RODELIO C. GUINTU, M.D.Department of Pathology andLaboratory Medicine®

guintur@cshs.org

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

Cedars-Sinai was founded on Judaic principles, which foster an environment that is respectful to all faiths and culturesCedars-Sinai maintains the Judaic environment and traditions:

— Rabbinical presence— Jewish religious services — Chapel— Observance of Jewish holidays— Sabbath observances – elevators and candelabras— Kosher kitchen facilities— Star of David on the Medical Center— Mezuzot— Jewish medical ethics— Holocaust memorial— Sukkah on premises— Relationships with other Jewish-sponsored organizations

Right lower lobe showing diffuse changes of panacinar emphysema

Cedars-Sinai serves theLos Angeles County market area –with more than 9 million people. Our primary service area, comprising some 2.5 million people, represents approximately 73 percent of Cedars-Sinai’s total inpatient discharges.

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

Cedars-Sinai Health SystemOur MissionOur CredoOur Values

Our Strategic VisionJudaic TraditionOur Community

Our LocationAn Academic Medical Center

Medical StaffInnovative Research

Licensed Beds

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

Cedars-Sinai Cancer ProgramCedars-Sinai Center for Digestive DiseasesCedars-Sinai Heart CenterImaging ServicesCedars-Sinai Center for Chest DiseasesMulti-Organ Transplant Program

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