Thorax (1960), 15, 325.

PLASTIC BRONCHITISBY

R. SLEIGH JOHNSON AND E. G. SITA-LUMSDENFrom Southend-on-Sea Hospital, Essex

(RECEIVED FOR PUBLICATION MAY 18, 1960)

The specific feature of plastic bronchitis is theformation of obstructive bronchial plugs or castsof thick, tenacious mucus during an attack ofbronchitis, when one or more lobes or even anentire lung may collapse. Casts are often expec-torated, but they may be discovered only atbronchoscopy, or be found lying in the bronchialtree at necropsy. They consist largely of mucus,and may also contain fibrin, pus cells, and blood,as well as elements commonly found in asthmaticattacks such as eosinophil cells, Charcot-Leydencrystals, and Curshmann's spirals.

CASE REPORTSCASE 1.-A married woman, previously in good

health, had her first respiratory illness, left lowerlobe pneumonia, in 1937 at the age of 27, followedby another attack three years later. In 1941, herhouse was demolished by a land-mine, when she washeavily exposed to dust and sustained severe nervousshock. Shortly afterwards she developed attacks ofcough, dyspnoea, and wheezing, relieved by adrenalinand antispasmodic inhalations. The attacks occurredevery few weeks during the winter, often followinga head cold; a small amount of yellow sputum wasproduced. Other precipitating factors were emotionalupset, excitement, or exertion.

In April, 1951, she had an attack of sinusitis withwheezing, following the death of her mother. Thefirst known incident of major pulmonary collapseoccurred after using an adrenalin spray. She noticeda "peculiar noise " in the retrosternal region withincreasing dyspnoea, cough with yellow sputum,malaise, and severe retrosternal pain spreading to theleft chest and interscapular area. She was admittedto hospital with signs of complete collapse of the leftlung, temperature of 1000 F., leucocytosis of 11,200per c.mm., and erythrocyte sedimentation rate of33 mm. in one hour (Westergren). There were nospecific bacteriological findings in the sputum.Postural drainage and breathing exercises provedineffective, so she was bronchoscoped, and the leftmain bronchus was found to be blocked by viscidsecretion, just distal to the carina. The secretion wassuccessfully aspirated, revealing inflammatory changesin the mucosa of the left bronchus. Restoration ofthe airway was followed by rapid re-expansion at theleft base and recovery.

She remained free from serious attacks until May,1954, when after moving house a coryzal attackdeveloped into acute bronchitis with asthma. She hadsevere, stabbing pain in the centre of the back andlower chest mainly on the left, induced by movement,coughing, or breathing. Dyspnoea was so severe thateven speaking caused distress, and she became very illwith fever, sweating, cough, and creamy sputum.Admitted as an emergency, she had an immobile, silentleft lung, and the heart and mediastinum weredisplaced to the left (Fig. 1).As she did not improve, bronchoscopy was carried

out: the left stem bronchus was completely obstructedby a plug of tough, inspissated mucus which appearedto be lightly adherent to the bronchial wall but provedtoo viscid to be dislodged by aspiration alone. Itrequired to be broken up and removed in small pieceswith forceps; when one large piece approximately3 x 1 cm. was withdrawn, the air entry was imme-diately restored to the lower lobe bronchi with rapid

FIG. 1.-Radiograph of Case 1, showing total collapse of the leftlung on her second admission.

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relief of dyspnoea and general improvement. Laterin the day a plug of dense, viscid mucus was expelledafter a bout of coughing, resulting in furtherimprovement in air entry. Treatment was continuedwith steam inhalation, antibiotics and antispasmodics,postural coughing, and breathing exercises. A smalleffusion developed at the left base, but six days afterbronchoscopy the radiographic appearances werealmost normal.The pathological findings pointed to a combination

of infection and allergy. A blood count on the davof admission showed a slight leucocytosis of 11,000per c.mm. (840o polymorphonuclears and 100eosinophils). Culture of sputum and bronchialsuckings gave a heavy growth of pneumococci, but noacid-fast bacilli or fungi were found; clumps ofhyperplastic bronchial epithelial cells were present, butno neoplastic cells were seen. The plug was ofirregular shape and consisted of mucoid. eosinophilicacellular material in which were included numerouspyknotic cells and foci of leucocytes. Among thelatter were needle-shaped and hexagonal Charcot-Leyden crystals, and outside the main mass were smallacellular clumps of highly refractile, non-stainingmaterial resembling portions of Curshmann's spirals.The biopsy consisted of corrugated bronchial mucosacovered by regular epithelium, in places somewhatflattened, beneath which was a heavv infiltration bylymphocytes, plasma cells, and eosinophils, presentalso in the muscularis mucosae and mucous glands;there was no evidence of malignancy. Bronchogramsmade after discharge were normal.The patient had a third episode of pulmonarv

collapse, once again in the spring, in March, 1957.Cough with a little sputum developed, attributed toinhalation of smoke from a train. Five days latershe had severe pain in the centre of the back to theleft of the midline, of constant intensity and radiatingdiffusely across the chest on breathing, accompaniedby dyspnoea, malaise, and anxiety. On readmissionshe had faucial injection, tachycardia, and respiratorydistress with signs again of left-sided pulmonarycollapse, confirmed by radiographic examination. Shehad a leucocytosis of 21,000 per c.mm., 80% beingpolymorphonuclear cells with no excess of eosinophils,and the sedimentation rate was 21 mm. in one hour(W;ntrobe). Sputum gave a growth of commensalsonly.Bronchoscopy was performed shortlv after

admiss on, and the left stem bronchus found to beblocked by a greenish-grey plug of viscid glassymaterial to I in. below the carina, with much thin,bubblv mucus in each bronchial tree but no plug inthe right bronchus. The mass was partly removed infragments with difficulty by forceps and sucker, afterwh:ch the almost complete remainder of the plug,about 61 cm. in length, was removed by forceps(Fig. 2). Part of a similar plug in the left upper lobebronchus was also sucked out and air entry re-estab-lished to all main bronchi. Relief was rapid, andnext day air entry throughout the left lung was muchimproved, though some mediastinal displacement

remained. There was still partial collapse of theanterior segment of the left upper lobe with a pleuralreaction at the left base and fluffy shadowing hadappeared in the lower two-thirds of the right lung.The temperature was raised to 100° F. but rapidl\became normal, though after postural percussion andbreathing exercises she coughed up copious mucoidsputum, which was slightly blood-stained at first.Penicillin and corticotrophin were given for four days,and five days after bronchoscopy the radiograph wasnormal and she was discharged (Fig. 3). Histologicalexamination of the bronchial plug showed a mucoidmatrix containing a large number of inflammatorycells, including many eosinophils. The material wasacetic-acid-coagulable, and on hydrolysis, followed bychromatography, glucose. galactose, and ribose wereidentified, confirming that the main constituent wasmucin.A year later, after a cold, she developed wheezing

with fever and bronchitis, more marked on the leftside, with thick stringy mucus. She recovered rapidlyafter prompt treatment with antibiotics and pred-nisolone, which probably prevented a pulmonarycollapse as in the three previous similar attacks. Afilm taken a week after recovery showed increasedpulmonary basal markings only, and bronchographydemonstrated slight dilatation of a single bronchus inthe left posterior basal segment, not shown in thebronchograms of 1954.

Since March, 1958, the patient has not had an\further major illness, but she has had occasionalcough and mucoid sputum with mild wheezing at

FIG. 2.-Fragments of the plig removed at bronchoscopy onthe third admission of Case 1.

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FIG. 3.-Radiograph of Case I on her third admission, showingcomplete re-expansion of the left lung after bronchoscopy.

times, readily controlled by ephedrine. The lungshave continued to show normal air entry on each sidewith a few scattered wheezes but no other signs.During one such attack in December, 1958, treatedby tetracycline, she coughed up mucous plugs withoutchest pain. Skin tests in August, 1959, gave a stronglypositive reaction to aspergillus antigen and mixedfungi (Bencard). There was no sputum at that timeand her serum did not show any precipitating anti-bodies to Aspergillus fumigatus.

This patient had three major attacks ofpulmonary collapse in seven years, and on eachoccasion was bronchoscoped with rapid relief.CASE 2.-A retired nurse, aged 61, was admitted to

hospital in March, 1948. Apart from heavy colds herhealth had been good until five months previously,when she had an influenzal illness with cough, highfever, and mucoid sputum. Similar febrile attackshad recurred, culminating in a sudden severe pain inthe left chest and dyspnoea. There were signs of leftbasal pulmonary collapse with jugular engorgementand tracheal and mediastinal displacement. There wasno bronchospasm and she was not febrile. Radio-graphs showed collapse of the left lower lobe andpatchy collapse of the left upper lobe, the right lungfield being clear. Leucocyte count was 5,100 perc.mm.; Friedlander's bacillus was grown from themucopurulent sputum. Improvement was rapid oninhalations and alkaline expectorants, and radio-graphic examination six days later showed normalappearances.

In July, 1948, she was readmitted with similarsymptoms, 12 days after breaking her right wrist.

Cough and dyspnoea developed on the night of theaccident, followed by severe stabbing pain and tight-ness in the chest. She had a low fever and acontinuous cough, producing a cupful daily of thick,tenacious, white sputum. Venous engorgement wasseen in the neck, and there was diminished air entry,prolonged expiration, and a loud expiratory wheezeover the whole of the right chest as well as diffusebronchitic signs. Cough and viscid sputum persisteduntil two weeks after admission, when increasinglysevere dyspnoea and cyanosis developed with intract-able cough and difficulty in expectoration. Chestradiographs then showed collapse of the pectoralsegment of the right upper lobe. Her sputum wasblood-stained and contained thick pieces of mucopusgiving a predominant growth of B. friedlanderi; notubercle bacilli were found, and the leucocyte countwas normal at 9,000 per c.mm. Five days later, aftera severe bout of coughing in which no sputum couldbe raised spontaneously, she was greatly relieved whenhard percussion resulted in the expectoration of aplug of tough mucus. Penicillin and streptomycinwere given, together with an antihistamine andephedrine, and the right lung re-expanded completelyduring the next four weeks.

In April, 1949, a further episode of pulmonarycollapse led to her third hospital admission. She wasable to bring up only a l.ttle sticky sputum with greateffort and without relief of the tightness in the chest,and had a collapsed left upper lobe. Shortly afteradm'ssion she coughed up a large plug of mucus withimmediate relief of symptoms and rapid re-expansionof the left upper lobe. The plug was a cylindrical bodyof tough yellow mucus, 4 cm. long by 1.5 cm.diameter, containing small white flecks which provedto be colonies of haemolytic streptococci. Micro-scopy showed scanty pus cells, most of the cells beingepithelial; no eosinophils or elastic fibres were seenbut many crystals were present. A month later shehad a sharD pain under the left scapula, and onceagain the left upper lobe and lingula had partiallycollapsed. The sedimentat on rate had risen fromnormal to 72 mm. in one hour (Westergren). Spon-taneous improvement occurred and she was dischargedhome in a few days.The fifth incident occurred in January, 1950, when

she was readmitted for a cough following a headcold with increasingly tenacious sputum and dyspnoea.She had severe bronchitis with transient signs ofcollapse at the left lung base. Pneumococci, staphylo-cocci, and coliform bacilli were obtained from thesputum and she had a mild leucocytosis of 11,200 perc.mm. The chest infection responded quickly tostreptomycin, breathing exercises, and posturalpercussion.She was admitted for the sixth time two months

later with increasing cough, dyspnoea, and sharp painin the left lower chest. The left lower lobe wascollapsed and the neck veins were engorged ; scatteredhigh-pitched wheezes were heard over the right lung.No specific organisms were found in the sputum andthere was no leucocytosis. Bronchoscopy showed

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the presence of thick secretions in the left lower lobebronchus, which were removed by aspiration; biopsyfrom the right upper lobe showed no evidence ofneoplasm. Vigorous physiotherapy was given as wellas methylephedrine and penicillin, and she wasdischarged two weeks later with nearly completeexpansion of the left lower lobe.

This patient illustrates the clinical features ofrelapsing plastic bronchitis with recurrent suddenatelectases occurring five times in the left lung andonce in the right, with apparently completerecovery between attacks. She was bronchoscopedonce only, for diagnosis and therapeutic aspiration.

CASE 3.-A married woman, aged 65, was admittedto hospital in October, 1942, with a provisionaldiagnosis of carcinoma of the left lung. She had hada winter cough for many years, but recently had hadtwo attacks of sudden aching in the left shoulder andpraecordium, with dry cough and breathlessness, eachlasting a week. She had no sputum but was cyanosed,with congested neck veins, a temperature of 100° F.,and signs of extensive collapse of the left lung, con-firmed by a chest radiograph. At bronchoscopy amass of jelly-like mucus was found in the left mainbronchus; after this had been removed with difficultyby suction the bronchi appeared normal. Within twodays she was apyrexial, free of pain, and the left lunghad completely re-expanded.

In September, 1943, she had a similar attack andwas again found to have complete collapse of the leftupper and lower lobes. Bronchoscopy showed that theleft main bronchus was again obstructed by a mass ofthick mucus, which was removed by suction andforceps; the mucosa appeared chronically inflamedbut showed no evidence of neoplasm, and she recoverdrapidly.

Similar events recurred three months later, necessi-tating her third admission. This time she had a slightfever and a leucocytosis of 15,000 c.mm., with collapseof the left lower lobe. A plug of thick black mucuswas coughed up with great difficulty, and she thenrecovered uneventfully with a subsequent normalbronchoscopy and bronchogram.She remained well for more than a year, but in

February, 1945, had to be bronchoscoped for arecurrence of cough and pain in the left chest, andthick purulent secretion was aspirated from thetrachea and left main bronchus.

In March, 1946, the left lung totally collapsed again.Spontaneous improvement occurred and broncho-scopy on the fourth day after admission showed onlya little inspissated mucus in the trachea, which wasremoved by aspiration; the left bronchial treeappeared normal and the mucosa showed no signifi-cant atrophy or metaplasia or other histologicalchange on biopsy. Bronchograms demonstratedminimal bronchiectasis in the left lower lobe.She then remained well until 18 months later, when

pain in the left shoulder was followed by cough, fever,

and expectoration of purulent sputum containingpneumococci, and a chest radiograph showed massivecollapse of the left lung. She was postured and herchest thumped vigorously, when she coughed up alarge mucous cast of the shape of the left mainbronchus and its main subdivision. Immediateimprovement of air entry on the left side was notedand was confirmed radiographically. Bronchogramslater showed normal filling of the left lower lobebronchi, demonstrating that their previous dilatationhad been a reversible process resulting fromtemporary block.A winter cold led to her seventh admission, in

December, 1948. There was no response of the left-sided collapse to postural percussion, but three dayslater partial re-expansion followed spontaneousejection of a lump of thick greyish mucus. Broncho-scopy then showed the presence of much tenaciousmucus in the left main bronchus, and this was suckedaway. Re-expansion remained incomplete andbronchoscopy had to be repeated a month later, whenfurther extremely thick mucus was aspirated fromthe trachea and left lower lobe. Re-expansionoccurred rapidly, but relief was brief and within amonth the left lung collapsed completely, necessitatingher eighth admission. Despite a week's intensivephysiotherapy and expectorant treatment the lung didnot re-expand and she had to be bronchoscoped again.The left main bronchus was found to be completelyfilled with thick, tenacious mucus requiring patientremoval with sucker and forceps, an operation taking45 minutes. Relief this time was immediate.

She was admitted for the ninth time in August, 1950,with partial collapse of the left upper lobe, whichresponded well to physiotherapy, and she completelyrecovered in a fortnight. She then remained well forsix years apart from transient collapse of the leftupper lobe treated successfully in the Out-patientDepartment. In December, 1956, she again developedcollapse of the greater part of the left lung. Afterstrenuous physiotherapy she coughed up a large plugof mucopurulent sputum and the lung re-expanded.

In June, 1957, she was found to have carcinoma ofthe cervix, which was treated with radium. She diedin December of that year at the age of 80, andnecropsy showed bronchopneumonia at both lungbases, with mucopurulent material in the bronchi, andcarcinomatosis of liver, lungs, brain, and bones.

This patient had 14 proven episodes of pulmonarycollapse at irregular intervals, all involving the leftlung, and was bronchoscoped eight times. Sheprobably had many other more transient and lesssevere attacks.CASE 4.-A married woman of 66, with a history

of winter bronchitis for the previous six years,developed pneumonia in February, 1958. There wasan area of collapse in the lower part of the left upperlobe seen on a chest radiograph taken because coughand sputum persisted with pain around the left lowerchest. A film in April still showed collapse of mostof the left upper lobe, including the lingula. Tomo-

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grams showed the left lower lobe bronchus to be clearbut the left upper lobe bronchus appeared completelyobstructed. Her sputum was negative for tuberclebacilli and other pathogens. She had further treat-ment with penicillin for 10 days, and after two weeksthe chest film was clear. The leucocyte count wasthen 13,600 per c.mm., with 23% eosinophils.

In October, 1958, she had a further episode ofrespiratory illness, treated with penicillin and strepto-mycin, but a subsequent chest film showed nopulmonary collapse, and when reviewed in February,1959, she was well and had only a little whitishmucoid sputum. The sedimentation rate was elevated,however, and a radiograph showed a small linearatelectasis in the left mid-zone. In May, 1959, sheattended again, complaining of increased cough withyellow sputum for six weeks and pain and tightnessin the left chest and epigastrium. She had beeninvestigated for cholecystitis with negative result.Radiographs showed collapse of the left upper lobeand lingula, the appearances being similar to thoseof April, 1958. She was coughing up hard plugs ofmucus with difficulty and was very dyspnoeic.Bronchoscopy showed the left upper lobe bronchusto be blocked by thick, tenacious mucus; this wasaspirated and the orifices of the segmental bronchiwere seen to be clear but narrowed by oedema.Biopsy of the bronchial mucosa showed a chronicinflammatory infiltrate in the submucosa. Pneumo-cocci and haemolytic streptococci were found in thesputum, but no fungi were isolated. The white cellcount was 6,000 per c.mm., of which 10% wereeosinophils. Rapid re-expansion of the left upperlobe followed bronchoscopy.A month later she had a further attack of cough

and pain in the left hypochondrium, and the radio-graph showed partial collapse of the lingula. Spon-taneous improvement occurred and after three weeksonly a small linear atelectasis remained.She was readmitted in October, 1959, following a

heavy cold, with mucopurulent sputum, pain, anddyspnoea. There were no pellets or casts in thesputum. The right upper lobe was now completelycollapsed, and bronchoscopy showed that the orificeof the right upper lobe bronchus was occluded by verythick, tenacious, brownish-grey mucus. This wasaspirated and the patency of the orifice restored, butthe bronchus was seen to be narrowed by oedema.No tubercle bacilli were found in the aspirate butAspergillus fumigatus grew on culture. Only 2% ofthe cells were eosinophils. Skin tests were stronglypositive to mixed moulds (Bencard) and to aspergillusantigen (kindly supplied by Dr. R. W. Riddell), butno precipitating antibodies against A. fumigatus couldbe detected by Dr. Riddell in her serum. The rightupper lobe did not re-expand for several days afterbronchoscopy, but expansion was almost complete afterphysiotherapy and prednisolone had been given for10 days. She has since had several minor attacks ofbronchitis with wheezing but no further pulmonarycollapse.

DISCUSSIONPlastic bronchitis is one of the rarer bronchial

diseases, and there have been only about 300 casesin the world's literature, most of them in thenineteenth century. It is also described as fibrinous,pseudo-membranous or Hoffman's bronchitis, oras bronchial croup. It was first mentioned in thesecond century A.D. by Galen, who thought theexpectorated masses were vessels of the lung(Bettmann, 1902). It was recognized by Morgagni,who believed the casts to be composed of inspis-sated mucus. In 1716 Tulp described " an entirevein cast up from the lungs" and his illustrationshows a bronchial cast branching to the sixthdegree. Warren in 1772 described " a largepolypous concretion " of the bronchus, showing thatit followed exactly the shape of the bronchial treeand that it was solid and of laminated construction(Major, 1939). It affects all ages, the youngestpatient recorded being 3 weeks old and the oldest75 years. In some there is a history of precedingrespiratory disorder such as bronchiectasis, tuber-culosis, asthma or chronic bronchitis, or of chronicheart disease (Bettmann, 1902; Woolley, 1953;Leggat, 1954; Le Melletier and Caulet, 1958); inothers, however, there has been no previous oraccompanying respiratory condition, as in the fatalcase reported by Johnstone (1945). Apart frompulmonary disease an association has been claimedin some instances with rheumatoid arthritis(Woolley, 1953), amyloidosis (Ranta, 1956),membranous colitis (Lamaison, 1922), enlargedthymus and also mediastinal compression bypericardial effusion (Saracoglu, 1960), althoughin no case on convincing evidence.The formation of the characteristic mucous

plugs can usually be traced to a combination offactors. Moderate inflammatory change in thebronchial wall is the most constant of these, whichmay include congestion and oedema of the mucosa,sometimes with haemorrhage, and infiltration bypolymorphonuclear leucocytes, eosinophils, orlymphocytes, extending into the deeper layers.Clinical signs of infection include fever, coughwith yellowish sputum, and leucocytosis. In thefour patients described above the white cell countwas only moderately raised, and varied from 5,100to 15,400 cells per c.mm., reaching 21,000 on asingle occasion. Mucosal biopsies showed sub-acute or chronic inflammatory changes. In thefirst patient the epithelium was flattened in places,beneath which was a moderate to heavy infiltra-tion by lymphocytes, plasma cells and eosinophils.In the second patient biopsy was reported asshowing chronic inflammation only, and in the

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fourth patient there was a chronic inflammatoryinfiltrate in the submucosa. Biopsy in the thirdcase was reported merely as showing nomalignancyCondorelli, Cappellini, and Rocca Rossetti (1958)

described the histological findings in two collapsedlobes removed from patients with plastic bron-chitis. The chief change in the bronchial mucosawas the presence of areas of epithelial flatteningwith zones of metaplasia in which there was anexcess of goblet cells and of mucin. Inflammatorychanges in the bronchial wall were relatively slight.Accumulations of mucogelatinous material werefound in medium and small bronchi and in someof the alveoli; vascular changes included endo-thelial proliferation, subendothelial oedema, elasticdegeneration, hyaline degeneration of the media,muscular hypertrophy, and numerous enlargedarteriovenous anastomoses. In the authors' viewthe essential lesion was not a true inflammationbut a dysfunction of the bronchial mucosa, whichbecame hypersecretory. The solidified secretioncould be expectorated only with difficulty becauseof the absence of the ciliary ladder, which resultedfrom replacement of columnar ciliated epitheliumby mucus-secreting cells. They considered thepulmonary vascular changes to be secondary toobstruction of the bronchial circulation.The bacteria found in the bronchial tree or

expectorated in plastic bronchitis are inconstantand not of specific aetiological significance; in ourcases pneumococci, B. friedlanderi, haemolyticstreptococci, staphylococci, coliforms, neisseriae,and other organisms were isolated at various times.The possible role of Aspergillus is discussed below.

Occasionally the immediate cause of thebronchial irritation may be physical (as by inhala-tion of dust or smoke), rather than bacteriological,though these two factors may act together. Afurther precipitating factor may be an allergy, asin our fourth patient, who had a blood eosinophiliaon two occasions and was found to be stronglysensitive to aspergillus antigen. There appears tobe a close relationship to the type of bronchialasthma in which areas of segmental collapse areoften found radiographically; these lesions differfrom those of plastic bronchitis mainly in theirmore peripheral distribution. They rarely involvemore than a small segment (never a lobe or lung),and the intense eosinophilia of the blood whichis frequently associated with them is not character-istic of plastic bronchitis. The special features ofthe bronchial casts also lend support to an allergicbasis, since they often have Charcot-Leydencrystals and Curschmann's spirals with large

numbers of eosinophil cells among the inspissatedmucus. Seasonal factors may be important, as inour first patient, and include a repeated seasonalallergy or recurrent infection at the same time ofyear (Bettmann, 1902). Emotional disturbancemay similarly provoke attacks; previous broncho-pulmonary damage may facilitate collapse anddetermine its site.

Plastic bronchitis is more common in women;our four patients were women in middle age, and,of 18 cases reported recently by 10 authors, 14were women. Plastic bronchitis may occur as asingle attack without recurrence, or in attackswhich may recur over a period of weeks or monthsand then cease, or yet again their incidence maybe spread at long intervals over many years.During a bout of acute or subacute bronchitis thepatient complains of shortness of breath and afeeling of suffocation, with pain in the chest andsometimes a localized wheeze. The pain is sharp,characteristically intense, constant and unaffectedby respiration, and its severity in patients withextensive pulmonary collapse is of diagnosticsignificance. Cough is troublesome and persistentwith great difficulty in expectoration, so that aftermuch effort the patient may finally bring up plugsor casts followed by frothy or purulent mucus,which is frequently blood-stained. If the plugs arenot coughed up the patient may become very illand life may be endangered. The signs are thoseof bronchitis, often with localized wheezing, andthere may be areas of impaired percussion noteand diminished air entry which can be segmental,lobar, or involve a whole lung. A pathognomonicbruit de drapeau or Ventilgerausch has occasion-ally been reported, and this is said to be caused bythe flapping or vibration of loosened portions ofcast in the trachea or main bronchus (Bettmann,1902; Johnstone, 1945 ; Woolley, 1953). This signmay also be apparent to the patient, but it wasnot detected in any of our four. Radiographsduring an attack will show one or more areas ofpulmonary collapse, and lung abscesses mayappear later from secondary sepsis. There doesnot seem to be a predilection for any particularpart of the lung; in a series of cases reportedduring the last 30 years in which the side of thecollapse is definitely stated, it occurred equally onthe two sides. Our four patients, however, had atotal of 27 observed attacks of pulmonarycollapse, 25 of which occurred on the left; onepatient had 14 attacks all on the left side. Theradiograph is usually normal between acuteepisodes, but recurrent attacks may lead to fibrosisand bronchiectasis.

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Bronchial casts are cylindrical, look like boiledspaghetti or macaroni, and may branch to theseventh degree. They are frequently expectoratedcoiled up in the form of pellets and take theircharacteristic shape only when floated in water.The casts usually separate quite readily from thebronchial wall, but may be difficult to cough upbecause of their softness, stickiness, and severalbifurcations. They are normally pale greyish orgreenish-white, but may be brown from alteredblood. They are peculiarly tough and viscid; thesurface is smooth and may reproduce the striationsof the bronchial mucosa. They not infrequentlycontain crystals and Curschmann's spirals witheos.nophils, pus cells, necrotic bronchial epithelialcells, and bacteria, which may form visiblecolonies. Microscopically they have a laminatedfibrillary structure, and Leggat (1954) showed thatthe matrix contains an acid mucopolysaccharidesimilar to mucin, which was confirmed in our firstcase. Variable amounts of fibrin may also bepresent.

Collapse may be due to obstructive mucus: ifwe remember this, the possibility of plastic bron-chitis will not be overlooked, though it may bedifficult if there is no sputum or the sputum doesnot contain casts. Where the patient has producedmucous plugs in the past the diagnosis is no longerdifficult. The plugs differ from those expectoratedin bronchial infection by Aspergillus fumigatus, inwhich they are characteristically brownish-yellow,friable rather than tenacious, and can be shown tocontain fungal mycelia on silver impregnation(Hinson, Moon, and Plummer, 1952). Aspergillusinfection is excluded by negative skin and aggluti-nation tests, but positive tests may be obtained incases of sensitivity to casually inhaled fungalspores without actual mycelial infection of thebronchi. The immediate weal reaction to asper-gillus antigen is said to be positive in about 15%of all asthmatics, and was positive in the two casesof our series in which the test was done. In onlyone case, however, was Aspergillus fumigatusobtained from the sputum and this patient differedfrom the others in showing a considerable bloodeosinophilia. Although her serum did not showany agglutinins aspergillus may have played a partin producing bronchitis, asthma, and attacks ofatelectasis. The casts were not examined by thesilver impregnation technique in any of our fourcases, but we hope to do this in future.An acute onset with fever, dyspnoea, and

purulent sputum may simulate a pneumonia, whilethe suppression of breath sounds may suggestpleural effusion or pneumothorax, both of which

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would be excluded by full physical and radio-logical examination. A more difficult differentia-tion is from bronchial obstruction by inhaledforeign body or neoplasm, in which a carefulhistory should help. These conditions will nor-mally call for bronchoscopy, by which means anaccurate diagnosis will be reached. Clinical pointsof special help in the diagnosis of plastic bronchitisare the relatively acute onset, the severity of chestpain, marked mediastinal shift, and, where present,stridor.

In general the prognosis is good, recovery froman attack usually occurring spontaneously, thoughthe patient may be very ill and greatly distressedfor several days. The prognosis is worse at theextremes of life. Fatal cases have been reportedby Johnstone (1945), Woolley (1954), and LeMelletier and Caulet (1958). Necropsy ofWoolley's patient, a man of 61, showed a castoccluding the right bronchial tree to the fifthsubdivision and extending across the trachealbifurcation to include the left main bronchus. LeMelletier and Caulet's patient, a woman of 32with old-standing pulmonary tuberculosis and athoracopLasty, was found to have a cast occupyingthe whole of the bronchial tree.

In mild cases the warm moistened air of steaminhalations may give sufficient relief, or a trypsinaerosol may be tried, and these measures shouldbe combined with postural coughing and percus-sion. Where bronchospasm is present adrenalinand other antispasmodics may be of benefit, andcorticosteroids may be helpful especially wherethere is a history of asthma (Pilgerstorfer, 1956).Antibacterial drugs should be given to combatinfection in the collapsed lung. In all severe casesbronchoscopic aspiration should be carried out atthe earliest opportunity, for by this means only canan exact diagnosis and assessment be made andspeedy relief of bronchial obstruction obtained.Bronchoscopic aspiration, repeated if necessary inthose cases not responding at once to simplemeasures, has been used by Jackson and Jackson(1934), Frank (1937), Merica (1950), and LeMelletier and Caulet (1958). In bilateral cases itmay be a life-saving measure.

SUMMARYRecurrent plastic bronchitis producing acute

obstructive pulmonary collapse is described in fourpatients. Each patient required bronchoscopy onone or more occasions. The aetiology, treatment,and prognosis of the condition are discussed, andthe value of bronchoscopy as a diagnostic andtherapeutic measure is stressed.

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R. SLEIGH JOHNSON and E. G. SITA-LUMSDEN

We thank Dr. Kenneth Perry for the details of thefirst patient's original hospital admission, and Dr.Neville Oswald for the details of the second and thirdpatients, who were under his care.

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