We present an unusual case of vascular sling, tracheal stenosis by complete cartilaginous ring, and aberrant left subclavian artery with right aortic arch that underwent successful surgical repair for the sling. These abnormalities were suspected from unusual multiple indentations found on esophagogram. Complete preoperative diagnosis was established with chest computerized tomogram combined with angiography.
Aorta, Thoracic/abnormalities* ; Case Report ; Child ; Human ; Male ; Pulmonary Artery/abnormalities* ; Subclavian Artery/abnormalities* ; Tracheal Stenosis/diagnosis ; Tracheal Stenosis/congenital

Congenital long-segment tracheal stenosis with complex cardiac anomaly has generally been regarded as a fatal disease. This report described the successful concomitant repair of unexpected congenital tracheal stenosis and complex cardiac anomaly with the use of cardiopulmonary bypass. The patient was a 3-month-old girl with coarctation of aorta, VSD, and PDA. The presence of tracheal stenosis was not discovered until when difficulty with endotracheal intubation was encountered at operating room. Thus, we decided concomitant repair of both lesions and performed anterior pericardial tracheoplasty combined with one stage repair of coarctation of aorta, VSD, and PDA under the cardiopulmonary bypass. The patient is doing well without any signs of complication at present, 2 years and 1 month after the operation.
Aortic Coarctation ; Cardiopulmonary Bypass ; Female ; Heart Defects, Congenital ; Humans ; Infant ; Intubation, Intratracheal ; Operating Rooms ; Tracheal Stenosis*

Pulmonary artery sling is a rare congenital condition in which the left pulmonary artery arises anomalously fmm the right pulmonary artery extrapericardially and encirdes the right main stem bronchus and passes between the trachea and esophaghus to reach the hilum of the left lung. Respiratory obstruction vesults from compression of the lower area of trachea and right main stem bronchus. It is rare cause of respiratory distress in the new bom and may be associated with significant morbidity and mortality. We experienced the case of pulmonary artery sling with diffuse trachea stenosis and congenital heart disease (pulmonary atresia and PDA) and confirmed the anomaly by bronchogram and MRI. The presumed cause of death was respiratory obsruction due to pulmonary artery sling.
Bronchi ; Cause of Death ; Constriction, Pathologic ; Heart Defects, Congenital ; Lung ; Magnetic Resonance Imaging ; Mortality ; Pulmonary Artery* ; Trachea ; Tracheal Stenosis*

Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; complications ; Humans ; Infant ; Infant, Newborn ; Male ; Tracheal Stenosis ; diagnosis ; therapy

OBJECTIVETo retrospectively analyze the experience of one-stage surgical correction for children with congenital heart diseases and tracheal stenosis and further to clear the principle of treatment for these patients.

METHODSFrom January 2006 to June 2013, 48 patients with congenital heart diseases and tracheal stenosis underwent surgical correction. There were 36 male and 12 female patients. The mean age at operation was (23 ± 27)months (range: 3-72 months) and the mean weight was (12 ± 8) kg (range: 3.4-46.0 kg). The underlying diagnoses were pulmonary sling in 33 patients, double aortic arch in 3, tetrology of Fallot in 6, ventricular septal defect in 4, double outlet right ventricle in 1, and pulmonary atresia in 1 patient. Among them, short tracheal stenosis was present in 15, long tracheal stenosis in 25 and bridging bronchus in 8 patients. Repairs with autogenous tracheal tissue were performed in 6, and end-to-end anastomosis in 11 and slide tracheoplasty in 31 patients. Two patients had granulation tissue growing in the airway postoperatively and were re-operated by autogenous rib tissue. All of patients were followed up after operation 1, 3, 6, 12 months and if the patient had symptoms that should be examined by bronchoscopy. The patients should be examined by CT post-operation one year.

RESULTSThere were 7 deaths in all 48 cases and the early mortality was 14.6%. Two deaths were not related to tracheal stenosis, and other 5 were associated with long segment tracheal stenosis. Forty-one patients were followed for 2 months to 6 years, and no patients required re-operations. Clinical symptoms of tracheal stenosis disappeared and the results of CT scan were satisfied.

CONCLUSIONSOne stage surgical repair of the patients with congenital heart diseases and tracheal stenosis have a good effect. Slide tracheoplasty is the effective surgical method for long segment tracheal stenosis. Postoperative granulation tissue growing in the airway is the leading cause of death.

Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; complications ; surgery ; Humans ; Infant ; Male ; Retrospective Studies ; Tracheal Stenosis ; complications ; surgery

BACKGROUND: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. MATERIAL AND METHOD: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF& pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). RESULT: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. CONCLUSIONS: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.
Angiography ; Arteries ; Bronchi ; Constriction, Pathologic ; Coronary Vessels ; Diagnosis ; Echocardiography ; Heart Defects, Congenital ; Humans ; Pulmonary Atresia ; Tomography, X-Ray Computed* ; Tracheal Stenosis

OBJECTIVETo investigate etiology and airway management in children with tracheobronchomalacia.

METHODBronchoscopic examinations were performed in 671 children. The cases with tracheomalacia and bronchomalacia were analyzed in etiopathogenesis and summarized their therapy simultaneously.

RESULTBronchoscopic examination indicated tracheomalacia and bronchomalacia in 148 cases, tracheomalacia in 77 cases and bronchomalacia in 71 cases. Among the cases with tracheomalacia, compression by vascular rings was found in 46 cases, incorporated congenital esophageal atresia with tracheoesophageal fistula was found in 5 cases, tracheomalacia was associated with tracheostoma and mechanical ventilation in 6 cases, with congenital airway malformation in 11 cases and isolated tracheomalacia was found in 4 cases. Among the cases with bronchomalacia, incorporated congenital cardiovascular malformation was found in 64 cases, congenital airway malformation in 6 cases and isolated bronchomalacia in 1 case. Ten children with anomalous innominate artery underwent aortopexy, twelve children with dextro-aorta arch with concomitant aberrant left subclavian artery and double aorta underwent arches vascular ring lysis, six children with pulmonary sling underwent plasty. Severe malacia segments were resected directly in four children during operation. Mechanical ventilation was performed in 38 children. Tracheostoma was performed in 4 children to treat tracheomalacia and bronchomalacia, it could relieve symptom to a certain extent. In 2 children metal stents were inserted into the bronchus for the treatment of bronchomalacia, one was successful and the other needed re-insertion of stent again, these two patients underwent balloon-dilatation in distal part of stent afterwards.

CONCLUSIONThe congenital cardiovascular malformation was the main reason to develop tracheobronchomalacia in children. The symptom of majority of the cases with cardiovascular malformation would be improved within 6 months after surgical intervention. In severe cases, treatments included mechanical ventilation and tracheostoma. Stenting could be applied in refractory cases, but it had certain limitations.

Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; complications ; therapy ; Humans ; Infant ; Male ; Stents ; Trachea ; pathology ; Tracheal Stenosis ; etiology ; therapy ; Tracheobronchomalacia ; diagnosis ; etiology ; therapy

Long-segment tracheal stenosis in infants and small children is difficult to manage and can be life-threatening. A retrospective review of 12 patients who underwent surgery for congenital tracheal stenosis between 1996 and 2004 was conducted. The patients' median age was 3.6 months. All patients had diffuse tracheal stenosis involving 40-61% (median, 50%) of the length of the trachea, which was suspected to be associated with complete tracheal ring. Five patients had proximal bronchial stenosis also. Ten patients had associated cardiac anomalies. Three different techniques were performed; pericardial patch tracheoplasty (n=4), tracheal autograft tracheoplasty (n=6), and slide tracheoplasty (n=2). After pericardial tracheoplasty, there were 2 early and 2 late deaths. All patients survived after autograft and slide tracheoplasty except one who died of pneumonia one year after the autograft tracheoplasty. The duration of ventilator support was 6-40 days after autograft and 6-7 days after slide tracheoplasty. The duration of hospital stay was 13-266 days after autograft and 19-21 days after slide tracheoplasty. Repeated bronchoscopic examinations were required after pericardial and autograft tracheoplasty. These data demonstrate that pericardial patch tracheoplasty show poor results, whereas autograft or slide tracheoplasty gives excellent short- and long-term results.
Body Weight ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Length of Stay ; Male ; Pulmonary Artery/pathology ; Respiratory System Abnormalities/surgery ; Thoracic Surgical Procedures ; Trachea/anatomy & histology ; Tracheal Stenosis/*congenital/*surgery ; Treatment Outcome

Bronchi ; abnormalities ; pathology ; Bronchial Diseases ; complications ; congenital ; diagnosis ; pathology ; Bronchoscopy ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Infant ; Male ; Pneumonia ; etiology ; physiopathology ; Respiratory System Abnormalities ; complications ; diagnosis ; pathology ; Trachea ; abnormalities ; pathology ; Tracheal Stenosis ; etiology ; pathology

We report a case of de novo 7q interstitial deletion detected by conventional karyotyping and by microarray of amniotic fluid sampled during the prenatal period. A 32-year-old pregnant woman was evaluated at our hospital following detection of increased nuchal translucency at 12 weeks and 5 days of gestation. Conventional karyotyping revealed 46,XX,del(7)(q21q22) in 20 interphase mitotic cells, and high-resolution microarray revealed 12.8 Mb (90,625,014-103,430,901) deletion in the region 7q21.13q22.1. Both parents had normal karyotypes. After birth, the neonate displayed several anomalies, including palatine cleft, upslanted and wide palpebral fissure, low-set ears, micrognathia, microcephaly, ventriculomegaly, subglottic tracheal stenosis, hearing loss, and hand/foot deformities, including brachydactyly, polydactyly, and cutaneous syndactyly. This case study helps explain the phenotype-genotype relationship in patients with 7q21.13q22.1 deletion.
Adult ; Amniotic Fluid ; Brachydactyly ; Congenital Abnormalities ; Ear ; Female ; Hearing Loss ; Humans ; Infant, Newborn ; Interphase ; Karyotype ; Karyotyping ; Microcephaly ; Nuchal Translucency Measurement ; Parents ; Parturition ; Polydactyly ; Pregnancy ; Pregnant Women ; Prenatal Diagnosis* ; Syndactyly ; Tracheal Stenosis