RODELIO C. GUINTU, M.D.Department of Pathology andLaboratory Medicine®
Clinical Summary
32 year-old nonsmoker male with life-long history of asthma
Had 2 hospitalizations during childhood
Presented to CSMC with progressive dyspnea on exertion for several months, back pain and a non- productive cough
Pulmonary function tests: — obstructive pattern with significant air trapping — evidence of small airway disease
V/Q scan severely decreased tracer activity in the right lung which received only 10% of the total lung perfusion and the remaining 90% was from the left lung
CXR showed hyperlucency of the hyperinflated right lung
Non-functional right lung compressing the opposite lung and the heart
To further characterized this disease process
The patient doesn't need it
Right pneumonectomy
Gross Photo- Right Lung
Right middle lobe showing the presence of severe bullous and panacinar emphysema, bronchiectases and peribrochiolar fibrosis
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Right lower lobe showing diffuse changes of panacinar emphysema
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Panacinar Emphysema
Peribronchiolar fibrosis
What is the diagnosis ?
Recurrent childhood asthma
Right lung hyperlucency on CXRNon functioning right lung
Massive unilateral emphysemaBronchiectasisPeribronchiolar fibrosisPleural bullae
EmphysemaPanacinar (panlobular) emphysema: acini are uniformly enlarged from the level of the respiratory bronchiole to the terminal blind alveoli
Centriacinar emphysema: central and proximal part of the acini are affected; distal alveoli are spared
Paraseptal (distal acinar) emphysema: proximal portion of the acinus is normal, but the distal part is predominantly involved
Obstructive emphysema: lung expands because air is trapped within it
Bullous emphysema: emphysema that produces large subpleural blebs or bullae
Differential Diagnosis of Unilateral Lung Hyperlucency Unilateral
radiolucency Lung volume
Arterial perfusion
Air trapping Bronchogram
Swyer-James-MacLeod syndrome
Moderate Moderate Present Moderate Bronchiectasis
Absence of Pulmonary artery
Slight Small Absent None Diminutive bronchial tree
Partial obstruction of main bronchus
Marked Large Present Marked Lesion in major bronchus
Congenital lobar emphysema
Marked Large Present Marked Bronchi displaced around emphysematous area
Daniel et T et al Clinical Pediatrics 1984 ;23 :393-397
DIAGNOSIS
Historical Background
First reported case by Swyer and James in 1953 characterized by repeated respiratory infection with right-sided pulmonary hyperlucency on chest X-rayIn 1954, MacLeod reported 9 adult cases showing similar featuresTraditionally known as: Idiopathic unilateral hyperlucent lung or unilateral pulmonary transradiancy
“MacLeod’s syndrome” first used by Rivett in 1960“Swyer-James syndrome” first used by Rakower & Moran in 1962
Swyer-James-MacLeod Syndrome
Clinical Features:History of recurrent respiratory infections during earlier lifeSymptomatic patients frequently diagnosed in childhoodAsymptotic patients usually diagnosed later in life when CXR is done for other reasons
— Increasing dyspnea, tightness of the chest, chronic cough and easy fatigability
Characteristic Radiographic Findings:Unilateral pulmonary hyperlucency on CXR secondary to a decrease in vascular shadows over the lung field
Etiology
Etiology and pathogenesis remain unknown
Yet to be determined whether congenital or acquired
Two theories raised regarding its pathogenesis: A. Bronchial Pathogenic Theory B. Pulmonary Artery Pathogenic Theory
Bronchial Pathogenic Theory
Bronchioles are first obstructed
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Bronchioles are first obstructed due to pulmonary infection
Emphysematous change takes place leading to dilated alveoli
Subsequently press pulmonary vessels
Decreased pulmonary blood supply
Congenital dysplasia of pulmonary artery
Inhibiting the bronchi growth
Making emphysematous changes inevitable along with bronchiolitis
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Pulmonary Artery Pathogenic Theory
Other pathologic findings: What is this?
Placental transmogrificationTransmogrification means to change into different form or shape especially one that is fantastic or bizarre:
First described by McChesney in 1979 in a case report
Morphologically bears superficial resemblance to placental villi
Represents a rare disease with only 23 cases reported in the literature to date
Adults and young adults, predominantly male with history of pneumonic process
All cases have been described in patients with intrapulmonary cystic lesions, may appear as a bulla or enlarging cyst
Different opinions postulated on its pathogenesis but no single explanation
Different opinions on its pathogenesisRepresents primarily a benign proliferation of peculiar interstitial clear cells with secondary cystic changes (Cavazza et al)
Its presence in patient with emphysema maybe the result of metaplastic mesenchymal differentiation (Hochholzer et al)
Related to chronic segmental edema due to destruction or blockage of the lymphatics in the affected areas (Koss, M)
May have been induced by proliferation of the epithelial cells lining the hamartomas (Xu et al)
Our opinion, probably results from the development of edema, fibrosis and chronic inflammation in the residual alveolar tissue of patients with severe emphysema
Conditions associated with Placental Transmogrification
Severe cigarette-smoking induced emphysema
Giant bullous emphysema of the lung
Fibrochondromatous hamartomas
Ours is the first case associated with Swyer-James-MacLeod syndrome
DIAGNOSIS
References:
Swyer PR, James GCW. A case of unilateral pulmonary emphysema. Thorax 1953; 8: 133-6Macleod WM. Abnormal transradiancy of one lung. Thorax 1954; 9: 147-53McNamara JJ. Urschel HC, Arndt JH, Ulevitch H, Kingsley WB. Idiopathic
unilateral hyperlucent lung, The Swyer-James Syndrome. Annals of Thoracic 1969; 7: 351-56Daniel TL, WoodringJH, Vandiviere HM Wilson MD. Swyer-James Syndrome-unilateral hyperlucent lug syndrome. Clin Pediatr 1984; 23: 393-7Morita, K, Shimizu J, Kamesui T, Watanabe Y, Nonomura A, Kita K, A case of surgical treatment of Swyer-James syndrome. Jpn J Thorac Cardiovasc Surg 1994; 42: 1949-52Koyama T, Osada H, Kitanka Y, Funaki S, Hiekata T. Surgically treated Swyer-
James syndrome. Jpn J Thorac Cardiovasc Surg 2001; 49: 671-4