Spontaneous pneumothorax in subjectsSpontaneous pneumothorax in young subjects Table continued Side No. of Case AgeiSex Affetdece EpisodesPrevious Clinical Presentation Histological

Thorax (1971), 26, 409.

Spontaneous pneumothorax in young subjectsA clinical and pathological study

I. LICHTER and J. F. GWYNNE

Departments of Thoracic Surgery and Pathology,University of Otago Medical School, Dunedin, New Zealand

Spontaneous pneumothorax may complicate lung disease which is clinically or radiologicallyapparent in patients suffering from chronic bronchitis, asthma, tuberculosis, bronchiectasis, and,less commonly, bronchial cancer, silicosis, pulmonary infarction, and other more rare disorders.These patients are usually in the older age group, and the commonest predisposing factor ischronic bronchitis. Pneumothorax occurring in these diseases is well recognized and needs nofurther elaboration.Spontaneous pneumothorax may also occur in apparently healthy people with no demonstrable

pulmonary lesion. The subjects are often young, usually male, and have been in good healthprior to their first episode. They are often athletic and tend to be of tall, thin physique. A groupof 20 cases which falls into this latter category forms the basis of this study. They were all treatedby wedge resection of apical lung disease. The clinical and histological findings are presented andthe literature is briefly reviewed.

Approximately 120 patients were admitted to theThoracic Surgical Unit of the Wakari Hospitalwith spontaneous pneumothorax between the years1962 and 1968. Most of these patients had clinicaland radiological evidence of established bilateralpulmonary disease such as bronchitis and emphy-sema, bronchiectasis or tuberculosis and have notbeen included in this study.

Fifty of the patients stimulated particularinterest because they gave no antecedent historyof chest disease and they form the basis of thisstudy. More than half of this group respondedto therapy by intercostal tube drainage. In sixthe air leak persisted despite drainage; 14 patientswere readmitted because of recurrent pneumo-thorax. These 20 patients, two of whom sufferedbilateral pneumothoraces, were subjected tothoracotomy and wedge resection. The clinicaland pathological data from this group have beenreviewed.

CLINICAL DATA

Relevant clinical findings are summarized in theTable.The indication for resection was recurrence in

14 patients and persistent leak in six. The pneumo-thorax was bilateral in two patients. Each lungwas affected with approximately equal frequency.

Predilection for the male sex is noted, only threeof the patients being females. Sixteen were betweenthe age of 16 and 30 (range 16 to 47). Two ofthe older patients were females.Measurements of body stature were not

recorded but most of the patients tended to betall and thin. A history of athletic activity wasfrequent.

OPERATIVE FINDINGS

At operation the characteristic findings were asfollows:At the apex of the lung there was a small area

of fibrosis, usually no larger than 3 x 2 cm, sur-mounted by a thin-walled bullous cyst or cysts.Commonly there were several cysts, and thesemeasured from about 0-2 cm in diameter to 1 cm ormore (Fig. 1). Only occasionally was a small pinholeleak apparent at the apex of the cyst. In the caseof leaks that failed to seal within 48 hours of drainage,a tear was sometimes found in a large cyst that hadbeen responsible for considerable air leak and failureto heal. The remainder of the lung appeared normal.Adhesions were uncommon.

PATHOLOGY

The histological material from the specimens in theseries has been reviewed and a similar pattern ofabnormalities was observed throughout.

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1. Lichter and J. F. Gwynne

TABLESUMMARY OF CLINICAL FEATURES

Case Age/Sex

1 18 M

LeI

2 25 M Left

3 27 M Left

4 ~21 M

5

6

Left

24 M Right

19 M

7 19M

8

9

10

11

24 F

16 M

20 M

31 M

Left

Right

Left

Right

Right

revious Clinical Presentation Histological Findings Follo"-uppisodes

I One previous episode of spontaneous Extensive fibrosis and

pneumothorax. Recurred 10 mth later. inflammation with linear

Wedge resection for recurrence lymphocytic infiltration

Prominent emphysema with large cysts,some with surrounding chronic in-flammation. Numerous intra-alveolarmacrophages. Mesothelial and alveolarcell proliferation. Dilated bronchi filledwith mucus. Vessels showed endarter-itis

3 Three previous episodes of spontaneous Widespread fibrosis Yrpneumothorax. Wedge resection for chronic inflammation. re.ur-encefurther recurrence with fibrous-walled subpleuralcysts.

Abundant pigmented macrophages.Distended bronchi containing mucus.Marked endarteritis obliterans

Previous pneumothorax 2k yr earlier Extensive linear fibrosisyrtreated by intercostal tube drainage. incorporating distorted recurrenceWedge resection for recurrence Emphysema not seen. pig-

mented macrophages. Superficial cystsPrevious episode of spontaneous pneu- Patchy fibrosis.

mthmothorax 5 wk earlier. Further episode, polymorphs surroundedrecurrencetreated by intercostal tube drainage inflammation. Focal emphysema. Pig-with continuing air leak for 2 days. mented macrophages

Wedge resection for persistent air leak relation to cholesterol.

prominent cellular lining. Cysts linedby simple

- First episode of pneumothorax. Treated Widespread vrby intercostal tube drainage. Air leak flammation. Patchy emphysema No re..urrencecontinued for 6 days, when wedge fibrous walled cysts. Pigmented macro-

resection was done for persistent air phages. Collapsed distorted

leak scars. Vessels show endarteritis

2 Left side: Two previous episodes of left Severe focal fibrosis

spontaneous pneumothorax and one on chronic inflammation. Emphysema recurrenceright. Wedge resection on left for fur- many fibrous-walled cysts. Mesothelial.ther recurrence bronchiolar and alveolar cell prolifera-ation.

Right side: One year later presentedafter 6 episodes of right-sided spon- Abundant pigmented yrtaneous pneumothorax, 4 of which had Many bronchioles which

recurrencewithin the past 2 mth. Wedge macrophages and pigment.

resection for recurrence show endarteritis

7 Seven previous episodes of spontaneous Focal fibrosis markedyrpneumothorax over previous 4 yr. chronic inflammation. Emphysema

recurrenceWedge resection for recurrence fibrous-walled cysts. pig-

mented macrophages with focal choles-terol deposition. Alveolar cell pro-liferation. Dilated bronchioles contain-ing macrophages

Tension pneumothorax treated by inter- Large subpleural scar 7s rcostal tube drainage. Air leak continued emphysema. Solitary fibrous-walled recurrencefor 6 days. Wedge resection for per- cyst with pigmented macrophages

sistent air leak wall

I

2 Two episodes of spontaneous pneumo- Fibrosis involving almost speci- vrthorax over previous 7 mth. Wedge men. Emphysema notrecurrenceresection for further episode chronic inflammation

Cysts

only on surface

2 Two previous episodes of spontaneous Multifocal scarring 3 yrpneumothorax over preceding 2 mth inflammation and lipoid granulomata recurrencetreated by intercostal tube drainage. On (history of camphorsecond occasion camphor oil was pleural cavity). Severe emphy-instilled into pleural cavity. Third sema. Fibrous-walled cysts.

episode treated by wedge resection alveolar cell proliferation. Collapsed

andbronchioles containing

secretion.endarteritis of small

One previous episode of spontaneous Subpleural and intrapulmonary fibro- 4 yrpneumothorax 5 mth earlier. On sis. Alveolar cell hyperplasia No recurrencesecond occasion treated by intercostal areas. Single cyst lined by tissue

tube drainage. Continued air leak over with haemosiderincholesterol

2-wk period. Wedge resection for per- deposition suggesting haemorrhage

sistent air leak

(cont.)

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Spontaneous pneumothorax in young subjects

Table continued

Side No. ofCase AgeiSex Affetd Previous Clinical Presentation Histological Findings Follow-upece Episodes

Two episodes of spontaneous pneumo-thorax over previous 2 mth. Thirdepisode treated by wedge resection

First episode of spontaneous pneumo-thorax treated by intercostal tube drain-age. Continued air leak for 3 days.Wedge resection for persistent air leak

Second spontaneous pneumothoraxwithin 5 wk. Treated by wedge resection

Spontaneous pneumothorax 5 yr earlier.Second episode treated by wedge re-section

Second episode of spontaneous pneu-mothorax within 2 mth. Treated bywedge resection

Two episodes of spontaneous pneumo-thorax over previous 31 yr. Thirdspontaneous pneumothorax treated bywedge resection

Three episodes ofspontaneous pneumo-thorax over previous year. Furtherrecurrence treated by wedge resection

Three episodes ofspontaneous pneumo-thorax over previous year. Furtherrecurrence treated by wedge resection

Left side: Asthma since age 13. Fiveepisodes of spontaneous pneumothoraxon left side and one on right overprevious 2 yr. Further recurrence onleft side treated by wedge resection

Right side: Admitted 18 mth later after4 episodes of spontaneous pneumo-thorax on right side. Wedge resectionfor recurrence

Widespread focal fibrosis with patchychronic inflammation. Emphysemawith fibrous-walled cysts, some lined bycellular layer. Alveolar walls lined byprominent cells. Dilated bronchi con-taining macrophages

Extensive fibrosis with active chronicinflammation and lymphoid hyper-plasia. Emphysema with cysts lined byprominent cellular layer. Abundantpigmented macrophages. Alveolar cellhyperplasia in scars. Small thick-walledbronchi. Vessels show marked endar-teritis

Extensive focal fibrosis with a solitarycavity surrounded by macrophages.Widespread emphysema. Numerouspigmented macrophages. Alveolar cellproliferation. Severe endarteritis

Intrapulmonary fibrosis with intensechronic inflammation. Emphysemawith large fibrous-walled cysts. Abun-dant pigmented macrophages. Meso-thelial, bronchiolar, and alveolar cellproliferation. Bronchioles containmucus and pigmented macrophages.Endarteritis ofsmall vessels

Large areas of fibrosis with adjacentemphysema. Pigmented macrophagesin scars. Chronic bronchiolitis withmucus plugs. Endarteritis of smallvessels. Cysts lined by simple fibroustissue

Patchy fibrosis and active chronic in-flammation with focal emphysema.Abundant pigmented macrophages.Cysts lined by flat cellular layer

Patchy fibrosis and emphysema, pig-mented macrophages, small fibrosedbronchi. Cysts lined by prominentcellular layer

Patchy fibrosis and emphysema. Alveo-lar and bronchiolar cell proliferation,pigmented macrophages, distendedbronchi with chronic inflammation.Cysts lined by fibrous tissue

Dense vascular subpleural scar withpatchy chronic inflammation. Emphy-sema and subpleural fibrous-walledcysts. Distended bronchi with cartilagein walls. Vessels show endarteritis inone biopsy only

5 yrNo recurrence

7 yrNo recurrence

4 yrNo recurrence

3 yrNo recurrence3 mth laterpneumothoraxon contra-lateral side

5 yrNo recurrence

6 yrNo recurrence

5 yrNo recurrence

5 yrNo recurrence

7 yrNo recurrence

5 yrNo recurrence

In each case the portion of lung was disorganizedby fibrosis, collapse, and cyst formation, all variablein degree. There was no evidence of adhesions tothe chest wall.

MICROSCOPIC FINDINGS Histologically the changeswere non-specific. Although common features werepresent, these varied in prominence from case to case,suggesting that the process was in different stagesof progression at the time of presentation.

The changes consisted of emphysema and cystformation, atelectasis, fibrosis, chronic inflammation,pigment deposition, bronchiolar, alveolar cell, andmesothelial proliferation, bronchial lesions, andvascular changes.

Emphysema and cyst formation Emphysema wasconstant and focally distributed, being compensatoryin some areas and destructive in others (Fig. 2).Cysts were usually lined by fibrous tissue (Fig. 3)

20 M

47 M

21 M

37 F

13

14

15

Right

Right

Left

Right

Left

Left

Left

Left

LeftRight

3

2

3

4-55-6

16 33 F

17 26 M

18 19 M

19 26 M

20 18 M

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FIG. 1. Appearance at operation showing cysts andscarred lung tissue.

but a prominent mesothelial layer was present insome instances (Fig. 4). Cysts were usually subpleuralbut occasionally were seen in scars.

Atelectasis This was a constant feature, being distri-buted either in linear fashion or in ill-defined circular

- J

areas. Atelectatic foci were often invaded by fibroustissue and were in many instances related to adjacentcompensatory emphysema.

Fibrosis This process varied in nature and distribu-tion. Dense subpleural scars were common (Fig. 5)but in addition the lung tissue itself was fibrosed withreplacement of architecture (Fig. 6). In other areas,fibrous tissue caused thickening of alveolar walls,especially in foci of atelectasis.

Chronic inflammation Active infiltration of densescar tissue was seen in some cases but this was notthe general rule. When present, the cells consistedmainly of lymphocytes and plasma cells, polymorphsbeing inconspicuous (Fig. 6). Intra-alveolar macro-phages were seen frequently. In case 10 there wasflorid inflammation with foamy macrophages. Thispatient had been treated elsewhere by camphor oilinstillation.

Pigment deposition This was inconstant and, whenpresent, was found in macrophages, both in scar tissueand also lying free in air spaces. Both haemosiderinand carbon were identified (Fig. 7).

,4.

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FIG. 2. Photomicrograph showing compensatory emphysema. H. and E. x 44.

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FIG. 3. Photomicrograph of subpleural cyst lined by fibrous tissue. H. and E. x 48.

t* ^' sZ f +~.0114.7' 9

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FIG.4.Photomicrograph °Scyst lined by prominent mesothelium. H. anzl E. x 226.

FIG. 4. Photomicrograph of cyst lined by prominent mesothelium. H. and E. x 226.



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FIG. 5. Photomicrograph of vascular subpleural scar with a focus of residual infl27mmation. H. and E. x 50.

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FIG. 7. Photomicrograph ofpigmented macrophages in alveolar space. Pigment is also deposited in adjacentfibrous tissue. H. and E. x 256.

FIG. 8. Photomicrograph ofsmall bronchi distended with secretion. There isperibronchial inflammation. H. and E.x 115.



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Alveolar cell proliferation This occurred in dis-organized scarred areas (Fig. 6). The cellular reactionwas considered to be related to nearby scarring andinflammation.

Bronchial lesions When bronchi were found in thebiopsies, they were on occasion filled with eosinophilicsecretion as though obstructed by scar tissue (Fig. 8).Some showed peribronchial fibrosis and mild inflam-mation of their walls.

Vascular lesions No specific inflammatory or occlu-sive lesions were seen but endarteritis obliterans wascommon.

DISCUSSION

AETIOLOGY The special site of the damage in eachcase suggested the possibility of a tuberculousaetiology, but there was no histological evidencefor this in any of the cases.

Localized congenital cystic lung was entertainedas a possible explanation but the constant relationof cysts to scar tissue made this difficult to assess.

Post-inhalational damage was excluded by thesite and by the absence of any evidence of inhaledmaterial apart from carbon.

Localized trauma to the lung suggested itselfin view of the youth of the patients and the historyof athletic activity, but there was no real evidenceof organizing haematomata. The finding of haemo-siderin pigment was inconstant and insufficient toexplain the findings on a basis of haemorrhage.On histological grounds alone the changes are

best explained on a basis of post-inflammatorydisorganization, the inflammation being mostlikely due to non-specific infection. The curiouslocalization of the lesions and their occurrencepredominantly in young males are obscure featureswhich suggest some local inherent predisposition.Many of the previous reports relating to primary

spontaneous pneumothorax in young subjects havenot included detailed accounts of the histologicalchanges (DuBose, Price, and Guilfoil, 1953;Bernhard, Malcolm, Berry, and Wylie, 1962;Carpathios and Bogedain, 1963; Leading article,1965; Lynn, 1965; Mills and Baisch, 1965; Levy,1966; Shields and Oilschlager, 1966).Most authors have favoured a congenital origin

for the cysts or have postulated inflammation andfibrosis with cyst formation. An earlier hypothesisby Brock (1948), that air may leak through animperfect visceral pleura, has not been reiteratedin recent studies, all of which record the presenceof bullae.The hyposthenic habitus of the patients is of

interest and it has been suggested (Withers, Fish-

back, Kiehl, and Hannon, 1964) that in tall thinindividuals there is a rapid growth rate relativeto pulmonary vasculature accounting for relativeischaemia and bleb formation during growth.Killen and Gobbel (1968) describe bleb formationwith associated fibrosis and chronic inflammation.They point out that the aetiology is unknown butconsider that a congenital fault may be presentor that the lesion may be secondary to local stressinjury or degenerative change. Similarly, it wassuggested in the British Medical Journal (Leadingarticle, 1968) that the lesion might result from acongenital fault or an inflammatory scar. Hyde(1963) drew attention to the long narrow chestand related this to the development of blebs asa congenital anomaly. The same author (1962)had noted the fact that the patients were 20-30 lb(9-13 kg) underweight. Aust (1961) presumed thatthe blebs were congenital in nature. Thomas (1959)divided his cases into those with congenital cystsand those with acquired blebs or bullae. Heindicated that loss of elasticity, fibrosis, andadhesions resulted from pulmonary vascularinsufficiency, causing bleb formation.None of the above authors has incriminated

tuberculosis in the aetiology of primary pneumo-thorax, and this disease was not in evidence inany of our cases.

INCIDENCE The true incidence of apical lungcysts in the population is not known, recognitiondepending on the complication of rupture. Radio-logical study prior to the development of pneumo-thorax is usually negative (Baronofsky et al.,1957; Bernhard et al., 1962; Reid, Stevenson andMcSwan, 1963; Leading article, 1968). Our ownexperience confirms the frequent absence of radio-logical changes.

According to a leading article in the BritishMedical Journal (1968) the incidence of the com-plication of pneumothorax rose from 025 perthousand in the 1950s to 04 per thousand in the1960s. This is probably due to better recognitionof the condition. Several large series have beenreported from military sources and these all stressthe importance of the condition in young appar-ently fit men (Thomas, 1959; Withers et al.,1964; Mills and Baisch. 1965).The frequency of involvement of both sides at

different times in the same patient is worthy ofnote, and suggests that bullous disease may bepresent in an intact state on the contralateral sidein a significant proportion of patients with appar-ently unilateral disease. Pneumothorax occurredbilaterally in two of our patients (10%) and this

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Spontaneous pneumnothorax in young subjects

may rise to a higher figure as our patients arefollowed up for longer periods. Baronofsky et al.(1957) were so impressed with the bilateralnature of the bullous disease that they performedthoracotomy on both sides in each of their 26patients. Bullae were found on both sides in allbut one of their patients. Details of the patho-logical findings in their series were not given.

THERAPY Wedge resection of diseased lung tissueas used in this series of patients reflects thelocalized apical nature of the disease process whichcaused the pneumothorax. Failure to understandthe focal affection of lung tissue has in the pastled to irrational therapy such as pleural cavityobliteration by the instillation of irritating fluids,a procedure liable to fail. When leakage persistsfor longer than 48 hours, or when the pneumo-thorax recurs after simple intercostal tubedrainage, thoracotomy seems to be the only logicalapproach to permanent cure. So far, in our series.the results have been good with no recurrences.Baronofsky et al. (1957) recommended resectionwhile Withers et al. (1964) found that the tech-nique compared favourably with other methods.Reid et al. (1963) reported similarly when theycompared the results of intercostal tube drainage,kaolin pleurodesing agent, and segmental resection.

CONCLUSIONS

1. A series of 20 patients with recurrent or per-sistent spontaneous pneumothorax is presentedfrom the clinical and pathological standpoints.All the patients were treated by wedge resectionof diseased lung tissue.2. The pathological features have been reviewedand an attempt has been made to explain theaetiology.3. The histological lesions are non-specific butsuggest post-inflammatory fibrosis and cyst forma-tion. An underlying congenital abnormality relatedto the anatomy of the thorax in tall thin athleticindividuals appears to be an attractive hypothesis.Defects in blood supply and aeration in thesecircumstances may be important factors.4. Treatment by wedge resection seems to be arational approach to the underlying pathology.

We wish to thank the staff of the PhotographicDepartment, University of Otago Medical School, forthe photomicrographs, and Mr. D. Tingle for technicalassistance. Mrs. D. Schmelz typed the manuscript.

REFERENCES

Aust, J. B. (1961). Spontaneous pneumothorax. Postgrad.Med., 29, 368.

Baronofsky, 1. D., Warden, H. G., Kaufman, J. L., Whatley,J., and Hanner, J. M. (1957). Bilateral therapy forunilateral spontaneous pneumothorax. J. thorac. Surg.,34, 310.

Bernhard, W. F., Malcolm, J. A., Berry, R. W., and Wylie,R. H. (1962). A study of the pathogenesis and manage-ment of spontaneous pneumothorax. Dis. Chest, 42, 403.

Brock, R. C. (1948). Recurrent and chronic spontaneouspneumothorax. Thorax, 3, 88.

Carpathios, J., and Bogedain, W. (1963). Spontaneouspneumothorax: experience with 50 cases. Amer. Surgn,29, 525.

DuBose, H. M., Price, H. J., and Guilfoil, P. H. (1953).Spontaneous pneumothorax: medical and surgicalmanagement. Analysis of 75 patients. New Engl. J. Med.,248, 752.

Hyde, L. (1962). Benign spontaneous pneumothorax. Ann.intern. Med., 56, 746.(1963). Spontaneous pneumothorax. Dis. Chest, 43, 476.

Killen, D. A., and Gobbel, W. G. (1968). SpontaneousPneumothorax, pp. 43-58. Little, Brown, Boston.

Leading article (1965). Spontaneous pneumothorax. Brit.med. J., 1, 1262.

Leading article (1968). Spontaneous pneumothorax. Brit.med. J., 1, 720.

Levy, I. J. (1966). Spontaneous pneumothorax. Treatmentbased on analysis of 170 episodes in 135 patients. Dis.Chest, 49, 529.

Lynn, R. B. (1965). Spontaneous pneumothorax. Dis. Chest,48, 251.

Mills, M., and Baisch, B. F. (1965). Spontaneous pneumo-thorax. A series of 400 cases. Ann. thorac. Surg., 1, 286.

Reid, J. M., Stevenson, J. G., and McSwan, N. (1963). Themanagement of spontaneous pneumothorax. Scot. med.J., 8, 171.

Shields, T. W., and Oilschlager, G. A. (1966). Spontaneouspneumothorax in patients 40 years of age and older.Ann. thorac. Surg., 2, 377.

Thomas, P. A. (1959). Spontaneous pneumothorax-MoJernconcepts in etiology and treatment of an importantsyndrome in military practice. Milit. Med., 124, 116.

Withers, J. N., Fishback, M. E., Kiehl, P. V., and Hannon,J. L. (1964). Spontaneous pneumothorax. Suggestedetiology and comparison of treatment methods. Amer. J.Surg., 108, 772.

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Spontaneous pneumothorax in subjectsSpontaneous pneumothorax in young subjects Table continued Side No. of Case AgeiSex Affetdece EpisodesPrevious Clinical Presentation Histological