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Plastic Bronchitis

Plastic Bronchitis . Pavel Ba š ek Kinderspital, Universitätsklinikum Salzburg 9. Winterschool, 11. – 14.3. 2013, Obergurgl . Historical Perspective and Epidemiology.

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Plastic Bronchitis

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  1. Plastic Bronchitis Pavel Bašek Kinderspital, Universitätsklinikum Salzburg 9. Winterschool, 11. – 14.3. 2013, Obergurgl

  2. Historical Perspective and Epidemiology • Galen (AD 131/200) first described bronchial casts and hypothesized that they were expectorated blood vessels of the lung: ”venae arteriosae expectoranti” • Osler’s Textbook of Medicine: ”thickened bronchial mucus ” • 1902 Bettmann’s review of 102 cases • Prevalence unknown • ca. 500 cases reported • Patients of all age groups • Female predominance

  3. Brogan et al. Pediatric Pulmonology 2002

  4. Reports of Plastic Bronchitis • Tuberculosis • ALL • Metastatic lung tumors • Smoke inhalation • ABPA • Influenza H1N1 Infection • Thalasemia alpha • Noonan’s syndrome • Turner’s syndrome • Idiopathic 1972

  5. Clinical Presentation • Hallmark: expectorationof large andbranchingcasts • whitish-yellowishcolloration • morecohesivethanmucuspluggs, rubberyconsistency • Acute/dramaticonset • Productivecough • Dyspnoea • Cyanosis • Wheezing/ ‘bruit de drapeau’ Clinic imitates foreign body aspiration

  6. Radiologic Appearance Veras T et al. J. Pediatr. 2005

  7. Diagnostic Bronchoscopy tissue glue bronchial cast Eberlein M, Am J Med Sci 2008

  8. Classification of casts • Type I (inflammatory) casts • Fibrin, eosinophilic or neutrophilic infiltrates • Acute presentation • Associated with bronchial disease • Type II (acellular) casts • Mucin, few mononuclear cells • Chronic/recurrent • Associated with cyanotic congenital heart disease Seear et al. Am J Respir Crit Care Med 1997

  9. Histology of bronchial casts Typ II. mucin in dark pink Typ I. cellular casts Deng J. Chest 2010

  10. lineary architecture of mucins reticulate pattern of mucins Rubin B., Chest 2009

  11. Asthma Vitium Andere Brogan et al. Pediatric Pulmonology 2002

  12. Pathophysiology in cardiac patients • abnormalities in lymphatic drainage • endobronchial lymph leakage • poor cardiac output • elevated venous pressure • disruption of the integrity of the bronchial mucosa • leakage of proteinaceous material into the airways Fontan hemodynamics

  13. Cardiacpatients with casts Grutter G. Ann Thorac Surg 2012

  14. Pathophysiology in non-cardiac patients • Inflammation/irritation leads to induction of mucin’s hypersecretion (TNFα, IL-1β, IL-6, IL-17, neutrophil eleastase, FFA… Rubin B. Chest 2009

  15. Inflammation/Infection high pulmonary pressure high pressure in the lymphatic system ? Eberlein et al. [Am J Med Sci 2008

  16. Therapy • Acute/unspecific: expectoration/removal of casts • Rigid bronchoscopy • Physiotherapy, high-frequency chest wall oscillation • Inhalation therapy • rhDNase • Acetylcystein • Urokinase • tPA • Long-term • Optimal control of primary disease • Cast-specific

  17. Endoscopic treatment

  18. Inhalation Therapy/Approaches

  19. Systemic therapy

  20. Therapy in the future?

  21. Prognosis/Mortality Asthma: 10-50% Cardiac pat: 60%

  22. Conclusion • Rare but potentially life-threatening • Clinic mimics foreign body aspiration • Therapy: • acute removal • optimal treatment of primary disease • cast-specific therapy

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