sweyer james macleod syndrome

1. Swyer-James (Macleod's) 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
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2. 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 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
AE
A 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, the
six-year-old boy with recurrent attacks of bronchitis pulse was 80 beats per minute and irregular, the respiration
consisted of a chest x-ray film showing a hyperlucent rate was 20/minute, and the temperature was 37.2OC
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. 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 to
1200 N. State Street, Los Angeles, 90033 .
hyperlucent side ( case 1)
62 GOTTLIEB, TURNER CHEST, 69: 1, JANUARY, 1976
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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 waves
FIGURE2. 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
(99'F). The abnormal findings were limited to the chest. blood glucose, blood urea nitrogen, and serum enzymes, were
Dullness, absent breath sounds, and scattered crackling basal all within normal limits.
rilles were found over the right lung. The heart was not On the patient's admission to the haspital, the diagnostic
enlarged, 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 oxygen
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 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
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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 proximal
uniformly 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 right
was 45/10 mm Hg and the right ventricular pressure was bronchial arterv revealed extensive radioactivity distributed
40/5 mm Hg. At this point, the diagnosis was more consistent throughout the bronchial vasculature.
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 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
h
shift to the unaffected side, with no density changes in the shortness of breath, and slight tenderness in the left calf
aEected lung. Bronchographic studies (Fig 4 ) showed dis- muscles. Her past history revealed that the patient was
tinctive abnormal findings in the hyperlucent lung; major and hospitalized for an acute pulmonary embolism one year
segmental bronchi were normally patent and distributed, but ago.
the smaller bronchi were clubbed with a wide peripheral Physical examination disclosed a well-developed woman in
unfilled wne between the clubbed bronchi and the chest no acute distress. The blood pressure was 108/62 mm Hg, the
wall and there was almost complete absence of "alveolar pulse was 92 beats per minute, the respiration rate was
filling." 20/minute, and the temperature was 37OC (98.6'F). The
Bronchial arteriographic studies (Fig 5 ) of the hyper- abnormal chest findings were on the left side and consisted of
lucent lung were performed by selective catheterization of a slight respiratory lag, decreased vocal fremitus, increased
the right bronchial artery. A precurved radiopaque catheter resonance to percussion, and coarse breath sounds. The heart
was introduced into the femoral artery by the Seldinger was slightly enlarged, and the rhythm was regular. A grade 1
percutaneous technique and was advanced under fluoro- systolic murmur was heard in the third left interspace; the
scopic guidance to the thoracic aorta, where the catheter was second pulmonic sound was greater than Az. Moderate calf
positioned into the right bronchial artery. The contrast tenderness was elicited in the left leg. Findings from the
medium 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 covering
FIGURE Right bronchial angiogram showing enlargement,
5.
proliferation, and hypervascularity of bronchial artery and the chest represent only a few of the entities that
proximal branches ( case 1) . may be confused with the Swyer-James syndrome.
64 GOTTLIEB, TURNER CHEST, 69: 1, J N A Y 1976
AUR,
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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 ( 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 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
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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 predisposes
no 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
hs
chospirometric 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. Surgical
markedly 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 have
pointed out that at thoracotomy the small pulmo- REFERENCES
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66 GOTTLIEB, TURNER CHEST, 69: 1, JANUARY, 1976
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7. Swyer-James (Macleod's) 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, 2011
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