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Congenital right bronchial stenosis with high bifurcation: Successful management with side to side tracheobronchial anastomosis
International Journal of Pediatric Otorhinolaryngology 111 (2018) 180–182
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Congenital right bronchial stenosis with high bifurcation: Successful management with side to side tracheobronchial anastomosis
T
Rachel Georgopoulosa,∗, Courtney Hilla, Jeffrey S. Heinleb, Deepak Mehtaa a b
Department of Pediatric Otolaryngology Texas Children's Hospital, United States Department of Cardiovascular Surgery Texas Children's Hospital, United States
A R T I C LE I N FO
A B S T R A C T
Keywords: Congenital Bronchial Stenosis Bifurcation Double trachea 3D model
Congenital bronchial stenosis is a rare entity [1,2]. While there are some reports of congenital bronchial anomalies and their repair in the thoracic literature this is first report in the literature of a bronchial take off just below the larynx managed with surgical success. Here we present a case of a former 33-weeker born with a tracheoesophageal fistula, Tetralogy of Fallot, and a high bifurcation of the right mainstem bronchus with a concomitant long segment bronchial stenosis. Preoperative planning included printing 3-D reconstructed models of the airway to analyze various treatment options. At 3 months of age the patient was taken to the operating room for surgical repair of the Tetralogy of Fallot and side-to-side tracheobroncheoplasty. The patient was extubated on postoperative day 3 and was discharged home without need for any ventilatory support.
1. Case report A 6 week-old former 33-weeker was transferred to our tertiary care facility with Tetralogy of Fallot, tracheoesophageal fistula repaired at the outside hospital, and a known congenital airway anomaly. On the first day of the life the patient underwent echocardiography which revealed Tetralogy of Fallot with a left-sided arch. On day two of life, he was taken to the operating room for repair of the tracheoesophageal fistula. Due to persistent issues with intermittent desaturations and hemoptysis, the patient underwent a CT chest which revealed a right bronchial take off just below the level of the true vocal folds. The right mainstem bronchus had a 22 mm segment of stenosis with concomitant hyperinflation of the right lung (Fig. 1). The patient when transferred was having significant problems with desaturation as a result of right lung hyperinflation when on continuous positive airway pressure. In light of this, he was weaned to high flow nasal canula and did significantly better. A 3-D printed model of the airway was created, and its utility was three-fold (Fig. 2). The model was used for preoperative surgical planning, and to enhance discussions about the patient in a multidisciplinary fashion. The model was invaluable for discussing the diagnosis and treatment with the patient's family and health care providers.
∗
Corresponding author. 6701 Fannin Suite D.0640.00, Houston TX, 77030, United States. E-mail address: [email protected] (R. Georgopoulos).
https://doi.org/10.1016/j.ijporl.2018.06.011 Received 24 April 2018; Received in revised form 8 June 2018; Accepted 9 June 2018 Available online 12 June 2018 0165-5876/ © 2018 Elsevier B.V. All rights reserved.
At 3 months of age, the patient was placed on cardiopulmonary bypass using direct aorto-bicaval cannulation. After completion of the sternotomy and exposure of the airway, a longitudinal incision was made on the coapting surfaces of the left main trachea and the right bronchus distal to the stenotic segment (Fig. 3). These were enlarged using an aortic punch, and then a side-to-side tracheal anastomosis was performed using a running 7-0 PDS suture. After repair of the airway, the remainder of the cardiac procedure was undertaken. The patient was extubated on postoperative day 3 and did well. He was taken back to the operating room for bronchoscopy 1 month later for surveillance, which revealed a well healing suture line and a patent airway (Fig. 4). Postoperative chest x-rays demonstrated improved aeration without airtrapping (Fig. 5). 2. Discussion Congenital bronchial stenosis with a high right bifurcation can be a challenging entity to treat. Long segment bronchial stenosis can lead to air-trapping, hyperinflation of the lung and subsequent respiratory distress. Management of these lesions can be complicated as traditional techniques used to manage airway stenosis such as dilation, cricotracheal reconstruction and slide tracheobronchioplasty, which are not
International Journal of Pediatric Otorhinolaryngology 111 (2018) 180–182
R. Georgopoulos et al.
Fig. 1. CT chest demonstrating the high take off of the right bronchus with significant air trapping.
Fig. 3. Red markings demonstrate the coapting surfaces of the airway that were opened with an aortic punch and a side to side tracheal anastomosis performed. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
possible given the lesions proximity to the vocal folds [2,3,4]. Furthermore, compared to the trachea, the bronchi have a significantly smaller intraluminal diameter making surgical repair more challenging [2]. Of the patients with congenital tracheal or bronchial stenosis described in the literature, all have had associated cardiovascular anomalies [1,2]. In light of this, patients will often require airway reconstruction and concurrent repair of their cardiac anomalies, as both often require a median sternotomy. 3-D printing of complex airway anomalies can act as an invaluable resource in the planning process. In this case it allowed for improved discussions between the surgical teams in planning surgical approach as well as helping to educate care providers and family members. In order to increase the intraluminal diameter, a side-to-side anastomosis from the left sided trachea and the portion of the right bronchus distal to the stenotic segment was undertaken. Due to the rarity of congenital bronchial stenosis, little is known about the longterm outcomes of patients with these lesions. To date, the patient continues to do well from a respiratory standpoint without the need for supplementation.
Fig. 2. 1:1 sized 3-D printed model of the airway demonstrating the high right bronchial take off with a long stenotic segment.
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International Journal of Pediatric Otorhinolaryngology 111 (2018) 180–182
R. Georgopoulos et al.
Fig. 4. A. Preoperative imaging showing the malacic opening of the right high bronchus just below the level of the true vocal folds. B 1 month postoperative bronchoscopy showing a healed suture line.
Sources of funding There is no funding or financial incentives for this study. Conflicts of interest There are no conflicts of interest. References [1] S. Kamata, N. Usui, S. Ishikawa, et al., Experience in tracheobronchial reconstruction with a costal cartilage graft for congenital tracheal stenosis, J. Pediatr. Surg. 32 (1997) 54–57. [2] E. Wu, M. Yang, C. Wang, et al., Congenital right intermediate bronchial stenosis with carina trifurcation: successful management with slide tracheobronchial plasty, Ann. Thorac. Surg. 98 (2014) 357–359. [3] W. Beierlein, M.J. Elliott, Variations in the technique of slide tracheoplasty to repair complex forms of long-segment congenital tracheal stenoses, Ann. Thorac. Surg. 82 (2006) 1540–1542. [4] C. Stock, M. Nathan, R. Murray, R. Rahbar, F. Fynn-Thompson, Modified end-to-end anastomosis for the treatment of congenital tracheal stenosis with a bridging bronchus, Ann. Thorac. Surg. 99 (2015) 346–348.
Fig. 5. Postoperative chest x-ray showing improved aeration.
182
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case Report
Congenital right bronchial stenosis with high bifurcation: Successful management with side to side tracheobronchial anastomosis
T
Rachel Georgopoulosa,∗, Courtney Hilla, Jeffrey S. Heinleb, Deepak Mehtaa a b
Department of Pediatric Otolaryngology Texas Children's Hospital, United States Department of Cardiovascular Surgery Texas Children's Hospital, United States
A R T I C LE I N FO
A B S T R A C T
Keywords: Congenital Bronchial Stenosis Bifurcation Double trachea 3D model
Congenital bronchial stenosis is a rare entity [1,2]. While there are some reports of congenital bronchial anomalies and their repair in the thoracic literature this is first report in the literature of a bronchial take off just below the larynx managed with surgical success. Here we present a case of a former 33-weeker born with a tracheoesophageal fistula, Tetralogy of Fallot, and a high bifurcation of the right mainstem bronchus with a concomitant long segment bronchial stenosis. Preoperative planning included printing 3-D reconstructed models of the airway to analyze various treatment options. At 3 months of age the patient was taken to the operating room for surgical repair of the Tetralogy of Fallot and side-to-side tracheobroncheoplasty. The patient was extubated on postoperative day 3 and was discharged home without need for any ventilatory support.
1. Case report A 6 week-old former 33-weeker was transferred to our tertiary care facility with Tetralogy of Fallot, tracheoesophageal fistula repaired at the outside hospital, and a known congenital airway anomaly. On the first day of the life the patient underwent echocardiography which revealed Tetralogy of Fallot with a left-sided arch. On day two of life, he was taken to the operating room for repair of the tracheoesophageal fistula. Due to persistent issues with intermittent desaturations and hemoptysis, the patient underwent a CT chest which revealed a right bronchial take off just below the level of the true vocal folds. The right mainstem bronchus had a 22 mm segment of stenosis with concomitant hyperinflation of the right lung (Fig. 1). The patient when transferred was having significant problems with desaturation as a result of right lung hyperinflation when on continuous positive airway pressure. In light of this, he was weaned to high flow nasal canula and did significantly better. A 3-D printed model of the airway was created, and its utility was three-fold (Fig. 2). The model was used for preoperative surgical planning, and to enhance discussions about the patient in a multidisciplinary fashion. The model was invaluable for discussing the diagnosis and treatment with the patient's family and health care providers.
∗
Corresponding author. 6701 Fannin Suite D.0640.00, Houston TX, 77030, United States. E-mail address: [email protected] (R. Georgopoulos).
https://doi.org/10.1016/j.ijporl.2018.06.011 Received 24 April 2018; Received in revised form 8 June 2018; Accepted 9 June 2018 Available online 12 June 2018 0165-5876/ © 2018 Elsevier B.V. All rights reserved.
At 3 months of age, the patient was placed on cardiopulmonary bypass using direct aorto-bicaval cannulation. After completion of the sternotomy and exposure of the airway, a longitudinal incision was made on the coapting surfaces of the left main trachea and the right bronchus distal to the stenotic segment (Fig. 3). These were enlarged using an aortic punch, and then a side-to-side tracheal anastomosis was performed using a running 7-0 PDS suture. After repair of the airway, the remainder of the cardiac procedure was undertaken. The patient was extubated on postoperative day 3 and did well. He was taken back to the operating room for bronchoscopy 1 month later for surveillance, which revealed a well healing suture line and a patent airway (Fig. 4). Postoperative chest x-rays demonstrated improved aeration without airtrapping (Fig. 5). 2. Discussion Congenital bronchial stenosis with a high right bifurcation can be a challenging entity to treat. Long segment bronchial stenosis can lead to air-trapping, hyperinflation of the lung and subsequent respiratory distress. Management of these lesions can be complicated as traditional techniques used to manage airway stenosis such as dilation, cricotracheal reconstruction and slide tracheobronchioplasty, which are not
International Journal of Pediatric Otorhinolaryngology 111 (2018) 180–182
R. Georgopoulos et al.
Fig. 1. CT chest demonstrating the high take off of the right bronchus with significant air trapping.
Fig. 3. Red markings demonstrate the coapting surfaces of the airway that were opened with an aortic punch and a side to side tracheal anastomosis performed. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
possible given the lesions proximity to the vocal folds [2,3,4]. Furthermore, compared to the trachea, the bronchi have a significantly smaller intraluminal diameter making surgical repair more challenging [2]. Of the patients with congenital tracheal or bronchial stenosis described in the literature, all have had associated cardiovascular anomalies [1,2]. In light of this, patients will often require airway reconstruction and concurrent repair of their cardiac anomalies, as both often require a median sternotomy. 3-D printing of complex airway anomalies can act as an invaluable resource in the planning process. In this case it allowed for improved discussions between the surgical teams in planning surgical approach as well as helping to educate care providers and family members. In order to increase the intraluminal diameter, a side-to-side anastomosis from the left sided trachea and the portion of the right bronchus distal to the stenotic segment was undertaken. Due to the rarity of congenital bronchial stenosis, little is known about the longterm outcomes of patients with these lesions. To date, the patient continues to do well from a respiratory standpoint without the need for supplementation.
Fig. 2. 1:1 sized 3-D printed model of the airway demonstrating the high right bronchial take off with a long stenotic segment.
181
International Journal of Pediatric Otorhinolaryngology 111 (2018) 180–182
R. Georgopoulos et al.
Fig. 4. A. Preoperative imaging showing the malacic opening of the right high bronchus just below the level of the true vocal folds. B 1 month postoperative bronchoscopy showing a healed suture line.
Sources of funding There is no funding or financial incentives for this study. Conflicts of interest There are no conflicts of interest. References [1] S. Kamata, N. Usui, S. Ishikawa, et al., Experience in tracheobronchial reconstruction with a costal cartilage graft for congenital tracheal stenosis, J. Pediatr. Surg. 32 (1997) 54–57. [2] E. Wu, M. Yang, C. Wang, et al., Congenital right intermediate bronchial stenosis with carina trifurcation: successful management with slide tracheobronchial plasty, Ann. Thorac. Surg. 98 (2014) 357–359. [3] W. Beierlein, M.J. Elliott, Variations in the technique of slide tracheoplasty to repair complex forms of long-segment congenital tracheal stenoses, Ann. Thorac. Surg. 82 (2006) 1540–1542. [4] C. Stock, M. Nathan, R. Murray, R. Rahbar, F. Fynn-Thompson, Modified end-to-end anastomosis for the treatment of congenital tracheal stenosis with a bridging bronchus, Ann. Thorac. Surg. 99 (2015) 346–348.
Fig. 5. Postoperative chest x-ray showing improved aeration.
182