Case #1 Surgical Pathology Evening Session USCAP, 2015 Samuel A. - PowerPoint PPT Presentation

Case #1 Surgical Pathology Evening Session USCAP, 2015 Samuel A. Yousem, MD University of Pittsburgh School of Medicine UPMC – Presbyterian – C620 Pittsburgh, PA

Case #1 Clinical History 48-year-old WM smoker s/p CABGx2 with thoracic duct repair two years previously presents with cough and production of large mucous plugs (see image). Diagnosis ?

Diagnosis: Plastic bronchitis A large and small airway inflammatory process characterized by the formation of large gelatinous or rigid branching airway mucous casts, that may or may not be spontaneously expectorated. Syn: Hoffman’s bronchitis, fibrinous bronchitis, pseudomembranous bronchitis.

Plastic Bronchitis History/Background 1. Initially described by Galen as “venae arteriosae expectorate” – expectorated pulmonary blood vessels. 2. Misinterpreted by others as regurgitated noodles or chicken meat. 3. Most comprehensive description by Osler in his Textbook of Medicine. Madsen et al. Paed Resp Review, 2005

Plastic Bronchitis The clinical presentation and histopathology of the mucous plug/bronchial cast are closely inter-related. Cajaiba et al Intl J Surg Path 2008

Clinical Presentation S&S: dyspnea, wheeze, chest pain, fever Exam: wheeze, “bruit de drapeau”. CXR/CT: collapse with secondary hyperinflation, patchy consolidation. Bronk: obstruction with casts. Gross appearance: cast reflects the pathology of the underlying bronchial tree.

Clinical Scenarios of Plastic Bronchitis 1. Congenital/structural heart disease with repair (Fontan procedure/B-T shunt; includes disorders of lymphatics) - 2º to increased blood flow, mucous hypersecretion, disrupted lymphatics w/ retrograde flow 2. Asthma/atopy/allergic bronchopulmonary microbial disease--mucoid impaction of bronchus. 3. Sickle cell disease – acute chest syndrome. 4. Infection – CF, post-obstructive, middle lobe syndrome.

Histopathology Plastic Bronchitis 1. Mucus with fibrin, foamy macrophages, few cells (CHD) . 2. Mucus with fibrin, eosinophils, Charcot-Leyden crystalloids, “allergic mucin” (asthma related). 3. Mucus with fibrin, bile stained macrophages (Sickle cell). 4. Mucus with PMNs – infection. • Type I / II plugs (Seear et al. AJRCCM, 1997) • Do not throw plugs away in cyto/surgical pathology laboratory. • Look for histologic clues. • Grocott stains/culture studies. Brogan et al Ped. Pul., 2002

Treatment/Prognosis Steroids/mucolytics/proteases/antibiotics Prognosis depends on clinical setting – 5 year mortality CHD – 30-60% Asthma – 5-50% Sickle cell – 0-5% Infection – 30-60% Eberlein et al, 2008

Plastic Bronchitis Summary/Conclusions 1. Bronchial casts may be informative – do not discard them. 2. Gross and microscopic appearance can give clues to etiology. 3. Clinical scenarios and pathology correlate Heart/lymphatic disease → mucin, fibrin, few cells. Asthma → mucus, fibrin, eos, allergic mucin Sickle cell → mucus, fibrin, sickled cells, bile Infection → mucus, PMNs, CF