Tracheobronchomegaly or Mounier-Kuhn syndrome a is rare disease characterized by marked dilatation of the trachea and main bronchi. It is thought to be due to a congenital anomaly, but is uncertain. It has variable clinical manifestations from causing chronic respiratory infections and bronchiectasis to being asymptomatic for the lifetime. Recently, we experienced a case of Mounier-Kuhn syndrome patient with tracheal diverticulum, who had lung cancer. Our case is reported with literature reviews.
Bronchi ; Bronchiectasis ; Dilatation ; Diverticulum ; Humans ; Lung Neoplasms* ; Lung* ; Rare Diseases ; Respiratory Tract Infections ; Trachea ; Tracheal Diseases ; Tracheobronchomegaly*

OBJECTIVETo evaluate the efficacy and safety of argon plasma coagulation (APC) in the treatment of large airway obstruction.

METHODSTotally 389 patients with treacheobronchial stenosis were treated with APC (ARCO3000 type) by bronchoscopy. The stenoses were caused by carcinomas (203 cases, 52.2%), metastatic tumors (67 cases, 17.2%), benign tumors (18 cases, 4.6%), granulomas (93 cases, 23.9%) and other lesions (8 cases, 2.1%). The rate of recanalization, relief of the symptoms, and complications were analyzed.

RESULTS1121 times of APC treatment were performed in the 389 patients. Complete recanalization was achieved in 138 cases (35.5%), partial in 143 (36.8%), mild in 55 (14.1%) and none in 53 (13.6%). The major complications included: super-ventricular tachycardia in 136 cases (34.9%), bleeding in 51 (13.1%), decrease in blood oxygen saturation in 48 (12.3%), asphyxia in 33 (8.5%), ventricular or super-ventricular arrhythmia in 24 (6.2%), short-term aggravation of airway obstruction in 18 (4.6%), and tracheal perforation in 3 (0.78%). All those complications were treated with various measures and no patient died of the complications during the procedure.

CONCLUSIONArgon plasma coagulation is effective and relatively safe in relieving the obstruction and dyspnea in patients with large airway obstruction caused by various reasons. However, for the patients with severe airway obstruction, argon plasma coagulation sometimes may cause severe or even lethal complications. Critical consideration of the indication, operators' skill and taking more precautions during the procedure are required to ensure the safety of argon plasma coagulation treatment.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Argon ; therapeutic use ; Bronchial Diseases ; etiology ; surgery ; Bronchial Neoplasms ; complications ; Bronchoscopy ; Constriction, Pathologic ; Electrocoagulation ; methods ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Tracheal Neoplasms ; complications ; Tracheal Stenosis ; etiology ; surgery ; Young Adult

Tracheal bronchus is an aberrant bronchus that arises most often from the right tracheal wall above the carina and is the result of an additional tracheal outgrowth early in embryonic life. It; incidence ranges between 0.1 and 5%. This anomaly is usually diagnosed incidentally during bronchoscopy, bronchography or computed tomography. Occasionally, it represents the underlying etiology for chronic pulmonary disease, especially if it involves the right upper lobe and reflects an abnorrnal pulmonary clearing mechanism. The tracheal bronchus may be associated with other bronchopulmonary anomalies, tracheal stenosis, or Down's syndrome. Asymptornatic tracheal bronchus does not require any treatment. In case of tracheal bronchus associated recurrent right upper lobe diseases, tracheal bronchus therapy should include resection of the aberrant bronchus as well as the lob it supplies. (J Korgan Pediatr Soc 2000;43:1501-1504)
Bronchi* ; Bronchography ; Bronchoscopy* ; Down Syndrome ; Equipment and Supplies ; Incidence ; Lung Diseases ; Tracheal Diseases ; Tracheal Stenosis

Tracheobronchopathia osteochondroplastica (TO) is an uncommon benign disease of an unknown etiology and it affects the cartilaginous walls of large airways. Most cases of TO have been reported to involve the lower two-thirds of the trachea and the proximal bronchi. Unlike the usual cases of TO, exclusive bronchial involvement and the formation of a solitary mass are very rare. We experienced an unusual case that had exclusive bronchial involvement and the formation of a solitary mass and this all mimicked lung malignancy. After surgical resection, we were finally able to diagnose the mass as bronchopathia osteochondroplastica.
Bronchi ; Cartilage Diseases ; Lung ; Lung Neoplasms ; Osteochondrodysplasias ; Trachea ; Tracheal Diseases

Idiopathic tracheal stenosis is a type of benign stenosis that possesses specific characteristics but is of unknown origin. It is a rare disease characterized by extensive fibrosis of a portion of trachea, and predominantly found in women. The lesion presents as circumferential fibrotic stenosis that usually occurs at upper trachea and the subglottic larynx, but lower trachea may also be involved. Diagnosis is made from the clinical characteristics accompanied by compatible pathologic features and by exclusion of other etiologies. Conservative management such as laser resection, dilatation and stent insertion can be tried initially, but surgical resection is recommended for definitive treatment due to frequent restenosis and maintenance problems of conservative approach. We report a case of idiopathic tracheal stenosis treated with tracheal resection and anastomosis followed by insertion of a retrievable stent for immediate relief of airway obstruction.
Airway Obstruction ; Constriction, Pathologic ; Diagnosis ; Dilatation ; Female ; Fibrosis ; Humans ; Larynx ; Rare Diseases ; Stents* ; Trachea ; Tracheal Stenosis*

Adult ; Female ; Granuloma ; etiology ; surgery ; Humans ; Male ; Middle Aged ; Postoperative Complications ; surgery ; Tracheal Diseases ; etiology ; surgery ; Tracheal Stenosis ; etiology ; surgery ; Tracheostomy ; adverse effects

PURPOSE: Infants and children with cardiovascular diseases often present with respiratory symptoms. However, missed or delayed evaluation for potential airway problem may complicate overall prognosis. The aim of this study is to determine the clinical characteristics of these patients and explore the cause of airway problem. METHODS: We reviewed the medical records of 64 patients (M: F=33: 31, mean age: 6.3+/-7.5 months) whose airway problems were proven by computed tomography or bronchoscopy in perioperative periods at the Asan Medical Center from January 1997 to June 2004. Patients were divided into two groups based on the duration of ventilator care: < or =7 days (group 1: 23 cases, M: F=10: 13) and > 7 days (group 2: 41 cases, M: F=23: 18). RESULTS: The patients in group 2 significantly developed more post-operative respiratory symptoms than group 1 (P< 0.001) and had more airway problems including extrinsic obstruction, intrinsic anomaly, and combined problem than group 1 although not significantly different (P=0.082). Among underlying diseases, the most common diseases were vascular anomaly (26.2 percent) and aortic arch anomaly (26.2 percent) in group 1 and pulmonary atresia with ventricular septal defect (22.4 percent) in group 2. The most frequent respiratory symptoms were recurrent wheezing pre-operatively and failure of ventilator weaning post-operatively. The major types of airway anomaly were tracheomalacia and tracheal stenosis (in each case 18.2 percent). Nineteen patients with persistent airway problems underwent aortopexy or other vascular correction. Of the 19 patients, 13 (68.4 percent) were improved, but 2 failed in weaning ventilator and 4 died of non-airway problems. CONCLUSION: Early evaluation and treatment for potential airway problems may affect natural or surgical prognosis in patients with cardiovascular diseases presenting with respiratory symptoms.
Aorta, Thoracic ; Bronchoscopy ; Cardiovascular Diseases* ; Child* ; Chungcheongnam-do ; Heart Septal Defects, Ventricular ; Humans ; Infant* ; Medical Records ; Perioperative Period ; Prognosis ; Pulmonary Atresia ; Respiratory Sounds ; Tracheal Stenosis ; Tracheomalacia ; Ventilator Weaning ; Ventilators, Mechanical ; Weaning

OBJECTIVETo analyze the effect and safety of bronchoscopic cryosurgery in children with lower airway stenosis caused by granulation and fiber hyperplasia.

METHODTwenty-two patients had undergone bronchoscopic examination and cryosurgery, 18 patients with atelectasis caused by Mycoplasma pneumoniae pneumonia, 2 patients with endobronchial tuberculosis (EBTB), 1 patient with atelectasis caused by granulation tissue after bronchial foreign body, and 1 case who had severe dyspnea, with tracheal stenosis after long-term endotracheal intubation during surgery for heart disease. All the patients under went bronchoscopic cryosurgery for several times, 1 case with severe tracheal stenosis was operated by electric coagulation before cryosurgery. Before and after the treatment, all the patients were evaluated based on clinical symptoms, tracheal lumen by bronchoscopy, chest CT for atelectasis and pulmonary function for tracheal stenosis.

RESULTThe patients were treated with cryosurgery for 1-4 times. Sixteen cases were markedly improved, clinical symptoms disappeared completely, no granulation and fiber hyperplasia were found during bronchoscopy, and all the atelectasis were cured. After the treatment, the bronchial lumen diameter of the patient with tracheal stenosis was increased from 2 mm to about 5 mm, the tidal volume increased from 3.0 ml/kg to 8.8 ml/kg. Five cases were effectively improved, the clinical symptoms also disappeared, during bronchoscopy and bronchial alveolar lavage (BAL), no granulation and fiber hyperplasia were found, but some distal bronchial tubes were atresia or narrowed, the lumen was patent, the atelectasis recovered partially. In 1 case the treatment was ineffective, as the clinical symptoms and granulation disappeared, but because almost all distal bronchi were atresia or narrow, the lumen was not patent during BAL, there was no change on chest CT. The total effective rate was 21/22 (95.5%). No severe complications occurred during and after the procedures. All the patient were followed up for 1 - 12 months, no case had recurrence.

CONCLUSIONTreatment with bronchoscopic cryosurgery is a safe and effective methods to tracheal stenosis caused by granulation tissue and fiber hyperplasia in children.

Bronchial Diseases ; etiology ; surgery ; Bronchoscopy ; Child ; Child, Preschool ; Cicatrix ; complications ; Constriction, Pathologic ; Cryosurgery ; Female ; Granuloma ; complications ; Humans ; Lung Diseases ; etiology ; surgery ; Male ; Tracheal Stenosis ; etiology ; surgery

BACKGROUND AND OBJECTIVES: Adequate evaluation of the upper airway is critical in the management of pediatric patients with stridor or extubation failure. For this purpose, we designed an evaluation protocol of the upper airway for these patients, in collaboration with Dept. of Pediatrics, Intensive care team and Anesthesiology. Here we present the clinical results of our evaluation protocol and provide information about the etiology and management of the upper airway problems. SUBJECTS AND METHOD: Clinical data of 380 pediatric patients (M:F=231:149) having airway evaluation for their problems (stridor or extubation failure) were retrospectively analyzed. Among them, patients of age less than 3 months ranked first (30.0%). Comorbidities of pulmonary diseases (30.8%) and cardiovascular diseases (29.5%) were found. The pre and post-evaluation diagnosis, management and prognosis were evaluated and the usefulness of an airway evaluation protocol was discussed. RESULTS: Frequent pre-evaluation diagnoses were subglottic stenosis (55.2%), laryngomalacia (12.6%) and tracheal stenosis (9.2%) and these were changed to subglottic stenosis (44.5%), laryngomalacia (9.7%), tracheal stenosis (6.6%) and no abnormality (14.5%). Particularly, 50% of pre-diagnosis laryngomalacia, 25% of subglottic stenosis and 37% of tracheal stenosis were corrected to other causes by airway evaluation. The procedures were exam only (41.6%), endoscopic dilatation (20.8%) and tracheostomy (17.9%). In 190 out of 380 (50.0%), extubation was successful, but 151 patients (39.7%) had tracheostomy tube. CONCLUSION: Adequate evaluation of the upper airway in pediatric patients with stridor or extubation failure can facilitate the diagnosis and management of their problems.
Anesthesiology ; Cardiovascular Diseases ; Comorbidity ; Constriction, Pathologic ; Cooperative Behavior ; Diagnosis ; Dilatation ; Humans ; Critical Care ; Laryngomalacia ; Lung Diseases ; Pediatrics ; Prognosis ; Respiratory Sounds* ; Retrospective Studies ; Tracheal Stenosis ; Tracheostomy

Congenital tracheal stenosis (CTS), though rare, is important because the mortality and morbidity rates are high in infants. Especially, associated congenital heart disease (CHD) in these infants may compound the effects of airway pathology. A 3-week-old patient with long-segmental tracheal stenosis below an anomalous right-upper lobe (RUL) bronchus had undergone a total correction of double outlet right ventricle. On third postoperative day, hypercarbia developed, and severe airway obstruction and atelectasis were detected. An emergency slide tracheoplasty was performed under cardiopulmonary bypass (CPB). The patient recovered well after the surgery. Thus, special attention needs to be paid during the postoperative intensive care of patients with congenital tracheal anomalies. Early detection and prompt diagnosis of airway obstruction can help reduce the morbidity and mortality rates. Further, it is important to select the suitable treatment of CTS associated with CHD.
Airway Obstruction ; Bronchi ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Double Outlet Right Ventricle ; Emergencies ; Heart ; Heart Diseases ; Humans ; Infant ; Critical Care ; Pulmonary Atelectasis ; Trachea ; Tracheal Stenosis