Overview

Definition:
-An aortoenteric fistula (AEF) is an abnormal communication between the aorta or a prosthetic aortic graft and the gastrointestinal tract
-It is a rare but life-threatening complication, most commonly occurring as a secondary event after aortic reconstructive surgery, particularly with prosthetic grafts
-Primary AEF without prior surgery is exceptionally rare.
Epidemiology:
-Secondary AEF occurs in 0.4-2% of patients with prosthetic aortic grafts
-The incidence of primary AEF is exceedingly low
-Risk factors for secondary AEF include prosthetic graft infection, previous abdominal surgery, radiation therapy to the abdomen, and immunocompromise
-The most common location for a secondary AEF is between the duodenum and an infrarenal aortic graft.
Clinical Significance:
-AEF represents a surgical emergency due to the high risk of massive hemorrhage and sepsis
-Prompt diagnosis and aggressive management are crucial for patient survival
-Mortality rates remain high, often exceeding 50%, emphasizing the need for rapid intervention and multidisciplinary care.

Clinical Presentation

Symptoms:
-Abdominal pain, often severe and colicky
-Gastrointestinal bleeding, ranging from melena to massive hematemesis or hematochezia
-Intermittent herald bleeding episodes followed by frank exsanguination
-Fever and systemic signs of infection if graft sepsis is present
-Signs of hemodynamic instability: hypotension, tachycardia, pallor, diaphoresis.
Signs:
-Abdominal tenderness, guarding, or rigidity
-Palpable abdominal pulsatile mass (if aneurysm is present)
-Signs of hypovolemic shock
-Evidence of ongoing gastrointestinal bleeding
-Signs of sepsis: fever, tachycardia, hypotension.
Diagnostic Criteria:
-No specific diagnostic criteria exist
-diagnosis is based on high clinical suspicion in the context of risk factors and confirmed by imaging and/or endoscopy
-The classic triad of abdominal pain, gastrointestinal bleeding, and a pulsatile abdominal mass is often absent or may not present simultaneously.

Diagnostic Approach

History Taking:
-Detailed history of prior aortic surgery, particularly the type and timing of graft placement
-History of abdominal radiation or infection
-Onset, severity, and character of abdominal pain
-Pattern and volume of gastrointestinal bleeding
-Presence of systemic symptoms like fever
-Red flags: any patient with a history of aortic graft presenting with unexplained gastrointestinal bleeding or abdominal pain requires a high index of suspicion.
Physical Examination:
-Thorough abdominal examination to assess for tenderness, masses, and pulsatility
-Complete cardiovascular assessment for signs of shock
-Systemic examination for signs of infection.
Investigations:
-Laboratory tests: Complete blood count (CBC) for anemia and leukocytosis
-Coagulation profile (PT, PTT, INR)
-Blood type and crossmatch for urgent transfusion
-Renal function tests
-Imaging modalities: Computed Tomography Angiography (CTA) is the investigation of choice, visualizing the aorta, graft, and potential fistulous tract, and identifying extraluminal air or fluid suggestive of infection
-Upper or lower endoscopy may be diagnostic if bleeding is active and imaging is inconclusive, but carries a risk of precipitating massive hemorrhage and should be performed with extreme caution and in conjunction with surgical readiness
-Ultrasound may detect perigraft fluid collections but is less sensitive for the fistula itself.
Differential Diagnosis:
-Peptic ulcer disease with bleeding
-Esophageal varices with bleeding
-Malignancy of the gastrointestinal tract
-Mesenteric ischemia
-Other causes of massive GI bleeding
-Ruptured abdominal aortic aneurysm (AAA) without fistula.

Management

Initial Management:
-Immediate resuscitation with aggressive fluid and blood product replacement to stabilize hemodynamics
-Secure at least two large-bore intravenous access lines
-Prompt consultation with vascular surgery and gastrointestinal services
-Preparation for urgent surgical intervention
-Nasogastric tube insertion to decompress the stomach and monitor for bleeding
-Broad-spectrum antibiotics for suspected graft infection.
Medical Management:
-Primarily supportive and aimed at hemodynamic stabilization
-Transfusion of packed red blood cells to maintain hemoglobin levels >7-8 g/dL
-Fresh frozen plasma and platelet transfusions as guided by coagulation status and active bleeding
-Vasopressors may be required in severe shock
-Antiemetics to control nausea and vomiting
-Proton pump inhibitors to reduce gastric acidity.
Surgical Management:
-The definitive management is surgical repair
-Options include: 1
-Excision of the infected graft with extra-anatomic bypass (e.g., axillofemoral bypass) and wide debridement of infected tissue
-2
-In situ graft repair (less common, higher risk of recurrence)
-3
-Ligation of the aorta if bypass is not feasible
-The choice of procedure depends on the patient's hemodynamic status, the extent of infection, and the surgeon's expertise
-If the fistula involves the duodenum, repair of the enteric defect is also necessary, often with diversion (e.g., duodenal diversion, gastrostomy).
Supportive Care:
-Continuous hemodynamic monitoring in an intensive care unit
-Close monitoring of urine output
-Nutritional support, often parenteral initially
-Strict glycemic control
-Prophylaxis for deep vein thrombosis
-Postoperative physiotherapy.

Complications

Early Complications:
-Massive hemorrhage leading to exsanguination and death
-Sepsis and septic shock
-Acute kidney injury
-Respiratory distress
-Myocardial infarction
-Stroke
-Graft dehiscence.
Late Complications:
-Graft limb occlusion or stenosis
-Pseudoaneurysm formation
-Recurrent graft infection
-Chronic abdominal pain
-Enterocutaneous fistula
-Incisional hernia.
Prevention Strategies:
-Judicious use of prosthetic grafts, considering biologic grafts in high-risk patients
-Meticulous surgical technique during aortic reconstruction to minimize contamination
-Early diagnosis and management of graft infection
-Careful antibiotic selection and duration for infected grafts.

Prognosis

Factors Affecting Prognosis:
-The most significant factor is the speed of diagnosis and intervention
-Hemodynamic stability at presentation
-Presence and severity of sepsis
-The extent of aortic and enteric involvement
-Patient comorbidities
-The type of surgical repair performed.
Outcomes:
-Mortality rates remain high, historically ranging from 40-90%, although aggressive modern management has led to some improvement
-Survival is significantly improved with prompt surgical treatment before exsanguination
-Long-term survival depends on successful graft limb salvage and absence of recurrent infection.
Follow Up:
-Long-term follow-up is essential, typically with vascular surgery and gastroenterology
-Regular clinical assessment for signs of recurrent infection or graft complications
-Imaging studies (CTA, ultrasound) may be used to monitor the integrity of any repaired or replaced graft
-Monitoring for gastrointestinal symptoms and nutritional status.

Key Points

Exam Focus:
-AEF is a surgical emergency
-prioritize immediate resuscitation and urgent surgical consultation
-CTA is the investigation of choice
-The management involves controlling the hemorrhage, treating infection, and reconstructing the aorta, often with extra-anatomic bypass
-Primary vs
-secondary AEF distinction is important
-Recognize the "herald bleed" phenomenon.
Clinical Pearls:
-Always consider AEF in patients with a history of aortic grafts presenting with unexplained GI bleeding or abdominal pain
-Maintain high index of suspicion and involve vascular surgery early
-Aggressive resuscitation is paramount before any diagnostic intervention that could precipitate massive bleeding
-Surgical mortality is high, but preventable if treated promptly
-Consider biologic grafts in certain high-risk scenarios to reduce infection rates.
Common Mistakes:
-Delaying surgical consultation due to uncertainty or attempts at conservative management
-Performing endoscopy or colonoscopy without adequate surgical preparedness, risking catastrophic hemorrhage
-Underestimating the severity of bleeding and failing to transfuse adequately
-Inadequate debridement of infected tissue
-Not addressing the enteric defect appropriately.