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DOI 10.1378/chest.69.1.62 1976;69;62-66Chest

 L S Gottlieb and A F Turner pulmonary-bronchial arterial blood flow.Swyer-James (Macleod's) syndrome. Variations in

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Swyer-James (Macleod's) 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 (Macleod's) syndrome (or unilateral hyperlucency of the lung) frequently presents a diagnostic problem. TWO cases of this entity are reported that demo onshte its simhity to and differentiation from pulmo- nuy embolism and other intraplllmonic disorders. De-

bnormal hyperlucency of one lung with the hy- A perlucent lung of normal size or smaller than the contralateral lung is an unusual entity. This abnormality may be encountered on routine chest x- ray examination in an asymptomatic individual or may occur with clinical features suggestive of a serious disease.

In 1953, Swyer and James1 first described this entity in a report entitled "A Case of Unilateral Pulmonary Emphysema." Their clinical findings on a six-year-old boy with recurrent attacks of bronchitis consisted of a chest x-ray film showing a hyperlucent right lung with the hemithorax and lung of the affected side smaller than the contralateral side, pe- ripheral bronchiectasis, and angiocardiographic evi- dence of a right pulmonary artery much diminished in caliber.

In 1954, Macleod2 reported his observations on nine cases of abnormal "transradiancy" of one lung. The distinctive clinical features of his study were quiet breath sounds over the abnormal lung, hyper- lucency, diminished vascular pattern, and small or normal size of the affected lung. Of this group, two patients were free of symptoms, and their abnormal- ity had been discovered on routine examination; the other seven patients had shortness of breath and periodic attacks of bronchitis.

The purpose of this report is to describe two patients with unilateral hyperlucency of the lung who had clinical features suggestive of either a pul- monary embolism, bronchiectasis, bronchitis, or a congenital pulmonary disorder. A detailed pulmo- nary-bronchial arterial investigation of the affected lung 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 accepted January 2 1. Reptint requests: Dr. Gottlieb, LAC-USC Medical Center, 1200 N. State Street, Los Angeles, 90033

t d e d roentgenologic and physiologic studies were per- formed to define the diagnostic criteria of this syndrome. The reciprocal d o n s h i p of the bronchial arterial cir- culation in the hyperincent lung was described.

CASE REPORTS

A 52-year-old woman was admitted to the hospital com- plaining of dyspnea, chest palpitations, weakyss, and expee toration of pink sputum for the past ten days. Her past history was unremarkable. The patient denied having signiikant respiratory illnesses in childhood or as an adult. No acute upper or lower respiratory episodes had been present prior to this hospital admission.

Physical examination revealed a well-developed woman in no acute distress. The blood pressure was 14/70 mm Hg, the pulse was 80 beats per minute and irregular, the respiration rate was 20/minute, and the temperature was 37.2OC

FIGURE 1. Inspiratory roentgenogram of chest showing hyper- lucent right lung. There is slight swing of mediastinurn to hyperlucent side ( case 1 ) .

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FIGURE 2. Lung scan indicating almost absent perfusion over right lung ( case 1 ) . (99'F). The abnormal findings were limited to the chest. Dullness, absent breath sounds, and scattered crackling basal rilles were found over the right lung. The heart was not enlarged, and the rhythm was irregular. A grade 2 systolic ejection murmur was audible at the apex, and the second pulmonic sound was of greater intensity than A*. The ex- tremities showed no clubbing or edema. There was no evidence of thrombophlebitis, venous varicosities, or calf tenderness.

The chest roentgenogram (Fig 1 ) revealed a hyperlucent right lung with decreased vascularity. The right hilum was less prominent than the left. Perfusion lung scan (Fig 2)

FIGURE 3. Pulmonary angiogram indicating diminutive right pulmonary artery with sparse branches throughout right lung (case 1).

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 mm Hg, and a pH of 7.43.

The admission electrocardiogram revealed frequent pre- mature atrial contractions and inverted or biphasic T waves in leads V1 through V4. Findings from complete blood counts, urinalysis, and liver function tests, as well as the levels of blood glucose, blood urea nitrogen, and serum enzymes, were all within normal limits.

On the patient's admission to the haspital, the diagnostic consideration included an acute pulmonary embolism, bron- chiectasis, bronchitis, or a congenital pulmonary disorder. The patient was initially treated with supplemental oxygen

FIG~RE 4. Bronchogram of right lung showing terminal, club- like bronchiectatic changes. There is no alveolar filling (case 1).

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administered by face mask and intravenous heparin therapy. Subsequently, pulmonary angiographic studies (Fig 3 ) showed that the right pulmonary artery and branches were uniformly markedly reduced in caliber. Right cardiac cath- eterization showed that the pulmonary arterial pressure was 45/10 mm Hg and the right ventricular pressure was 40/5 mm Hg. At this point, the diagnosis was more consistent with Swyer-James syndrome, and the heparin therapy was discontinued.

Further investigation of pulmonary function disclosed moderate to severe obstructive airway disease (Table 1). A chest roentgenogram during expiration showed a mediastinal shift to the unaffected side, with no density changes in the aEected lung. Bronchographic studies (Fig 4 ) showed dis- tinctive abnormal findings in the hyperlucent lung; major and segmental bronchi were normally patent and distributed, but the smaller bronchi were clubbed with a wide peripheral unfilled wne between the clubbed bronchi and the chest wall and there was almost complete absence of "alveolar filling."

Bronchial arteriographic studies (Fig 5 ) of the hyper- lucent lung were performed by selective catheterization of the right bronchial artery. A precurved radiopaque catheter was introduced into the femoral artery by the Seldinger percutaneous technique and was advanced under fluoro- scopic guidance to the thoracic aorta, where the catheter was positioned into the right bronchial artery. The contrast medium flowed readily through the bronchial artery.

#.,a.

FIGURE 5. Right bronchial angiogram showing enlargement, proliferation, and hypervascularity of bronchial artery and proximal branches ( case 1 ) .

64 GOTTLIEB, TURNER

The bronchial angiogram demonstrated abnormalities con- sisting of hypertrophy and tortuosity of the bronchial artery, with proliferation and hypervascularity of the proximal branches. A bronchial arterial lung scan (Fig 6 ) with 1311- macroaggregated albumin injected into the catheterized right bronchial arterv revealed extensive radioactivity distributed throughout the bronchial vasculature.

A 25-year-old woman was admitted to the hospital com- plaining of pain in the left side of the chest, moderate shortness of breath, and slight tenderness in the left calf muscles. Her past history revealed that the patient was hospitalized for an acute pulmonary embolism one year ago.

Physical examination disclosed a well-developed woman in no acute distress. The blood pressure was 108/62 mm Hg, the pulse was 92 beats per minute, the respiration rate was 20/minute, and the temperature was 37OC (98.6'F). The abnormal chest findings were on the left side and consisted of a slight respiratory lag, decreased vocal fremitus, increased resonance to percussion, and coarse breath sounds. The heart was slightly enlarged, and the rhythm was regular. A grade 1 systolic murmur was heard in the third left interspace; the second pulmonic sound was greater than Az. Moderate calf tenderness was elicited in the left leg. Findings from the remainder of the physical examination were normal.

Findings from the hemograrn and blood chemistry studies 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 covering the chest represent only a few of the entities that may be confused with the Swyer-James syndrome.

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' my- +-?;

, .

FIGURE 6. Right bronchial arterial lung scan indicating extensive radioactivity throughout bronchial vasculature ( case 1 ) .

The following tabulation represents a classification of disease entities presenting unilateral hyper- lucency of the lung:

A. Factitious unilateral hyperlucency of lung 1. Mastectomy 2. Congenital absence or atrophy of pectoral muscles 3. Congenital absence or atrophy of shoulder girdle

B. Unilateral hyperaeration of lung (compensatory or ob- structive emphysema) C. Absence or maldevelopment of lung (agenesis or hypo- ~lasia of a lobe or lobes) 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 ( Macleod's ) 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 lS3xenon during the ventilation lung scan is &o indicative of obstructive airways in the hyperlucent lung. The bronchographic findings are striking and

limited to the abnormal lung. The major bronchi are normal, but the smaller branches are club-like, and occasionally small buds project from the ends of the peripheral divisions. Generally, there is almost com- plete absence of alveolar filling, demonstrated by a well-demarcated clear zone between the bron- chiectatic smaller bronchi and the chest waU This finding may explain the air trapping as a result of check-valve obstructive processes in the peripheral

Frcum 7. Pulmonary angiogram showing small left pulmonary artery with very diminished peripheral vascular branches (-2).

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b r ~ n c h i . ~ Bronchograms of the uninvolved lung are normal. Bronchoscopic examination usually reveals no abnormality of the major bronchi.

Pulmonary-function studies performed on pa- tients with Swyer-James syndrome show the pattern of obstructive airway d i s e a ~ e . ~ , ~ Differential bron- chospirometric analysis shows normal or diminished minute volume but marked impairment of oxygen uptake in the hyperlucent

Angiographic study in these patients reveals a markedly diminished pulmonary artery in the af- fected lung, with almost complete absence of per- fusion on lung scan; however, previous reports have pointed out that at thoracotomy the small pulmo- nary artery, when visualized, may be larger in caliber than suspected on the basis of the angio- gram. ls8

Selective bronchial arteriographic studies and bronchial arterial lung scans were performed to as- sess the relationship of pulmonary and bronchial arterial blood flow in this disorder. In both patients the bronchial artery in the hyperlucent lung devel- oped extensive branching and hypervascularity. Bronchial arterial lung scans showed increased per- fusion in the regions corresponding to the hyper- vascular areas.

The two basic pathophysiologic defects in the Swyer-James syndrome, unilateral bronchiectasis and hypoplasia of the pulmonary artery on the same side, may result in reciprocal increase in bronchial arterial blood flow. Other studies have reported striking morphologic and hemodynamic changes in the bronchial arterial circulation resulting from bronchie~tasis.~~'~

The etiology of this entity has not been clearly defined. One theory supports the view that the initial abnormality occurs in the peripheral bronchial tree following infection at an early age, with secondary hypoplasia of the pulmonary artery." Another view

upholds the concept that a primary unilateral pul- monary vascular abnormality exists that predisposes to bronchial and alveolar changes.12

Most patients are asymptomatic and have no com- plications; however, there are pitfalls in the diag- nosis of this abnormality. This disorder may mimic pulmonary embolism, a i d patients have been sub- jected to protracted courses of anticoagulant ther- apy. Acute infections associated with bronchiectasis are managed with conventional therapy. Surgical resection may be indicated for recurrent infections, severe bronchiectasis, or hemorrhage.

REFERENCES

1 Swyer PR, James GCW: A case of unilateral pulmonary emphysema. Thorax 8: 133-136, 1953

2 Macleod WM: Abnormal transradiancy of one lung. Thorax 9: 147-153, 1954

3 Dornhorst AC, Heaf PV, Semple SVG: Unilateral "emphysema." Lancet 2:873-875, 1957

4 Darke CS, Chrispin AR, Snowden GS: Unilateral lung transradiancy : A physiological study. Thorax 15:74-81, 1960

5 Margolin HN, Rosenberg LS, Felson B, et al: Idiopathic unilateral hyperlucent lung: A roentgenologic syndrome. Am J Roentgen01 82:63-75, 1959

6 Figueroa-Casas JC, Jenkins DE: Unilateral hyperlucency of the lung (Swyer and James syndrome). Am J Med 44:301-309, 1968

7 Culiner MM: The hyperlucent lung: A problem in differ- ential diagnosis. Dis Chest 49:578-586, 1986

8 Rakower J, Moran E: Unilateral hyperlucent lung ( Swyer- James syndrome ) . Am J Med 33: 864-872,1962

9 Viamonte M Jr, Parks RE, Smoak WM IV: Guided catheterization of the bronchial arteries. Radioloy 85:205- 229, 1965

10 Miyazawa K, Katori R, Ishikawa K, et al: Selective bronchial arteriography and bronchial blood flow: Corre- lative study. Chest 57:416-422, 1970

11 Reid L, Simon G, Zorab PA: The development of unilat- eral hypertransradiancy of the lung. Br J Dis Chest 61 : 190-192, 1967

12 Kent D: Physiologic aspects of unilateral hyperlucent lung. Am Rev Respir Dis 90:202-212, 1964

66 GOTTLIEB, TURNER CHEST, 69: 1, JANUARY, 1976

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DOI 10.1378/chest.69.1.62 1976;69; 62-66Chest

L S Gottlieb and A F Turnerarterial blood flow.

Swyer-James (Macleod's) syndrome. Variations in pulmonary-bronchial

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