Abstract
Congenital tracheal stenosis is a rare but serious condition with high mortality and morbidity. We present a 6-month-old patient with complex congenital tracheal stenosis involving the trachea, carina and right bronchus intermedius, which was corrected with a combination of slide tracheoplasty and side-to-side bronchoplasty.
Keywords: Congenital tracheal stenosis, Tracheoplasty, Bronchoplasty
INTRODUCTION
Congenital tracheal stenosis (CTS) is a rare but serious condition with high mortality and morbidity [1]. We present a case of complex CTS, which was corrected with combined slide tracheoplasty and side-to-side bronchoplasty.
CASE REPORT
A 3-month-old boy was admitted for 1-week history of cough and dyspnoea, on a background of intermittent wheeze since 1-month of age. He was in respiratory distress with bilateral wheeze and was treated for Human Coronavirus NL63 detected on nasopharyngeal swab. Continuous positive airway pressure (CPAP) was commenced for desaturations. However, he was unable to wean off CPAP despite the resolution of infection.
Computed Tomography (CT) thorax revealed a left pulmonary artery (LPA) sling, right tracheal bronchus and a long-segment tracheal stenosis. The smallest calibre of the trachea measured 1.4 mm in diameter at the level of LPA sling. It gave rise to a stenotic pseudocarina (Fig. 1A). 2D echocardiogram confirmed the findings of LPA sling. Flexible bronchoscopy showed complete tracheal rings. Distal trachea appeared slit-like due to compression from LPA sling. The proximal left main bronchus appeared severely malacic. The right bronchus intermedius was not well visualized.
Figure 1:
(A) CT thorax showing the 2.6-cm long-segment tracheal stenosis, giving rise to a stenotic carina. (B) Schematic representation of the regions of stenosis and relations to left pulmonary artery sling. (C) Incision sites for tracheoplasty and bronchoplasty. (D) Slide tracheoplasty and side-to-side bronchoplasty.
Patient underwent slide tracheoplasty, side-to-side bronchoplasty and reimplantation of LPA at 6-month-old. Suprahyoid release was performed to facilitate the mobilization of trachea prior to median sternotomy. LPA was detached from the RPA after cardiopulmonary bypass was instituted. Trachea was transected and anterior slit was created to the lower half while posterior slit to the upper half. The LPA was passed to the left hilum thereafter. The stenosis at proximal right bronchus intermedius was confirmed. The extent of bronchoplasty was determined by the accommodation of a 3-mm probe in the lumen of right bronchus intermedius. Slide tracheoplasty, which encompassed the proximal end of the tracheal stenosis, and side-to-side bronchoplasty were performed (Fig. 2). Finally, LPA was re-implanted to the main pulmonary artery.
Figure 2:
Postoperative bronchoscopy images. (A) Trachea-right tracheal bronchus bifurcation. (B) Carina to right bronchus intermedius.
Postoperative course was complicated by LPA stenosis due to pericardial hitching requiring a revision of LPA with pericardial patch augmentation on postoperative day (POD) 1. He was extubated on POD 8 to CPAP support. Postoperative bronchoscopy showed normal tracheobronchial anatomy, although distal trachea still appeared severely malacic. He was discharged at 1 month after operation on CPAP. Follow-up bronchoscopy at 5 months postoperation (Fig. 2) showed mild lower tracheomalacia. At 6 months follow-up, he has successfully weaned off CPAP.
DISCUSSION
Slide tracheoplasty is a widely used approach for long-segment tracheal stenosis [2]. This technique, however, does not correct carinal or proximal bronchial stenosis. To address this limitation, several modifications have been described. For carinal stenosis, a longitudinal incision on the anterior wall of the inferior part of the trachea that terminates at the carina in an inverted ‘Y’ shape, followed by the usual sliding and anastomosis, has been reported [3]. Alternatively, side-slide tracheoplasty is used to treat carinal and proximal bronchial stenosis, whereby incision is made longitudinally down to the carina distally, forming an oblique anastomosis [4]. All forms of slide tracheoplasty inevitably shorten the airway and increase the risk of causing anastomotic tension especially in longer lesions, leading to restenosis and dehiscence [1].
To avoid anastomotic tension, side-to-side tracheoplasty is an alternative technique that provides tension-free anastomosis without shortening or significantly distorting the native tracheobronchial anatomy. Side-to-side tracheoplasty was first described for the augmentation of long-segment tracheal stenosis using a normal bronchus suis [5]. Although this technique was originally for long-segment tracheal stenosis, it can also be used to treat carinal and proximal bronchial stenosis.
When planning for our patient’s operation, we decided against modified slide tracheoplasty in view of risks damaging the lateral blood supply from the longitudinal incision and excessive shortening of the trachea. Therefore, the side-to-side technique was adopted for bronchoplasty to augment the carina and proximal bronchial stenosis, in addition to the standard slide tracheoplasty for long-segment tracheal stenosis. The combination of these techniques can address all the stenotic lesions in 1 setting while avoiding the disadvantages of each technique.
CONCLUSION
Surgical management should be tailored for each patient with CTS. The combination of slide tracheoplasty and side-to-side bronchoplasty avoids excessive tracheal shortening and at the same time, addresses the carina and proximal bronchial stenosis.
Conflict of interest: none declared.
Reviewer information
Interactive CardioVascular and Thoracic Surgery thanks Shu-Chien Huang and the other, anonymous reviewer(s) for their contribution to the peer review process of this article.
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