Clinical Characteristics Of Bridging Bronchus In 87 Children

ObjectiveTo highlight the clinical characteristics data of bridging bronchus(BB)by review the clinical data of this rare disease.MethodsThe clinical data of 87 children with BB who were hospitalized in the Children’s Hospital,Zhejiang University School of Medicine from January 2003 to December 2019 were collected.The characteristics of gender,age distribution,clinical classification,clinical manifestations,other congenital malformations and outcome were analyzed retrospectively.Results1.Among 87 patients,66 were Wells type I(76.9%)and 21 were type II(24.1%)with a median age of 8.97 months(ranged from 4 hours-177 months).87 patients including 44 males,with 37 case of Wells type I and 7 of type II,and the age of diagnosis ranged from 6 days-170 months.And 43 cases were women,including 29 cases of type I BB,14 of type II,and the age of diagnosis ranged from 4 hours-177 months.There was no statistically significant difference between men and women in the age of diagnosis and the incidence of dichotomy.2.Of the 87 children enrolled,the main clinical manifestations were respiratory and cardiovascular symptoms,including cough(74.4%),wheezing(59.8%),anhelation(63.2%),cyanosis(26.4%),cardiac murmur(49.4%)etc.The wheezing rate of Wells type II BB was higher than that of type I(P=0.005).The duration of symptoms after admission ranged from 2 to 42 days.The clinical symptoms of children with BB combined with airway stenosis lasted longer than those without airway stenosis(P=0.002).3.55 patients(63.2%)with airway stenosis were included in 87 cases of BB,including 21 cases of main tracheal stenosis(38.2%),24 cases of bronchial stenosis(43.6%),and 10 cases of main trachea with bronchial stenosis(18.2%).There were 36 cases of Wells type I BB with airway stenosis(54.5%),19 cases of Wells type II BB with airway stenosis(90.5%),and the ratio of airway stenosis in Wells type II BB was higher than that of type I(P=0.002).4.Among 87 children,69(79.3%)were congenital cardiovascular deformity,including 56(81.2%)with congenital heart disease(40 were complex congenital heart disease),13(18.8%)with congenital heart disease and extracardiovascular malformation and 12(13.8%)with normal.BB had congenital heart disease accompanied with airway stenosis in 30 cases(75%),and those 47 case of BB without complex congenital heart disease existed airway stenosis in 25 cases(53.2%).The ratio of airway stenosis in BB combined with complex congenital heart disease was higher than that in BB without complex congenital heart disease(P=0.036).5.Among the 66 patients with Wells type I BB,49(74.2%)were accompanied with congenital heart disease,including 21(42.9%)with complex congenital heart disease.Twenty(95.2%)of the 21 patients with Wells type II BB accompanied with congenital heart disease,including 19 patients with complex congenital heart disease(95.0%).There was no statistically significant difference in the ratio of Wells types BB with congenital heart disease when that with complex congenital heart disease Wells type II was higher than that of Wells type I(P<0.001).6.Among 87 patients,42 cases were treated by surgery while 45 were medical treatment.80 patients were discharged healthy while 7 died,including 5 patients in surgical treatment group and 2 patients in internal treatment group.All seven of the deaths were associated with congenital heart disease,including 6 were complex congenital heart disease.And the main causes of death included low cardiac output syndrome,secondary infection,arrhythmia and automatic abandonment.Conclusion1.BB mostly occurs in infancy,Wells type I is more common,and the age of diagnosis and the proportion of typing are similar among children of different genders2.The clinical manifestations of BB were lack of specificity and similar between the two types.And airway stenosis often occurs in BB patients with Wells type II,complex congenital heart disease,and clinically long-term symptoms of cough,wheezing,and anhelation,etc.3.BB often associated with respiratory and circulatory system malformations.Chest CT is the first choice for diagnosis of BB.The proportion of Wells type II BB with trachea malformation and complex congenital heart disease was higher than that of type I.4.Complicated factors influence the prognosis of BB.Cardiopulmonary failure tends to occur in BB with organ deformities,especially when accompanied with complex congenital heart disease.