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Tracheomalacia R3 吳俊璟 Weakness of trachea Reduction of the longitudinal elastic fibers of the pars menbranacea Impaired cartilage integrity, flaccidity of the supporting tracheal cartilage Reduced anterior-posterior airway caliber Histopathologic characteristics Z The membranous trachea: larger Z The ration of cartilage to muscle: reduced Major airway collapse classification Type Presentation Type I Congenital or intrinsic tracheal abnormalities that can be associated with a tracheoesophageal fistula Type II Extrinsic defects or anomalies, such as a vascular ring causing undue pressure on the trachea Acquired tracheomalacia that occurs with prolonged Type III intubation or chronic tracheal infections Pediatric tracheomalacia Congenital TM Z most common congenital anomaly of the trachea, more in premature Z Most common associated disease Æ tracheoesophageal fistulaÆ faulty division of the foregut into the trachea and esophagus Z Primary TM is a self limiting disease Pediatric tracheomalacia Acquired TM Z More common Z Male predominance Associated conditions Z Cardiovascular: 20~58% Z Bronchopulmonary: 52% Z GE reflex: 50~78% Z Neurologic impairment Z Development delay Incidence and nature history 1/1445 infants 30% in children ≤ 3 y/o undergoing bronchoscopy for respiratory distress. Primary TM is a self limiting disease Infants outgrow the condition by 2 y/o The mortality rate from severe TMÆ80% Symptoms Mild: respiratory difficulties associated with infectious process Moderate: stridor, wheezing, recurrent infection and cyanosis Severe: marked sputum retention, reflex apnea and cardiac arrest Diagnosis (I) History and physical examination Radiography: Z Sensitivity:62% Z Inspiratory and expiratory views Z Airway Fluoroscopy Z Barium Swallow Bronchoscopy Z General anesthesia Z Decrease 50% diameter Diagnosis (II) CT scan Z High sensitivity Z Dynamic CT Z Multidetector helical CT Z Virtual bronchoscopy MRI scan Z Evaluate extrinsic compression Z Vascular compression syndromes Treatment (I) Conservative therapy ZTreat respiratory infection ZHumidified oxygen therapy ZPulmonary physiotherapy Tracheostomy and mechanical ventilation Z12%~62% ZElongated tracheostomy tube Continuous positive airway pressure (CPAP) Treatment (II) Surgery Z Indication: recurrent pneumonia, intermittent respiratory obstruction, inability to extube the airway, dying spell Z Aortopexy Z External splinting with autologous and prosthetic materials Treatment (III)-internal tracheal stent Silicone prothesis Z Montgomery,1965 Z Granulation Z Easy to remove Metal stent Z Minimal thickness Z Easy deployment Z Granulation Z Migration Z Difficult removal Z Halitosis Adult TM Isolated tracheal enlargement, Mounier-Kuhn, 1932 Acquired tracheal enlargement, Lemoine, 1949 Classification by macroscopic finding: Z Lateral wall narrowÆ saber-sheath type, fissure shape Z Anterioposterior wall narrowÆ crescent type, scabbard shape Z Circumferential narrowing, combination type Classification of adult tracheomalacia Primary (congenital) Z Polychondritis Z Idiopathic (Mounier-Kuhn syndrome) Secondary (acquired) Z Posttraumatic (postintubation, posttracheotomy, external chest trauma, post-lung transplantation) Z Emphysema Z Chronic bronchitis Z Chronic inflammation (relapsing polychondritis) Z Chronic external compression of trachea (malignancy, benign tumors, cysts, abscesses, aortic aneurysm) Z Vascular rings (diagnosed in childhood) Incidence and symptoms Majority of adult TMÆ acquired, in men > 40 y/o 1% in patient with respiratory disease for bronchoscope. TM is progressive in some patient Symptoms: asymptomatic until infection, dyspnea, cough, sputum production, hemoptysis, wheezing, stridor, syncope Diagnosis Bronchoscope: Z Mild:50~75% Z Moderate:>75% Z Severe: posterior wall touches the anterior wall CT MRI Pulmonary function study Z not diagnostic Z Decreased FEV1, low peak flow rate with repeat decrease in flow Treatment Supportive, treat underlying disease CPAP Tracheostomy Stent Z Metal: flexible bronchoscope, Z Silicone: rigid bronchoscope, GA Z External stenting Surgery Z Surgical supportÎ bone graft to posterior wall Z Resection and reconstruction Z Surgical placation of posterior wall with mesh References Weinberger M, Abu-Hasan M. Pseudo-asthma: when cough, wheezing, and dyspnea are not asthma. Pediatrics. 2007 Oct;120(4):855-64. Agarwal A, Mishra AK, Gupta SK, Arshad F, Agarwal A, Tripathi M, Singh PK. High incidence of tracheomalacia in longstanding goiters: experience from an endemic goiter region. World J Surg. 2007 Apr;31(4):832-7. Epstein SK.Late complications of tracheostomy. Respir Care. 2005 Apr;50(4):542-9. Carden KA, Boiselle PM, Waltz DA, Ernst A. Tracheomalacia and tracheobronchomalacia in children and adults: an in-depth review. Chest. 2005 Mar;127(3):984-1005. Adliff M, Ngato D, Keshavjee S, Brenaman S, Granton JT. Treatment of diffuse tracheomalacia secondary to relapsing polychondritis with continuous positive airway pressure.Chest. 1997 Dec;112(6):1701-4. Aquino SL, Shepard JA, Ginns LC, Moore RH, Halpern E, Grillo HC, McLoud TC. Acquired tracheomalacia: detection by expiratory CT scan. J Comput Assist Tomogr. 2001 May-Jun;25(3):394-9 References Madden BP, Loke TK, Sheth AC.Do expandable metallic airway stents have a role in the management of patients with benign tracheobronchial disease? Ann Thorac Surg. 2006 Jul;82(1):274-8 Isa AY, Macandie C, Irvine BW. Nitinol stents in the treatment of benign proximal tracheal stenosis or tracheomalacia. J Laryngol Otol. 2006 Jan;120(1):32-7. Masters IB, Chang AB, Patterson L, Wainwright C, Buntain H, Dean BW, Francis PW. Series of laryngomalacia, tracheomalacia, and bronchomalacia disorders and their associations with other conditions in children. Pediatr Pulmonol. 2002 Sep;34(3):189-95 McNamara VM, Crabbe DC.Tracheomalacia. Paediatr Respir Rev. 2004 Jun;5(2):147-54. Göbel G, Karaiskaki N, Gerlinger I, Mann WJ. Tracheal ceramic rings for tracheomalacia: a review after 17 years. Laryngoscope. 2007 Oct;117(10):1741-4.