeding. Additionally, this patient had recently been discharged from the hospital after a colonoscopy, and nuclear bleeding scan failed to visualize an active source of bleeding. The Emergency Department work-up and management are critical. They begin with rapid assessment and supportive care as well as considering the possibility of the entity. It is imperative to obtain diagnostic studies promptly as well as to involve the Vascular Surgeon early in the decision and management process. The majority of the literature supports that an abdominal CT scan with i.v. contrast is the diagnostic procedure of choice to diagnose PAEF (2,5,6). Angiography, which is useful in planning the operative approach, rarely demonstrates the actual fistula tract (2,3,5). Esophagogastroduodenoscopy rarely identifies the actual fistula, but can supplement other diagnostic modalities by ruling out other causes of GI bleeding (2– 4). Treatment is surgical and involves emergent exploratory laparotomy with graft repair of the aorta and closure of the fistula tract (3– 6). CONCLUSION Although a PAEF is rare, it should be included in the differential diagnosis of GI bleeding, especially when
27
such bleeding is recurrent. With mortality rates of 80 – 100%, the key to its diagnosis and successful management is to consider the diagnosis, rapid assessment to confirm its presence, and immediate surgery. PAEF should be considered in the differential diagnosis of any patient with recurrent GI bleeding, massive exsanguination, known AAA and GI bleeding or known AAA and unexplained fever. CT scan is the diagnostic procedure of choice and treatment is immediate surgery.
Acknowledgments—We would like to formally acknowledge Dennis P. Hanlon, MD, from the Department of Emergency Medicine for his clinical support, and Anthony R. Lupetin, MD, from the Department of Radiological Services, for his technical support.
REFERENCES 1. Kaushik SP, Cowlishaw JL. Primary aortoenteric fistula. Aust NZ J Med 1998;28:471–2. 2. Berry SM, Fisher JE. Classification and pathophysiology of enterocutaneous fistulas. Surg Clin North Am 1996;76:1009 –18. 3. Brown PW, Sailors DM, Headrick JR, et al. Primary aortoduodenal fistula: a case report. Am Surg 1999;65:139 – 41. 4. Voorhoeve R, Moll FL, de Letter JAM, et al. Primary aortoenteric fistula: report of eight new cases and review of the literature. Ann Vasc Surg 1996;10:40 – 8. 5. Bernhard V. Aortoenteric fistulae. In: Rutherford RS., ed. Vascular surgery, 4th edn. Philadelphia: WB Saunders Company; 1995: 611–18. 6. Voorhoeve R, Moll FL, Bast TJ. The primary aortoenteric fistula in the Netherlands—the unpublished cases. Eur J Vasc EndoVasc Surg 1996;11:429 –31. 7. Hickey NC, Downing R, Hammer JD, et al. Abdominal aortic aneurysms complicated by spontaneous ileocaval or duodenal fistulae. J Cardiovasc Surg 1991;32:181–5. 8. Nagy SW, Marshall JB. Aortoenteric fistula. Postgrad Med 1993; 93:211–22. 9. Peck JJ, Eidemiller LR. Aortoenteric fistulas. Arch Surg 1992;127: 1191– 4. 10. Rhim JA, Rubin RA, Katzka DA, et al. Primary aortoduodenal fistula: a unique presentation of a pseudoaneurysm associated with cystic medial necrosis. Am J Gastroenterol 1993;88:447–50. 11. Calligaro KD, Bergen WS, Saverese RP, et al. Primary aortoduodenal fistula due to septic aortitis. J Cardivasc Surg 1992;33: 192– 8. 12. Vlassopoulos D, Noussias C, Dagounaki P, et al. Aortoenteric fistula: an unusual cause of gastointestinal bleeding in a hemodialysis patient. Nephrol Dial Transplant 1997;12:2171–3. 13. Antinori CH, Andrew CT, John S, et al. The many faces of aortoenteric fistulas. Am Surg 1996;62:344 –9.