Congenital Tracheal Stenosis Research Articles

Updates on diagnosis and treatment of tracheal stenosis in infants

Airway stenosis is the narrowing of airway lumen caused by congenital or acquired factors, resulting in respiratory and vocal dysfunction in infants.In recent years, the application of fiber bronchoscopy in NICU and PICU improve the diagnosis in infants with congenital tracheal stenosis significantly.With the increased survival of very low birth weight and very early premature infants, tracheal stenosis associated with intubation has become increasingly populated.With the advancement of surgical approach and the utilization of fiber bronchoscopy, the survival and prognosis of the airway stenosis in infants make a huge promotion, which was considered deadly in the past.This article reviews progress in the diagnosis and treatment of airway stenosis in infants. Key words: Airway stenosis in infants; Fiber bronchoscopy; Endoscopic balloon dilation; Endobronchial stents; Tissue-engineering trachea

Pediatric airway surgery.

Pediatric airway surgery is a challenging field in pediatric surgery. Laryngotracheal stenosis has a variety of congenital and acquired conditions that require precise assessment and tailored treatment for each individual patient. About 90% of acquired conditions are represented by subglottic stenosis (SGS) resulting as a complication of tracheal intubation. Congenital tracheal stenosis (CTS) is a rare and life-threatening malformation, usually associated with complete tracheal rings along a variable length of the trachea. Tracheomalacia (TM) is a process characterized by flaccidity of the supporting tracheal cartilage, widening of the posterior membranous wall, and reduced anterior-posterior airway caliber. The clinical presentation can vary from almost asymptomatic patients to near fatal airway obstruction. There is considerable variation in both the morphologic subtypes and the prognosis of pediatric airway. The patients are divided into three clinical groups (mild, moderate, and severe). A further division was proposed according to the presence or absence of associated anomalies. The definitive diagnosis of pediatric airway was made by means of rigid bronchoscope and computed tomography scan with three-dimensional reconstruction (3D-CT). Rigid bronchoscopy and 3D-CT confirmed the diagnosis in all the cases. Other associated anomalies include congenital heart disease, vascular anomalies, and BPFM (maldevelopment of aerodigestive tract). After definitive diagnosis of pediatric airway lesions, surgical intervention should be considered. Surgical strategy was presented on each lesion.

The role of adjunctive procedures in reducing postoperative tracheobronchial obstruction in single lung patients with congenital tracheal stenosis undergoing slide tracheoplasty

PurposeCongenital tracheal stenosis (CTS) and a single right lung treated with slide tracheoplasty (ST) has relatively high rates of mortality and morbidity. We report a single institution's experience with adjunctive procedures at the time of ST to reduce postoperative tracheal obstruction in patients with a single right lung and CTS. MethodsWith IRB approval, 8 patients with a single right lung and CTS who underwent ST in our institution between 2008 and 2016 were reviewed. ResultsSeven of 8 patients (88%) survived, with a median follow-up period of 35 (3–89) months. The only mortality was because of complications unrelated to the airway. Six of 8 patients underwent ST anterior to the aortic arch (tracheal translocation, TT). This was combined with tracheopexy concomitantly in 3 patients and subsequently in one patient. Two patients had insufficient tracheal length for TT. Both underwent aortopexy. Of three patients undergoing TT with tracheopexy concomitantly, two have been successfully extubated, and a third has required tracheostomy for subglottic stenosis. All patients undergoing TT without tracheopexy have experienced severe tracheobronchomalacia. ConclusionWe conclude that adjunctive TT with tracheopexy might be associated with lower rates of tracheobronchial obstruction in patients with a single right lung undergoing ST for CTS. Level of evidenceLevel IV.

Congenital tracheal stenosis challenging the management of patent ductus arteriosus in an infant

Congenital tracheal stenosis (CTS) has an incidence between 0.3 and 1% of all laryngotracheal stenosis [1]. In about 84% of such cases, it is associated with cardiac, skeletal or oesophageal anomalies. The associated cardiac anomalies include ventricular septal defect, atrial septal defect, patent ductus arteriosus, atrioventricular canal defect and pulmonary artery sling [2]. The diagnosis of CTS is based on high degree of suspicion in infants with respiratory distress. Classically, infants have a stridor that is exacerbated by lower respiratory tract infection and present with intermittent cyanosis, leading to reflex apnoea or dying spells. A 1 mm decrease in the cross-sectional area (CSA) of the airway results in 44% decrease in CSA. Infants become symptomatic only when the decrease in CSA is more than 50% and become dyspnoeic when the CSA decreases by more than 75% [3]. We present the hospital course of an infant who presented with CTS and a large patent ductus arteriosus. The present case is reported with parental consent.

先天性気管狭窄を合併した Gross C 型食道閉鎖症の1例

先天性気管狭窄を合併した Gross C 型食道閉鎖症の1例

先天性気管狭窄症に対するslide tracheoplasty : 周術期管理の諸問題

先天性気管狭窄症に対するslide tracheoplasty : 周術期管理の諸問題

Bridging bronchus, type six, as a new rare case of a bronchial anomaly.

In 1976, Gonzales-Crussi et al. (Am. J. Dis. Child. 130:1015–18, 1976) introduced the first case of bridging bronchus as a rare bronchial branching anomaly; since then, only 14 worldwide cases was described. We suggest our case might be number 15 and could be the first case of type six of this bronchial anomaly. We present a case of a 10-month-old infant with bridging bronchus, congenital tracheal stenosis, and double outlet right ventricle who underwent major laparoscopic surgery for repair of gastrointestinal anomalies to raise awareness of this rare underdiagnosed congenital anomaly and a thorough discussion of the tracheobronchial anomalies and its clinical implications.

Congenital tracheal stenosis associated with left pulmonary artery sling accompanied by tracheal diverticula: A case report

Abstract We present the case of an eleven-year-old boy with history of stridor since birth with suspicion of tracheal stenosis due to a vascular ring. CT-evaluation revealed a pulmonary artery sling (PAS) and complete tracheal rings with a long segment funnel type tracheal stenosis. Histopathological evaluation additionally revealed a septum within the stenotic part dividing the trachea into a stenotic left part and a right part which turned out to only be an evagination like a small diverticulum. The long segment tracheal stenosis was opened in the middle and an anterior-posterior tracheal sliding plasty was performed after resecting the diseased part of the obstructive cartilage. The patient was extubated on the third postoperative day and the stridor disappeared immediately. To the best of our knowledge, this is the first report of tracheal stenosis with a pulmonary artery sling accompanied with tracheal diverticulum.

A case of pulmonary artery sling where onset Was induced by therapeutic hypothermia

A neonate with severe neonatal asphyxia was treated with therapeutic hypothermia. He developed hypothermia-induced respiratory deterioration, after which congenital tracheal stenosis and pulmonary artery sling were diagnosed. Even low-grade hypothermia is likely to induce bronchial narrowing in neonates, especially in neonates with congenital respiratory tract anomalies. Congenital tracheal stenosis represents a potential pitfall in differential diagnosis and should be carefully ruled out in cases of bronchial narrowing episode induced by therapeutic hypothermia. Pediatr Pulmonol. 2017;52:E7-E10. © 2016 Wiley Periodicals, Inc.

Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis.

The aim of this study was to determine the appropriate surgical intervention strategies for congenital tracheal stenosis (CTS) associated with a tracheal bronchus based on the location of stenosis. The medical records of 13 pediatric patients with CTS associated with a tracheal bronchus at a single institution between January 2006 and December 2015 were retrospectively reviewed. Type 1: tracheal stenosis above the right upper lobe bronchus (RULB) (n=1). One patient underwent slide tracheoplasty and was successfully extubated. Type 2: tracheal stenosis below the RULB (n=7). Tracheal end-to-end anastomosis was performed before 2014, and one patient failed to extubate. Posterior-anterior slide tracheoplasty was performed since 2014, and all three patients were successfully extubated. Type 3: tracheal stenosis above the RULB to the carina (n=5). One patient underwent posterior-anterior slide tracheoplasty and was successfully extubated. Two patients with left-right slide tracheoplasty and another two patients with tracheal end-to-end anastomosis for the stenosis below the RULB could not be extubated. Tracheal end-to-end anastomosis or slide tracheoplasty can be selected for tracheal stenosis above the RULB according to the length of stenosis. Posterior-anterior slide tracheoplasty appears feasible for tracheal stenosis below the RULB or above the RULB to the carina.

Rapid Expansion of Human Epithelial Stem Cells Suitable for Airway Tissue Engineering

Stem cell-based tracheal replacement represents an emerging therapeutic option for patients with otherwise untreatable airway diseases including long-segment congenital tracheal stenosis and upper airway tumors. Clinical experience demonstrates that restoration of mucociliary clearance in the lungs after transplantation of tissue-engineered grafts is critical, with preclinical studies showing that seeding scaffolds with autologous mucosa improves regeneration. High epithelial cell-seeding densities are required in regenerative medicine, and existing techniques are inadequate to achieve coverage of clinically suitable grafts. To define a scalable cell culture system to deliver airway epithelium to clinical grafts. Human respiratory epithelial cells derived from endobronchial biopsies were cultured using a combination of mitotically inactivated fibroblasts and Rho-associated protein kinase (ROCK) inhibition using Y-27632 (3T3+Y). Cells were analyzed by immunofluorescence, quantitative polymerase chain reaction, and flow cytometry to assess airway stem cell marker expression. Karyotyping and multiplex ligation-dependent probe amplification were performed to assess cell safety. Differentiation capacity was tested in three-dimensional tracheospheres, organotypic cultures, air-liquid interface cultures, and an in vivo tracheal xenograft model. Ciliary function was assessed in air-liquid interface cultures. 3T3-J2 feeder cells and ROCK inhibition allowed rapid expansion of airway basal cells. These cells were capable of multipotent differentiation in vitro, generating both ciliated and goblet cell lineages. Cilia were functional with normal beat frequency and pattern. Cultured cells repopulated tracheal scaffolds in a heterotopic transplantation xenograft model. Our method generates large numbers of functional airway basal epithelial cells with the efficiency demanded by clinical transplantation, suggesting its suitability for use in tracheal reconstruction.

Clinical equivalency of cardiopulmonary bypass and extracorporeal membrane oxygenation support for pediatric tracheal reconstruction.

Pediatric surgery for congenital tracheal stenosis continues to be a therapeutic challenge, and it often requires cardiopulmonary bypass (CPB) or extracorporeal membrane oxygenation (ECMO) for intra-operative extracorporeal cardiorespiratory support. The purpose of this study was to compare the peri-operative outcomes of CPB with ECMO in pediatric tracheal reconstruction. Forty-three consecutive patients who underwent tracheal reconstruction without intra-cardiac repair for congenital tracheal stenosis at Kobe Children's Hospital between January 2000 and August 2012 were enrolled in this retrospective study. They were divided into two groups according to intra-operative extracorporeal cardiopulmonary support [CPB (n=17) or ECMO (n=26)]. Peri-operative variables were compared between the two groups. The CPB and ECMO groups had similar patient and operative characteristics. However, the CPB group required larger priming volume and higher doses of total heparin injection. Although the ECMO group exhibited less peri-operative bleeding and lower red blood cell requirement, there were no statistically significant differences between the two groups. Compared to the ECMO group, patients in the CPB group had significantly less positive fluid balance during surgery and in the first 24h after surgery, and exhibited a trend towards higher ratios of PO2 to the fraction of inspired oxygen and lower PCO2 at the time of ICU admission and on post-operative day 1. Pediatric tracheal reconstruction should be performed with intra-operative CPB or ECMO, after considering the advantages of utilizing each extracorporeal cardiorespiratory support type according to the patient's specific condition and situation.

Surgery for Congenital Tracheal Stenosis in Children

Surgery for Congenital Tracheal Stenosis in Children

Emergency management for congenital tracheal stenosis with endoluminal stenting in pediatric intensive care units.

The objective of this study was to share our experience in the management of congenital tracheal stenosis (CTS) using self-expanding intraluminal stents in infants. From January 2010 to August 2012, 31 infants with CTS treated using stents by bronchoscope in pediatric intensive care units (PICUs) were recruited for this study. Among the 31 patients, 17 were male and 14 were female with their ages ranging from 55 days to 22 months. CTS was associated with congenital heart disease (CHD) in 22 patients. There were no immediate stent-related airway complications. A significant and immediate improvement of respiratory obstruction following stent placement in all 31 patients led to successful weaning from the breathing machine and extubation. Granulation tissue formation as a complication was observed in three (9.7%) patients, but all were successfully managed using cryotherapy. During follow up (up to 24 months), stents in 29 infants remained functional. In seven cases, stents were retrieved under bronchoscope (the same day to 10 months), and three cases required stent replacement (each of the three cases had the stent replaced three times). Nine infants died after stenting due to other causes and two infants abandoned treatment. Airway stenting may provide an important therapeutic option in infants with CTS.

Congenital tracheobronchial stenosis

Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Successful management of this complex condition requires a highly individualized approach delivered by an experienced multidisciplinary team, which is best delivered within centralized units with the necessary diverse expertise. In such settings, surgical correction by slide tracheoplasty has become increasingly successful over the past 2 decades such that long-term survival now exceeds 88%, with normalization of quality of life scores for patients with non-syndrome-associated congenital tracheal stenosis. Careful assessment and planning of treatment strategies is of paramount importance for both successful management and the provision of patients and carers with accurate and realistic treatment counseling.

The association of congenital tracheobronchial stenosis and cardiovascular anomalies

ObjectivesCongenital tracheobronchial stenosis (CTBS) is a rare disorder characterized by the presence of focal or diffuse complete tracheal or bronchial cartilage rings resulting in a fixed lumen narrowing. The aim of this study was to expose the association of various cardiovascular (CV) anomalies with various types of CTBS. MethodsA retrospective review of 58 patients who had bronchoscopically proven CTBS between 1997 and 2011 was conducted. Totally, 48 cases who had received echocardiography, computed tomography and other examinations including cardiac catheterization were enrolled. There were 33 boys and 15 girls, and the median age of diagnosis was 4 months (0–44 months). The image findings of associated CV anomalies were reviewed and analyzed, along with the clinical manifestations and patients’ outcome. ResultsThere were 14 generalized hypoplasia (29.2%), 10 funnel type (20.8%), 15 segmental type (31.3%), and 9 isolated bronchial stenosis cases (18.7%). Among 48 cases of CTBS, 32 patients (66.7%) had various CV anomalies including atrial septal defect (n=9), pulmonary artery sling (n=7), ventricular septal defect (n=7), patent ductus arteriosus (n=6), tetralogy of Fallot (n=3), peripheral pulmonary stenosis (n=3), etc. No significant difference existed in the incidence and types of comorbid CV anomalies among 4 types of CTBS, except that funnel type had significantly higher comorbidity of pulmonary artery sling (50%, p<0.01). ConclusionPatients with CTBS had high incidence of various associated CV anomalies. Isolated bronchial stenosis had similar high comorbidity of CV anomalies as did the 3 classical categories of congenital tracheal stenosis. The higher comorbidity of pulmonary artery sling in patients with funnel CTBS might be related to its gradual caudal narrowing of the trachea.

Cardiopulmonary Bypass (CPB) in Low-birth Weight Premature Neonate with Double Aortic Arch, Congenital Heart Disease and Tracheal Stenosis

Introduction: Prenatal diagnosis of aortic arch anomalies associated with critical tracheal stenosis is challenging and management is related to high morbidity and mortality. We present successful surgical treatment of low-birth weight premature neonate with vascular ring, congenital heart disease and tracheal stenosis.

Advances in Surgical Treatment of Congenital Airway Disease

Tracheobronchomalacia (TBM) is frequently present in infants and children with congenital heart disease (CHD). Infants with CHD and TBM appear to do worse than those without TBM. The principle of operative intervention for TBM is to improve function of the airway and clinical status. When indicated, conventional surgical options include tracheostomy, aortopexy, tracheoplasty, and anterior tracheal suspension. There is no consensus on the optimal treatment of severe tracheobonchomalacia, which can be associated with a mortality rate as high as 80%. Congenital tracheal stenosis is also frequently associated with CHD (vascular rings, atrioventricular canal defects, and septal defects) and may require concomitant repair. Repair of tracheal stenosis is often associated with distal TBM. This article addresses new techniques that can be performed in corrective surgery for both TBM and congenital tracheal stenosis.

Evolution of Surgical Approaches in the Management of Congenital Tracheal Stenosis: Single-Center Experience.

Congenital tracheal stenosis (CTS) is a serious and rare condition that often presents a complex surgical challenge. We reviewed the evolution of surgical approaches to the management of CTS at a single institution. We conducted a retrospective clinical review of all patients who underwent surgical repair of CTS at our institution. From January 1992 to January 2014, a total of 30 patients (median age 92 days; range 3 days-1.7 years) underwent surgery for CTS at our institution. Techniques included slide tracheoplasty (n = 16), tracheal resection (n = 10), pericardial patch tracheoplasty (n = 3), and costal cartilage tracheoplasty (n = 1). Twenty-two (73%) patients had associated intracardiac or great vessel anomalies, including 18 (60%) with left pulmonary artery sling. Five (17%) patients had associated single lung malformation. All procedures were performed via a median sternotomy with cardiopulmonary bypass. Median postoperative length of stay was 25 days (range, 5-431 days). Late airway reintervention was required in seven (26%), including two (15%) infants after slide tracheoplasty, two (22%) posttracheal resection, and two (100%) post pericardial patch tracheoplasty. The one patient who underwent costal cartilage tracheoplasty required multiple reinterventions. Overall mortality was 13% (n = 4), two deaths occurred post slide tracheoplasty, one death occurred after tracheal resection, and one after pericardial patch tracheoplasty, respectively. Slide tracheoplasty is the procedure of choice for repair of CTS, while tracheal resection is a viable option for patients with discreet, short-segment stenosis.

Surgical management of congenital tracheal stenosis associated with tracheal bronchus and congenital heart disease.

Congenital tracheal stenosis (CTS) is variable in patients with tracheal bronchus and congenital heart disease (CHD). Tracheoplasty remains a high-risk surgical treatment. From January 2007 to December 2014, 24 CTS patients (10 males and 14 females; age 20.6 ± 13.6 months) with tracheal bronchus and CHD underwent one-stage surgical correction. Clinical features of all patients included dyspnoea, or recurrent pulmonary infections. There was long-segment CTS in 13 cases (54%), and 4 cases were associated with a bridging bronchus. Less than 50% of normal tracheal size was identified in 21 cases. Complete tracheal or bronchial rings were identified in all cases. Operative techniques included: tracheal end-to-end anastomosis in 11 cases and slide tracheoplasty in 13 cases, which included 11 cases of right upper lobe bronchus (RULB) opposite side-slide tracheoplasty. There were 2 operative deaths, due to postoperative tracheomalacia or residual main bronchial stenosis. The duration of postoperative hospital stay was 7-59 days, with an average of 19 days. Follow-up was completed in 19 cases. The follow-up duration was from 1 month to 7 years. Tracheal granulation occurred in 1 case. Clinical symptoms were significantly improved in the remaining patients. Tracheal bronchi have special anatomical features. The techniques of tracheal end-to-end anastomosis or side-slide tracheoplasty can be used to correct tracheal stenosis with satisfactory outcomes.