Impact of preoperative respiratory distress on outcomes of slide tracheoplasty.

OBJECTIVES: Children with congenital tracheal stenosis born in the developing world face a high risk of mortality due to limited access to proper treatment. Patients who required preoperative respiratory support were suspected to have poor survival after slide tracheoplasty; however, this was not clearly demonstrated in the previous studies. This study aims to investigate the impact of preoperative respiratory conditions on outcomes of slide tracheoplasty. METHODS: From 2016 to 2022, children who underwent slide tracheoplasty were retrospectively reviewed. Patients with respiratory distress requiring emergency operations (group A) were compared with patients in stable condition who were scheduled for surgery (group B). RESULTS: Perioperative results revealed that group A (n = 43) had a longer bypass time (P < 0.001), operation time (P = 0.01), postoperative ventilation time (P < 0.001) and length of intensive care unit stay (P = 0.00125) than group B (n = 60). The early mortality rate was 7.8%, and the actuarial 5-year survival rate was 85.3%. The cumulative incidence test revealed that group A was highly significant for overall mortality [sudistribution (SHR) 4.5; 95% confidence interval (CI) 1.23-16.4; P = 0.023]. Risk factors for overall mortality were prolonged postoperative ventilation time (hazard ratio 3.86; 95% CI 1.20-12.48; P = 0.024), bronchial stenosis (hazard ratio 5.77; 95% CI 1.72-19.31; P = 0.004), and preoperative tracheal mucositis (hazard ratio 5.67; 95% CI 1.51-21.31; P = 0.01). Four patients needed reintervention during a follow-up of 28.4 months (interquartile range 15.3-47.3). CONCLUSIONS: Preoperative respiratory distress negatively affected the outcomes of patients who required slide tracheoplasty. Therefore, early detection of congenital tracheal stenosis and aggressive slide tracheoplasty are crucial and obligatory to enhance long-term survival in this lethal congenital airway disease.

Slide tracheoplasty for congenital tracheal stenosis: A systematic review.

INTRODUCTION: Slide tracheoplasty has become the gold standard surgery for congenital tracheal stenosis (CTS). This condition is rare and the surgery can be challenging and is performed by experienced surgeons in tertiary centers. A few reports involving relatively small cohorts have been published. The aim of this review is to evaluate the post-operative mortality and morbidity of pediatric slide tracheoplasty for CTS. METHODS: A systematic literature review was performed according to PRISMA guidelines. The Medline and EMBASE databases were screened using a search strategy defined in collaboration with a librarian. We included articles reporting the post-operative mortality rate of slide tracheoplasties for treatment of CTS in children, when at least 10 patients were included. RESULTS: A total of 932 articles were reviewed, and 15 studies were eligible with a total of 845 patients. The overall post-operative mortality rate was 9.3 %, and most deaths were airway related. The open revision surgery rate after surgery was 2.8 % and the endoscopic revision rate was 27.6 %. DISCUSSION: This study highlights key factors to consider before the surgery and helps anticipate post-operative follow-up considerations for children with CTS. Several factors were identified as predictors of mortality including young age, weight at the time of surgery and association with lung hypoplasia or aplasia. CONCLUSION: Although slide tracheoplasty has gained popularity in recent years due to better outcomes, it remains a major surgery with mortality risk and the need for multidisciplinary management.

Aerodynamic evaluation of surgical design for the stenosis correction of airway.

Introduction: Congenital tracheal stenosis (CTS) is a rare but life-threatening disease that can lead to respiratory dysfunction in children. Obstructive sleep apnea syndrome (OSAS) in children is characterized by prolonged partial upper airway obstruction and/or intermittent complete obstruction. Both of the diseases require surgical intervention. Although respective treatments of these two diseases are clear, there is a lack of literature discussing the surgical treatment of patients with CTS complicated by OSAS. Methods: We conducted a patient-specific study of patient with CTS complicated by OSAS. Computer-aided design was used to simulate surgical correction under different surgical sequences. Computational fluid dynamics was used to compare the outcomes of different sequences. Results: Aerodynamic parameters, pressure drop, velocity streamlines, wall shear stress (WSS), and the ratio of airflow distribution and energy loss rate were evaluated. An obvious interaction was found between the two diseases in different surgical sequences. The order of correction for CTS or OSAS greatly affected the aerodynamic parameters and turbulence flows downstream of tracheal stenosis and upstream of epiglottis. The CTS and OSAS had mutual influences on each other on the aerodynamic parameters, such as pressure drops and WSS. Discussion: When evaluating the priority of surgical urgency of CTS and OSAS, surgeons need to pay attention to the state of both CTS and OSAS and the physiological conditions of patients. The aerodynamic performance of the uneven airflow distribution and the potential impact caused by the correction of CTS should be considered in surgical planning and clinical management.

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.

Outcomes of not using tracheoplasty in asymptomatic tracheal stenosis found during open-heart surgery.

OBJECTIVES: We aimed to review the outcomes of treating incidentally encountered asymptomatic airway stenosis during open-heart surgery conservatively without the use of tracheoplasty. METHODS: Between January 2002 and October 2022, 25 patients were incidentally diagnosed with tracheal stenosis during open-heart surgery. Intraoperative bronchoscopy and/or laryngoscopy revealed tracheal stenosis; however, this was not consistent with the findings of the preoperative computed tomography. Patients who were diagnosed with a pulmonary artery or vascular sling or had moderate-to-severe respiratory symptoms before open-heart surgery were excluded. RESULTS: The median age and weight of the patients at operation were 3.0 months and 5.1 kg, respectively. They were categorized as those having tracheal stenosis on preoperative computed tomography (n = 12) or not having tracheal stenosis (n = 13). The narrowest diameter was significantly smaller in the former group (3.0 vs 5.8 mm, P < 0.05). The rates of reintubation and the tracheostomy, and intubation days tended to be higher in former group without statistical significance. Stenotic degree improved 2 months and 1 year or more after the operation (39.3% at operation, 28.4% at 2 months, 12.5% after 1 year). All patients were Ross class 1 or 2 at follow-up (mean, 7.1 years). CONCLUSIONS: Patients with tracheal stenosis showed tolerable long-term outcomes without using tracheoplasty. Accordingly, if tracheal stenosis, that would cause intubation difficulty, was incidentally revealed, concomitant tracheoplasty may not be required during open-heart surgery if the stenosis did not cause considerable symptoms or signs preoperatively.

Regeneration Process of an Autologous Tissue-Engineered Trachea (aTET) in a Rat Patch Tracheoplasty Model.

The treatment of long-tracheal lesion is difficult because there are currently no viable grafts for tracheal replacement. To solve this problem, we have developed an autologous Tissue-Engineered Trachea (aTET), which is made up of collagenous tissues and cartilage-like structures derived from rat chondrocytes. This graft induced successful long-term survival in a small-animal experiment in our previous study. In this study, we investigated the regeneration process of an aTET to attain reproducible success. We prepared an aTET by using a specially designed mold and performed patch tracheoplasty with an aTET. We assigned twenty-seven rats to three groups according to the three types of patch grafts used: aTET patches (the aTET group), fresh tracheal autograft patches (the Ag group), or polylactic acid and polycaprolactone copolymer sheets (the PPc group). In each group, gross and histological evaluations were performed at 1 month (n = 3), 3 months (n = 3), and 6 months (n = 3) after implantation. We obtained high survival rates in all groups, but only the PPc group attained thick tracheal walls with granular tissues and no tracheal regeneration. On the other hand, the aTET and Ag groups reproducibly achieved complete tracheal regeneration in 6 months. So, an aTET could be a promising candidate for tracheal regeneration grafts.

[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.

TBX5 pathogenic variant in a patient with congenital heart defect and tracheal stenosis.

Congenital tracheal stenosis is a rare life-threatening disorder caused by narrow O-shaped tracheal ring without smooth muscle. Its underlying genetic cause has not been elucidated. We performed whole exome sequencing in a patient with congenital tracheal stenosis and congenital heart defect, and identified a de novo pathogenic TBX5 variant (NM_181486.4:c.680T>C, p.(Ile227Thr)). The Ile227Thr-TBX5 protein was predicted to have a decreased stability by in silico protein structural analyses, and was shown to have a significantly reduced activity for the NPPA promoter by luciferase assay. The results, together with the expression of mouse Tbx5 in the lung and trachea and the development of tracheal cartilage dysplasia in the lung-specific Tbx5 null mice, imply the relevance of TBX5 pathogenic variants to congenital tracheal stenosis.

Incidence of congenital tracheal stenosis in left pulmonary artery sling diagnosed by bronchoscopy.

PURPOSE: Congenital tracheal stenosis (CTS) has been reported to occur in 50-65% of cases of left pulmonary artery sling (LPAS), but the exact incidence rate is unknown. This study aimed to determine the actual rate using bronchoscopy and to elucidate morphological features in computed tomography (CT) diagnosis. METHODS: We performed a single institutional retrospective review of all patients with LPAS between January 2010 and March 2022. The percentage of complete tracheal rings in patients with LPAS was evaluated using bronchoscopy. The anteroposterior/lateral diameter ratios at the smallest and largest diameters of each CTS patient's trachea were measured on CT. The Wilcoxon signed-rank test was used to analyze the differences between the two parts. RESULTS: Fifty-two patients with LPAS were enrolled. All patients had complete tracheal rings on bronchoscopy. CT analysis of 32 patients with CTS was performed. The median anteroposterior/lateral diameter ratio at the smallest diameter was 1.05 (interquartile range [IQR] 0.95-1.15); the median ratio at the largest diameter was 0.94 (IQR 0.89-0.99). There was a significant difference between the two parts (p = 0.013). CONCLUSION: CTS might be universally associated with LPAS. The circular tracheal cross-section on CT might imply the existence of a complete tracheal ring.

Use of Virtual Reality and Three-Dimensional Printing in the Surgical Planning of Slide Tracheoplasty.

Purpose: To describe our experience using virtual reality (VR) and three-dimensional (3D) printing as complements for the surgical planning process of slide tracheoplasty (ST) in patients with congenital tracheal stenosis (CTS). Description: VR and 3D printing are used for the surgical planning of ST as a therapeutic option in three female patients under five years of age with CTS. Evaluation: We assessed the planned surgical procedure, procedural time, postoperative complications, and outcomes, as well as the main surgeon's experience with the use of the applied technologies. Conclusions: The interaction within the VR environment allowed for collaboration of the surgical plan between surgical staff and enhancement of the radiologist-surgeon communication, while procedural simulation with 3D printing prototypes allowed for refining technical abilities for the surgical interventions. Based on our experience, the application of these technologies have added value to the surgical planning of ST and its outcomes in the treatment of CTS.

Missed bronchial web in a 4-year-old boy with foreign body aspiration: A case report.

Congenital bronchial webs are extremely rare and usually remain undiagnosed due to nonspecific symptoms. Herein, we reported a 4-year-old case of the bronchial web who was initially undiagnosed upon bronchoscopy following foreign body aspiration and afterward misdiagnosed as childhood asthma through his consistent cough and exertional dyspnea for several months.

Computational fluid dynamics assessment of congenital tracheal stenosis.

PURPOSE: The severity of congenital tracheal stenosis (CTS) is commonly evaluated based on the degree of stenosis. However, it does not always reflect the clinical respiratory status. We applied computational fluid dynamics (CFD) to the assessment of CTS. The aim of this study was to evaluate its validity. METHODS: CFD models were constructed on 15 patients (12 preoperative models and 15 postoperative models) with CTS before and after surgery, using the computed tomographic data. Energy flux, needed to drive airflow, measured by CFD and the minimum cross-sectional area of the trachea (MCAT) were quantified and evaluated retrospectively. RESULTS: The energy flux correlated positively with the clinical respiratory status before and after surgery (rs = 0.611, p = 0.035 and rs = 0.591, p = 0.020, respectively). Although MCAT correlated negatively with the clinical respiratory status before surgery (rs = -0.578, p = 0.044), there was not significant correlation between the two after surgery (p = 0.572). CONCLUSIONS: The energy flux measured by CFD assessment reflects the respiratory status in CTS before and after surgery. CFD can be an additional objective and quantitative evaluation tool for CTS.

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.

Simulated slide tracheoplasty for congenital tracheal stenosis using three-dimensional printed models.

PURPOSE: The slide tracheoplasty (STP) is the standard treatment for severe congenital tracheal stenosis (CTS). Understanding the features of the tracheal stenosis in each case and choosing an appropriate incision design are very important for successfully executing the procedure. The present study aimed to evaluate the advantages of three-dimensional (3D) printed models of the trachea for improving CTS. METHODS: Three-D tracheal models were created using computed tomography (CT) data from ten patients undergoing STP for CTS. Simulated surgery was performed using the hollow models after reinforcing with them with a coating of gum spray. Clinical outcomes, including patient survival, postoperative surgical interventions, and time required for STP, were compared with the corresponding values in the last ten patients before the introduction of 3D model simulations. RESULTS: All ten patients for whom simulated surgery using a 3D tracheal model were conducted achieved good airway patency after their STP. The surgeons reported feeling that the 3D model simulations were highly effective although there was no significant difference in the clinical outcomes of the groups with or without simulated STP. The models were useful not only for surgical planning but also for sharing important information among the multidisciplinary team and the patients' family. CONCLUSION: Our experience using 3D tracheal models demonstrated several features enabling improvement in the surgical treatment of CTS.

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.

An Infant With Congenital Tracheal and Bronchial Stenosis Diagnosed by Chest Three-Dimensional Computed Tomography.

In this case report, we describe the case of an infant with repeated wheezing diagnosed relatively early with congenital tracheal and bronchial stenosis after evaluation by chest three-dimensional computed tomography (3D-CT). The patient was a six-month-old male infant with a one-month history of cough and wheezing. His symptoms worsened the day before admission, and he was admitted with pneumonia and wheezing. However, wheezing continued after treatment with intravenous steroids and inhalation of a short-acting ß2-stimulant. 3D-CT of the chest revealed tracheal stenosis, right bronchial stenosis, and right tracheobronchial bronchus. The patient was finally diagnosed with congenital tracheal and bronchial stenosis via bronchoscopy. A virtual bronchoscopic navigation image of the tracheal lumen was created based on the CT images. Although virtual bronchoscopic navigation is more difficult for the dynamic evaluation and evaluation of mucosal lesions than bronchoscopy, it has the advantage of not directly invading the airway. Therefore, if a fixed stenotic lesion is suspected at a facility where bronchoscopy is difficult, evaluation using chest 3D-CT and virtual bronchoscopic navigation may be helpful for diagnosis. In conclusion, congenital tracheal/bronchial stenosis should be considered in patients with prolonged wheezing and recurrent airway infections, and evaluation by chest 3D-CT and virtual bronchoscopic navigation may be helpful for diagnosis.

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.

A case of successful slide tracheoplasty for long-segment congenital tracheal stenosis in a neonate with a congenital diaphragmatic hernia and Fallot's tetralogy.

BACKGROUND: Congenital tracheal stenosis (CTS) is a rare and life-threatening airway disorder, which is often associated with cardiac malformations. Among them, neonatal symptomatic CTS with cardiac malformations has an extremely poor prognosis. In contrast to cardiac malformation, congenital diaphragmatic hernia (CDH) has rarely been associated with CTS. We report a neonatal case in which slide tracheoplasty and intracardiac repair were performed simultaneously for CTS and Fallot's tetralogy (TOF). CASE PRESENTATION: An infant with left CDH and Fallot's tetralogy (TOF) was born by cesarean section at 38 weeks of gestation. At the time of resuscitation, a 2.5 mm (ID) endotracheal tube could only be inserted just below the vocal cords. After repairing the CDH at 3 days of age, planned extubation was performed at 7 days of age. However, the patient required re-intubation due to life-threatening episodes after 2 days of the extubation. Enhanced CT revealed a long segment CTS from the upper trachea to the right bronchus (length of stenosis: 40 mm, minimum inner diameter: 2 mm). At 24 days of age, veno-arterial extracorporeal membrane oxygenation (ECMO) was introduced due to severe respiratory failure. At 28 days of age, slide tracheoplasty and palliative right ventricular outflow tract reconstruction (RVOTR) was performed with cardiopulmonary bypass (CPB). After tracheoplasty, a 3.5 mm tracheal (ID) tube could be placed in the reconstructed trachea in a patient with CTS. ECMO was completed 7 days after the operation. On the 17th day after the operation, he was extubated successfully. He was discharged 5 months after birth with home oxygenation therapy. CONCLUSIONS: We reported the successful simultaneous correction of slide tracheoplasty and palliative RVOTR for a neonate with CDH. ECMO was used for respiratory management before and after surgery.