Tracheoesophageal fistula and severe esophageal stenosis in a patient with Behçet’s disease. Report of a case and literature review
Konstantinos Tsalis, Konstantinos Blouhos, Leonidas Sakkas, Emmanouel Christoforidis, Charalampos Lazaridis
Am J Case Rep 2009; 10:47-51
Available online: 2009-04-28
Background: In Adamandiades-Behçet’s Disease (ABD), controversy has existed over the incidence of esophageal involvement, which may be a direct threat to the patient’s life.
Case Report: We describe a case of tracheoesophageal fistula after stent placement in a 50-year-old female with ABD. The onset of ABD was typical. For the last three years she complained of diffi culty in swallowing both solid and liquid foods. Endoscopy revealed ulcerative esophagitis and a markedly stenotic
esophagus in the middle and distal portion. She was on a treatment regime including immunosuppressivetherapy. Balloon and bougie dilations were necessary to relieve her symptoms.After an additional episode of restenosis, an esophageal stent was placed, but her clinical course was complicated with a tracheoesophageal fistula. The patient was taken to the operating room where a right thoracotomy was performed. The fistula was dissected free, and the tracheal defect was closed with a two-layer esophageal poutch and with a rotated diaphragmatic flap, based on a well-vascularized pericardiophrenic pedicle. Staged reconstruction of the alimentary continuity was planed. The postoperative course complicated with severe haemorrhage from the chest tube. A second look operation was performed to identify and control the bleeding sources. Unfortunately, the patient died during her transfer from the theatre to the ICU.
Conclusions: All ABD patients should be evaluated for esophageal involvement. Although selected ABD patients with esophageal stenosis may benefit with temporary stenting, we believe that in patients with a persisting fistula track or in the presence of severe symptoms surgical intervention with a tailored approach is inevitable.
Keywords: esophageal involvement, Behçet’s disease, Adamandiades-Behçet’s disease, Tracheoesophageal Fistula, esophageal stenting