Tracheal bronchus associated with VACTERL

Tracheal bronchus (TB) associated with VACTERL has not been reported previously. A 5-month-old girl with VACTERL association was ventilator-dependent following surgical closure of a patent ductus arteriosus (PDA). Chest radiographs showed persistent hyperinflation of the right upper lobe. Bronchoscopy showed a laterally displaced right main bronchus with stenosis and malacia of the left main bronchus. Dynamic bronchography revealed a TB associated with tracheal stenosis and malacia below the abnormal bronchus. TB may represent an additional tracheal (T) anomaly in VACTERL association. Dynamic bronchography is a useful diagnostic tool in tracheobronchial anomalies. CONCLUSION: TB may represent an "associated" tracheal (T) anomaly in children with VACTERL association. Bronchoscopy may fail to diagnose an ectopic bronchus. Dynamic bronchography is a useful diagnostic tool in tracheobronchial anomalies.     

先天性血管环12例诊断与治疗分析

摘要目的探讨先天性血管环的临床特征、诊断与治疗。方法回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院诊断为先天性血管环患儿的临床特征、影像学检查、诊断和治疗。结果12例先天性血管环患儿进入分析,男3例,女9例。就诊年龄1个月至21岁,<1岁7例。反复咳喘11例,喂养困难2例,青紫3例,心脏杂音10例。12例超声心动图示主动脉双弓4例、右位主动脉弓伴左位动脉导管未闭1例、肺动脉吊带7例,其中10例伴其他心脏畸形。10例64排CT气道重建示主气管狭窄5例、左主支气管狭窄1例,右侧气管狭窄3例,食管狭窄2例;2例纤维支气管镜示主气管狭窄1例,右主支气管狭窄1例。7例行手术治疗,根据血管环的病理类型选择术式,包括2例切断双主动脉弓中的次弓、1例切断右位主动脉弓伴随的左位动脉导管、4例左肺动脉移植术。1例术后死亡,6例术后3～6个月呼吸道症状逐渐好转,生长发育改善。结论儿童中反复出现原因不明的咳喘等呼吸道症状者,应考虑到先天性血管环的可能,超声心动图联合多排CT可明确诊断,及早手术解除气管狭窄是改善预后的关键。     

Bridging bronchus and posterior left pulmonary artery: a unique association

A 6-month-old female with a lifelong history of respiratory distress became increasingly difficult to manage and required right upper and middle pulmonary lobectomies for worsening emphysema and mediastinal shift. The postoperative course was stormy and confusing and the patient died despite emergency tracheostomy. The autopsy disclosed an anomalous bronchus to the right lower lobe, originating from the left mainstem bronchus. In addition, the left main pulmonary artery was positioned posterior to the left mainstem bronchus. Two other cases of bridging bronchus have been reported, but the association with posterior left pulmonary artery has not been described.     

Congenital lobar emphysema due to a pre-eparterial tracheal bronchus

This report presents a case of congenital lobar emphysema due to a pre-eparterial tracheal bronchus with a slitlike orifice. Resection of the right upper lobe was required because of compression of the surrounding lung. A search of the hospital records found three other cases of aberrant right upper lobe bronchus in the years 1977–1987. All three cases were incidental findings on bronchoscopy.     

Interrupted aortic arch with a right descending aorta and right ductus arteriosus,causing severe right bronchial compression

Summary We report here a patient with interrupted aortic arch and a right descending aorta, in whom the surgical management was complicated by the development of right bronchus compression and unilateral emphysema, due to the presence of the right ductus arteriosus.     

Asthma-like Attacks Resulting from the Isolated Congenital Left Pulmonary Artery Agenesis with Right Main Bronchus Stenosis

A 5-year-old girl with isolated congenital left pulmonary artery agenesis suffered from recurrent attacks of dyspnea and right-sided pneumonia due to the stenosis of the right main bronchus. The division of ligamentum of the ductus arteriosus and suspension of the right pulmonary artery resulted in the disappearance of symptoms. It is notable that the compression of contralateral bronchus by the remaining pulmonary artery can cause respiratory symptoms in patients with isolated unilateral pulmonary artery agenesis.     

纤维支气管镜结合多层螺旋CT检查对无脾综合征伴双侧气管性支气管的评估作用

目的使用多层计算机断层扫描(MSCT)和纤维支气管镜对8例双侧气管性支气管病例进行评估。方法在接受纤维支气管镜检查的1 382例患儿中有8例诊断为双侧气管性支气管,对此8例同时进行MSCT检查。结果 7例患儿内镜及MSCT均诊断为双侧气管性支气管,另1例患儿胸部CT加三维气道重建提示右侧气管性支气管,伴右上肺不张,经支气管镜检查并清理阻塞物后提示为双侧气管性支气管。该8例儿童均诊断为无脾综合征。结论纤维支气管镜结合MSCT检查对无脾综合征合并双侧气管性支气管病变的诊断具有较好的价值,并为复杂心血管畸形纠治中的气道管理提供了保障。     

三维CT对支气管异物诊断价值的临床研究

目的 探讨三维CT在支气管异物诊断和鉴别诊断中的作用.方法 对37例可疑支气管异物的患儿行螺旋CT检查,并进行支气管三维CT重建,通过支气管镜检术、保守及手术治疗的结果 判断三维CT检查结果 的准确性.结果 37例可疑支气管异物的患儿中,X线胸部透视提示支气管异物8例(A组);X线胸部透视不提示支气管异物29例(B组).A组中,CT提示肺内感染5例、先天性肺发育异常-肺叶缺如2例、气管食管瘘1例.B组29例患儿中,CT提示支气管异物25例,经支气管镜检证实其中24例存在植物性异物,1例为气管内肿物;CT不提示支气管异物4例,证实为肺内感染、小儿急性喉炎各2例.手术证实存在植物性异物的24例中,CT显示气管支气管内异物影共14例次,不规则狭窄共6例次;阻塞性肺不张、肺气肿、肺炎共7例次;前两项为直接征象,占74%(20/27),后三项为间接征象,占26%(7/27).结论 三维CT检查对支气管异物的诊断及鉴别诊断有重要意义,有助于疑难病例的诊断和鉴别诊断.     

Spontaneous regression of left upper lobe emphysema after division of large ductus arteriosus in an infant

Congenital lobar emphysema is cause of infantile respiratory distress and diagnosed by lobar overaeration, mediastinal shift and/or compression of the adjacent lobe. However, concomitant congenital heart disease with this clinical condition is not uncommon, and there is no uniform consensus about the treatment strategy in these particular cases. We present a child who was treated with division of large patent ductus arteriosus without the lobectomy.     

Tracheal bronchus: association with respiratory morbidity in childhood

An aberrant right upper lobe (RUL) bronchus arising from the trachea (tracheal bronchus) can be responsible for recurrent pneumonia. In this hospital, 2% of children requiring bronchoscopy for respiratory symptoms are found to have a tracheal bronchus, which is frequently thought to be an incidental finding. We reviewed findings in 18 patients to determine when a tracheal bronchus is of clinical significance. The age at presentation ranged from 1 day to 54 months (mean 17 months). The children had recurrent pneumonia (nine), stridor (six), respiratory distress (two) and a thoracic mass (one). Other congenital abnormalities were present in 14, including Down syndrome (two), tracheoesophageal fistula (two), and fused or hypoplastic first and second ribs (four). Recurrent RUL pneumonia was present in five. Bronchiectasis or bronchial stenosis was shown by bronchography in four of five; in all five the right upper lobe was surgically resected, with resolution of the recurrent pneumonias. The presence of a clinically significant tracheal bronchus should be considered in every child with recurrent RUL pneumonia, especially in children with Down syndrome or rib abnormalities; if bronchiectasis or bronchial stenosis is found, surgical resection should be performed.     

Acute Respiratory Distress Secondary to Severe Compression of the Left Main Bronchus by the Ductus Arteriosus

Two newborn infants presented with acute respiratory distress. In both cases, the left lung was opaque, hyperinflated, and associated with a rightward shift of the mediastinum. A diagnosis of retained fetal fluid secondary to vascular compression of the left bronchus by the ductus arteriosus was made by combining various imaging methods including chest radiograph, computed tomography (CT), and echocardiography. Although the initial chest radiographs were similar, the mechanisms of obstruction were different. The imaging emphasizes the importance of CT angiography to understanding the three-dimensional relationships resulting in bronchial compression.     

Selective intubation in pulmonary interstitial emphysema: experience in five patients

Abstract Selective bronchial intubation can be useful in the management of severe localized pulmonary interstitial emphysema (PIE). Five infants between 9 and 20 days old with severe left sided PIE had the right main bronchus intubated for between 4 to 30 h. Four patients showed permanent and one patient temporary improvement and the experience from these cases suggests that prolonging the procedure after obtaining radiological evidence of improvement may not be necessary. There were no serious complications although right upper lobe atelectasis occurred in three cases and could not be prevented by using an endotracheal tube with a side hole. Nevertheless, active management should only be considered in PIE causing respiratory failure or pressure effects on surrounding structures.     

Coil Embolization Therapy in Congenital Coronary Arterial Fistulas

Three pediatric patients (8&frac12; years, 3 years, and 1 month) presented with congenital coronary arterial fistulas. In all cases the fistulas entered into the right side of the heart (main pulmonary artery, n = 1; right ventricle, n = 2). In the first patient, the fistula and an open ductus arteriosus were closed during the same intervention. The second patient presented with a single left coronary ostium and residual shunt from the coronary artery system to the right ventricle after surgery. The third child had pulmonary atresia with intact ventricular septum and a fistula from the left coronary artery to the right ventricle. The fistulas in all patients were managed with coil occlusion. Fistula occlusion was documented with angiocardiography.     

Congenital thrombotic occlusion of the ascending aorta and the aortic arch

Summary In a one-day-old male newborn with severe heart failure, the skin of the upper right thorax was pink, whereas the remaining areas were cyanotic. No peripheral pulses were palpable and the blood pressure could not be measured. On cardiac catheterization, systolic and diastolic pressures were elevated in the left ventricle (137/4/12 mmHg), but in the descending aorta, reached via a patent ductus arteriosus, the pressure was only 55/45 mmHg. O₂ saturation was 97% in the left ventricle and 67% in the descending aorta. Angiocardiography showed an extreme obstruction of the ascending aorta and the aortic arch. The infant died on the second day. Postmortem examination revealed a wall-adherent calcified thrombus that totally occluded the lumen. No etiologic explanation could be obtained from the histologic examination, anamnestic data, or clinical findings.     

Large patent ductus arteriosus and interventricular communication associated with congenital absence of the pulmonary valve

A case of pulmonary valve agenesis with ventricular septal defect and large patent ductus arteriosus is reported in a neonate. Heart failure occurred at 10 days of life; clinical examination showed continuous murmur. Echocardiography and angiocardiography visualized a major dilatation of the pulmonary artery. At cardiac catheterization there was an important left to right shunt through a large ductus, and pulmonary hypertension. There was also marked pulmonic regurgitation. Because of respiratory distress, ligation of ductus arteriosus was performed at 5 weeks of life. Five years later the patient is still in good condition. Patent ductus arterious is rarely associated with pulmonary valvular agenesis and ventricular septal defect. Our case is not explained by the fetal circulation theory described in this heart malformation.     

Truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle

Summary The association of truncus arteriosus with congenital left heart abnormalities is rare. We describe a case of truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle. In 11 other patients diagnosed with truncus arteriosus, none had left heart hypoplasia. Despite the truncal root being primarily or entirely committed to the right ventricle, more right than left heart abnormalities have been found. This is in contrast to double-outlet right ventricle in which left heart abnormalities are more often associated.     

Double right tracheal bronchus. A case report in an infant

An apparently unique case of double right tracheal bronchus supplying the whole right upper lobe is described in a 12-month-old infant presenting with a right paratracheal opacity, persisting cough, and ventricular septal defect. The two tracheal bronchi, initially discovered on tomography, were confirmed by tracheobronchography, which demonstrated also the absence of other upper lobe branches. At surgery, the upper lobe was atelectatic, and its blood supply was abnormal.     

Patent ductus arteriosus in preterm neonates

Failure of the ductus arteriosus to close within 48–96 hours of postnatal age results in a left to right shunt across the ductus and overloading of the pulmonary circulation. This is more likely to happen in premature neonates with respiratory distress syndrome. Deterioration in the respiratory status on day 3–4 in a ventilated neonate and unexplained metabolic acidosis may be the earliest indicators of a patent ductus arteriosus (PDA). Indomethacin is the main stay of medical management of PDA in preterm neonates. Guidelines for administration of indomethacin have been described in the protocol. Restricted fluid therapy may be beneficial in the prevention of PDA in preterm neonates. Presence of PDA in a term neonate should be investigated to rule out an underlying congenital heart disease.     

Patent ductus arteriosus in preterm neonates

Failure of the ductus arteriosus to close within 48–96 hours of postnatal age results in a left to right shunt across the ductus and overloading of the pulmonary circulation. This is more likely to happen in premature neonates with respiratory distress syndrome. Deterioration in the respiratory status on day 3–4 in a ventilated neonate and unexplained metabolic acidosis may be the earliest indicators of a patent ductus arteriosus (PDA). Indomethacin is the main stay of medical management of PDA in preterm neonates. Guidelines for administration of indomethacin have been described in the protocol. Restricted fluid therapy may be beneficial in the prevention of PDA in preterm neonates. Presence of PDA in a term neonate should be investigated to rule out an underlying congenital heart disease.     

Lobar emphysema complicating hyaline membrane disease

A pre-term infant with severe hyaline membrane disease (HMD), requiring intubation and mechanical ventilation, who developed bronchopulmonary dysplasia (BPD), which was followed by lobar emphysema in the right lower lobe is presented. Surgical removal of the right lower lobe led to prompt improvement in the oxygen requirements and subsequent recovery of the infant's respiratory distress. Pathology revealed marked stenosis of the right lower lobe bronchus and air-trapping, without destruction of alveoli. The lobar bronchus contained adequate cartilage, but deficient muscle at the site of stenosis. It seems likely that this bronchial stenosis, and resultant lobar emphysema was not congenital, but was a complication of HMD, BPD or therapy.