Long- term outcomes of congenital tracheal stenosis after slide tracheoplasty.

PURPOSE: Long-term outcomes of slide tracheoplasty in patients with congenital tracheal stenosis (CTS) have rarely been reported. This study aimed to clarify the long-term outcomes of CTS after slide tracheoplasty. METHODS: The medical records of 33 patients who underwent slide tracheoplasty for CTS at our institution between January 2005 and July 2018, with a follow-up duration > 5 years, were retrospectively reviewed. Patients' characteristics, perioperative condition, operative management, postoperative course, tracheal stenosis rates and growth data, were collected from medical records. RESULTS: The median operative age, minimum tracheal diameter, length of stenosis, duration of hospital stays, and follow-up duration were 8 months, 2.4 mm, 35 mm, 39 days, and 90 months, respectively. One patient died of bleeding in the right lung at 126 months postoperatively. Among the 10 patients requiring postoperative tracheostomy, seven were successfully decannulated at a median of 65 months postoperatively. Tracheal stenosis rates improved postoperatively and were subsequently maintained. Growth impairment and psychomotor delay were observed in 9 and 16 patients, respectively with significant differences found only in cases with genetic abnormalities and not in tracheal stenosis severity. CONCLUSION: Slide tracheoplasty for CTS leads to favorable long-term outcomes. However, various associated anomalies may influence growth and psychomotor development, emphasizing the importance of adequate support.

[Thoracoscopic tracheal resection in a child under extracorporeal membrane oxygenation]. / Torakoskopicheskaya rezektsiya trakhei u rebenka v usloviyakh ekstrakorporal'noi membrannoi oksigenatsii.

Congenital tracheal stenosis in children is a rare, severe and life-threatening respiratory tract malformation characterized by respiratory failure. We have performed 74 surgical interventions in these patients under extracorporeal membrane oxygenation between 2013 and 2022. In this article, we present surgical treatment of a newborn with congenital tracheal stenosis. For the first time in the world, the patient underwent thoracoscopic resection and reconstruction of the trachea under extracorporeal membrane oxygenation.

Management Strategies for Congenital Heart Disease Comorbid with Airway Anomalies in Children.

OBJECTIVE: To assess management strategies for pediatric patients with the challenging combination of congenital heart diseases (CHDs) and airway anomalies. STUDY DESIGN: Patients diagnosed with CHD and airway anomalies in the Pediatric Cardiac Surgery Centre of Fuwai Hospital from January 2016 to December 2020 were included in this retrospective study. Patients were divided into three groups based on different management, including the conservative group, the slide group (slide tracheoplasty), and the suspension group (suspension with external stenting). Patients' data and computed tomography measurements from medical records were reviewed. RESULTS: A total of 139 patients were included in the cohort; 107 had conservative airway treatment (conservative group), 15 had slide tracheoplasty (slide group), and 17 had tracheal suspension operation (suspension group). The top three associated intracardiac anomalies were ventricular septal defect (n = 34, 24%), pulmonary artery sling (n = 22, 16%), and tetralogy of Fallot (n = 15, 11%). Compared with patients with conservative airway management (100 minutes [median], 62-152 [IQR]), the extra airway procedure prolonged cardiopulmonary bypass duration, with 202 minutes (IQR, 119-220) for the slide group and 150 minutes (IQR, 125-161) for the suspension group. Patients who underwent slide tracheoplasty required prolonged mechanical ventilation (129 minutes [median], 56-328 [IQR]). Of the total cohort, 6 in-hospital deaths, all in the conservative group, and 8 mid-to long-term deaths, with 6 in the conservative group, occurred. CONCLUSIONS: Both conservative and surgical management of CHD patients with airway anomalies have promising outcomes. Extra tracheobronchial procedures, especially the slide tracheoplasty, significantly prolonged cardiopulmonary bypass duration. Based on multidisciplinary team assessment, individualized management strategies should be developed for these patients.

Congenital nasal pyriform aperture stenosis; our experience of 34 cases.

OBJECTIVES: To study our population of patients with congenital nasal pyriform aperture stenosis (CNPAS) in terms of incidence and socioeconomic status; the effect of pyriform aperture size, gestational age, birth weight, and whether congenital abnormalities are associated with surgical requirement. METHODOLOGY: Retrospective case note review of all patients treated for CNPAS at a single tertiary paediatric referral site was undertaken. Diagnosis was made on the basis of a pyriform aperture of <11 mm on CT scanning; patient demographics were collected to explore risk factors for surgery and surgical outcomes. RESULTS: 34 patients were included in the series, 28 (84%) of whom underwent surgery. 58.8% of subjects had an associated mega central incisor. A smaller pyriform aperture size was seen in neonates requiring surgery (4.87 mm ± 1.24 mm vs 6.55 mm ± 1.41 mm, p = 0.031). There was no difference in gestational age in neonates requiring surgery (p = 0.074). Requirement for surgery was not associated with co-existing congenital anomalies (p = 0.297) or lower birth weight (p = 0.859). Low socioeconomic status was not significantly associated with requiring surgery but a potential link between CNPAS and deprivation was identified (p = 0.0583). CONCLUSION: These results suggest that a pyriform aperture of less than 6 mm requires surgical intervention. Associated birth anomalies add additional management considerations but in this cohort were not associated with increased need for surgery. A potential association between CNPAS and low socioeconomic status was identified.

Impact of Tracheal Arborization and Lung Hypoplasia in Repair of Pulmonary Artery Sling in Combination With Long-Segment Tracheal Stenosis.

Background: Reimplantation of the left pulmonary artery (LPA) and slide tracheoplasty has been our standard approach of care for patients with pulmonary artery sling (PAS) and tracheal stenosis. We present our experience, with emphasis on tracheal arborization and hypoplastic lungs; and their impact on long-term outcome of children with PAS and tracheal stenosis. Methods: It is a retrospective comparative study. Data were classified and analyzed based on the type of tracheobronchial arborization and normal versus hypoplastic lungs. Results: Seventy-five children operated between January 1994 and December 2019 (67 with normal lungs and 8 with lung hypoplasia/agenesis) were included. Patients with hypoplastic lungs had higher rates of preoperative ventilation (87.5%), postoperative ventilation (10 vs 8 days, P = .621), and mortality (50% vs 9%, P = .009) compared with those with normal lungs. Nineteen patients had tracheal bronchus (TB) variety and 30 patients had congenital long-segment tracheal stenosis (CLSTS) variety of tracheobronchial arborization. Endoscopic intervention was needed in 47.4% of patients with TB type and 60% with CLSTS type. CLSTS patients had higher rates of preoperative ventilation (60% vs 47.4%, P = .386), longer periods of postoperative ventilation (13 vs 6.5 days, P = .006), and ICU stay (15 vs 11 days, P = .714) compared with TB type. Conclusion: Surgical repair of PAS with tracheal stenosis has good long-term outcomes. All variations of tracheal anatomy can be managed with slide tracheoplasty. Persistence of airway problems requires intervention during follow-up as tracheal stenosis continues to be the Achilles heel.

Surgical management of congenital tracheal stenosis associated with complex cardiovascular anomalies.

PURPOSE: Although surgical outcomes have improved in patients with congenital tracheal stenosis (CTS), the management of such patients with complex cardiovascular anomalies (CVAs) remains a challenge. This study aimed to clarify the clinical features of this combination. METHODS: Medical records of 14 patients with complex CVAs who had undergone slide tracheoplasty for CTS between May 2016 and February 2022 were retrospectively reviewed. Complex CVAs were defined as CVAs without left pulmonary artery sling and simple cardiac shunts. Data collected included age and body weight at tracheal reconstruction, preoperative respiratory support, surgery details, and mortality. RESULTS: The median age and body weight at tracheal reconstruction were 3.5 (range, 1-17) months and 4.7 (range, 2.3-8.2) kg, respectively. Simultaneous repair of CTS and CVAs was performed in eight patients and staged repair in six patients. Patients who underwent simultaneous repair required preoperative respiratory support more frequently than those who underwent staged repair (8/8 [100%] vs 2/6 [33.3%]; P = 0.015). No mortality occurred. CONCLUSIONS: Patients with complex CVAs often require tracheal reconstruction in early infancy. Surgical management strategy for this combination was generally decided on the basis of the severity of respiratory symptoms due to CTS.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Evaluation and management of congenital tracheal stenosis.

OBJECTIVES: To outline an expert-based consensus of recommendations for the diagnosis and management of pediatric patients with congenital tracheal stenosis. METHODS: Expert opinions were sought from members of the International Pediatric Otolaryngology Group (IPOG) via completion of an 18-item survey utilizing an iterative Delphi method and review of the literature. RESULTS: Forty-three members completed the survey providing recommendations regarding the initial history, clinical evaluation, diagnostic evaluation, temporizing measures, definitive repair, and post-repair care of children with congenital tracheal stenosis. CONCLUSION: These recommendations are intended to be used to support clinical decision-making regarding the evaluation and management of children with congenital tracheal stenosis. Responses highlight the diverse management strategies and the importance of a multidisciplinary approach to care of these patients.

Successful surgical treatment of congenital tracheal stenosis combined with tracheal bronchus and left pulmonary artery sling: a 10-year single-institution experience.

PURPOSE: Radical surgery for congenital tracheal stenosis (CTS) is technically demanding. CTS combined with tracheal bronchus (TB) and pulmonary artery (PA) sling is a particularly challenging condition. We herein report our successfully modified surgical techniques for CTS combined with TB and PA sling. METHODS: Nine patients treated at our institution from July 2010 to December 2020 for CTS with TB and PA sling were enrolled. The patients' characteristics, operative results, and clinical outcomes were reviewed and analyzed retrospectively. RESULTS: The mean age at the operation and body weight were 8.0 ± 4.4 months old and 6.5 ± 0.8 kg, respectively. The mean tracheal diameter and length of the stenotic lesion were 3.2 ± 1.0 mm (mean stenosis rate 46.2%) and 25.4 ± 4.9 mm, respectively. All cases were complicated with PA sling at bifurcation stenosis with tracheobronchomalacia. All patients underwent modified posterior-anterior slide tracheoplasty with an inverted Y-shaped incision at the bifurcation and repositioning of the PA. The mean postoperative intubation period was 25.0 ± 32.1 days. There were no major intraoperative or postoperative complications, including hypoxic-ischemic encephalopathy. The mean hospital stay was 92.2 ± 73.4 days. All patients were discharged home without tracheostomy or oxygen support. CONCLUSION: Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes. LEVEL OF EVIDENCE: Level IV.

Airway performance in infants with congenital tracheal stenosis associated with unilateral pulmonary agenesis: effect of tracheal shape on energy flux.

Congenital tracheal stenosis (CTS) with unilateral pulmonary agenesis (UPA) is characterized by the absence of one or both lungs in the hemithorax and is often associated with airway distortion. Some UPA patients have high mortality and morbidity even postoperatively, and it remains unclear whether surgery increases the energy flux needed to drive airflow. Here, we used pre- and postoperative patient-specific airway models to numerically investigate tracheal flow in patients with CTS, especially flow associated with right UPA (CTS-RUPA). Airflow was simulated with the large-eddy model, and energy flux was investigated to quantify airway performance and the contribution of surgical intervention. Although energy flux decreased postoperatively, clinical respiratory status did not improve. Standard surgical intervention for CTS, which expands the minimal cross-sectional area, decreased energy flux, i.e., improved airway performance. The simulation also included artificial airways with a straightened bend or reduced tracheal lumen roughness. The numerical results clearly showed interindividual differences in the percent reduction of energy flux caused by straightening the tracheal bend versus correcting tracheal lumen roughness. Although this study was limited to small sample size, these numerical results indicated that energy flux alone is insufficient to evaluate breathing performance in patients with CTS-RUPA but it can be used to estimate airway performance.

Outcomes of slide tracheoplasty for congenital tracheal stenosis in 80 children: A 22-year single-center experience.

PURPOSE: The application of slide tracheoplasty (STP) in the treatment of congenital tracheal stenosis (CTS) has improved patient outcomes over the past few decades. We reviewed our experiences with the procedure, elucidated risk factors, and discussed important aspects of perioperative management to improve outcomes. METHOD: Patients with CTS undergoing STP between July 1998 and December 2020 were enrolled. Patient characteristics, perioperative condition, management, operative details, and outcomes, including mortality and postoperative intervention, were collected from medical records. RESULTS: Eighty patients underwent STP. Sixty-five patients (81.3%) had an associated cardiovascular anomaly. Thirteen patients (16.3%) had unilateral lung agenesis or hypoplasia. Preoperative mechanical ventilation was necessary in 54 (67.5%) patients, and extracorporeal membrane oxygenation (ECMO) was required in eight patients (10.0%). An endotracheal tube was placed before the stenotic entrance to avoid granulation. During STP, the trachea was dissected as little as possible to maintain the blood supply. The one-year survival rate was 88.8% (nine patients died). One patient (1.3%) required postoperative balloon dilation, and none required stenting or granulation removal. Of the survivors, 62 (92.5%) achieved successful extubation without tracheostomy. Multivariable analysis revealed complex cardiovascular anomaly (P = 0.05) and preoperative ECMO (P = 0.019) to be adverse predictors of survival. CONCLUSION: Although STP can be performed successfully in CTS patients, surgeons and families should be aware of factors that may lead to a more difficult postoperative course or increase the mortality. Meticulous, perioperative positioning of the endotracheal tube and preserving the tracheal blood flow can minimize the need for postoperative intervention.

Surgical outcomes of pulmonary artery sling and congenital tracheal stenosis with right lung anomaly.

OBJECTIVES: This study aimed to investigate whether computed tomographic indices support surgical decision-making in patients with pulmonary artery sling, congenital tracheal stenosis and right lung underdevelopment. METHODS: A total of 38 patients with pulmonary artery sling and congenital tracheal stenosis underwent tracheoplasty. Patients were divided into 3 groups based on anatomical categorization: group normal lung (NL; n = 21), group H (right lung hypoplasia, n = 12) and group A (right lung agenesis or aplasia, n = 5). Using preoperative computed tomographic images, the severity of the tracheal bending due to the right posterior deviation of the aortic arch and the relative length of the left pulmonary artery for reimplantation was evaluated. RESULTS: Slide tracheoplasty posterior to the aortic arch with pulmonary artery reimplantation was performed in 32 patients (21, 10 and 1 in groups NL, H and A, respectively). Slide tracheoplasty anterior to the aortic arch was performed in 4 patients (2 patients each in groups H and A). Among the 6 patients with severe tracheal bending who underwent slide tracheoplasty posterior to the aortic arch, 5 required aortopexy for tracheomalacia. The overall mortality rate was 3% (group NL, n = 1). The relative length of the left pulmonary artery in group H (0.85) was significantly shorter than that in group NL (1.36, P < 0.0001). CONCLUSIONS: In patients with right lung underdevelopment, preoperative computed tomography elucidated the tracheal bending due to right posterior deviation of the aortic arch, which compromised tracheoplasty and shortness of the left pulmonary artery for pulmonary artery sling repair.

Tracheal Reconstruction for Congenital Tracheal Stenosis: A 950-Gram Neonate.

Congenital tracheal stenosis is a rare but life-threatening malformation of the trachea. Surgical reconstruction is high risk, and not frequently performed in neonates born of extreme prematurity and low birth weight. We present the case of an extremely premature 950-gram neonate with severe congenital tracheal stenosis who underwent tracheal reconstruction. Complete repair, with no residual stenosis, was achieved with slide tracheoplasty without the need for cardiopulmonary bypass.

[Surgical tracheoplasty for children with congenital tracheal stenosis undergoing previous balloon dilatation or tracheal metal stent placement: a series of 9 cases].

Objective: To examine the outcomes of Slide tracheoplasty for the children with severe congenital tracheal stenosis received previous repeated balloon dilatation or metal stent placement under endoscopy. Methods: A retrospective study was conducted in 9 children with congenital tracheal stenosis undergoing previous interventional therapy under tracheoscopy and later received Slide tracheoplasty due to obvious respiratory symptoms at Department of Cardiac Surgery, Qilu Children's Hospital of Shandong University between February 2017 and July 2021. There were 7 males and 2 females with a median age at operation of 72.4 months (range: 13.3 to 98.9 months), and the median weight was 19.0 kg (range: 9.0 to 33.0 kg). Among the 9 patients, 2 patients began to receive repeated balloon dilatation (more than 3 times) 17.8 and 51.8 months ago respectively. One patient received metal stents placement into the trachea for 4 days and the other 6 children for median 56.8 months (range: 21.6 to 74.2 months). Complete tracheal cartilage rings and long segmental stenosis were present. in all 9 children. Operative details and outcome measures, including the need for endoscopic airway intervention and mortality, were collected. Results: Slide tracheoplasty was performed in all cases. Two patients with repeated balloon dilatation had different thickness of tracheal wall, local scar hyperplasia and irregular lumen. Among them, 1 case had obvious local calcification of tracheal wall, which was difficult to suture. The metal stent in one patient with short time of placement was completely removed. However, only part of the metal stents could be removed due to the long placement time in the other 6 cases. There was no operative death in the 9 children. The median postoperative tracheal intubation time was 25.3 hours (range: 17.4 to 74.5 hours). A silicone stent was placed in the trachea of 1 child due to obvious respiratory symptoms. Follow-up of median 11 months (range: 1 to 23 months) showed that no death occurred after discharge and all children had basically normal activity tolerance with no obvious respiratory symptoms. Conclusions: Slide tracheoplasty is feasible for children undergoing prior balloon dilatation or metal stents placement. Previously repeated balloon dilatation or metal stent placement under endoscopy increased the difficulty of slide tracheoplasty, the metal stent could not be completely removed after a long time.

Surgical management of long-segment congenital tracheal stenosis with tracheobronchial malacia.

OBJECTIVES: Slide tracheoplasty has become the mainstream treatment for long-segment congenital tracheal stenosis (LSCTS). However, technical improvements are still needed to improve the clinical outcomes of patients exhibiting LSCTS with tracheobronchial malacia. METHODS: LSCTS patients who underwent tracheoplasty from January 2010 to December 2020 were reviewed. According to the time of surgical technique modifications for reconstructing a supportive carina, the patients were divided into 2 groups: group A (2010-2018) and group B (2019-2020). We identified a well-balanced cohort matched by propensity score to evaluate the differences in surgical outcomes between the 2 groups. RESULTS: There were no significant differences between group A and group B in any of the 8 characteristics before and after propensity score matching. In the propensity score-matched cohort, the number of patients who accepted anterior carina tracheopexy (75/77 vs 4/77, P < 0.001) and in situ pericardium insertion (75/77 vs 14/77, P < 0.001) in group B was significantly greater than that in group A. The mechanical ventilation time [48.3 (interquartile range: 29.6, 116.3) h vs 73.3 (interquartile range: 47.9, 111.6) h, P = 0.009] and cumulative mortality (P = 0.023) were significantly lower in Group B than Group A. CONCLUSIONS: Reconstructing a supportive, stable carina of the neotrachea and tracheobronchopexy are helpful to improve the outcomes of slide tracheoplasty.

Anomalous Origin of the Right Pulmonary Artery From the Aorta and Congenital Tracheal Stenosis: Staged Repair in a Neonate.

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA), sometimes referred to as hemitruncus, is a rare malformation. We report a unique case of AORPA associated with Ebstein's anomaly and with congenital tracheal stenosis due to complete tracheal rings. The AORPA and tracheal stenosis were both successfully corrected in the neonatal period.

Surgical management strategy of slide tracheoplasty for infants with congenital tracheal stenosis.

OBJECTIVE: The study objective was to evaluate the outcomes of slide tracheoplasty in infancy and identify predictors of adverse outcomes. METHODS: We retrospectively reviewed the clinical data of infants aged less than 1 year with congenital tracheal stenosis who underwent slide tracheoplasty at a single center from April 2010 to September 2020. RESULTS: Of 120 infants, 71.7% (86/120) had a pulmonary artery sling and 37.5% (45/120) had simultaneous intracardiac repairs. Additionally, 52.5% (63/120) of the patients had anomalous tracheobronchial arborization, and 17.5% (21/120) had diffuse tracheal stenosis. Six airway reoperations (5%) and 6 deaths (5%) occurred, and the mortality decreased annually. Multivariate analysis revealed that a low body weight, cardiovascular anomalies, and normal tracheobronchial arborization predicted a longer intubation duration. Univariate analysis revealed that a low body weight, preoperative invasive ventilation, a long cardiopulmonary bypass time, and granulation tissue were associated with death. After surgery, 26 patients had dysphagia, 24 of whom resumed oral feeding during follow-up. Ninety-two patients underwent chest computed tomography reexamination, and the trachea diameter had increased significantly from 2.32 ± 0.72 mm to 5.46 ± 1.24 mm. Nineteen and 29 patients underwent spirometry before and after surgery, respectively, and showed improvements in ventilation function, with the ratio of time to peak tidal expiratory flow to total expiratory time and ratio of volume to peak tidal expiratory flow to total expiratory volume values significantly improved from 19.80% (interquartile range, 16.90-23.80) and 23.10% (interquartile range, 21.10-25.90) to 26.80% (interquartile range, 21.20-34.40) and 30.20% (interquartile range, 25.00-34.50), respectively (P < .05). CONCLUSIONS: A tailored individual management strategy of slide tracheoplasty in infancy facilitates favorable clinical outcomes. Close postoperative follow-up and long-term functional evaluations including clinical symptoms and pulmonary function are still needed.