Aortoesophageal
fistulas (AEFs) are rare and life-threatening conditions. Till date, an association between an
AEF and
sarcoidosis has not been reported yet. The aim of this report is to demonstrate a case of
AEF secondary to
sarcoidosis and its multistage interdisciplinary surgical
therapy. A 66-year-old male was diagnosed with
sarcoidosis in 2014. He has been treated with
glucocorticoids since then and no severe health restrictions due to the disease have occurred. In December 2015, the patient presented with acute thoracic
pain and
hematemesis: an esophagogastroscopy revealed an
AEF. First,
stent-graft implantation in the thoracic aorta was urgently performed as a "bridging" procedure. Second,
esophagectomy and local
debridement were performed, followed by explantation of the
stent graft and reconstruction by means of xenograft replacement of the stented aorta in a third operation. Finally, retrosternal gastric pull-up was performed in a fourth operative procedure. Sixteen days after the last operation the patient could be discharged to a rehabilitation clinic. Follow-up is uneventful so far; the
antibiotic therapy was stopped at the time of hospital discharge. The pathogenesis of
sarcoidosis, a rare autoimmunological disease, has not been completely clarified yet. The diagnosis relies on clinical symptoms and radiological as well as histopathological findings. Many cases of
sarcoidosis show
spontaneous regression, but severe complications may occur. While
tracheoesophageal fistulas have been described in the literature, AEFs related to
sarcoidosis have not been mentioned yet. Despite surgical and
antibiotic treatment, the morbidity and mortality rates of
AEF are high. Because the endovascular treatment has been established for emergency procedures of the aorta, it is considered as an appropriate first-line "bridging" treatment option. To achieve good long-term results, surgical treatment has to involve
esophagectomy with secondary reconstruction of the upper gastrointestinal tract, as well as open aortic replacement using xenograft or homograft material.
Sarcoidosis may lead to
AEF as demonstrated in this case. Successful treatment can be realized by a multistage interdisciplinary surgical approach.