Case Presentation: A 61 year-old man with a history of aortitis of uncertain etiology and pseudoaneurysm s/p thoracic endovascular aortic repair (TEVAR) with subclavian coiling eight months earlier presented with progressive worsening left upper and lower extremity pain beginning post-operatively. On admission the patient developed hemoptysis, usually scant, but intermittently as much as 100cc. He was otherwise asymptomatic, hemodynamically stable and with an unremarkable physical exam. Over the next 48 hours, his hemoglobin dropped from 10.4 to 6.4 mg/dL. There was concern for tracheo-aortic fistula given the history of TEVAR, however a CTA of his chest did not reveal contrast extravasation. The patient responded appropriately to pRBC transfusion and his hemoptysis resolved without further intervention. Several days later he again had another hemoglobin drop from 8.3 to 6.5 mg/dL but had no signs or symptoms of overt bleeding including no hematemesis, melena, or hematochezia. A CT scan of his abdomen and pelvis demonstrated no bleeding source and his hemolysis labs were negative. Given no identified culprit for this patient’s continued acute anemia and transfusion dependence he underwent endoscopy that revealed his aortic graft protruding through his esophagus and no evidence of recent bleeding.
Discussion: Aortoesophageal fistula (AEF) is a rare, but recognized complication of TEVAR and cause of upper gastrointestinal bleeding (UGIB). An international multicenter registry including over two thousand TEVAR procedures over ten years found an incidence of AEF of 1.5%. The most common clinical presentations were fever of unknown origin (81%) and hematemesis (53%). 92% were diagnosed by CT and 50% were confirmed on endoscopy (Czerny et al. 2014). It is unclear if the remaining 8% had negative CT scans, or if clinical suspicion was so high that upper endoscopy was pursued emergently before scanning. A meta-analysis of studies on the role of CT scan in diagnosing AEF found sensitivity ranging from 40-100% and specificity 33.3-100%, representing a questionable diagnostic reliability (Malik et al. 2015). In this patient with prior aortitis and pseudoaneurysm who had undergone TEVAR presenting with hemoptysis and transfusion dependent anemia was highly suspicious for fistulation, prompting upper endoscopy despite a negative CT scan.
References:
Czerny, M et al. New insights regarding the incidence, presentation and treatment options of aorto-oesophageal fistulation after thoracic endovascular aortic repair: the European Registry of Endovascular Aortic Repair Complications. European Journal of Cardio-Thoracic Surgery. 2014:45;452-457.
Czerny M et al. Secondary organ fistulation after thoracic endovascular repair. Minimally Invasive Therapy & Allied Technologies, 2015. 24:5. 305-310.
Malik, MU, et al. Critical gastrointestinal bleed due to secondary aortoenteric fistula. Journal of Community Hospital Internal Medicine Perspectives. 2015(5).
Nazarewicz, GV and Jain, R. Upper Gastrointestinal Bleeding Caused By Aortoesophageal Fistula. Clinical Gastroenterology and Hepatology 2016:14(12);xxii. Image of the month
Conclusions: AEF is a rare, but perilous cause of UGIB in patients with a history of prior TEVAR that may require endoscopic evaluation to diagnose when clinical suspicion is high.