reinoXh - Thorax · had measles and chickenpox as a child and tonsil-lectomy had been performed in...

Thorax (1954), 9, 167.

A CONGENITAL BRONCHOPULMONARY CYST ASSOCIATEDWITH AN ANOMALOUS ARTERY

BY

M. ABUL-WAFAFrom the Victoria Hospital, Blackpool

(RECEIVED FOR PUBLICATION DECEMBER 23, 1953)

Although bronchogenic cysts of the lungs arenot very common, the widespread use of massminiature radiography and check radiography ofthe chest is revealing more and more of such con-genital anomalies. It may therefore be of advan-tage for radiographers and chest physicians to bearin mind this possibility in the differential diagnosisand appropriate treatment of such cases.The present case is of interest because there was

also a widespread murmur over the chest due toan associated vascular anomaly.

CASE HISTORYA 15-year-old boy who left school before Christ-

mas, 1952, had a routine mass miniature radiographtaken with his schoolmates, and this revealed ashadow lying anteriorly in the right lung (Figs. 1 and2). He started to work as an apprentice joiner in

anterol in thereinoXhmdloe

FIG. 1.-Postero-anterior radiograph ofthe lungs showing a cyst witha fluid level in the right paracardiac area.

1953, but was admitted to hospital for investigationlater in the year.He was asymptomatic, and there was no cough,

expectoration, dyspnoea, or loss of weight. He hadhad measles and chickenpox as a child and tonsil-lectomy had been performed in 1952. The familyhistory was not available as he is an adopted child,but his father is believed to have died of pulmonarytuberculosis.On examination he looked a well enough nourished

boy of fair intelligence with no cyanosis or fingerclubbing. No abnormal signs were discovered in thelungs. The apex beat lay in the fifth space in themidclavicular line; it was forcible and vibrating, butno definite thrill at the apex could be felt (Fig. 3).

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FiGs. 6-8.-Phonocardiographs and electrocardiographs after operation.

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M. ABUL-WAFA

The outstanding sign was a loud machinery murmurheard over the right upper chest, maximal in thesecond and third intercostal spaces near the rightsternal border (Fig. 4). The murmur was continuousand high pitched with systolic accentuation and wasconducted over a wide area anteriorly and to theback. A systolic thrill could also be felt over theright upper sternum. The blood pressure was 130/75mm. Hg.

In the electrocardiogram T3 was negative, butbiphasic radiological examination showed that thepulmonary conus might be slightly enlarged. Aphonocardiogram confirmed the presence of a systolicand diastolic murmur in the second and fourth rightinterspace (Fig. 5).The patient was given 1.8 million units of dista-

quaine penicillin daily for two and a half weeks with-out alteration in the radiological picture. During thatperiod his evening temperature rose to 990 F. on twooccasions but was otherwise normal. The highestwhite count recorded was 11,000 cells with 5,750neutrophils. A bronchoscopy was performed, but re-vealed nothing abnormal. A diagnosis of a pulmonaryabscess or infected pulmonary cyst in the right middlelobe was made, and it was thought that this wasinvolving the pulmonary arterial circulation in someway to cause the machinery murmur.

Mr. J. S. Glennie operated on April 15, 1953. Thepleura was approached through the bed of the re-sected fourth rib. It was noted that the vessels ofthe chest wall bled very fiercely. The pleura wasdensely adherent to the chest wall over the anteriorsurface of the middle lobe, and a network of arterieswas found in the region of the cyst, which was thoughtto lie in the middle lobe.The internal mammary artery was three times its

normal size. On palpation a thrill was felt over theanterior surface of the upper lobe. A right middlelobectomy was carried out in the course of which itwas found that the cyst was attached to the anterioraspect of the upper lobe, and that there was a verylarge artery going into the cyst. When the interna'mammary artery was tied the thrill over the upperlobe ceased abruptly. This shortly returned, however,and was not finally abolished until the artery con-necting the upper lobe and cyst was ligatured. Afterclosing the chest no vascular murmur was audibleand the thrill could no longer be felt. A considerablequantity of blood was lost during the operation andconvalescence was stormy; but the patient made anexcellent recovery. An electrocardiogram now sug-gests increased right ventricular strain. Post-operativetracings are shown in Figs. 6 to 8.

ifhe specimen consists of the rignt middle lobe andthe cyst, which contained necrotic debris, culture ofwhich was sterile. It was found that the cyst wasonly attached to the middle lobe by thin fibroustissue, but it was continuous with the anterior segmentof the upper lobe by means of a small bronchusand a large artery (Figs. 9 and 10).

Histological examination of different parts of thecyst wall shows a uniform picture of disorderly

bronchial epithelium surrounded by a connectivetissue capsule rich in vessels. There is no evidenceof recent infection in the cyst (Figs. 11, 12, and 13).

It is evident that the rich vascular ramificationin the wall of the cyst was contributed to by (1) abranch of the anomalous artery, (2) by branchesof intercostal arteries, and (3) by tributaries ofintercostal veins. No normal pulmonary vesselstook part in its vascular supply, and the venousreturn and drainage was via the intercostal veins.The remaining and major part of the systemic

anomalous artery continued upwards and piercedthe upper lobe, where a systemic pulmonary shuntwas established, and this accounted for the mach-inery murmur and the thrill.The resumption of the thrill over the upper lobe

eveil after ligature of the internal mammary arterycan be explained by the presence of rich vascularcollateral channels in the wall of the cyst.

It was observed by Pryce (1946) that the distri-bution of the anomalous artery could either be tonormal lung with no evidence of other pulmonaryabnormality, or to the cystic mass plus adjacentnormally connected lung (as in the case underdiscussion) or be confined to the cystic mass.As to the nature of bronchial attachment to the

upper lobe, the absence of any history of respira-tory infection, the bacteriological sterility of thecyst contents together with the histological appear-ance of its wall indicate that the attachment of thevestigial bronchus was primary as distinct fromsecondary bronchial communication which mighthave resulted from cyst infection. Infection isknown to occur in congenital cysts, even withoutdemonstrable communication with a bronchus bymeans of direct spread or by haematogenousorigin. This implies that the bronchopulmonarymass in this case was sequestrated from the rightupper lobe.The association of pulmonary sequestration with

an anomalous pulmonary blood supply has re-cently been reported by several observers includingPryce (1946), Pryce, Sellors, and Blair (1947),Bruwer, Clagett, and McDonald (1950), andKergin (1952).The usual clinical picture is a recurrent produc-

tive cough with or without haemoptysis; theremay be bouts of fever or a state consistent withrecurrent pneumonia. The presumptive diagnosiswas commonly of pneumonia, bronchiectasis, orbasal empyema, or was an accidental finding asin cases reported by Wyman and Eyler (1952).

Radiologically there is some abnormality at thebase of one or other lower lobes, more commonlythe left, which may show as an opacity or cystwith a fluid level.

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CONGENITAL BRONCHOPULD'JONAR Y CYST

Artery

FIG. 9 Fin. 10

FIGS. 9 and 10.-The operation specimen. In Fig. 10 the middle lobe is seen behind the mass.

FIG. 1 1 FIG. 12

FIGS. 11 and 12.-Photomicrographs of the cyst wall. x 15 and x 70.

On thoracotomy a systemic artery emergingfrom the aorta above or below the diaphragm isfound to pass in the pulmonary ligament and tosupply a sequestrated segment of the lower lobe,usually the posterior basal lobe. The latter showsa fairly constant pathological picture, namely, it

consists of a mass of unaerated primitive lungtissue devoid of pigment and containing one or

more bronchial cysts. A connexion with the bron-chial system of the normal lobe may or may notbe demonstrable, even when the cyst is found tobe aerated. No vein accompanies the anomalous

Bronchus

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M. ABUL-WAFA

FIG. 13.-Photomicrograph of cyst wall. x 320

artery except in some cases of extralobar seques-tration, and the veins then terminate directly orindirectly into the hemi-azygous veins.

It is suggested by Kergin (1952) that there isevidence of direct and considerable anastomosisbetween the systemic anomalous arterial supplyand the tributaries of the pulmonary vein, whichleads, if of sufficient degree, to burdening the leftventricle by a useless shunt analogous to an arterio-venous aneurysm (case 5 in his series).The blood carried by the anomalous artery,

whether to the norm-1 or to the sequestrated lung,is already oxygenated and passes unaltered to thevenous side. Further, the sequestrated mass isincapable of any function.Pulmonary arterial ramifications may or may not

be demonstrable in the primitive tissue.In the case cited above, although such a shunt

was not anatomically demonstrated, the character-istic loud continuous murmur can only be ex-plained by an abundant communication betweenthe high pressure systemic vessel and the lowpressure circulation, namely, the pulmonary veintributaries in the upper lobe. This fact is an argu-ment in favour of Kergin's view.

The accepted conception for the aetiology ofsuch combined anomalies is that introduced byPryce (1946) and Pryce and others (1947). Hestates that the vascular anomaly is the primarycondition, and that in the course of developmentof the lung the bulbous tip of one branch of theprimitive bronchus is appropriated by one of thegrowing embryonic vessels, and therefore remainscut off to a greater or less extent from the rest ofthe lobe.

SUMMARY AND COMMENTA condition, radiologically consistent with pul-

monary abscess, was accidentally revealed on massminiature radiography in a boy of 15 who wassymptomless.

Clinically the only sign was a loud continuoussystolic-diastolic murmur heard maximally overthe right second and third intercostal spaces nearthe sternum accompanied by a systolic thrill.

Subsequent thoracotomy has shown the abnor-mality to be a congenital bronchopulmonary masswith an anomalous systemic arterial supply andto be situated in front of the right middle lobe.A brief review of some other combined con-

genital anomalies in the lung is cited.The case differs from most of the series described

because (1) it was revealed before infection tookplace, (2) the lobe was sequestrated from the rightupper lobe, (3) its anomalous systemic supply camefrom the internal mammary artery, and (4) itshowed a peculiar machinery murmur to the rightof the sternum, due to a systemic pulmonary shuntin the substance of the right upper lobe.

I wish to express my thanks to Dr. W. J. Hay, ofthe Royal Infirmary, Lancaster, and to Mr. J. S.Glennie, of Victoria Hospital, Blackpool, for theirkind permission to publish this case, and I am in-debted to Dr. Hay for providing the specimen, photo-micrographs, and E.C.G. tracings.

REFERENCES

Bruwer, A., Clagett, 0. T., and McDonald, J. R. (1950). J. thorac.Surg., 19, 957.

Kergin, F. G. (1952). Ibid., 23, 55.Pryce, D. M. (1946). J. Path. Bact., 58, 457.- Sellors, T. H., and Blair, L. G. (1947). Brit. J. Surg., 35, 18.WVyman, S. M., and Eyler, W. R. (1952). Radiology, 59, 658.

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