Spontaneous Pneumothorax in Young Subjects a Clinical and Pathological Study

Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from

Thorax (1971), 26, 409.

Spontaneous pneumothorax in young subjects A 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 radiologically apparent 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 is chronic bronchitis. Pneumothorax occurring in these diseases is well recognized and needs no further 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 health prior to their first episode. They are often athletic and tend to be of tall, thin physique. A group of 20 cases which falls into this latter category forms the basis of this study. They were all treated by wedge resection of apical lung disease. The clinical and histological findings are presented and the literature is briefly reviewed.

Approximately 120 patients were admitted to the Predilection for the male sex is noted, only three http://thorax.bmj.com/ Thoracic Surgical Unit of the Wakari Hospital of the patients being females. Sixteen were between with spontaneous pneumothorax between the years the age of 16 and 30 (range 16 to 47). Two of 1962 and 1968. Most of these patients had clinical the older patients were females. and radiological evidence of established bilateral Measurements of body stature were not pulmonary disease such as bronchitis and emphy- recorded but most of the patients tended to be sema, bronchiectasis or tuberculosis and have not tall and thin. A history of athletic activity was been included in this study. frequent. Fifty of the patients stimulated particular interest because they gave no antecedent history OPERATIVE FINDINGS on September 25, 2021 by guest. Protected copyright. of chest disease and they form the basis of this At operation the characteristic findings were as study. More than half of this group responded follows: to therapy by intercostal tube drainage. In six At the apex of the lung there was a small area the air leak persisted despite drainage; 14 patients of fibrosis, usually no larger than 3 x 2 cm, sur- were readmitted because of recurrent pneumo- mounted by a thin-walled bullous cyst or cysts. thorax. These 20 patients, two of whom suffered Commonly there were several cysts, and these bilateral pneumothoraces, were subjected to measured from about 0-2 cm in diameter to 1 cm or thoracotomy and wedge resection. The clinical more (Fig. 1). Only occasionally was a small pinhole and pathological data from this group have been leak apparent at the apex of the cyst. In the case reviewed. of leaks that failed to seal within 48 hours of drainage, a tear was sometimes found in a large cyst that had been responsible for considerable air leak and failure CLINICAL DATA to heal. The remainder of the lung appeared normal. Relevant clinical findings are summarized in the Adhesions were uncommon. Table. The indication for resection was recurrence in PATHOLOGY 14 patients and persistent leak in six. The pneumo- The histological material from the specimens in the thorax was bilateral in two patients. Each lung series has been reviewed and a similar pattern of was affected with approximately equal frequency. abnormalities was observed throughout. 409 Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from

410 1. Lichter and J. F. Gwynne TABLE SUMMARY OF CLINICAL FEATURES

Case Age/Sex revious Clinical Presentation pisodes Histological Findings Follo"-up

1 18 M 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 inflammation. Numerous intra-alveolar LeI macrophages. Mesothelial and alveolar cell proliferation. Dilated bronchi filled with mucus. Vessels showed endarteritis 2 25 M Left 3 Three of spontaneous Widespread fibrosis previous episodes Yr pneumothorax. Wedge resection for chronic inflammation. re.ur-ence further recurrence with fibrous-walled subpleural Abundant pigmented macrophages.cysts. Distended bronchi containing mucus. Marked endarteritis obliterans 3 27 M Left Previous pneumothorax 2k yr earlier Extensive linear fibrosis treated yr by intercostal tube drainage. incorporating distorted recurrence Wedge resection for recurrence Emphysema not seen. pigmented macrophages. Superficial cysts

4 21 M ~ Left Previous episode of spontaneous Patchy fibrosis. pneu- mth mothorax 5 wk earlier. Further episode, polymorphs surrounded treated recurrence 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 lined

by simple

- 5 24 M Right First episode of pneumothorax. Treated Widespread vr

by intercostal tube drainage. Air leak flammation. Patchy emphysema continued for No re..urrence 6 days, when wedge fibrous walled cysts. Pigmented macro-

resection was done for persistent air phages. Collapsed distorted

leak scars. Vessels show endarteritis http://thorax.bmj.com/ 6 M 19 2 Left side: Two previous episodes of left Severe focal fibrosis

spontaneous Emphysema pneumothorax and one on chronic inflammation. recurrence

right. Wedge resection on fur- many fibrous-walled cysts. left for Mesothelial. ther recurrence bronchiolar and alveolar cell ation. prolifera- Right side: One year later presented after 6 episodes of right-sided spon- Abundant pigmented yr pneumothorax, taneous 4 of which had bronchioles which Many recurrence within the past 2 mth. Wedge macrophages and pigment. Left resection for recurrence show endarteritis

M 7 19 7 Seven previous episodes of spontaneous Focal fibrosis marked yr pneumothorax over previous 4 yr. chronic inflammation. Emphysema recurrence

Wedge resection for recurrence fibrous-walled cysts. on September 25, 2021 by guest. Protected copyright. mented macrophages with focal choles-pig- terol deposition. Alveolar cell proliferation. Dilated bronchioles contain- Right ing macrophages

8 24 F Tension pneumothorax treated by inter- Large subpleural scar r tube Air 7s costal drainage. leak continued emphysema. Solitary fibrous-walled recurrence I for 6 days. Wedge resection for per- cyst with pigmented macrophages Left sistent air leak wall

9 16 M 2 of Two episodes spontaneous pneumo- Fibrosis involving almost speci- vr thorax over 7 men. not previous mth. Wedge Emphysema recurrence resection for further episode chronic inflammation Cysts only on surface

10 20 M Right 2 Two previous of Multifocal episodes spontaneous scarring 3 yr pneumothorax over and preceding 2 mth inflammation lipoid granulomata recurrence treated by intercostal tube drainage. On (history of camphor second occasion camphor oil was pleural cavity). Severe emphy- instilled into pleural cavity. Third sema. Fibrous-walled cysts. episode treated resection alveolar cell by wedge proliferation. Collapsed

and bronchioles containing secretion. endarteritis of small

1 1 31 M Right One previous of and episode spontaneous Subpleural intrapulmonary fibro- 4 yr pneumothorax 5 mth On sis. Alveolar cell earlier. hyperplasia No recurrence second occasion treated intercostal areas. lined by Single cyst by tissue tube Continued air leak over with haemosiderin drainage. cholesterol

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

(cont.) Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from

Spontaneous pneumothorax in young subjects 411 Table continued

of Case AgeiSex Side PreviousNo. Clinical Presentation Histological Findings Affetdece Episodes Follow-up 20 M Right 3 Two episodes of spontaneous pneumo- Widespread focal fibrosis with patchy 5 yr thorax over previous 2 mth. Third chronic inflammation. Emphysema No recurrence episode treated by wedge resection with fibrous-walled cysts, some lined by cellular layer. Alveolar walls lined by prominent cells. Dilated bronchi containing macrophages

13 47 M Right First episode of spontaneous pneumo- Extensive fibrosis with active chronic 7 yr thorax treated by intercostal tube drain- inflammation and lymphoid hyper- No recurrence age. Continued air leak for 3 days. plasia. Emphysema with cysts lined by Wedge resection for persistent air leak prominent cellular layer. Abundant pigmented macrophages. Alveolar cell hyperplasia in scars. Small thick-walled bronchi. Vessels show marked endarteritis 14 21 M Left Second spontaneous pneumothorax Extensive focal fibrosis with a solitary 4 yr within 5 wk. Treated by wedge resection cavity surrounded by macrophages. No recurrence Widespread emphysema. Numerous pigmented macrophages. Alveolar cell proliferation. Severe endarteritis 15 37 F Right Spontaneous pneumothorax 5 yr earlier. Intrapulmonary fibrosis with intense 3 yr Second episode treated by wedge re- chronic inflammation. Emphysema No recurrence section with large fibrous-walled cysts. Abun- 3 mth later dant pigmented macrophages. Meso- pneumothorax thelial, bronchiolar, and alveolar cell on contra- proliferation. Bronchioles contain lateral side mucus and pigmented macrophages. Endarteritis ofsmall vessels 16 33 F Left Second episode of spontaneous pneu- Large areas of fibrosis with adjacent 5 yr mothorax within 2 mth. Treated by emphysema. Pigmented macrophages No recurrence wedge resection in scars. Chronic bronchiolitis with mucus plugs. Endarteritis of small vessels. Cysts lined by simple fibrous

tissue http://thorax.bmj.com/ 17 26 M Left 2 Two episodes of spontaneous pneumo- Patchy fibrosis and active chronic in- 6 yr thorax over previous 31 yr. Third flammation with focal emphysema. No recurrence spontaneous pneumothorax treated by Abundant pigmented macrophages. wedge resection Cysts lined by flat cellular layer 18 19 M Left Three episodes ofspontaneous pneumo- Patchy fibrosis and emphysema, pig- 5 yr thorax over previous year. Further mented macrophages, small fibrosed No recurrence recurrence treated by wedge resection bronchi. Cysts lined by prominent cellular layer 19 26 M Left 3 Three episodes ofspontaneous pneumo- Patchy fibrosis and emphysema. Alveo- 5 yr thorax over previous year. Further lar and bronchiolar cell proliferation, No recurrence recurrence treated by wedge resection pigmented macrophages, distended bronchi with chronic inflammation. on September 25, 2021 by guest. Protected copyright. Cysts lined by fibrous tissue 20 18 M Left 4-5 Left side: Asthma since age 13. Five Dense vascular subpleural scar with 7 yr Right 5-6 episodes of spontaneous pneumothorax patchy chronic inflammation. Emphy- No recurrence on left side and one on right over sema and subpleural fibrous-walled previous 2 yr. Further recurrence on cysts. Distended bronchi with cartilage left side treated by wedge resection in walls. Vessels show endarteritis in one biopsy only Right side: Admitted 18 mth later after 5 yr 4 episodes of spontaneous pneumo- No recurrence thorax on right side. Wedge resection for recurrence

In each case the portion of lung was disorganized The changes consisted of emphysema and cyst by fibrosis, collapse, and cyst formation, all variable formation, atelectasis, fibrosis, chronic inflammation, in degree. There was no evidence of adhesions to pigment deposition, bronchiolar, alveolar cell, and the chest wall. mesothelial proliferation, bronchial lesions, and vascular changes. MICROSCOPIC FINDINGS Histologically the changes were non-specific. Although common features were Emphysema and cyst formation Emphysema was present, these varied in prominence from case to case, constant and focally distributed, being compensatory suggesting that the process was in different stages in some areas and destructive in others (Fig. 2). of progression at the time of presentation. Cysts were usually lined by fibrous tissue (Fig. 3) Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from

412 1. Lichter and J. F. Gwynne

areas. Atelectatic foci were often invaded by fibrous tissue and were in many instances related to adjacent compensatory 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 with replacement of architecture (Fig. 6). In other areas, fibrous tissue caused thickening of alveolar walls, especially in foci of atelectasis. Chronic inflammation Active infiltration of dense scar tissue was seen in some cases but this was not the general rule. When present, the cells consisted mainly of lymphocytes and plasma cells, polymorphs being Intra-alveolar macro- FIG. 1. Appearance at inconspicuous (Fig. 6). operation showing cysts and phages were seen frequently. In case 10 there was scarred lung tissue. florid inflammation with foamy macrophages. This patient had been treated elsewhere by camphor oil but a prominent mesothelial layer was present in instillation. some instances (Fig. 4). Cysts were usually subpleural but occasionally were seen in scars. Pigment deposition This was inconstant and, when present, was found in macrophages, both in scar tissue Atelectasis This was a constant feature, being distri- and also lying free in air spaces. Both haemosiderin buted either in linear fashion or in ill-defined circular and carbon were identified (Fig. 7). http://thorax.bmj.com/

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A FIG. 2. Photomicrograph showing compensatory emphysema.Ja~~~~~~~~~~~~~~~~~~~~tH. and E. x 44. Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from http://thorax.bmj.com/ FIG. 3. Photomicrograph ofsubpleural cyst lined by fibrous tissue. H. and E. x 48.

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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. Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from

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FIG. 5. Photomicrograph of vascular subpleural scar with a focus of residual infl27mmation. H. and E. x 50. http://thorax.bmj.com/

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FIG. 7. Photomicrograph ofpigmented macrophages in alveolar space. Pigment is also deposited in adjacent fibrous tissue. H. and E. x 256. http://thorax.bmj.com/ on September 25, 2021 by guest. Protected copyright.

FIG. 8. Photomicrograph ofsmall bronchi distended with secretion. There isperibronchial inflammation. H. and E. x 115. Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from

416 1. Lichter and J. F. Gwynne

Alveolar cell proliferation This occurred in dis- back, Kiehl, and Hannon, 1964) that in tall thin organized scarred areas (Fig. 6). The cellular reaction individuals there is a rapid growth rate relative was considered to be related to nearby scarring and to pulmonary vasculature accounting for relative inflammation. ischaemia and bleb formation during growth. Bronchial lesions When bronchi were found in the Killen and Gobbel (1968) describe bleb formation biopsies, they were on occasion filled with eosinophilic with associated fibrosis and chronic inflammation. secretion as though obstructed by scar tissue (Fig. 8). They point out that the aetiology is unknown but Some showed peribronchial fibrosis and mild inflam- consider that a congenital fault may be present mation of their walls. or that the lesion may be secondary to local stress injury or degenerative change. Similarly, it was Vascular lesions No specific inflammatory or occlu- suggested in the British Medical Journal (Leading sive lesions were seen but endarteritis obliterans was article, 1968) that the lesion might result from a common. congenital fault or an inflammatory scar. Hyde DISCUSSION (1963) drew attention to the long narrow chest and related this to the development of blebs as AETIOLOGY The special site of the damage in each a congenital anomaly. The same author (1962) case suggested the possibility of a tuberculous had noted the fact that the patients were 20-30 lb aetiology, but there was no histological evidence (9-13 kg) underweight. Aust (1961) presumed that for this in any of the cases. the blebs were congenital in nature. Thomas (1959) Localized congenital cystic lung was entertained divided his cases into those with congenital cysts as a possible explanation but the constant relation and those with acquired blebs or bullae. He of cysts to scar tissue made this difficult to assess. indicated that loss of elasticity, fibrosis, and Post-inhalational damage was excluded by the adhesions resulted from pulmonary vascular site and by the absence of any evidence of inhaled insufficiency, causing bleb formation. material apart from carbon. None of the above authors has incriminated Localized trauma to the lung suggested itself tuberculosis in the aetiology of primary pneumo- in view of the youth of the patients and the history thorax, and this disease was not in evidence in of athletic activity, but there was no real evidence any of our cases. http://thorax.bmj.com/ of organizing haematomata. The finding of haemosiderin pigment was inconstant and insufficient to INCIDENCE The true incidence of apical lung explain the findings on a basis of haemorrhage. cysts in the population is not known, recognition On histological grounds alone the changes are depending on the complication of rupture. Radio- best explained on a basis of post-inflammatory logical study prior to the development of pneumo- disorganization, the inflammation being most thorax is usually negative (Baronofsky et al., likely due to non-specific infection. The curious 1957; Bernhard et al., 1962; Reid, Stevenson and

localization of the lesions and their occurrence McSwan, 1963; Leading article, 1968). Our own on September 25, 2021 by guest. Protected copyright. predominantly in young males are obscure features experience confirms the frequent absence of radio- which suggest some local inherent predisposition. logical changes. Many of the previous reports relating to primary According to a leading article in the British spontaneous pneumothorax in young subjects have Medical Journal (1968) the incidence of the com- not included detailed accounts of the histological plication of pneumothorax rose from 025 per changes (DuBose, Price, and Guilfoil, 1953; thousand in the 1950s to 04 per thousand in the Bernhard, Malcolm, Berry, and Wylie, 1962; 1960s. This is probably due to better recognition Carpathios and Bogedain, 1963; Leading article, of the condition. Several large series have been 1965; Lynn, 1965; Mills and Baisch, 1965; Levy, reported from military sources and these all stress 1966; Shields and Oilschlager, 1966). the importance of the condition in young appar- Most authors have favoured a congenital origin ently fit men (Thomas, 1959; Withers et al., for the cysts or have postulated inflammation and 1964; Mills and Baisch. 1965). fibrosis with cyst formation. An earlier hypothesis The frequency of involvement of both sides at by Brock (1948), that air may leak through an different times in the same patient is worthy of imperfect visceral pleura, has not been reiterated note, and suggests that bullous disease may be in recent studies, all of which record the presence present in an intact state on the contralateral side of bullae. in a significant proportion of patients with appar- The hyposthenic habitus of the patients is of ently unilateral disease. Pneumothorax occurred interest and it has been suggested (Withers, Fish- bilaterally in two of our patients (10%) and this Thorax: first published as 10.1136/thx.26.4.409 on 1 July 1971. Downloaded from

Spontaneous pneumnothorax in young subjects 417 may rise to a higher figure as our patients are We wish to thank the staff of the Photographic followed up for longer periods. Baronofsky et al. Department, University of Otago Medical School, for (1957) were so impressed with the bilateral the photomicrographs, and Mr. D. Tingle for technical nature of the bullous disease that they performed assistance. Mrs. D. Schmelz typed the manuscript. thoracotomy on both sides in each of their 26 patients. Bullae were found on both sides in all but one of their patients. Details of the patho- REFERENCES logical findings in their series were not given. Aust, J. B. (1961). Spontaneous pneumothorax. Postgrad. Med., 29, 368. THERAPY Wedge resection of diseased lung tissue Baronofsky, 1. D., Warden, H. G., Kaufman, J. L., Whatley, as used in this series of patients reflects the J., and Hanner, J. M. (1957). Bilateral therapy for localized apical nature of the disease process which unilateral spontaneous pneumothorax. J. thorac. Surg., caused the pneumothorax. Failure to understand 34, 310. tissue has in the Bernhard, W. F., Malcolm, J. A., Berry, R. W., and Wylie, the focal affection of lung past R. H. (1962). A study of the pathogenesis and manage- led to irrational therapy such as pleural cavity ment of spontaneous pneumothorax. Dis. Chest, 42, 403. obliteration by the instillation of irritating fluids, Brock, R. C. (1948). Recurrent and chronic spontaneous a procedure liable to fail. When leakage persists pneumothorax. Thorax, 3, 88. for longer than 48 hours, or when the pneumo- Carpathios, J., and Bogedain, W. (1963). Spontaneous thorax recurs after simple intercostal tube pneumothorax: experience with 50 cases. Amer. Surgn, drainage, thoracotomy seems to be the only 29, 525. logical DuBose, H. M., Price, H. J., and Guilfoil, P. H. (1953). approach to permanent cure. So far, in our series. Spontaneous pneumothorax: medical and surgical the results have been good with no recurrences. management. Analysis of 75 patients. New Engl. J. Med., Baronofsky et al. (1957) recommended resection 248, 752. while Withers et al. (1964) found that the tech- Hyde, L. (1962). Benign spontaneous pneumothorax. Ann. nique compared favourably with other methods. intern. Med., 56, 746. Reid et al. (1963) reported similarly when they (1963). Spontaneous pneumothorax. Dis. Chest, 43, 476. Killen, D. A., and Gobbel, W. G. (1968). Spontaneous compared the results of intercostal tube drainage, Pneumothorax, pp. 43-58. Little, Brown, Boston. kaolin pleurodesing agent, and segmental resection. Leading article (1965). Spontaneous pneumothorax. Brit. http://thorax.bmj.com/ med. J., 1, 1262. CONCLUSIONS Leading article (1968). Spontaneous pneumothorax. Brit. med. J., 1, 720. 1. A series of 20 patients with recurrent or per- Levy, I. J. (1966). Spontaneous pneumothorax. Treatment sistent is based on analysis of 170 episodes in 135 patients. Dis. spontaneous pneumothorax presented Chest, 49, 529. from the clinical and pathological standpoints. Lynn, R. B. (1965). Spontaneous pneumothorax. Dis. Chest, All the patients were treated by wedge resection 48, 251. of diseased lung tissue. Mills, M., and Baisch, B. F. (1965). Spontaneous pneumo- 2. The pathological features have been reviewed thorax. A series of 400 cases. Ann. thorac. Surg., 1, 286. on September 25, 2021 by guest. Protected copyright. and an attempt has been made to explain the Reid, J. M., Stevenson, J. G., and McSwan, N. (1963). The aetiology. management of spontaneous pneumothorax. Scot. med. 3. The histological lesions are non-specific but J., 8, 171. Shields, T. W., and Oilschlager, G. A. (1966). Spontaneous suggest post-inflammatory fibrosis and cyst forma- pneumothorax in patients 40 years of age and older. tion. An underlying congenital abnormality related Ann. thorac. Surg., 2, 377. to the anatomy of the thorax in tall thin athletic Thomas, P. A. (1959). Spontaneous pneumothorax-MoJern individuals appears to be an attractive hypothesis. concepts in etiology and treatment of an important Defects in blood supply and aeration in these syndrome in military practice. Milit. Med., 124, 116. factors. Withers, J. N., Fishback, M. E., Kiehl, P. V., and Hannon, circumstances may be important J. L. (1964). Spontaneous pneumothorax. Suggested 4. Treatment by wedge resection seems to be a etiology and comparison of treatment methods. Amer. J. rational approach to the underlying pathology. Surg., 108, 772.