We experienced a refractory case of acquired TEF with double fistula that was repaired by gastric transposition through the retrosternal route. Double fistula formation is relatively rare. The clinical course in this case suggests some aspects of acquired TEF development. The mechanisms of acquired TEF and the strategies to repair this complex case will be discussed based on the current study.
With regards to the mechanisms of acquired TEF development, this case suggests the possibility that the acquired TEF is derived from the severe inflammation. Furthermore, it may have been complicated by a network of tiny fistulas which are not always fully visualized in a contrast study. In the current case, before the right upper lobectomy was performed, the contrast study and bronchoscopy only detected a fistula to the right upper lobe bronchus. However, 1 month after lobectomy, a new fistula to right lower lobe bronchus appeared. This happened despite no adverse events in the postoperative course. Taking the improvement of the respiratory symptom after lobectomy into consideration, we assumed that several fistulas existed from the beginning and the esophageal contents only flowed into the relatively evident fistula (towards the upper lobe) due to the low pressure inside it. Therefore, the tinier fistula directed towards the right lower lobe could not be detected. Majority of acquired TEFs were reported to be related with anastomotic leakage, not only in esophageal atresia, but also in other diseases such as esophageal cancer [8, 9]. This suggests that severe inflammation derived from a leakage could form a fistula. If the original inflammation spread extensively, multiple fistulas could be formed. Possibly, some of the reported cases of “re-recurrence” might have developed through this mechanism and may have existed before the operation.
In the current case, the 3 cm stenosis of the esophagus and the severe adhesion around the anastomosis site were expected. Esophagoesophagostomy was thought to be difficult; hence, esophagus replacement was planned. However, the respiratory symptoms were so severe that we had to prioritize fistula removal by lobectomy and reduction of gastroesophageal reflex (GER) by esophageal banding. These operations, however, reduced the residual pulmonary function and shortened the available distal esophagus for esophageal replacement. There is no consensus about the optimal way for esophageal replacement, which are usually done in cases of long-gap esophageal atresia. Gastric transposition permits a good blood supply and a lower rate of leakage; however, respiratory morbidities have been reported to be slightly higher than other methods in the long term [10]. Gastric tube esophagoplasty is a relatively physiological method that minimizes the postoperative GER if it is performed with the Collis–Nissen procedure. However, it tends to have a shorter mobilized esophagus length and a higher leakage rate due to the high anastomotic tension [11,12,13]. Jejunal and colon interpositions are reported to have good outcomes and enough lengths of interposition. These procedures, however, need several anastomotic sites and require a precarious blood supply [14].
In the current case, aside from the long-gap esophageal atresia, two points were considered. First, the reconstruction route could have become longer if the mediastinal route could not be formed because of the severe adhesion and adopt the retrosternal route. Second, the respiratory and perioperative risks had to be avoided as much as possible. Considering these risks, we chose gastric transposition as the appropriate reconstruction method. However, we remained vigilant for the probable postoperative risks of gastric transposition such as mediastinal compression and dislocation of the pulled-up stomach into the thoracic cavity. To minimize these risks, we mobilized the stomach with the assistance of a fiberscope. Periodic X-ray tests were planned to check for stomach dislocation after the operation.
In the current case, dissection of the esophagus in the posterior mediastinal route was impossible due to severe adhesion. We chose the retrosternal route and left the residual esophagus containing stenotic sites in the posterior mediastinum. Hirschi et al. reported 41 successful cases of gastric transposition through the posterior mediastinal route, including 15 cases with a previous esophageal operation [15]. Some of these were done with a thoracotomy incision. In the current case, even if thoracotomy was added, it was considered impossible to peel off the bronchus from the surrounding tissues and the stenotic esophagus due to severe adhesions. For these reasons, the retrosternal route was adopted.
A CT scan taken 2 years after the operation showed mucus pooling in the residual esophagus. However, it was not diluted and the mucus seemed to flow into the distal bronchus through the fistula. Although there have not been any adverse events caused by the residual esophagus for 2 years since the operation, the residual esophagus is blind lesion and should be strictly followed by CT scan supported by blood test to detect the possibility of carcinogenesis, inflammation, and ballooning. As a long-term complications, some cases of esophageal cancer are reported in EA patients and routine endoscopy is recommended [16]. If an adverse event was to occur, we assumed that this would be treated by puncture drainage or esophagectomy approached from the left thoracic cavity. The current case was believed to be derived from an anastomotic rupture following balloon dilation against the esophageal anastomotic stenosis. No consensus are exist about how many times of balloon dilation are accepted; however, most of the successful cases were achieved within four sessions [17,18,19,20]. Although the majority of cases with stenotic strictures improve with balloon dilation, if the acquired TEF developed after anastomotic rupture, it may become very difficult to repair. Surgeon should keep these important points in mind when dealing with similar cases.
