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  1. 1. Swyer-James (Macleods) syndrome. Variations in pulmonary-bronchial arterial blood flow. L S Gottlieb and A F Turner Chest 1976;69;62-66 DOI 10.1378/chest.69.1.62 The online version of this article, along with updated information and services can be found online on the World Wide Web at: http://chestjournal.chestpubs.org/content/69/1/62 Chest is the official journal of the American College of Chest Physicians. It has been published monthly since 1935. Copyright1976by the American College of Chest Physicians, 3300 Dundee Road, Northbrook, IL 60062. All rights reserved. No part of this article or PDF may be reproduced or distributed without the prior written permission of the copyright holder. (http://chestjournal.chestpubs.org/site/misc/reprints.xhtml) ISSN:0012-3692Downloaded from chestjournal.chestpubs.org by guest on September 25, 2011 © 1976 American College of Chest Physicians
  2. 2. Swyer-James (Macleods) Syndrome*Variations in Pulmonary-Bronchial Arterial Blood Flow Leon S. Gottlieb, M.D.,F.C.C.P., and A. Franklin Turner, M.D.The SwyerJames (Macleods) syndrome (or unilateral t d e d roentgenologic and physiologic studies were per-hyperlucency of the lung) frequently presents a diagnostic formed to define the diagnostic criteria of this syndrome.problem. TWO cases of this entity are reported that demo The reciprocal d o n s h i p of the bronchial arterial cir-onshte its simhity to and differentiation from pulmo- culation in the hyperincent lung was described.nuy embolism and other intraplllmonic disorders. De- bnormal hyperlucency of one lung with the hy- C S REPORTS AEA perlucent lung of normal size or smaller than the contralateral lung is an unusual entity. This A 52-year-old woman was admitted to the hospital com- abnormality may be encountered on routine chest x- plaining of dyspnea, chest palpitations, weakyss, and expee ray examination in an asymptomatic individual or toration of pink sputum for the past ten days. Her past history may occur with clinical features suggestive of a was unremarkable. The patient denied having signiikant serious disease. respiratory illnesses in childhood or as an adult. No acute In 1953, Swyer and James1 first described this upper or lower respiratory episodes had been present prior to this hospital admission.entity in a report entitled "A Case of Unilateral Physical examination revealed a well-developed woman in Pulmonary Emphysema." Their clinical findings on a no acute distress. The blood pressure was 14/70 mm Hg, thesix-year-old boy with recurrent attacks of bronchitis pulse was 80 beats per minute and irregular, the respirationconsisted of a chest x-ray film showing a hyperlucent rate was 20/minute, and the temperature was 37.2OCright lung with the hemithorax and lung of theaffected side smaller than the contralateral side, pe-ripheral bronchiectasis, and angiocardiographic evi-dence of a right pulmonary artery much diminishedin caliber. In 1954, Macleod2 reported his observations onnine cases of abnormal "transradiancy" of one lung.The distinctive clinical features of his study werequiet breath sounds over the abnormal lung, hyper-lucency, diminished vascular pattern, and small ornormal size of the affected lung. Of this group, twopatients were free of symptoms, and their abnormal-ity had been discovered on routine examination; theother seven patients had shortness of breath andperiodic attacks of bronchitis. The purpose of this report is to describe twopatients with unilateral hyperlucency of the lungwho had clinical features suggestive of either a pul-monary embolism, bronchiectasis, bronchitis, or acongenital pulmonary disorder. A detailed pulmo-nary-bronchial arterial investigation of the affectedlung was part of this study.*From the Pulmonary Service, Department of Medicine, and the Department of Radiology, Los Angeles County-Univer- sity of Southern California Medical Center, Los Angeles.Manuscript received December 2, 1974; revision acceptedJanuary 2 1. FIGURE Inspiratory roentgenogram of chest showing hyper- 1.Reptint requests: Dr. Gottlieb, LAC-USC Medical Center, lucent right lung. There is slight swing of mediastinurn to1200 N. State Street, Los Angeles, 90033 . hyperlucent side ( case 1)62 GOTTLIEB, TURNER CHEST, 69: 1, JANUARY, 1976 Downloaded from chestjournal.chestpubs.org by guest on September 25, 2011 © 1976 American College of Chest Physicians
  3. 3. Table 1 - R d t r of Pulmonary-Function Studia from Core 1 Measurement* Observed Predicted FVC (L) 1.5 2.63.0 Maximum breathing capacity (L/min) 45 75100 Maximum expiratory flow rate (L/min) 80 216285 Maximum inspiratory flow rate (L/min) 100 130-200 FEVI (L) 0.7 1.8-2.2 FEVl/FVC (percent) 47 6476 FVC, Forced vital capacity; FEVI, forced expiratory volume in one eecond. showed a marked decrease in perfusion to the entire right lung. A ventilation lung scan demonstrated delayed clearance of radioactive 183xenon from the right lung during the washout. Arterial blood gas measurements performed while the patient was breathing room air showed an arterial oxygen pressure of 86 mm Hg, an arterial carbon dioxide pressure of 40 m m Hg, and a pH of 7.43. The admission electrocardiogram revealed frequent pre- mature atrial contractions and inverted or biphasic T wavesFIGURE2. Lung scan indicating almost absent perfusion over in leads V1 through V4. Findings from complete blood counts,right lung ( case 1) . urinalysis, and liver function tests, as well as the levels of(99F). The abnormal findings were limited to the chest. blood glucose, blood urea nitrogen, and serum enzymes, wereDullness, absent breath sounds, and scattered crackling basal all within normal limits.rilles were found over the right lung. The heart was not On the patients admission to the haspital, the diagnosticenlarged, and the rhythm was irregular. A grade 2 systolic consideration included an acute pulmonary embolism, bron-ejection murmur was audible at the apex, and the second chiectasis, bronchitis, or a congenital pulmonary disorder.pulmonic sound was of greater intensity than A*. The ex- The patient was initially treated with supplemental oxygentremities showed no clubbing or edema. There was noevidence of thrombophlebitis, venous varicosities, or calftenderness. The chest roentgenogram (Fig 1 ) revealed a hyperlucentright lung with decreased vascularity. The right hilum wasless prominent than the left. Perfusion lung scan (Fig 2)FIGURE Pulmonary angiogram indicating diminutive right 3. FIG~RE Bronchogram of right lung showing terminal, club- 4.pulmonary artery with sparse branches throughout right lung like bronchiectatic changes. There is no alveolar filling (case(case 1). 1).CHEST, 69: 1, JANUARY, 1976 SWYER-JAMES SYNDROME 63 Downloaded from chestjournal.chestpubs.org by guest on September 25, 2011 © 1976 American College of Chest Physicians
  4. 4. administered by face mask and intravenous heparin therapy. The bronchial angiogram demonstrated abnormalities con-Subsequently, pulmonary angiographic studies (Fig 3 ) sisting of hypertrophy and tortuosity of the bronchial artery,showed that the right pulmonary artery and branches were with proliferation and hypervascularity of the proximaluniformly markedly reduced in caliber. Right cardiac cath- branches. A bronchial arterial lung scan (Fig 6 ) with 1311-eterization showed that the pulmonary arterial pressure macroaggregated albumin injected into the catheterized rightwas 45/10 mm Hg and the right ventricular pressure was bronchial arterv revealed extensive radioactivity distributed40/5 mm Hg. At this point, the diagnosis was more consistent throughout the bronchial vasculature.with Swyer-James syndrome, and the heparin therapy wasdiscontinued. Further investigation of pulmonary function disclosedmoderate to severe obstructive airway disease (Table 1). A A 25-year-old woman was admitted to the hospital com-chest roentgenogram during expiration showed a mediastinal plaining of pain in the left side of t e chest, moderate hshift to the unaffected side, with no density changes in the shortness of breath, and slight tenderness in the left calfaEected lung. Bronchographic studies (Fig 4 ) showed dis- muscles. Her past history revealed that the patient wastinctive abnormal findings in the hyperlucent lung; major and hospitalized for an acute pulmonary embolism one yearsegmental bronchi were normally patent and distributed, but ago.the smaller bronchi were clubbed with a wide peripheral Physical examination disclosed a well-developed woman inunfilled wne between the clubbed bronchi and the chest no acute distress. The blood pressure was 108/62 mm Hg, thewall and there was almost complete absence of "alveolar pulse was 92 beats per minute, the respiration rate wasfilling." 20/minute, and the temperature was 37OC (98.6F). The Bronchial arteriographic studies (Fig 5 ) of the hyper- abnormal chest findings were on the left side and consisted oflucent lung were performed by selective catheterization of a slight respiratory lag, decreased vocal fremitus, increasedthe right bronchial artery. A precurved radiopaque catheter resonance to percussion, and coarse breath sounds. The heartwas introduced into the femoral artery by the Seldinger was slightly enlarged, and the rhythm was regular. A grade 1percutaneous technique and was advanced under fluoro- systolic murmur was heard in the third left interspace; thescopic guidance to the thoracic aorta, where the catheter was second pulmonic sound was greater than Az. Moderate calfpositioned into the right bronchial artery. The contrast tenderness was elicited in the left leg. Findings from themedium flowed readily through the bronchial artery. remainder of the physical examination were normal. Findings from the hemograrn and blood chemistry studies #.,a. were within normal limits. Sputum cultures for acid-fast bacteria were negative. Fungal and tuberculin skin tests were negative. Pleural aspirates were negatives for pathogens. The chest roentgenogram revealed a hyperlucent left lung of normal size, with thin pulmonary vascular shadows and a small hilar shadow. The left costophrenic angle showed blunting and thickening of the pleura. A perfusion lung scan revealed almost complete absence of perfusion in the left lung. Pulmonary angiographic studies (Fig 7 ) showed a diminutive left pulmonary artery with sparse peripheral vascular branches. A left bronchial angiogram demonstrated the bronchial artery to be of normal caliber, but increased branching and hypervascularity were evident in the basal lung region. A bronchial arterial lung scan showed increased radioactivity chiefly distributed through the vascular ram%- cations of the bronchial artery. These patients were admitted to the hospital with clinical features suggestive of either an acute pul- monary embolism, bronchiectasis, or a congenital pulmonary disorder. The basis for these diagnoses in the &st patient was the chest roentgenographic evi- dence of a unilateral hyperlucent lung, lack of perfu- sion on lung scan, and a cough productive of pink- stained sputum. In the second patient, these diag- noses were suggested by the presence of a hyper- lucent lung, pain over the affected lung, dyspnea, and pain in the calf muscles. Pulmonary embolism, other causes for diminished pulmonary perfusion, hyperaeration, loss of pulmo- nary parenchyma, and changes in the tissue coveringFIGURE Right bronchial angiogram showing enlargement, 5.proliferation, and hypervascularity of bronchial artery and the chest represent only a few of the entities thatproximal branches ( case 1) . may be confused with the Swyer-James syndrome.64 GOTTLIEB, TURNER CHEST, 69: 1, J N A Y 1976 AUR, Downloaded from chestjournal.chestpubs.org by guest on September 25, 2011 © 1976 American College of Chest Physicians
  5. 5. m - y . +-?; , FIGURE Right bronchial arterial lung scan 6. indicating extensive radioactivity throughout . bronchial vasculature ( case 1 ) The following tabulation represents a classification limited to the abnormal lung. The major bronchi are of disease entities presenting unilateral hyper- normal, but the smaller branches are club-like, and lucency of the lung: occasionally s a l buds project from the ends of the ml peripheral divisions. Generally, there is almost com- A. Factitious unilateral hyperlucency of lung plete absence of alveolar filling, demonstrated by a 1. Mastectomy well-demarcated clear zone between the bron- 2. Congenital absence or atrophy of pectoral muscles 3. Congenital absence or atrophy of shoulder girdle chiectatic smaller bronchi and the chest waU This B. Unilateral hyperaeration of lung (compensatory or ob- finding may explain the air trapping as a result of structive emphysema) check-valve obstructive processes in the peripheral C. Absence or maldevelopment of lung (agenesis or hypo- ~lasia a lobe or lobes) of D. Primary defects of pulmonary artery 1. Congenital a. Unilateral pulmonary arterial agenesis or hypoplasia 2. Acquired a. Pulmonary embolism b. Unilateral pulmonary arterial occlusion by tumor E. Swyer-James ( Macleods ) syndrome The distinctive radiographic signs of the Swyer- James syndrome are a unilateral hyperlucent lung of normal or decreased size with diminished lung, markings, a smaller hilar shadow than the opposite lung, and slight displacement of the mediastinum to the affected side.14 A chest roentgenogram during expiration characteristically reveals a mediastinal swing to the normal side and almost complete ab- sence of volume or density changes in the hyper- lucent lung.2 These signs are consistent with air trapping in the affected lung. The delayed washout of lS3xenonduring the ventilation lung scan is &o Frcum 7. Pulmonary angiogram showing small left pulmonary indicative of obstructive airways in the hyperlucent artery with very diminished peripheral vascular branches lung. The bronchographic findings are striking and (-2). CHEST, 69: 1, J N A Y 1976 AUR, SWYER-JAMES SYNDROME 85 Downloaded from chestjournal.chestpubs.org by guest on September 25, 2011 © 1976 American College of Chest Physicians
  6. 6. b r ~ n c h i Bronchograms of the uninvolved lung are .~ upholds the concept that a primary unilateral pul-normal. Bronchoscopic examination usually reveals monary vascular abnormality exists that predisposesno abnormality of the major bronchi. to bronchial and alveolar changes.12 Pulmonary-function studies performed on pa- Most patients are asymptomatic and have no com-tients with Swyer-James syndrome show the pattern plications; however, there are pitfalls in the diag-of obstructive airway d i s e a ~ e . Differential bron- ~,~ nosis of this abnormality. T i disorder may mimic hschospirometric analysis shows normal or diminished pulmonary embolism, a i d patients have been sub-minute volume but marked impairment of oxygen jected to protracted courses of anticoagulant ther-uptake in the hyperlucent apy. Acute infections associated with bronchiectasis Angiographic study in these patients reveals a are managed with conventional therapy. Surgicalmarkedly diminished pulmonary artery in the af- resection may be indicated for recurrent infections,fected lung, with almost complete absence of per- severe bronchiectasis, or hemorrhage.fusion on lung scan; however, previous reports havepointed out that at thoracotomy the small pulmo- REFERENCESnary artery, when visualized, may be larger in 1 Swyer PR, James GCW: A case of unilateral pulmonarycaliber than suspected on the basis of the angio- emphysema. Thorax 8: 133-136, 1953gram.ls8 2 Macleod WM: Abnormal transradiancy of one lung. Thorax 9: 147-153, 1954 Selective bronchial arteriographic studies and 3 Dornhorst AC, Heaf PV, Semple SVG: Unilateralbronchial arterial lung scans were performed to as- "emphysema." Lancet 2:873-875, 1957sess the relationship of pulmonary and bronchial 4 Darke CS, Chrispin AR, Snowden GS: Unilateral lungarterial blood flow in this disorder. In both patients transradiancy: A physiological study. Thorax 15:74-81,the bronchial artery in the hyperlucent lung devel- 1960 5 Margolin HN, Rosenberg LS, Felson B, et al: Idiopathicoped extensive branching and hypervascularity. unilateral hyperlucent lung: A roentgenologic syndrome.Bronchial arterial lung scans showed increased per- Am J Roentgen01 82:63-75, 1959fusion in the regions corresponding to the hyper- 6 Figueroa-Casas JC, Jenkins DE: Unilateral hyperlucencyvascular areas. of the lung (Swyer and James syndrome). Am J Med The two basic pathophysiologic defects in the 44:301-309, 1968 7 Culiner MM: The hyperlucent lung: A problem in differ-Swyer-James syndrome, unilateral bronchiectasis ential diagnosis. Dis Chest 49:578-586, 1986and hypoplasia of the pulmonary artery on the same 8 Rakower J, Moran E: Unilateral hyperlucent lung ( Swyer-side, may result in reciprocal increase in bronchial James syndrome ) . Am J Med 33: 864-872,1962arterial blood flow. Other studies have reported 9 Viamonte M Jr, Parks RE, Smoak WM IV: Guidedstriking morphologic and hemodynamic changes in catheterization of the bronchial arteries. Radioloy 85:205- 229, 1965the bronchial arterial circulation resulting from 10 Miyazawa K, Katori R, Ishikawa K, et al: Selectivebronchie~tasis.~~~ bronchial arteriography and bronchial blood flow: Corre- The etiology of this entity has not been clearly lative study. Chest 57:416-422, 1970defined. One theory supports the view that the initial 11 Reid L, Simon G, Zorab PA: The development of unilat- eral hypertransradiancy of the lung. Br J Dis Chestabnormality occurs in the peripheral bronchial tree 61 :190-192, 1967following infection at an early age, with secondary 12 Kent D: Physiologic aspects of unilateral hyperlucenthypoplasia of the pulmonary artery." Another view lung. Am Rev Respir Dis 90:202-212, 196466 GOTTLIEB, TURNER CHEST, 69: 1, JANUARY, 1976 Downloaded from chestjournal.chestpubs.org by guest on September 25, 2011 © 1976 American College of Chest Physicians
  7. 7. Swyer-James (Macleods) syndrome. Variations in pulmonary-bronchial arterial blood flow. L S Gottlieb and A F Turner Chest 1976;69; 62-66 DOI 10.1378/chest.69.1.62 This information is current as of September 25, 2011Updated Information & ServicesUpdated Information and services can be found at:http://chestjournal.chestpubs.org/content/69/1/62Permissions & LicensingInformation about reproducing this article in parts (figures, tables) or in its entirety can be foundonline at:http://www.chestpubs.org/site/misc/reprints.xhtmlReprintsInformation about ordering reprints can be found online:http://www.chestpubs.org/site/misc/reprints.xhtmlCitation AlertsReceive free e-mail alerts when new articles cite this article. To sign up, select the "Services" link tothe right of the online article.Images in PowerPoint formatFigures that appear in CHEST articles can be downloaded for teaching purposes in PowerPointslide format. See any online figure for directions. Downloaded from chestjournal.chestpubs.org by guest on September 25, 2011 © 1976 American College of Chest Physicians