COMPLICATIONS OF LOCALIZED PULMONARYEMPHYSEMA IN CHILDHOOD

BY

S. van CREVELD, M.D., and J. van den BROECKE, M.B.

(From the Children's Clinic of the Municipal University of Amsterdam, Holland)

Our knowledge of the causes of interstitial pul-monary emphysema and its eventual complicationshas been greatly extended by clinical and experi-mental observations made principally during thelast ten years. We can now assume with confidencethat interstitial pulmonary emphysema is usuallycaused by rupture or leakage of the alveoli, due totrauma or raised intra-pulmonary pressure, oralternatively is due to spontaneous rupture ofalveoli. Air which enters the interstitial tissuesfrom the alveoli is able to shift position, and topass along the sheaths ofthe blood vessels. Macklin(1937, 1939) and Fischer and Macklin (1940) haveshown experimentally that the entrance of air intothe sheath-system is effected by way of multipleminute tears in the base of the alveoli, which areclosely connected with the finer ramifications of thepulmonary vessels. The cause of these tears isusually a rise of intra-alveolar pressure, occurringas a result of transient bronchial obstruction of thecheck-valve type; this we now know may some-times lead to localized pulmonary emphysema andultimately to cavitation of the lungs. It is, therefore,not surprising that localized bullous emphysemaand interstitial pulmonary emphysema (occurringapart from birth trauma or asphyxia) have oftenbeen observed in pneumonia, pertussis, stenosis ofthe larynx, and asthma.

Attention has been drawn to this cavity-formationresulting from localized builous emphysema inpneumonia in childhood by Tillema (1940) of thisclinic, and by a number of other authors (Caffey,1940; Wissler, 1938; Benjamin and Childe, 1939;and more recently by Leopold and Kratzman, 1945).In the above-mentioned conditions, thick and stickysputum readily leads to obstruction of the trachea,bronchi, and bronchioli, whilst coughing furtherincreases the intra-pulmonary pressure. Macklinhas also shown that distension of the alveolar wallscan exist for a long time without rupture occurring.When, however, interstitial pulmonary emphysemahas been established, air can then pass (principallyalong the sheaths of the blood vessels) to the media-stinum, resulting in mediastinal emphysema. In-creased pressure on the pulmonary vessels will atthe same time affect the circulation to a greater orless extent. In cases of post-operative atelectasis,associated with forcible coughing to clear theobstructed bronchus, pneumo-mediastinum has

occurred, owing to rupture of the mediastinal pleura.In the mediastinum, the air can be gradually

resorbed, or may lead to further obstruction of thecirculation from pressure on the heart and greatvessels. When the pressure of air increases to agreat extent, however, the air will seek an outletfrom the mediastinum; this has repeatedly beenobserved in animal experiments when the lungswere forcibly inflated. The easiest exit of air fromthe mediastinum appears to be by way of the con-nective tissue around the blood-vessels, and mayoccur in one of three different directions: (1) tothe subcutaneous and deeper tissues of the neck(sometimes also of the shoulders, face, trunk andextremities), (2) to the pleural cavity, and (3) alongthe aorta and oesophagus, through the diaphragmto the retroperitoneal tissues of the flanks andperirenal areas.

Subcutaneous emphysema in the neck, however,is not necessarily due to air in the mediastinum,and before the latter condition can be assumed,other possible causes such as wounds of the thoraxcommunicating with the lung, or air originatingfrom the mouth or nasal cavity, must be excluded.Escape of air from the mediastinum to the pleural

cavity causes a pneumothorax, and bullous emphy-sema can, therefore, lead to a pneumothorax byway of interstitial and mediastinal emphysema (cf.Hamman, 1945). Bullous emphysema may also giverise to a pneumothorax, however, by the burstingof a bulla situated on the surface of the lung. Thistook place, for example, in the patient with infec-tious reticulo-endotheliosis described by van Creveldand ter Poorten (1935), where a great many suchbullae were present on the surface of the lungs. Atautopsy, the opening in the ruptured bulla whichhad caused the pneumothorax could be identifiedimmediately.That air from the mediastinum can find its way

into the pleural cavity has clearly been demonstratedexperimentally; the forcible introduction of airinto the lungs of an animal results in a pneumo-thorax. When sudden death occurs in such anexperiment, bilateral pneumothorax with collapseof both lungs is found at autopsy. However, underthese circumstances, there is no trace of a ruptureor perforation of the visceral pleura. In Hamman'sopinion (confirmed by a recent observation of ourown), tension pneumothorax may be secondary to

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ARCHIVES OF DISEASE IN CHILDHOODpneumo-mediastinum rather than to a valvularrupture through the pleura which allows air toenter but not to leave the pleural cavity.The severity of the symptoms will depend on the

volume of air which enters and remains in themediastinum after the occurrence of interstitialemphysema. When mediastinal emphysema isfollowed by extensive subcutaneous emphysema inthe neck, the symptoms directly due to mediastinalemphysema are usually few and relatively insignifi-cant. Thus subcutaneous emphysema in the neckmay actually be the first symptom of mediastinaland interstitial emphysema respectively.

Although the causes of interstitial emphysemawith its sequelae, and those of localized bullcusemphysema, are usually the same, it is exceptionalfor both types of emphysema to be present simul-taneously. That this is possible, however, and alsothat symptoms of localized bullous emphysema canexist at the same time as the sequelae of interstitialemphysema (e.g. emphysema of the neck) is provedby one of the cases discussed below.The severe symptoms which arise when much air

is present in the mediastinum closely resemble thosecaused by increased intra-pericardial pressure.Dyspnoea and cyanosis may appear early, due topressure on blood-vessels; these symptoms gradu-ally become more severe, and unless relieved arelikely to prove fatal. In Macklin's experiments, itwas found that when air was blown into a lobe ofthe lung by means of a catheter, there appearedaround the tip of the catheter a balloon-shapedemphysematous portion of lung surrounded by alayer of atelectasis. Around the vessels descendingto the mediastinum, Macklin observed numerousair-bubbles, forming a canal in the sheath of thevessel. When emphysema was produced in animalsby the introduction of air directly into the media-stinum, and death followed, it was found that theheart and great vessels in the thorax were surroundedby air-bubbles.The presence of air in the mediastinum can some-

times be seen very clearly in the x ray, whilst inother cases it must be looked for more carefully.In the antero-posterior view, a sharply demarcatedtransparent band can sometimes be observed in theline of the pericardium. Hamman considers thatthis localization of mediastinal emphysema is some-times wrongly regarded as a pneumo-pericardium;as a rule, according to this author, it is in fact a' pneumo-praecordium.' Others, however, regardit as a true pneumo-pericardium. Such an appear-ance has been observed in cases of whooping-cough, pulmonary tuberculosis, broncho-pneumonia,stenosis of the larynx, after severe physical exertion,after operations on the neck, thorax and abdomen,following traumatic perforations of the oesophagus,in infections with gas-producing organisms, andafter necrosis due to radium (Graebner, 1939). Inview of Mackiln's experiments and of the casediscussed below, we believe that in these circum-stances it is quite possible for mediastinal emphy-sema to produce a pneumo-pericardium, the air

reaching the pericardium along the sheaths of theblood- and lymph-vessels (compare the passage ofdust along the same route).When interstitial emphysema occurs as a result

of excessive pressure in the alveoli, such factors asweakness of the alveolar walls due to infectious,toxic or hereditary influences, are likely to play apart in its production. Predisposed individualsmay suffer from repeated attacks of interstitialemphysema, and this is also true of spontaneouspneumothorax.

Case reportsCase 1. Baby J., aged ten weeks, was admitted

in August, 1945, to the Children's Clinic for feedingdisorder. The parents were healthy and the familyhistory essentially negative. The infant had beenborn at full term, weighing 3,500 g., and was notasphyxiated. Apart from the feeding difficulty atthe timne of admission, the infant had been healthy.ON ADMISSION. The child appeared to be

moderately ill; its nutritional state was poor, andits weight 2,700 g. The heart and lungs showed noabnormalities. Slight albuminuria was present.The blood picture was normal.

PROGRESS. The digestive disturbance soon im-proved, but recurrent abscesses of the skin ofvarying size appeared. The Pirquet tuberculin testwas negative. On November 5, 1945, the infantdeveloped rhinitis, and the following day began tocough. On the night of November 6-7, the infanthad two attacks of extreme dyspnoea, in which itbecame deeply cyanotic and perspired freely; thepulse was slow, and there was inspiratory retractionof the jugular region and lower part of the thorax.The temperature was not affected, and there wereno convulsions.On November 7, the infant had several attacks

of vomiting and coughing, and during the night afurther attack of dyspnoea and cyanosis. Noabnormal physical signs were detected in the heartand lungs. The following day the child's conditionhad deteriorated. Examination of the left flankand left upper chest posteriorly showed someimpairment of percussion note and diminishedbreath sounds. The heart was not displaced, andno murmur was heard. The heart sounds were loud.The blood picture showed 24,600 white blood cellsper c.mm., of which 64 per cent. were polymor-phonuclear, 33 per cent. lymphocytes, and 3 per cent.monocytes; toxic granulation was marked. Thesame afternoon, shortly after a feed, a further attackof severe dyspnoea and cyanosis occurred, lastingabout two minutes; the infant was ashen-colouredduring the attack, and this colour persisted forsome considerable time. The respiration remainedshallow, the alae nasi working, and severe inspi-ratory retractions continued. The pulse rate was106 per minute and the temperature 35-8- C. In theoxvgen tent the child became quieter and had nofurther attacks, though some dyspnoea remained.On November 9 there was impaired percussion

note and reduction of breath sounds over the leftupper and right lower lobes and in the left flank.X ray of the chest (fig. 1) showed a sharply demar-cated, rather wide transparent area along the wholeof the left border and right outer border of theheart. Translucency of this type can only repre-sent a pneumo-pericardium. There was also an

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LOCALIZED PULMONARY EMPHYSEMA

FIG. l-X ray of chest, Case 1, showing a sharply demarcated, rather wide transparent areaalong the whole of the left border and right outer border of the heart.

FIG. 2.-X ray of the chest, C&be i, taken foar days after x ray m fig. 1, and still showingsome air adjacent to the right border of the heart

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ARCHIVES OF DISEASE IN CHILDHOODaccumulation of air in the left and right sides of themediastinum. A second x ray (fig. 2) taken fourdays after the first, still showed some air adjacentto the right border of the heart, but a third x ray,taken two weeks after the first, showed that thisair had by then disappeared.The clinical condition of the child improved

rapidly, but some degree of dyspnoea remained fora considerable time.Comment Radiographic evidence was found of

a pneumo-pericardium with pneumo-mediastinumin a child with broncho-pneumonia, who sufferedfrom severe attacks of dyspnoea with cyanosis. Ofthe signs described in analogous cases, one waslacking, viz. the characteristic praecordial crepita-tion resembling the crackling of cellophane (Ham-man's sign). It is obvious that in an infant of thisage the subjective symptoms, e.g. pain caused bythe pressure of air on the ramifications of the pul-monary artery, or the anginal pain due to pressureof air on the coronary vessels, could not be elicited.

In this case the development of the pneumo-pericardium was probably slow and insidious. Inaddition, a definite pneumo-mediastinum waspresent. We imagine that the course of events wasas follows: air entered the mediastinum via thesheaths of the blood- or possibly lymph-vessels, asa result of rupture or leakage from the alveolarwalls which had become weakened by the respira-tory tract infection. The pneumo-mediastinum soproduced may itself have given rise to attacks ofdyspnoea and cyanosis. The air in the mediastinumthen passed by way of the lymph-clefts to the peri-cardium, producing a pneumo-pericardium. Thiswas manifested by loud heart sounds, and was alsopossibly partly responsible for the dyspnoea andcyanosis. As in the majority of such cases, the airwas resorbed moderately quickly, so that the mostserious results, viz. oedema of the lungs, increaseddyspnoea and cyanosis, and finally death, did notoccur. (These results can sometimes only beavoided by active intervention, when an outlet forthe compressed air must be provided.)

Case 2. A girl, aged five years two months, wastransferred on November 9, 1945, to the Children'sClinic from another hospital. A diagnosis ofcavernous phthisis had been made, mainly onaccount of the x-ray appearance of the lungs. Afortnight before admission to the Children's Clinic,the child had complained of pain in the rightelbow, and it was found that movement of the armcaused pain in the right shoulder. Three dayslater the temperature was 38- to 39-4J C., andthough the other joints were not involved andthere was no redness or swelling, a diagnosis ofrheumatism had been considered. The child hada poor appetite, and had been constipated anddrowsy; cough had become more frequent duringthe five days before transfer, and there was con-siderable expectoration. The previous history hadbeen essentially negative, and although there hadbeen recurrent cough and poor appetite for theprevious six months, there had been no loss ofweight. The family was healthy, and since thechild's illness both parents had been examrined toexclude tuberculosis, with negative result.ON ADMI55ON the child appeared moderately ill,

but was active, and her nutritional state was reason-ably good. She occasionally coughed up yellowish-

green sputum, but no blood. (Repeated examina-tion of the sputum for tubercle bacilli was negative.>There was no cyanosis, clubbing, dyspnoea oradenopathy. The pulse and respiration rates werenormal, the temperature 39" C. The tongue wasfurred. The trachea was central. On the rightside, in the region of the coracoid process betweenthe sternomastoid and trapezius muscles, it waspossible to feel coarse emphysema, which was moreobvious after coughing. This area was painful onpressure, as was the right shoulder. There was noredness or swelling of the joints, but passive move-ment caused pain in the right elbow and shoulder.The percussion note was impaired, anteriorly andposteriorly, over the right upper zone; on auscul-tation, sub-bronchial respiration could at first beheard with a single rhonchus and increased xocalfremitus. The apex beat was palpable in the fourth

FIG. 3.-X ray of Case 2, taken on Nov. 6.

intercostal space, just inside the nipple line; thearea ofcardiac dullness was not enlarged; the cardiacsounds were pure, not excessively loud, and regular.The urine showed a trace of albumin but was

otherwise normal. Blood examination showedhaemoglobin 59 per cent. (Sahli), red blood cells3-3 million per c.mm., white blood cells 15,000 perc.mm. (eosinophils 1 per cent., polymorphs 68 percent., lymphocytes 28 per cent., mononuclears 3 percent.); Loxic granulation was much in evidence.RADIOLOGICAL EXAMNATnON. The x ray taken

on November 6 (fig. 3), which was sent to us.showed some characteristic and very large areas ofdiminished density in the right upper lobe, about3 cm. in diameter, surrounded by wide borders ofincreased density. In addition, some shado-vvsindicating subcutaneous emphysema were visible inthe right supraclavicular region and over the rightside of the thorax. Signs of right-sided mediastinalemphysema were also present, and in the pericardialline a narrow area of translucency was visible(? pneumo-pericardium). The right hilar shadowwas enlarged, and calcified foci were present in thisarea, whilst on the right a linear shadow in theposition of the interlobar septum was present.A subsequent x ray of the chest taken on

November 16 (fig. 4) showed that the areas ofdecreased density in the lung, and also the areas ofsubcutaneous emphysema, had become smaller: inthe right upper iobe, evidence of infiltration "asstill visible.

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FIG. 4.-X ray of Case 2, taken on Nov. 16.

On December 27 radiological examination showedno abnormality apart from the somewhat enlargedright hilar shadow, containing calcified foci, andthe linear shadow previously noted in the region ofthe interlobar septum.TUBERcULIN sTES. The Pirquet test was nega-

tive on admission, but the Mantoux test (1 in 1,000)was positive a week later, and again on December 7.It was concluded that the child had had a previoustuberculous infection, but that her condition onadmission was due to localized bullous emphysemacomplicated by subcutaneous and mediastinalemphysema.

PRoGRESS. Following admission, the temperatureremained high, and cibazol was prescribed, since inthe majority of published cases bullous emphysemahas been associated with pneumonia. The tempera-ture then rapidly subsided, and both physical signsand expectoration rapidly decreased. The bloodsedimentation rate on November 21 was 24 mm. inone hour, and on December 7 blood examinationshowed haemoglobin 82 per cent. (Sahli), red bloodcells 44 million per c.mm., white blood cells 6,500per c.mm., of which 52 per cent. were polymorpho-nuclear; toxic granulation was no longer present.Subsequently the child's general condition rapidlyimproved.Comment. This second case is an example of

the association of symptoms which may followdistension of the alveoli. The occurrence of sub-cutaneous emphysema of the neck, in the absenceof other causes, may be regarded as proof of thepresence of mediastinal emphysema, particularlysince the initial symptoms (pain in the right shoulderand elbow) have frequently been observed in similarcases. They should be regarded as the result ofdistension and possibly rupture of tissues throughwhich the air escapes from the mediastinum. (Thepassage of air from the lung tissue to the media-stinum may also give rise to pains either radiatingto the shoulders or down the arms, or localized tothe substernal area, which may suggest a falsediagnosis of angina pectoris or rheumatism.)

In the first antero-posterior x ray of the chest,we found localized bullous. subcutaneous andmediastinal emphysema present at the same time.

There was also a narrow translucent area along themargin of the pericardium, but (unlike the appear-ance seen in the first case) we do not feel justifiedin ascribing this to a pneumo-pericardium. Weconsider that it is more probably due to the presenceof air between the heart and surrounding tissues.In this case, the bullous emphysema which arose asa consequence of a relative stenosis of the bronchusis regarded as primary, and the interstitial emphy-sema with its various complications as secondary.

Since the child showed a positive Mantouxreaction, the following conditions were consideredin differential diagnosis: (1) cavity formation inprimary tuberculosis of the lung; (2) localizedbullous emphysema resulting from valvular stenosisof a bronchus or bronchioli by tuberculous lesions;and (3) localized bullous emphysema due tovalvular stenosis associated with an acute specificinfection of the respiratory tract, in a patient whohad previously had a tuberculous infection. Thefirst of these diagnoses could be excluded in viewof the x-ray appearance of the cavities, their rapiddisappearance, and the absence of tubercle bacilliin the sputum. The second diagnosis cannot beexcluded in this case. Emphysematous bullae,which during life were regarded as being tuber-culous cavities, have most frequently been observedin cases of pulmonary tuberculosis (Miller, 1933;Freedman, 1936; Spivek, 1936), though Benjaminand Childe (1939) have not observed the conditionin children with pulmonary tuberculosis withoutpneumonia. We are, however, inclined to thethird diagnosis on account of the rapid disappear-ance of the cavities, the absence of tubercle bacilliin the sputum, the presence of calcified foci, therapid subsidence of pyrexia and local signs, andthe rapid improvement of the general conditionfollowing the administration of cibazol. In bothcases, no other treatment was required. Guinea-piginoculation with fasting stomach-contents gave anegative result. X ray of the lungs on February 2,1946, showed only some small calcified foci at theright hilum.

SwTwo cases have been described which clearly

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144 ARCHIVES OF DISEASE IN CHILDHOOD

illustrate the complications, clinical and radio-logical signs, and symptoms, to which localizedbullous and interstitial emphysema may give rise inchildhood.The first patient, an infant, showed a definite

pneumo-pericardium occurring as a complicationof broncho-pneumonia. Attacks of cyanosis andincreased cardiac sounds provided clinical con-firmation of the diagnosis.The second patient, a girl aged five years, showed

symptoms and signs of bullous and subcutaneousemphysema occurring simultaneously, with, inaddition, radiological evidence of mediastinalemphysema and air situated around the heart.Here the localized bullous emphysema must havepreceded the occurrence of the interstitial emphy-sema. Attention is drawn to the particular clinicalsigns which were present in this case.

REFERENCES

Benjamin, B., and Childe, A. E. (1939). J. Pediat., 15620.

Caffey, J. (1940). Amer. J. Dis. Child., 60, 586.Creveld, S. van, and Poorten, F. H. ter (1935). Arch.

Dis. Childh., 10, 125.Fischer, J. H., and Macklin, C. C. (1940). Amer. J.

Dis. Child., 60, 102.Freedman, E. (1936). Amer. J. Roentgenol., 35, 324.Graebner, H.(1939). Arch. Otolaryng. Chicago, 29,446.Harmman, L. (1945). J. Amer. med. Ass., 128, 1.Leopold, J. S., and Kratzman, E. A. (1945). Amer. J.

Dis. Child., 69, 287.Macklin, C. C. (1939). Arch. intern. Med., 64, 913.- (1937). Canad. Med. J., 36, 414.Miller, W. S. (1933). Amer. Rev. Tuberc., 28, 359.Spivek, M. L. (1936). Amer. J. Dis. Child., 51, 69.Tillema, E. A. (1940). Ned. Tj;dschr. Geneesk., 84, 3666.Wissker, H. (1938). Arch. Kinderheilk., 113, 16.

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