Thorax (1953), 8, 133.

* A CASE OF UNILATERAL PULMONARY EMPHYSEMABY

PAUL R. SWYER AND G. C. W. JAMESFrom Warwick Hospital

(RECEIVED FOR PUBLICATION AUGUST 18, 1952)

It is not uncommon to see unilateral pulmonaryemphysema compznsating for lobar collapse, or asa mechanical result of valvular obstruction of abronchus. The clinical details of a case whereneither of these factors was present, with a patho-logical report on the resected lung, form the sub-ject of this paper.

CLINICAL REPORTThe patient was a boy aged 6 years who came under

observation after an attack of acute bronchitis inSeptember, 1949. At the ages of 5 weeks, 2 years, and3 years he had had bronchopneumonia, and since theage of 3 years he had been subject to recurrent attacksof bronchitis, which affected mainly the right lung.The boy was mentally normally developed, but slightly

below standard height and weight for his age. Slightclubbing of the fingers was present. Chest expansionwas poor on both sides, but more so on the right. Airentry on the right side was diminished with scattered

FIG. I .-Postero-anterior radiograph showing relative transradiancvof the right lung. The right hi!ar vascular shadow is smallerand the left larger than normal.

crepitations throughout, and there were a few persistentcrepitations at the left base. Other systems wereapparently normal.A radiograph of the chest showed relative trans-

radiancy of the right lung field compared with the left,with considerable decrease of vascular markings. Theheart was slightly displaced to the right. The righthemithorax and lung were smaller than the left despitethe increased transradiancy on the right (Fig. 1). Fluoro-scopy showed movement of the heart to the right oninspiration and restricted excursion of the rightdiaphragm.Bronchography showed on the left side only slight

dilatation of the posterior basic segmental bronchus ofthe lower lobe, and on the right a peculiar appearancebecause the peripheral bronchi failed to fill, with asharp line of demarcation between the proximal filledand the peripheral unfilled parts (Figs. 2 and 3). Bron-choscopy showed that the main bronchi were normalwith very little secretion present. Angiocardiographyshowed a normal heart with a right pulmonary arterymuch diminished in calibre, and confirmed the poor

FIG. 2.-Postero-anterior bronchogram. Note that the peripheraldivisions of the right bronchial tree compared with the lefthave not filled.

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vascular filling of the right lung in contrast to the over-

full left side, which had been suspected from the

appearance of the radiograph (Fig. 4). In view of thesefindings demonstrating a small, functionally deficientlung, prone to recurrent infections, a right pneumonec-

tomy was advised.

FIG. 3.-Right lateral bronchogram showing normal distribution ofmain bronchi but failure of peripheral filling.

FIG. 4.-Angiogram 3 seconds after injection showing the contrastbetween the under-filled right pulmonary artery and the over-filled left side.

Right dissection pneumonectomy was performed onJanuary 26, 1950, by Mr. J. Leigh Collis. On openingthe chest the pleura was free, and it was noticed thatcarbon pigmentation was present on the anterior partof the right upper lobe only and that the horizontalfissure was absent. Surprisingly the right pulmonaryartery was of larger calibre than the angiograms sug-gested. It was also noticed that the pulmonary veinswere larger than normal and that the right bronchialarteries were within normal limits. The post-operativecourse was uneventful.

PATHOLOGICAL REPORTRIGHT LUNG: MACROSCOPIC.-The fissure between

the upper and middle lobes was absent. The cranialone-third was blackish and the remainder pink. Dis-section of the bronchial tree showed normal ramifications.The cut stump of the pulmonary artery was about

7 mm. in diameter, dividing almost at once into twobranches 5 mm. in diameter.A group of cystic cavities 5-10 mm. in diameter was

present at the apex of.the upper lobe. No macroscopiccysts were seen in the lower lobe, which appearedemphysematous.MICRo3COPIC.-There were many cystic spaces of

varying diameters. The larger were lined by ciliatedcolumnar epithelium with plates of cartilage, abundantplain muscle fibres, mucous glands, but rather scantyelastic fibres in their walls. Some had only a partialepithelial lining and resembled dilated respiratorybronchioles. Some alveoli were collapsed and othersemphysematous. Fairly numerous lymphadenoidnodules were seen in relation to bronchi which weredilated, some of which showed chronic inflammatorychanges in their walls (Figs. 5 and 6).One of the most striking features was the widespread

obliteration of peripheral lung capillaries in the wallsof emphysematous alveoli (Fig. 7).

DISCUSSIONClinically, there were recurrent attacks of

bronchitis mainly confined to the right side of thechest. The small size of the right lung and thenormal bronchoscopic appearances showed thatthe condition was not emphysema due to obstruc-tion in the main bronchi. Similarly emphysemacompensating for a tight segmental atelectasis wasexcluded by the normal distribution of the mainbronchi shown by bronchography. The clinicaland radiological findings, supported by the angio-cardiograms, made us conclude that irreversiblechanges in the lung, possibly secondary to hypo-plasia of the right pulmonary artery, were pro-ducing an almost functionless lung. The clinicalsuggestion of hypoplasia of the right pulmonaryartery, however, was not upheld either by dissectionof the pulmonary artery at operation, or by theexamination of the lung, which showed that the

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A CASE OF UNILATERAL PULMONARY EMPHYSEMA

pulmonary artery was only slightly diminished insize and that the anatomical distribution wasnormal.

Pathological study of the resected lung suggestedcystic disease of the lung with secondary diminu-tion in the size of the pulmonary artery. Dotter

of the pulmonary artery. But if the causativefactor was in fact not congenital, it must have beenoperative from a very early age, in view of therelative absence of carbon pigmentation of theaffected lung in a child who had spent all his lifein an industrial coalmining district. The history

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FIG. 5.-Peribronchial fibrosis and lymphadenoid nodule of theright lower lobe. Van Gieson stain, x 35.

and Steinberg (1949) and Dotter (1950) in theirseries of angiocardiograms have noted a similardiminution in size of the pulmonary arterysecondary to widespread unilateral lung disease.The origin of the lung condition in our case is un-certain. We think that the cystic condition in thelung was more likely to be acquired than con-genital. Microscopically there was evidence ofconsiderable bronchitis and bronchiolitis, both pastand present, and the emphysema may well havebeen secondary to infection and peripheral obstruc-tion in the smaller bronchi. Although it is usualin emphysematous lungs for the main pulmonaryarterial branches to dilate rather than to contractin size, in our case the more normal left lung mayhave offered an alternative route of less resistancefor the pulmonary arterial blood.The alternative is to assume that the pulmonary

artery was congenitally abnormal and that the lungdysplasia was secondary to the resultant diminishedblood flow. This seems unlikely in view of thenormal macroscopic and microscopic appearances

FIG. 6.-Small bronchus of the right upper lobe. Someof the surrounding alveoli are dilated and some col-lapsed. Haematoxyiin and eosins x 30.

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FIG. 7.-Bronchiole of the right lower lobe containing secretionsurrounded by emphysematous alveoli with thin walls and fewcapillaries. Haematoxylin and eosin. x 35.

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PAUL R. SWYER and G. C. W. JAMES

of pneumonia at the age of 5 weeks may be signi-ficant in this connexion.We therefore conclude that our case showed

functional insufficiency of the right pulmonaryartery secondary to widespread lung disease of un-

certain, but possibly of acquired, origin.

SUMMARYA case is reported of unilateral pulmonary

emphysema, with secondary deficiency of bloodsupply.

Right pneumonectomy was performed success-

fully.The emphysematous condition is considered to

be acquired in origin.We wish to thank Dr. M. E. MacGregor and.Mr. J.

Leigh Collis for their help and permission to publishthis case; Dr. J. G. Scadding, who saw the case inconsultation, for his help and advice; Dr. H. S. Baarfor the pathological report; and Dr. Glasgow for'theangiogram.

REFERENCESDotter, C. T. (1950). Personal communication.

and Steinberg, 1. (1949). J. Amer. ned. Ass.. 133, 566.

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