Thorax (1964), 19, 1 Congenital cystic adenomatoid malformation of the lung RAYMOND BELANGER, LEO R. LA FLfCHE, AND JEAN-LOUIS PICARD From the Departments of Radiology, Surgery, and Pediatrics, Notre Dame Hospital, Montreal, Quebec, Canada Congenital cystic adenomatoid malformation (C.C.A.M.) of the lung has seldom been reported in the medical literature, and, because of the necessity for early radiological diagnosis and the relative urgerncy of surgical resection once diagnosed, the authors feel that this case merits publication. According to Caffey (1953) true congenital cysts of the lung are rare, and many so-called cystic lesions are transient and disappear spontaneously without surgical resection. Various lesions includirng emphysema, bronchiectasis, malignant neoplasms, bronchogenic cysts, and pulmonary sequestrations have been grouped in the literature under the name 'cystic disease of the lung'. The congenital nature of cystic adenomatoid disease of the lung is without question, as we shall see from the description of the pathology of the lesions. The first case to be published in the English medical literature was reported by Ch'In and Tang (1949); these authors included 10 cases that were reported in German publications. In all these, premature children, frequently stillborn, were involved and nearly always these children exhibited anasarca; quite frequently the mother's pregnancy had been complicated by hydramnios. Craig, Kirkpatrick, and Neuhauser (1956) reported four cases wherein surgical resection had been carried out within the first two weeks of neo-natal life. At the same time they brought up to date the list of cases tabulated by Ch'In and Tang. We have attempted to list the cases reported to date (Table 1). PATHOLOGY One of the most important features of these lesions is the often considerable increase in weight and volume of the affected part of the lung. On section the absence of normal septa in the areas comprising the cystic adenomatoid malformation is a reflection of the absence of any well-defined bronchial organization. Microscopic examination reveals an identical picture in the majority of cases, the essential features having been enunciated by Kwittken and Reiner (1962) as follows: (1) an 'adenomatoid' increase of terminal respiratory structures as manifested by cysts of varying size communicating with each other and variably lined with either a pseudo-stratified ciliated columnar (bronchial- type) epithelium or a single-layered cuboidal epithelium; (2) polypoid configuration of the mucosa and increased amounts of elastic tissue in the walls of the cystic portions lined with bronchial-type epithelium; (3) absence of cartilage plates in the cystic parenchyma except as constituents of non-deformed bronchial structures trapped within the diseased lung; (4) occasional groups of alveoli lined with mucogenic cells; and (5) absence of inflammation. Bain (1959) described an additional feature which consisted in the absence of alveolar formation except in the immediate sub-pleural zone. CASE HISTORY A term male infant weighing 3-26 kg. (7 lb. 3 oz.) at birth was delivered by caesarean section because of cephalo-pelvic dystocia after a trial labour lasting 10 hours. The mother was a 32-year-old primipara who had experienced a completely normal pregnancy. The baby breathed spontaneously at birth. The initial examination of the newborn child was normal. A dry cough was noted during the first two days in the nursery; pulmonary auscultation remained nor- mal. He was considered a normal child until the eighth day when the mother was discharged from hospital. A chest film was requested as a precau- tionary measure because of the dry cough noted during the first days of life. Initial A.P. and lateral films (Fig. IA, IB) of the 8-day-old baby showed an increase in volume of the on March 11, 2021 by guest. Protected by copyright. http://thorax.bmj.com/ Thorax: first published as 10.1136/thx.19.1.1 on 1 January 1964. Downloaded from
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Thorax (1964), 19, 1
Congenital cystic adenomatoid malformationof the lung
RAYMOND BELANGER, LEO R. LA FLfCHE, AND
JEAN-LOUIS PICARD
From the Departments of Radiology, Surgery, and Pediatrics, Notre Dame Hospital, Montreal,Quebec, Canada
Congenital cystic adenomatoid malformation(C.C.A.M.) of the lung has seldom been reportedin the medical literature, and, because of thenecessity for early radiological diagnosis and therelative urgerncy of surgical resection oncediagnosed, the authors feel that this case meritspublication.
According to Caffey (1953) true congenital cystsof the lung are rare, and many so-called cysticlesions are transient and disappear spontaneouslywithout surgical resection. Various lesionsincludirng emphysema, bronchiectasis, malignantneoplasms, bronchogenic cysts, and pulmonarysequestrations have been grouped in the literatureunder the name 'cystic disease of the lung'. Thecongenital nature of cystic adenomatoid diseaseof the lung is without question, as we shall seefrom the description of the pathology of thelesions.The first case to be published in the English
medical literature was reported by Ch'In and Tang(1949); these authors included 10 cases that werereported in German publications. In all these,premature children, frequently stillborn, wereinvolved and nearly always these childrenexhibited anasarca; quite frequently the mother'spregnancy had been complicated by hydramnios.Craig, Kirkpatrick, and Neuhauser (1956) reportedfour cases wherein surgical resection had beencarried out within the first two weeks of neo-natallife. At the same time they brought up to datethe list of cases tabulated by Ch'In and Tang. Wehave attempted to list the cases reported to date(Table 1).
PATHOLOGY
One of the most important features of these lesionsis the often considerable increase in weight andvolume of the affected part of the lung. On sectionthe absence of normal septa in the areas
comprising the cystic adenomatoid malformationis a reflection of the absence of any well-definedbronchial organization.
Microscopic examination reveals an identicalpicture in the majority of cases, the essentialfeatures having been enunciated by Kwittken andReiner (1962) as follows: (1) an 'adenomatoid'increase of terminal respiratory structures asmanifested by cysts of varying size communicatingwith each other and variably lined with either apseudo-stratified ciliated columnar (bronchial-type) epithelium or a single-layered cuboidalepithelium; (2) polypoid configuration of themucosa and increased amounts of elastic tissue inthe walls of the cystic portions lined withbronchial-type epithelium; (3) absence of cartilageplates in the cystic parenchyma except asconstituents of non-deformed bronchial structurestrapped within the diseased lung; (4) occasionalgroups of alveoli lined with mucogenic cells; and(5) absence of inflammation.
Bain (1959) described an additional featurewhich consisted in the absence of alveolarformation except in the immediate sub-pleuralzone.
CASE HISTORY
A term male infant weighing 3-26 kg. (7 lb. 3 oz.) atbirth was delivered by caesarean section because ofcephalo-pelvic dystocia after a trial labour lasting 10hours. The mother was a 32-year-old primipara whohad experienced a completely normal pregnancy.The baby breathed spontaneously at birth. The
initial examination of the newborn child was normal.A dry cough was noted during the first two days inthe nursery; pulmonary auscultation remained nor-mal. He was considered a normal child until theeighth day when the mother was discharged fromhospital. A chest film was requested as a precau-tionary measure because of the dry cough notedduring the first days of life.
Initial A.P. and lateral films (Fig. IA, IB) of the8-day-old baby showed an increase in volume of the
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Age Site ofLesions
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Right lungAll lobes
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right lung with herniation of part of the right upperlobe into the left chest. This was associated withdisplacement of the heart and mediastinal structuresto the left. The posterior aspect of the rightdiaphragm was somewhat depressed.A radiolucent oval area, 3 x 4 cm. in diameter, was
noted in the anterior part of the lower third of theright lung. Below and posterior to this large cysticformation a smaller cyst-like area was presentmeasuring approximately 1 cm. in diameter.Non-homogenous shadows of increased density
were present throughout most of the right lung. Theysurrounded the previously described cysts and werethemselves separated by many smaller areas of gasdensity which were interpreted as smaller cysts.The left lung was normal in appearance. The diag-
nosis of C.C.A.M. of the right lung was suggested.Because the right diaphragm was not fully visualized,oblique views of the chest and a gastro-intestinalseries were recommended.The child was transferred to the paediatric service
from the nursery at 9 days of age. A throat cultureand a blood culture done on transfer were reportednegative. The haemogram was as follows: Haemato-crit 46 vol.°0, haemoglobin 13-8 g./100 ml., W.B.C.8,300 per c.mm., polymorphonuclear neutrophils 20.polymorphonuclear basophils l, polymorphonucleareosinophils 1, monocytes 11, lymphocytes 67. Urin-alysis was normal.
Additional chest films taken the following day,including oblique views (Fig. 2a and b) as well assubsequent chest films and a gastro-intestinal series(taken three days later), established the integrity ofthe right hemidiaphragm and permitted in addition amore detailed appreciation of the largest cyst. Thewalls of the largest cyst were not constituted by a thincurved line of uniform appearance but by a line, oreven several lines, which seemed to taper out only tobecome thicker again, thus varying in thickness fromI to 5 mm. and ev_n more. In certain areas the cystwall blended in with the adjoining dense tissue. Invarious planes the principal cystic cavity was notradiolucent but was partially traversed by non-homogenous shadows of increased density. Thesedense zones were thought to represent pathologicaltissue forming a common wall between the principalcyst and the loculated satellite cysts communicatingwith the principal cyst, as well as pathological tissuesituated immediately adjacent to the principal cyst.
It was impossible to determine within which lobesthe principal cysts were located.
Posterior to this large cyst, an apparently unaffectedzone of lung parenchyma could be detected. Thisarea seemed to correspond to the lower lobe or, atleast, to the posterior segment of the lower lobe.Nevertheless, it was impossible to be certain of thedegree of involvement of this lobe. It seemed com-pressed, and dense shadows situated in an almostvertical direction were visible, probably representingatelectatic zones. No cystic areas were discernible inthe areas of presumed atelectasis.
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Congenital cystic adenomatoid malformation of the lung
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FIG. Ia. A.P. view showing multiple densities riddled withcysts of varying sizes and herniation of part of the rightlung; mediastinal shift to the left.
FIG. lb. Lateral view showing depression of the posterioraspect of the diaphragm and atelectasis in the right lowerlobe.
FIG. lb
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Congenital cystic adenomatoid malformation of the lung
Intravenous pyelography was done and failed toreveal any anomaly of the urinary tract.On the tenth day a consultation in thoracic surgery
was requested. The child was of normal colourationwith no evidence of cyanosis, even when crying.Pulmonary auscultation revealed diminished breathsounds on the right, particularly at the base antero-laterally. The case was discussed at paediatric roundswhen it was suggested that, before deciding on asurgical intervention, the films should be shown toDr. Neuhauser of Boston, who substantiated thediagnosis of C.C.A.M. of the lung.A pre-operative chest film taken at 23 days of age
failed to show any appreciable change.
OPERATIVE FINDINGS Under endotracheal anaesthesia,with the infant in the left lateral decubitus position, aright postero-lateral thoracotomy in the fifth inter-costal space was performed. There was no evidenceof intra-pleural adhesions. The right upper and middlelobes were of considerable size; many bluish cystswere visible through the thin visceral pleura. Thelargest of the cysts was situated in the middle lobe.The pulmonary parenchyma in the area of the largercysts presented a certain resistance to manual com-pression. The apex of the supero-dorsal segment ofthe right lower lobe showed an area which on initialinspection was thought to be a cyst, but closer exami-nation revealed an area of atelectasis. The remainderof the right lower lobe was of normal appearanceand consistency.A right upper and right middle lobe lobectomy was
carried out without any difficulty, the dissection beingfacilitated by the absence of adhesions and peribron-chial nodes. After observing a re-expansion of theright lower lobe, which, while completely inflatedwas still inadequate to fill the enlarged right hemi-thorax, the chest was closed with under-water drain-age. A tracheotomy was considered but deemedunnecessary.
PATHOLOGY The specimen of the right upper andmiddle lobes was reported on as follows: The twolobes weighed 60 g. On the external surface irregularbluish areas, somewhat elevated from the surroundingtissues, were noted; these showed no particular local-ization or demarcation (Fig. 3a). Surrounding theseareas the pulmonary lobules were well demarcatedand normal in appearance, the inter-lobular septabeing thickened and oedematous. Near the apex areddish-blue nodular mass was easily discernible,measuring 1-5 cm. in diameter. The cut surface (Fig.3b) revealed cystic cavities within the pulmonaryparenchyma varying in size from a few millimetres toseveral centimetres. The largest of these cysts wasmultilocular and measured 3 cm. in diameter, beingsituated in the middle lobe. Within the parenchy-matous tissue of the upper lobe on cut section a smallamount of yellow mucopurulent material could bedemonstrated within the smaller bronchi. Within thelargest cyst itself a small collection of similar muco-purulent material could readily be seen.
FIG. 3a. Right upper and right middle lobes. Operatiiefindings showing cystic areas.
FIG. 3b. Right upper and right middle lobes. Cut sectionshowing multi-cystic appearance and purulent droplets.
Microscopically, the pulmonary parenchyma con-
tained a large number of cysts of all sizes. These were
lined by simple cuboidal or cylindrical epitheliumcontaining isolated mucus cells (Fig. 4a and b).
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FIG. 4a. Cystic adenomatoid malformation of the lung. FIG. 4b. Another area of the same lunig showing theOne area at the top shows cysts lined with a cuboidal variety in the volume of the cysts and also the differentepithelium. At th( bottom the mucosa is polypoid a-id lined types of epithelium. Note the absence of cartilage andwith a bronchial type of epithelium. In the middle the bronchial adnexa. x 85.alveoli are lined with mucogenic epithelium. x 53.
FIG. 4c. This area shows apolypoidmass within a cyst, lined with a tall mucogenicepithelium. On the left the b,onchiolar epithelium is clearly visible. x 170.
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Congenital cystic adenomatoid malformation of the lung
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FIG. 5. Chest film nine months after surgery showing moderate mediastinalshift to the right with slight hypertransparency of the right lung (remaining rightlower lobe).
Numerous papillary projections were noted arisingfrom the lining epithelium. These were lined with atall mucogenic epithelium (Fig. 4c). No cartilage orbronchial glands were found. The cysts were sur-rounded by a fine network of reticular fibres, conjunc-tive tissue, and smooth muscle. Within certain cavitiesa small amount of fluid was noted. An area of inflam-matory reaction consisting of lymphocytes andplasmocytes was also found. The lobular septa werethickened and oedematous. A diagnosis of C.C.A.M.of the lung was made.
POST-OPERATIVE COURSE The post-operative coursewas uneventful. The remaining right lower lobe ex-panded to fill the right hemithorax. The child was dis-charged on the eighteenth post-operative day inexcellent condition with radiological evidence of aslight mediastinal shift to the right. In the periodsince (nine months at the time of writing) the childhas continued to do very well and has progressednormally (Fig. 5).
RADIOLOGICAL DISCUSSION
The radiological aspects of C.C.A.M. of the lungin the living child have been studied and describedby Craig et al. (1956). Since that time a few photo-
graphs and descriptions of the radiological featuresof this disease have been published (Nanson,1962; Kwittken and Reiner, 1962; Caffey, 1961).Craig et al. (1956) included in their article a
discussion of the differential diagnosis anddescribed the radiological findings in four casesof C.C.A.M. These consisted of an 'intra-pulmonary mass of soft tissue density, containingscattered radiolucent areas, which were irregularin size and shape'. They also observed that 'theoutlines of the mass were usually quite sharp andthe malformation was limited to one lobe'. Theynoted that 'the heart and mediastinum weredisplaced to the opposite side by the distendedhypertrophic air-containing mass and the affectedlung frequently showed herniation across themidline'.
The radiological appearance of the affectedlung in the case reported in the present article iscomparable in some respects to case IV describedby Craig et al. (1956).
DIFFERENTIAL DIAGNOSIS OF THE RADIOLOGICALFEATURES We shall review the diagnoses mostlikely to be considered from the radiological
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Raymond Belanger, Leo R. La Fleche, and Jean-Louis Picard
features (bearing in mind the present case) andwe shall attempt to rule out certain other diagnoseswhich, while more remote, may still at times bearcertain similarities to C.C.A.M. of the lung.The radiological appearances of the following
conditions will be compared with those ofC.C.A.M.: pneumonitis or atelectasis surroundingcongenital cysts or bronchogenic cysts; congenitalcysts with small areas of adenomatoid tissue;pneumatocoele secondary to staphylococcal infec-tion, and acquired obstructive emphysematousblebs; pyopneumothorax; diaphragmatic hernia;pulmonary sequestration, angiomatous malforma-tion, and hamartoma.
(a) Pneumonitis or atelectasis surroundingcongenital cysts Since inflammatory zones mayoccur in the areas surrounding congenital cysts,one might interpret the shadows of increaseddensity as being zones of pneumonitis oratelectatic plaques surrounding congenital cysts.Zones of pneumonitis, or of atelectasis, do not
cause an increase in volume of the affected lungunless the cysts within the lung, by means of acheck-valve mechanism, are responsible. In suchcases the cysts would have to be of greater sizeto account for such an increase in volume of theright lung, and the repeat chest films would beexpected to show some change in the size of thecysts. It has been shown that 'characteristic of theC.C.A.M. is the massive size of the affected lobewhich displaced other thoracic structures' (Bain,1959). Further, 'in true cystic disease the affectedlobe may occasionally be enlarged, but here it isa result of hyperdistension due to a check-valvemechanism' (Bain, 1959).
In our case the increase in volume of the affectedlobes was due to dense pathological pulmonarytissue, disorganized and riddled with cysts.
If we consider a case, as described by Herrmann,Jewett, and Galletti (1959), of 'typical expandingpulmonary cyst with atelectasis and mediastinalshift', we note that the radiological findings canbe readily distinguished from C.C.A.M. Whilecertain radiological features, such as mediastinaldisplacement, depression of the hemidiaphragm,the presence of cysts, and the presence ofatelectatic pulmonary tissue taken together at thebase giving the impression of a mass, can simulateC.C.A.M., certain characteristics, such as the well-demarcated appearance, the evident radiolucency,the thinness of the cyst walls, and even the sizeof the cyst (s), distinguish the two conditions.The principal cystic cavity, while not necessarily
round or oval, can measure several centimetres inits greater diameter in certain cases of C.C.A.M.
Kwittken and Reiner (1962) reported a case wherethe principal cystic cavity measured 9 cm.
(b) Congenital cyst with small zones ofadenomatoid tissue In the article published byCraig et al. (1956) there were reported cases oftrue congenital cysts with small zones of tissueof adenomatoid type, and the authors stated thatthese cases could be distinguished from C.C.A.M.(as well as by the histopathological findings) bythe 'absence of a roentgenologically demonstrablemass in the area, the lack of increased pulmonarymass (volume), and usually by the presence oflarger and often single cysts'. They (Craig et al.,1956) also believed that 'transitional cases existbetween the two groups', i.e., true congenital cystand C.C.A.M.
(c) Pneumatocoele secondary to staphylococcalinfection and acquired obstructive emphysematousblebs It is sometimes difficult to differentiatebetween true congenital cysts and cases ofstaphylococcal infection with pneumatocoeleformation. Potts and Riker (1950) stated 'thatoccasional lesions reported in the literature ascongenital cysts have all the characteristics ofpost-infectious pneumatocoeles'.
This difficulty in differentiation can exist even invery young infants. For example, a case has beenreported (Caffey, 1953) of 'lobar pneumonia whichdeveloped on the 4th day of life, followed bytriple air-cyst formation identified on the 15th dayof life with gradual spontaneous regression of thecysts'.
Since congenital cysts are rare (Caffey, 1961)when compared to acquired cystic disease, onemust exercise extreme caution before calling acystic lesion congenital. Because of the charac-teristic radiological appearance, the diagnosis ofC.C.A.M. can probably be entertained withoutdifficulty, but one must not confuse the radio-logical picture of this condition with thatencountered in pneumatocoeles secondary tostaphylococcal infection.
Caffey (1961) reported that 'it is difficult in theinitial films to differentiate C.C.A.M. of the lungfrom acquired obstructive emphysematous blebswhich disappear spontaneously'. He gave anexample of a 5-month-old infant with C.C.A.M.and stated that 'the edges of the emphysematoussegments (C.C.A.M.) are not sharply outlined byannular walls, as in the case of acquiredemphysematous cysts'.
Ordinarily, notwithstanding the fact thatpneumatocoeles can occur at a very young age,the clinical history, the appearance of the cysticcavities which often possess thinner walls, and
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repzat chest films showing a tendency towardsconfluent cavities, and occasionally even the rapiddisappearance of such confluent cavities, will helpto establish the diagnosis. Further, empyema maybe associated with the complex of pneumatocoelessecondary to staphylococcal infections (Schultze,1959). Usually, the increased size of the affectedlung argues in favour of C.C.A.M.
'Pulmonary pneumatocoeles may contain liquidbut usually are completely air-filled' (Flaherty,Keegan, and Sturtevant, 1960). Kwittken andReiner (1962) stated that the radiological findingsin one case of C.C.A.M. showed 'multiple cysticareas with fluid levels throughout the left lungfield'.
(d) Loculated pyopneumothorax Since caseshave been reported of loculated pyopneumothoraxduring the first week of life (Caffey, 1953) andsince the radiological appearance at certain stagesmay resemble that of congenital cystic diseaseof the lung associated with an infectious process,one should not misinterpret these findings withthose of C.C.A.M.
(e) Diaphragmatic hernia Whitesell and White(1952) state that in the newborn 'multilocular air-containing cysts of the lung must not be confusedwith congenital diaphragmatic hernia' and theybelieve that occasionally 'the loculations in a cystmay be mistaken for loops of bowel. . .'. Again,it has been noted that 'pulmonary infections occurin the presence of diaphragmatic hernia and thepneumonic infiltration may obscure loops ofbowel, or it may be misinterpreted as localizedemphysema' (Reed and Lang, 1959). We believethat the investigation should be completed by agastro-intestinal series of radiographs if there isdouibt about the intactness of the diaphragm. Thediagnosis of diaphragmatic hernia with intra-thoracic loops of bowel should be ruled out; itshould be especially considered when loculatedcysts of moderate size are situated near the levelof the diaphragm and, even more, when thediaphragm has not been well visualized on thechest films. Recently, Reed and Lang (1959)studied a group of cases of diaphragmatic herniain the infant, and there is general agre,ement thatthe radiological findings are usually characteristicin almost all cases of pleuro-peritoneal hernia.They may be listed as follows: (1) 'the diaphragmon the affected side is seen only in part or notat all; (2) there are multiple radiolucenciesrepresenting loops of intestine within the thorax;some may contain fluid levels; (3) there is a shiftof the heart and upper mediastinum to the oppositeside of the chest; there may be associated
compression atelectasis ; (4) there is a relative orabsolute absence of the intestinal gas in theabdomen' (Reed and Lang, 1959).
(f) Pulmondry sequestration, angiomatousmalformation, and hamartoma Since 'the lesionin different cases varies from mainly solid,enlarged tumor-like lobes to multicystic lesions'(Bain, 1959), the differential diagnoses which mayon occasion be amongst the first considered arethose of well-circumscribed lesions such aspulmonary sequestration, angiomatous malforma-tion, and hamartoma. The latter may gain a con-siderable size (Graham and Singleton, 1955).
'Pulmonary sequestration ordinarily appears asa homogeneous opacity of the basal posteriorsegment of the lower lobe provided no communi-cation with the bronchial tree has been establishedsecondarily, possibly as a consequence of infection.One or more fluid levels indicating cystic degenera-tion would be then apparent' (Jensen and Wolff,1956).
In the present case the dominant cystic featureof the pulmonary mass, the involvement of thegreater part of the right lung, and the poorly-demarcated contours of the mass helped todifferentiate C.C.A.M. from any of the threeabove-named conditions.
CONCLUSION Based on the radiological appear-ance, C.C.A.M. of the lung should be suspectedwhenever there exists a pulmonary mass contain-ing cystic cavities, especially if there is a markedincrease in the volume of the affected lung.
SURGICAL CONSIDERATIONS Diaphragmatic herniaand diaphragmatic eventration are diagnosed onthe basis of auscultatory and radiological (gastro-intestinal series and barium enema) findings.Pneumatocoeles secondary to staphylococcalpneumonias are of frequent occurrence and areoften complicated by a pyopneumothorax. Theonly surgical treatment indicated is intercostaldrainage of the pyopneumothorax. These infantspresent a febrile, toxic picture unlike the clinicaltableau of C.C.A.M., which belies the apparentsimilarity in the radiological pattern. Pulmonarytension cysts, tension pneumothorax, and lobarobstructive emphysema generally are distinctradiologically from C.C.A.M., as well as causinga more marked symptomatology with progressivedyspnoea, cyanosis, diminished breath sounds, anda hyper-resonant enlarged hemithorax. Theseconditions nearly always require surgical inter-vention. Hamartomas of the lung, because of theoccasional radiological similarity and the absenceof symptoms, must also be considered in the
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differential diagnosis. However, they are usuallysmaller, situated peripherally, and, in any case,require surgical exploration and extirpation. Intra-lobar pulmonary sequestration may develop acommunication with a bronchus, becomesecondarily infected, and resemble a pulmonaryabscess. Here again the clinical picture differs fromthe usually mild symptomatology associated withC.C.A.M. Enterogenous cysts, if communicatingwith a bronchus or the oesophagus, may containair, but usually they are small and confined to themediastinum, as are diverticula of the digestivetract.Most of these air-containing lesions, particularly
when symptomatic, require surgical intervention,the most notable exceptions being the pneuma-tocoele and cystic disease of the lung secondaryto mucoviscoidosis in older children.Bronchoscopy and bronchography have not
been frequently us.d in the diagnosis of C.C.A.M.because of the difficulties and inherent dangers inthese procedures in the newborn. Craig et al.(1956) have however reported a case. Since surgicalexploration is indicated, such examinations areonly of academic interest.The importance of an early and precise diagnosis
is seen from the fact that surgical resection isindicated without delay. This is not to say thatresection must be carried out immediately thediagnosis has been established: it is a matter ofrelative urgency. The operation should not bedelayed four to six months or more in order toallow the infant to grow stronger with the idea ofrendering him in the optimum condition possiblefor undergoing an operation of such magnitude.Despite an experience still quite limited, it appearsthat the majority of surviving children underwentsurgical resection shortly after C.C.A.M. of thelung was diagnosed.There are two main reasons for not delaying
the operation. As already stated, the affected lobesare characterized by having a weight and volumewell above normal. In the majority of cases themediastinum is displaced to the contralateral side.It seems inevitable that, with time, the affectedlobes would occupy an increasing proportion ofthe volume of the thoracic cage at the expense ofthe normal lobes that would progressively undergocompression atelectasis. In addition the derangedphysiology results not only from the disturbanceof pulmonary function but also from the interfer-ence with the normal haemodynamics of the intra-thoracic organs, viz., in venous return, passivecongestion, diminished filling of the heart, anddiminished cardiac output.
Another reason strongly suggested by Craigand his colleagues (1956), one which appears to bewell-founded, especially when the cystic forma-tions are large, and was in fact borne out in thepresent case, is the danger of secondary infection.Because of the direct communication with thebronchial passages (even if such a communicationis not demonstrable by bronchography) thesecystic spaces constitute sites predisposed to infec-tion, which, once established, would be extremelydifficult, if not impossible, to control and whichmost assuredly would end in the death of theinfant. In this regard, antibiotic treatment withdrugs of large spectrum is strongly recommendedin the pre-operative period to diminish the chancesof infection.
DISCUSSION
Congenital cystic adenomatoid malformation ofthe lung is an apparently rare condition, althoughin recent years more reports have been publishedin the medical literature. According to ourresearch of the literature, 34 cases have beenreported and of these a good number werenecropsy diagnoses. Recently, these cases have,when properly diagnosed, undergone surgicalextirpation with excellent results. The casereported by us is, to the best of our knowledge,the seventh surviving case submitted for publica-tion.There is no question that, in the face of the
paucity of clinical symptoms and findings, thediagnosis becomes radiological, as in the case herepresented. Consideration of the history, clinicaltableau, and radiological findings leaves only a fewconditions to be seriously entertained. Of these,congenital cystic disease of the lung, diaphragmatichernia, pneumatocoele, and hamartoma come firstto mind.
SUMMARY
A case of congenital cystic adenomatoid mal-formation of the lung in a newborn male infantis reported. A review of the literature and a listingof known reported cases are submitted. Radio-logical and surgical considerations are discussedin addition to the pathological features.
REFERENCES
Altmann, F. (1929). Beitrage zur Lehre von den Lungenmissbildungen.Beitr. Path. Anat., 82, 199.
Bain, G. 0. (1959). Congenital adenomatoid malformation of thelung. Dis. Chest, 36, 430.
Caffey, J. (1953). On the natural regression of pulmonary cysts duringearly infancy. Pediatrics, 11, 48.
, (1961). Pediatric X-Ray Diagnosis, 4th ed., p. 282. Year BookPubl., Chicago.
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rotected by copyright.http://thorax.bm
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Thorax: first published as 10.1136/thx.19.1.1 on 1 January 1964. D
Congenital cystic adenomatoid malformation of the lung
Ch'In, K. Y., and Tang, M. Y. (1949). Congenital adenomatoidmalformation of one lobe of a lung with general anasarca. Arch.Path., 48, 221.
Couvelaire, A. (1904). Dig-nirescence kystique cong:nitale dupoumon. HistogIn6se des deganerescences kystiques congenitalesdes organes glandulaires (rein, fore, pancrdas, poumon). Rev.mens. Afaladies de l'Enfance, 22, 60.
Craig, J. M., Kirkpatrick, J., and Neuhauser, E. B. D. (1956). Con-genital cystic adenomatoid malformation of the lung in infants.Amer. J. Roentgenol., 76, 516.
Fischer, C. C., Tropea, F., Jr., and Bailey, C. P. (1943). Congenitalpulmonary cysts: report of infant treated by lobectomy withrecovery. J. Pediat., 23, 219.
Flaherty, R. A., Keegan, J. M., and Sturtevant, H. N. (1960). Post-pneumonic pulmonary pneumatoceles. Radiology, 74, 50.
Goodyear, J. E., and Shillitoe, A. J. (1959). Adenomatoid hamartomaof the lung in a newborn infant. J. clin. Path., 12, 172.
Gottschalk, W., and Abramson, D. (1957). Placental edema andfetal hydrops. A case of congenital cystic and adenomatoidmalformation of the lung. Obstet. and Gynec., 10, 626.
Graham, G. G., and Singleton, J. W. (1955). Diffuse hamartoma ofthe upper lobe in an infant. Amer. J. Dis. Child., 89, 609.
I{errmann, J. W., Jewett, T. C., Jr., and Galletti, G. (1959). Broncho-genic cysts in infants and children. J. thorac. Surg., 37, 242.
Huckel, R. (1927). Beitrage zur ar.geborenem Wabenlunge:Frankfurt Ztschr. Path., 35, 320.
Hunermann, C., and Sievers, H. (1930). Zur klinischen und rdnt-genologischen Diagnose angeborener cystischer Lungenmissbil-dungen im Sauglingsalter. Z. Kinderheilk., 50, 451.
Jensen, V., and Wolff, A. (1956). Congenital intralobar pulmonarysequestration with anomalous artery from the aorta. Acta radiol.(Stockh.), 45, 357.
Kwittken, J., and Reiner, L. (1962). Congenital cystic adenomatoidmalformation of the lung. Pediatrics, 30, 759.
Nanson, E. M. (1962). Pulmonary resection in infancy and childhood.Canad. med. Ass. J., 87, 275.
Pappenheimer, A. M. (1912). Congenital cystic malformation of thelung. Proc. N.Y. path. Soc., n.s., 12, 193.
Potter, E. (1952). Pathology of the Fetus and the Newborn. YearBook Publishers, Chicago.
Potts, W. J., and Riker, W. L. (1950). Differentiation of congenitalcysts of the lungs and those following staphylococcic pneumonia.Arch. Surg., 61, 684.
Reed, J. O., and Lang, E. F. (1959). Diaphragmatic hernia in infancy.Amer. J. Roentgenol., 82, 437.
Schultze, G. (1959). Unusual roentgen manifestations of primarystaphylococcal pneumonia in infants and young children. Ibid.,81, 290.
Thomas, M. R. (1949). A cystic hamartoma of the lung in a new-borninfant. J, Path. Bact., 61, 599.
Whitesell, F. B., Jr., and White, W. J. (1952). Congenital cystic diseaseof the lung in the newborn. Ann. Surg., 136, 299.
Wolman, I. J. (1930). A case of congenital cystic lung in a stillbornfetus. Bull. Ayer clin. Lab., 2, No. 12, p. 49.
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on March 11, 2021 by guest. P
rotected by copyright.http://thorax.bm
j.com/
Thorax: first published as 10.1136/thx.19.1.1 on 1 January 1964. D
