Adult Benign, Non‐Iatrogenic Bronchoesophageal Fistulae: Systematic Review and Descriptive Analysis of Individual Patient Data

Abstract

Background Adult, benign, non-iatrogenic bronchoesophageal fistula (BEF) is a rare condition, which is occasionally described in single case reports. Therefore, little is known about its possible causes, presentation and management. Methods A systematic search of the literature in MEDLINE, PubMed Central and EMBASE databases between 1990 and 2020 was carried out to identify all cases of BEF. The initial database search identified 19,452 articles, of which 183 (251 individual patient cases) were included in the final analysis. Results Main causes of BEF were congenital malformations (97/251, 38.7%) and infections (82/251, 32.7%), while 33/251 (13.1%) fistulae were regarded as idiopathic and 39/251 (15.5%) attributed to other causes. Esophagograpy was the most sensitive method of diagnosis (97.4%) compared with esophagoscopy (78.9%), computed tomography (49.6%) and bronchoscopy (46.0%). Definitive treatment was surgical for 176 patients (70%), endoscopic for 25 (10%) and medical for 37 (14.7%). Compared with congenital BEFs, infective BEFs had shorter median symptom duration and were distributed more proximally over the bronchial tree. Definitive treatment was almost only surgical for congenital BEFs, while infective BEFs were treated also endoscopically (12%) and by medical therapy (38%). Morbidity, treatment failure and recurrence rates were higher for infective BEFs. Conclusions BEFs are rare. Symptoms are non-specific and a high index of suspicion is necessary for diagnosis. Patients with infective BEF tend to have a more severe clinical picture than those with congenital BEF. Surgery is the main treatment for patients affected by congenital BEF, while infective BEFs may heal conservatively.

Endoscopic submucosal dissection-based suture combined with medical adhesive for complicated tuberculous bronchoesophageal fistula: a case report

“…13 Mammana et al reported that among 25 endoscopically-treated patients with bronchoesophageal fistula, 4 patients received combined endoscopic treatments, and the most common endoscopic tools were clips, stents, glue, and septal occluders. 14 Therefore, the combination of ESD-based suture and medical adhesive was proposed and implemented, in this case. Esophageal ESD around the fistulous orifice was intended to provide a larger mucosal defect for the subsequent endoscopic nylon loop suture.…”

Management of aero-digestive fistulas in adults: the bronchoscopist’s perspective

“…The median time from symptom development to diagnosis of malignant ADFs has been reported between 3 days to 7 months with a range of 0–58 months ( 4 , 6 , 11 ). The most common symptoms include coughing after swallowing (“Ono’s sign”) in 20–100% of patients, dysphagia in 8–100%, purulent tracheobronchitis in 15–100%, pneumonia in 5–95%, chronic cough in 20–81%, dyspnea in 18–69%, cachexia and malnutrition in 18–60%, aspiration in 23–46%, fever in 21–27%, chest pain in 5–23%, respiratory failure requiring mechanical ventilation in 6–15%, and hemoptysis or hematemesis in 2–3% ( 4 , 6 , 12 - 17 ).…”

“…Once an ADF is suspected, the first diagnostic study is typically an esophagram as it is easy to obtain with a sensitivity estimated between 70–97% ( 17 , 18 ). Oral barium sulfate is the contrast of choice over Gastrografin because aspiration of Gastrografin has been associated with fatal pneumonitis and pulmonary edema due to the iodine content as well as the hyperosmolarity of the contrast solution, respectively ( 19 ).…”

“…Oral barium sulfate is the contrast of choice over Gastrografin because aspiration of Gastrografin has been associated with fatal pneumonitis and pulmonary edema due to the iodine content as well as the hyperosmolarity of the contrast solution, respectively ( 19 ). Computed tomography has a lower reported sensitivity of 50% but may aid in diagnosis when an esophagram is nondiagnostic or cannot be performed due to an inability to swallow contrast ( 17 ).…”

“…Direct visualization can be performed by esophagoscopy and/or bronchoscopy, which have reported sensitivities of 80% and 46%, respectively ( 17 ). These procedures will also help guide treatment strategies, as discussed below.…”