| Congenital airway malformations are rare diseases with unknown pathogenesis in children.Lower airway malformations include bronchial tree malformation,as well as trachea malformations such as stenosis and malacia.According to the deformed lumen and severity,clinical manifestations vary greatly from stridor,wheeze,nosiy breathing to recurrent respiratory infections.Severe cases develop tachypnea and cynosis postmortem,sometime that can be life threatened.Part of congenital airway malformation combined with congenital heart disease,vascular ring malformation and digestive system malformation,or a part of the syndrome.Due to the lack of specificity of clinical manifestations of these diseases,they are often misdiagnosed as asthma,recurrent respiratory infection,laryngitis,laryngeal malacia,resulting in delayed treatment for some patients.In addition,the prognosis of different types of airway deformities is very different due to the location,degree of deformities and complications.So early diagnosis and treatment is very important.Bronchoscopy is the gold standard for the diagnosis of airway malformation.Severe tracheobronchial malacia(TBM)can cause respiratory failure requiring endotracheal intubation repeatedly even death.There is no mature surgical experience for TBM in China.Airway stents can improve the ventilation,is minimally invasive option for the treatment of severe TBM.This study is divided into two parts.The part I is a retrospective study of children with congenital lower airway malformation confirmed by bronchoscopy from January 2012 to December 2020.The diagnosis,clinical symptoms.,age,imaging,complications(congenital heart disease or digestive system malformation),treatment status and shortterm follow-up results were analyzed.The clinical phenotypic characteristics of various types of lower airway malformation were analyzed.The part II discusses the effectiveness and safety of airway stenting in the treatment of 44 children with congenital severe tracheobronchomalacia.All the children who were diagnosed with severe congenital tracheobronchomalacia and underwent airway stents in our hospital were selected to review their inpatient medical records to investigate the efficacy,safety and prognostic factors of endotracheal stent implantation in the treatment of severe tracheobronchomalacia.The improvement of clinical symptoms,survival time and complications of the children after stentplacement were followed up,and clinical data were summarized and analyzed.Part ? Clinical characteristics of congenital lower airway malformation in children Objective To retrospectively analyze the clinical characteristics of 1052 children with congenital lower airway malformation.Methods The datas of lower airway malformation were searched with keywords as"mirror bronchial tree,tracheal diverticulum,tracheomalacia,tracheoesophageal fistula,bronchial bridge,bronchial malacia,tracheal bronchial stenosis and tracheal bronchus"in hospital endoscopy system.Then,through the case system,the clinical data of chief complaint,onset age,sex,pregnancy,clinical phenotype,bronchoscopy,combined malformations,imaging examination and complications were collected.The collected datas were retrospectively analyzed using Oracle 19c+Navicat Premium+human flesh intelligence system.Result1.Clinical data of 1052 children with congenital lower airway malformation were collected in this retrospective research,including single airway malformation and compound airway malformation.The number of cases of airway malformation was systematically searched.The detection rates of lower airway malformation from high to low were trachea and bronchomalacia in 420 cases(39.92%),trachea and bronchus in 401 cases(38.11%),trachea and bronchial stenosis in 386 cases(36.69%),trachea and esophageal fistula in 165 cases(15.68%)and bronchial bridge in 45 cases(4.28%).,43 cases(4.09%)of tracheobronchial diverticulum,7 cases(0.67%)of mirror bronchial tree,6 cases(0.57%)of congenital tracheal cartilage ring loss,9 cases(0.86%)of bronchial atresia,3 cases(0.29%)of high bifurcation trachea,Tracheal atresia with tracheoesophageal fistula in 1 case(0.1%),congenital tracheobiliary fistula in 1 case(0.1%),and traffic-induced bronchopulmonary foregut malformation in 1 case(0.1%).2.The sex ratio in this group was 1.37 1.Tracheobronchomalacia,tracheobronchial stenosis and bridging bronchus were common types of lower airway obstruction,common clinical manifestations were wheezing,shortness of breath,dyspnea,laryngeal stirdor and recurrent respiratory infection,among which the fatal malformation was tracheoatresia.There were no specific clinical symptoms in tracheobronchi and tracheobronchial diverticulum,and most of the mirror bronchial tree transposition combined with visceral transposition cilia immobility and other genetic abnormalities.Tracheoesophageal fistula is a common type of trachea with digestive tract malformation.Bronchoesophageal fistula,tracheobiliary fistula and trafficbronchopulmonary anterior bowel malformation were also been selected.3.Among the 420 children with tracheobronchomalacia,153 cases(36.43%)were primary tracheobronchomalacia,including 112 cases complicated with tracheoesophageal fistula(TEF)and 10 cases complicated with laryngeal cleat.73 cases were complicated with laryngomalacia,10 cases with bronchopulmonary dysplasia.31 cases were isolated tracheomalacia.Secondary tracheobronchomalacia in 207 cases(49.29%),with 145 cases of congenital heart disease and 50 cases of vascular ring malformation,including 33 double aortic arch cases,right aortic arch with left subclavian artery in 5 cases,,anonymous artery compression syndrome every 4 cases;There were 5 cases of bronchogenic cyst,1 case of esophageal cyst,2 cases of thoracic cavity occupying,4 cases of vertebral and thoracic deformity.Another 60 cases could not be clearly classified due to incomplete cardiac ultrasound and chest CTA.4.Among the 386 cases of congenital tracheobronchial stenosis,56.2%of the children with tracheobronchial stenosis and 77.78%of the children with bronchial bridging bronchus were complicated with congenital heart disease or vascular malformation,and the most common types were pulmonary artery sling,patent ductus arteriosus,ventricular septal defect and atrial septa 1 defect.Slide tracheoplasty was performed in 79 children with congenital tracheal stenosis,71 cases survived and 8 cases died(total mortality 10.1%),which was an effective treatment.5.There were 165 cases of TEF,including 1 case of type ?(0.61%),1 case of type ?(0.61%),93 cases of type ?,48 cases of type ? A(29.09%),45 cases of type ? B(27.27%),3 cases of type ?(1.82%),24 cases of type ?(14.55%),and the rest could not be classified.The median diagnosis time of type ? was 0-1 day after birth,while the median diagnosis age of type ? tracheoesophageal fistula was 56 days(5-2190 days).According to T-test,the diagnosis age of type V tracheoesophageal fistula was significantly older than that of other types(P=0.011).6.In TEF group,the proportion of patients with tracheomalacia was 61.82%.T test was performed on the trachea length and tracheomalacia length of children with type? A and Type ?B esophageal atresia whild P value was 0.308,showing no statistical difference between the two groups.The length of tracheomalacia between the two groups was significantly different(P=0.001).Relevance ratio of laryngeal cleft was 9.09%in TEF group.Conclusion1.The most common types of congenital lower airway malformations in children include tracheobronchomalacia,tracheobronchia,tracheobronchial stenosis and tracheoesophageal fistula,with clinical manifestations of wheezing,shortness of breath and laryngeal stridor.2.Congenital tracheobronchomalacia,secondary to congenital heart disease and vascular ring malformation is the most common type,followed by primary tracheobronchomalacia combined with tracheoesophageal fistula.3.Congenital tracheobronchial stenosis is closely related to cardiovascular malformations.56.2%of children with tracheobronchial stenosis and 77.78%of children with bronchial bridge were complicated with congenital heart disease or vascular malformations,and the most common types were pulmonary atery sling,patent ductus arteriosus,ventricular septal defect and atrial septal defect.Surgery is an effective method to treat congeTnital tracheal stenosis.3.The common types of congenital tracheoesophageal fistula in this group were type ?and type ?.The diagnosis age of type ? was significantly higher than that of other types through statistics of the diagnosis age of all types,suggesting that type ?tracheoesophageal fistula was easy to be misdiagnosed.4.Laryngeal cleft and tracheomalacia are the most common respiratory malformations associated with tracheoesophageal fistula.In this study,the incidence of laryngeal cleft was 9.09%,and the proportion of tracheomalacia was 61.82%.The length of tracheomalacia segment was related to the classification,and the length of tracheomalacia segment in type ? A was larger than that in type ? B.Part? Effective reserch of 44 children with severe congenital tracheobronchomalacia treated by airway stentingObjective To investigate the efficacy and safety of airway stenting in the treatment of congenital severe tracheobronchomalacia in children.Methods From August 2012 to June 2021,44 children diagnosed with severe congenital trachea and bronchomalacia in our hospital were selected and treated with endotracheal stent placement.The inpatient medical records,bronchoscopy diagnosis and treatment data,cervical and chest CT,cardiac ultrasound and other imaging data were reviewed,and the weight and age of the children were recorded in detail.The location and degree of tracheobronchomalacia,complications,respiratory status before stent placement,mechanical ventilation time,stent placement and decannulation time,clinical symptom improvement and survival time after treatment were followed up,and the clinical data were summarized and analyzed.Forty-four children were divided into three groups according to different complications,one group was secondary to cardiovascular malformation;The second group was complicated with esophageal atresia and tracheoesophageal fistula.Three groups were primary bronchomalacia excluded cardiovascular malformation and tracheoesophageal fistula.For survival analysis among groups,Kaplan-Meier curve was drawn,and log-rank test was performed for comparison between groups.COX regression analysis was used to explore the risk factors affecting decannulation and survival.Results1.A total of 44 children were enrolled,including 31 males and 13 females,with a median age of 3.5m(1-84m)and a median weight of 4.5kg(2.9-18.5kg)at the time of stent implantation.Before stenting,34 patients required tracheal intubation mechanical ventilation,3 patients required continuous positive pressure ventilation(CPAP),7patients required nasal catheter oxygen inhalation The indications for stenting were decanulation failure in 34 cases,recurrent pneumonia in 3 cases,paroxysm cyanosis in 3 cases and dyspnea in 4 cases.2.44 children were placed with 59 stents,including 39 straight tube silicone stents,1 Montgomery T silicone stents,2 covered metallic stents,and 17 bare metallic stents.The statistical sites of stents were 43 tracheal stents,12 left main bronchial stents,3 right main bronchial stents and 1 right intermediate bronchial stents.3.Among the 34 children with decanulation failure,24 cases were successfully removed endotracheal tube after stent placement,,3 cases had recurrent endotracheal intubation,and 7 cases were failed to decanulation.3 cases of dyspnea and 6 cases of paroxysmal cyanosis were effectively.Of the 3 children with recurrent pneumonia,2 were effective,and the other one had improved after stent implantation,but the stent needed to be removed due to repeated displacement who received surgery subsequently.The total effective rate was 84.1%and the ineffective rate was 15.9%.4.Among 59 stents implanted in this group,45(76.3%)had stent-related complications,and 35(59.3%)had minor complications,including secretion retention(10.1%),mild granulation hyperplasia(27.1%),stent malapposition(16.9%),and 10 cases(16.9%)presented serious complications,6 cases of stent displacement(10.1%),which all occurred in silicone stent group,3 cases of hemoptysis(5.1%),1 case of gastrointestinal bleeding(1.7%),and 1 case of granulation hyperplasia affecting ventilation(1.7%).There were 2 cases of stent-related fatal hemoptysis(3.4%),all of which occurred in patients with vascular ring malformation.5.By June 2021,35 of 59 stents were removed,including 30 silicone stents and 5 metal stents,while 24 stents were not removed including 10 silicone scaffolds,2 covered metallic stents and 12 bare metallic stents.Among them,25 patients survived after all the stents were removed.The median time of stenting was 6.2m(0.6-24m).Seven patients survived with stents,and the median follow-up time after stenting was 61m(3106m).Eleven children died without stent removal,and one died during follow-up after stent removal.6.Kaplan-Meier survival curve showed that the survival rate between groups was group? The three groups,primary tracheomalacia combined with EA-TEF group had the highest survival rate.The survival rate of silicone stent group was better than that of metal stent.But log-rank tests showed there is no significant difference between those groups.Cox risk regression model analysis showed that the risk factors affecting the decanulation included carina involvement,TBM and preoperative ventilation time,while the risk factors affecting survival included carina involvement,long-segment tracheomalacia and upper segment tracheomalacia.Conclusion1.In this clinical study,airway stent implantation was applied in the treatment of severe tracheobronchomalacia,with a total effective rate of 84.1%,which is an effective minimally invasive treatment.2.Complications occurred in 76.3%of stents during the follow-up period,among which 59.3%were minor complications and 16.9%were serious complications.Stent displacement occurred in the silicone stent group,while hemoptysis occurred in patients with vascular ring malformation.3.Cox risk regression indicated that carina involvement was a risk factor affecting decanulation and survival,long segment tracheomalacia and upper segment tracheomalacia affected the survival rate of children,patients with TBM and preoperative ventilation time increased postoperative weaning time,but did not affect the survival rate of children.Research SignificanceCongenital lower airway malformation is one of the important causes of wheezing,recurrent lower respiratory tract infection and dyspnea in children.It is often missed and misdiagnosed due to lack of specificity and insufficient understanding of clinical manifestations.The clinical manifestations of more than 1000 patients of different types were included in this paper,which is a group of data with large sample and varied types in China.Severe tracheobronchomalacia severely affects the survival of children,and the treatment methods include palliative care,surgery and stenting.Aortopexy is the most commonly surgical option in foreign countries,but there is no mature surgical experience in China.Airway stents are minimally invasive interventional techniques that can support the airway to improve ventilation.There is no application evaluation of airway stents in the treatment of congenital tracheobronchomalacia in China,and experience is mainly based on small sample data.This study conducted a single-center retrospective case analysis of the application of airway stenting in 44 children with severe tracheobronchomalacia from 2012 to 2021,and described the effectiveness,safety,prognostic factors and other asp ects.The results showed that the short-term effective rate of stenting in the treatment of severe tracheobronchomalacia was 84.1%.The results of this study provide clinical suggestion for the application of airway stents in children with tracheobronchomalacia. |
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