The etiology of EA with or without TEF is reported to occur due to the failure of separation or incomplete development of the foregut. Therefore, EA is often comorbid with various forms of tracheobronchial anomalies [2]. cBPFM, one of these malformations, is defined by a patent congenital communication between the esophagus or stomach and an isolated portion of the respiratory tract [3]. Srikanth et al. [4] classified cBPFM into four groups. Among these, group IA is associated with EA/TEF and the entire lung arises from the lower esophagus or stomach. The corresponding mainstem bronchus is absent from the trachea.

cBPFM has diagnostic difficulties [5], and in our case, the challenge was to differentiate between a diagnosis of cBPFM type IA and an atypical case of type C EA. In general, a case of type C EA has the entry site of the lower esophagus located around the tracheal bifurcation, in which case the esophagus would need to be dissected at this site and an end-to-end esophageal anastomosis is performed. In the case of cBPFM group IA, although the lower esophagus needs to be dissected and anastomosed at the same site as in type C EA, tracheobronchial anastomosis [6] or lobectomy [7] of the esophageal-originating lung is additionally needed. Our case was different from both cases. In our patient, the malformation formed as a type C EA with an additional characteristic that the bronchial-like lumen was observed from tracheal trifurcation to the lower esophagus. Therefore, it was necessary to dissect the lower esophagus distal to this bifurcation and to resect the bronchial-like portion of the lower esophagus. In each of these cases, the surgical procedures are completely different, and inadequate operations can cause fatal complications. A preoperative surgical planning is crucial in cases of EA with an atypical clinical course.

Regarding the diagnosis of this case, there is an alternative explanation. We diagnosed this case as esophageal atresia with the bronchial-like lower esophagus which originates from the left lower lobe bronchus. However, this case could also be considered as a case of cBPFM Group IA with bronchial-like lower esophagus. The main difference between these diagnoses is whether the dorsal branch of tracheal trifurcation is considered as a left lower lobe bronchus or a bronchial-like lower esophagus. Since they present similar morphologies, it is difficult to distinguish between them. This is the first case report of this kind of malformation, making it difficult to classify this case.

The left lung was unilobed, and a left-sided approach with right one-lung ventilation was considered. However, the patient had a usual left-sided aortic arch; hence, a left-sided approach was expected to have a higher risk of intraoperative organ injury and postoperative esophageal obstruction due to flexion. This logic is similar to a case, in which the patient had an EA with a right-sided aortic arch that was treated surgically by a right-sided approach [8, 9]. Therefore, we selected a right-sided approach. Furthermore, a transpleural approach was applied to minimize the compression of the right lung. This was because the thoracoscopic approaches would compress the entire right lung, resulting in intraoperative respiratory distress. In addition, we thought it would be safer to compress the lung under direct observation by transpleural approach rather than by extrapleural approach.

The transected edge of the esophagus contained cartilage around two-thirds of its circumference, presenting as a bronchial-like appearance. It has been reported that small islands of cartilage are present within the lower esophageal wall in some cases of EA [10]. However, there have been no previous reports in which the lower esophagus showed a bronchial-like appearance and transitioned into the esophagus. This case is extremely uncommon among the types of tracheoesophageal malformations. In these types of cases, we recommend complete resection of the bronchial-like tissue at surgery. The remnant of the esophagus wall would certainly cause the same situation as congenital esophageal stenosis [11]. Extensive resection of the lower esophagus can make primary repair of EA/TEF challenging and conversion of the surgical procedure should be taken into consideration.

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