Cases reported "Tracheal Diseases"

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1/34. ehlers-danlos syndrome type IV with a unique point mutation in COL3A1 and familial phenotype of myocardial infarction without organic coronary stenosis.

    We report on a 43-year-old male patient with ehlers-danlos syndrome (EDS) type IV with acute myocardial infarction (MI) without organic coronary stenosis. The disease was complicated with pneumothorax, subcutaneous and mediastinal emphysema, and splenic artery rupture. Three of the patient's family members suffered sudden cardiac death or MI. A diagnosis of EDS type IV was confirmed by decreased production of type III collagen by 86%. Mutation analysis revealed a point mutation in the COL3A1 gene that substituted glycine for aspartate at amino acid position 877. This mutation had not been reported as pathogenic for EDS type IV. These findings suggest close linkage between the mutation and the phenotype with familial MI.
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ranking = 1
keywords = stenosis
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2/34. Metal airway stent implantation in children: follow-up of seven children.

    Long segment malacia of the trachea or main stem bronchi in children is not always suitable for surgical correction; patients may therefore remain ventilator-dependent and/or experience severe obstructive crises. We treated 7 children (ages, 4 months to 9 years) with extreme structural central airway obstruction with stent implantations. Six were mechanically ventilated; 5 had frequent life-threatening obstructive spells requiring deep sedation or paralysis. Diagnoses were: syndrome-associated tracheobronchomalacia (n = 4), malignancy infiltrating the carina (n = 1), congenital tracheal stenosis (n = 1), and tracheobronchial compression by a malpositioned aorta (n = 1). Six tracheal and 13 bronchial stents were endoscopically placed. The prostheses included mesh titan (n = 5), the newer shape memory material nitinol (n = 13), and 1 Y-shaped carina stent. Follow-up was reported for 7 weeks to 72 months. All patients showed marked improvement of their respiratory obstruction. Six children were weaned at least temporarily from ventilation. No significant bleeding, stenosis, or perforation was observed. Seven stents were changed after up to 14 months. Three children are well and at home. In 2 children airway stabilization was successful, but they later died from causes unrelated to stent placement, and 2 children died due to generalized airway disease. Soft metal mesh airway stents can offer a therapeutic option in life-threatening inoperable obstruction of the trachea and main stem bronchi in children.
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ranking = 134.00576505494
keywords = tracheal stenosis, stenosis
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3/34. Tracheobronchopathia osteochondroplastica: report of three cases.

    Tracheobronchopathia osteochondroplastica (TO) is a rare disease characterized by the presence of osseous and cartilaginous submucosal nodules in the tracheobronchial tree. The majority of patients remain asymptomatic; however, a small number develop severe airway stenosis. Symptoms may include dyspnea, hoarseness, cough, hemoptysis, and recurrent pneumonia. Plain chest X-ray films are often unremarkable but may demonstrate atelectasis, consolidation, tracheal nodularity, or narrowing. CT reveals tracheal nodularity with calcification and narrowing. This article reviews the cross-sectional imaging characteristics of TO.
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ranking = 0.2
keywords = stenosis
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4/34. Metallic tracheal stents: complications associated with long-term use in the upper airway.

    The use of metallic airway stents for the treatment of benign airway stenosis is increasingly advocated; however, the long-term safety and efficacy of these devices has not been established. Three case studies involving late but significant proximal tracheal stent complications are reported: 1 related to proximal mechanical stent failure and 2 related to obstructing granulation tissue and stenosis at the proximal stent orifice. Placement of these stents in close proximity to the cricotracheal junction is thought to be primarily responsible for these complications as a result of the excess shearing forces created at the stent-mucosal interface by the differential motion of the stent relative to the rigid subglottic airway and the more distensible trachea. Although the use of metallic stents remains appropriate in cases in which there is a defined and relatively short-term end point for treatment, caution is urged in using them in the proximal trachea for long-term management of benign airway disease.
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ranking = 0.4
keywords = stenosis
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5/34. A case of relapsing polychondritis involving placement of an expandable metallic stent.

    Relapsing polychondritis is a relatively uncommon disease characterized by recurrence of progressive inflammation of cartilaginous structures. Laryngotracheal involvement occurs in 50-70% of cases, and occasionally results in acute airway obstruction. We reported a case of relapsing polychondritis with severe tracheal stenosis that was treated by the placement of two expandable metallic stents. A 58-year-old man was hospitalized with fever and breathing difficulties. A tracheostomy was performed and a silicone T-tube was placed. A diagnosis of relapsing polychondritis was made based on the biopsy from auricular and tracheal cartilages. Two expandable metallic stents were placed in the trachea to bilateral main bronchus. This dilated the airway lumen and resulted in the dyspnea disappearing completely. However, 18 months later a sudden massive hemorrhage occurred through the tracheostoma, and he died of respiratory failure. autopsy showed a tracheoinnominate artery fistula. We discuss the management of relapsing polychondritis with severe tracheal stenosis.
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ranking = 267.61153010988
keywords = tracheal stenosis, stenosis
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6/34. tracheomalacia associated with Mounier-Kuhn syndrome in the intensive care Unit: treatment with Freitag stent. A case report.

    tracheomalacia is a process characterized by softness of the supporting tracheal cartilages, by the extension of the posterior membranous wall and by reduction of the tracheal antero-posterior diameter. Exceptionally, tracheomalacia can be associated with tracheobronchomegaly or Mounier-Kuhn syndrome. Fibro-bronchoscopy represents the ''gold standard'' for diagnosis. The case of a 79-year-old male observed after hospitalization in a medical ward for chronic pulmonary obstructive disease (COPD) decompensation, and with basal left bronchopulmonary focus, is described. During this period, a progressive worsening of clinical conditions occurred, despite cortisone and antibiotic therapy, and the patient was transferred to the ICU for dyspnea, hypoxia, hypocapnia and with a diagnosis of pulmonary fibrosis. bronchoscopy, performed during spontaneous breathing, revealed tracheomalacia which was responsible for tracheal dynamic complete stenosis during expiration and dynamic subtotal stenosis of the left primary bronchus in the first tract, together with sputum retention. Moreover, this investigation confirmed the diagnosis of tracheobronchomegaly already seen on CT. It was suggested to place a Freitag stent, since the insertion of another model would not have had enough chance of stability, due to the enormous extension of the tracheal lumen and could not have guaranteed good clearance of the secretions. Seven days after this intervention, performed in an outpatients' setting, the patient was dismissed from the ICU, without the help of O2, with good ventilation, saturation in line with his age and good expectoration.
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ranking = 0.4
keywords = stenosis
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7/34. Complications of metallic stents in the pediatric airway.

    OBJECTIVE: Our aim was to present our experience with complications caused by placement of metallic stents in the pediatric airway. DESIGN AND SETTING: We conducted a retrospective study of the medical records of patients with complications resulting from metallic stent placement, managed by the senior authors between 1993 and 2002. RESULTS: Nine children had complications associated with the placement of metallic airway stents. Of these, 8 children required stent removal. granulation tissue and tracheal stenosis were seen in all 7 children with long standing stent placement. There was 1 stent death in this series. CONCLUSIONS: Metallic airway stents can cause significant complications in the pediatric airway. These complications may supersede the airway compromise that necessitated their initial placement. As such, metallic stent placement should be approached with caution. The likelihood and severity of complications increase with time, as do the difficulties encountered upon removal. The proportion of patients in whom metallic stents may be placed "permanently" without complications is not known. Therefore we recommend that metallic airway stents be considered a temporizing measure of limited duration.
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ranking = 133.80576505494
keywords = tracheal stenosis, stenosis
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8/34. Interventional bronchoscopy in the management of airway stenosis due to tracheobronchial tuberculosis.

    STUDY OBJECTIVES: To assess the efficacy and complications of interventional bronchoscopic techniques in treating airway stenosis due to tracheobronchial tuberculosis. DESIGN: Case series. SETTING: Respiratory care centers at two tertiary care referral teaching hospitals in japan, Hiroshima City Hospital and Okayama red cross Hospital. PATIENTS AND INTERVENTIONS: A total of 30 patients were admitted to the hospital with a diagnosis of tracheobronchial tuberculosis between January 1991 and January 2002. Of those 11 patients received interventional bronchoscopy, including stent placement, laser photoresection, argon plasma coagulation (APC), balloon dilatation, cryotherapy, and endobronchial ultrasonography (EBUS). One patient with complete bronchial obstruction underwent a left pneumonectomy. RESULTS: Six patients underwent stent placement after balloon dilatation, while the remaining five patients underwent only balloon dilatation. In six patients, Dumon stents were successfully placed to reestablish the patency of the central airways. Two patients first had Ultraflex stents implanted but had problems with granulation tissue formation and stent deterioration caused by metal fatigue due to chronic coughing. Dumon stents then were placed within the Ultraflex stents after the patient had received treatment with APC and mechanical reaming using the bevel of a rigid bronchoscope. In four patients, EBUS images demonstrated the destruction of bronchial cartilage or the thickening of the bronchial wall. The main complications of Dumon stents are migration and granulation tissue formation, necessitating stent removal, or replacement, and the application of cryotherapy to the granuloma at the edge of the stent. CONCLUSION: Interventional bronchoscopy should be considered feasible for management of tuberculous tracheobronchial stenosis. Dumon stents seem to be appropriate, since removal or replacement is always possible. Ultraflex stents should not be used in these circumstances because removal is difficult and their long-term safety is uncertain. EBUS could provide useful information in evaluating the condition of the airway wall in cases of tracheobronchial tuberculosis with potential for bronchoscopic intervention.
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ranking = 1.2
keywords = stenosis
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9/34. Management of an ascending aortic aneurysm with coronary artery disease and tracheal compression from a substernal goiter.

    We report the case of a 61-year-old female, who presented with a history of chronic fatigue, dyspnea on exertion, a widened mediastinum with tracheal deviation on chest X-ray, and a neck mass. After a diagnostic workup, the patient was found to have a paratracheal mass extending into the chest in addition to a 6.5 cm ascending aortic aneurysm with aortic insufficiency, and a 70% stenosis of the right coronary artery. She underwent successful resection of a substernal goiter via a neck incision facilitated by a previously performed sternotomy for a concomitant ascending aortic root replacement and a bypass utilizing the RIMA to the distal RCA.
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ranking = 0.2
keywords = stenosis
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10/34. Infantile major airway stenosis and acute respiratory distress associated with cardiac tamponade.

    Coxsackie virus pericarditis caused cardiac tamponade in a 45-day-old infant with corrected total anomalous pulmonary venous drainage and a hypodynamic left heart. The pathophysiology comprised reduced heart compliance, venous return impairment, acute pulmonary hypertension, and increased airway microvascular permeability. Tracheal edema and external compression caused tracheal lumen narrowing and respiratory failure. laryngoscopy was difficult because of laryngeal inlet swelling. Endotracheal intubation was accomplished with a 3.0-mm tube. Pericardial cavity evacuation resulted in rapid recovery. A postprocedural chest radiograph revealed tracheal lumen enlargement. Repeated laryngoscopy revealed resolution of upper-airway edema. In infants, large pericardial effusions developing after corrective/palliative heart surgery may cause major airway compromise.
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ranking = 0.8
keywords = stenosis
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