Bullae and severe generalized disease Successful treatment with bullectomy Sheldon R. Braun, * Guillermo A. doPico, ** Marvin L. Birnbaum, *** and John R. Pellett, **** Madison, Wis.
SUrgiCal intervention for bullous disease of the lung has been accepted with varying degrees of enthusiasm for the last quarter of a century. Presently, there is substantial variability in the methods used for selection of patients who would potentially benefit from surgery; coupled with this is a degree of uncertainty in the rationale for surgical intervention.' The following is a report of a patient who was treated surgically for bullous disease in spite of having significant components of the disease which seemed to be contraindications for surgery. Postoperatively, she exhibited both subjective and objective improvement. Case report A 45-year-old white woman was admitted to the University Hospitals in severe respiratory distress. She had smoked one half to three quarters of a package of cigarettes per day since the age of 18 years. The first indication of pulmonary disease occurred 18 years prior to the present admission when she developed a spontaneous right pneumothorax. Subsequently, she suffered recurrent episodes of bronchitis. Eight years prior to admission she began to notice dyspnea with strenuous exercise. The severity of the dyspnea inFrom the Departments of Medicine, Surgery, and Physiology, University of Wisconsin Center for Health Sciences, Madison, Wis. 53706. Received for publication March 9, 1973. "Fellow supported by National Institutes of Health Grant No. 5TOI-HL-05626-09. "" Assistant Professor of Medicine. """ Assistant Professor of Medicine and Physiology. """"Professor of Surgery.
creased insidiously over the next several years. Two months prior to admission she developed a severe exacerbation of symptoms, and by the time of admission she was no longer able to perform her duties as a housewife and mother. The patient presented in acute respiratory distress with a ventilatory rate of 36 breaths per minute. The accessory muscles of respiration were hypertrophied and used for quiet breathing. The diaphragms were displaced downward with limited excursion. Breath sounds, especially over the apices, were distant. The remainder of the physical examination was noncontributory. A chest film revealed a large bulla filling the upper two thirds of the right hemithorax and a smaller bulla on the left. The arterial Po, was 37 mm. Hg, and the pH was 7.39, with no carbon dioxide retention. Low-flow oxygen by nasal prongs caused prompt elevation of the Pea" and it was necessary to insert a nasotracheal tube and assist ventilation with a volume-cycled ventilator. Ventilatory support, intensive nursing care, retraining of breathing, and physical therapy were required. With this regimen, there was some reexpansion of the remaining compressed lung. The course was complicated by several episodes of bronchopneumonia which responded to antimicrobial drugs and chest physiotherapy. The nasotracheal tube was removed on the nineteenth hospital day and a course of rehabilitation was begun. However, the severity of pulmonary impairment was incapacitating. Severe hypoxemia (Table I) persisted even with continuous oxygen administration. It became apparent that, unless some further improvement in the ability for gas exchange could be provided, she would be unable to contribute to the care of her family or home. Chest x-ray study at this time (Fig. 1) showed persistence of large bullae in both upper fields with probable compression of the remaining lung. This was confirmed
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Bullae and severe generalized disease
Nu mber 6
.)8 DEC 7/ Fig. 1. Posteroanterior chest film show ing extent of bullou s disease.
Fig. 2. Arteriogram of r ight pulmonar y artery revealing the de gree of compression of the vasculature by the bulla .
Table I. Mea sures of pulmonary function before and after bullectomy Before bull ectom y Test
TLC (rnl.) Vital capaci ty ( mI.) FEV , ( mI.) FE V,/ FVC % MVV (L./ min. ) DL"o (ml./min./mm. Hg) Pao, (mm. Hg) Pa"o, (mm. Hg) pH HCO :; (mEq./L.)
A fter bullectom y
4 Da ys
3 Month s
6 Mo nt hs
2,830 1,427 812 57 30.4 2.6 45.1 41.9 7.432 27.6
3,674 1,983 1,253 67 37.1 5.9 48.5 31.0 7.455 21.9
3,920 2,342 1,355 58 57.6 7.3 63.0 31.2 7.47 22.7
3,744 2,356 1,325 56 55.7 7.9 63.2 31.3 7.47 22.8
Legend: TL C, Tot al lung capacity . F EV" O ne seco nd for ced vital capacity. FEV,/FVC %, One second forced vital ca pacity as per cent of tot al forced vital capaci ty . MVV, Maximum ventilator y ventilat ion . O Leo, Single breath pulm on ar y di ffusing capacity for ca rbo n monoxide . Pan" Par tia l press ure of o xygen in a rterial blood ; Pacn, P arcial pre ssure of carbon diox ide in arteria l blood ; HCO" Pla sma bicarb on ate .
by pulmonary angiogra phy ( Fig. 2). At the time of angiography, pulmonary function studies (Ta ble I) indicated severe generalized impai rment of lung func tion . It was clear that the onl y possibility of improving lun g funct ion was to attempt remo val of the large bull a on the right with the hope that the remain ing lung coul d be re-expand ed and that pulmonary funct ion would improve. The chest was opened throug h a posterolatera l right thoracotom y incision. A lar ge bull a occupied most of the upper lobe . Also, there were diffuse emphysematous changes of the superior segment
of the middle lobe and anterior segment of the upper lobe. T he bull a was opened across its superior margin. Num erous str and-like septae extended acro ss the inne r aspect of the bulla, and mult iple areas which communicated freel y with surrounding lun g tissue were visible. Most of the a pical and poster ior segments of the uppe r lobe were excised. An attempt was mad e to save the a nterio r segment by sewing ac ross the residual lung tissue at the junction of the poster ior and ante rior segment s. This was acco mplished by mean s of a plicat ing technique with hor izont al ma ttre ss suture s of chromic gut. The technique allo wed con-
The Journal of
Braun et al.
Thora cic and Cardiovascular Surgery
Fig. 3. Posteroanterior chest film done 3 months postoperatively. trol of nearly all the air leaks. A single che st tube was inserted into a dependent position in the hemithorax, and the chest was closed in the routine manner. A nasotracheal tube was left in place . The postoperative course was difficult but uncomplicated. Ventilation was assisted with a volume-cycled ventilator. Small air leaks occurred postoperatively but were not a serious problem. Ventilatory support was gradually withdrawn, and the nasotracheal tube was removed on the tenth postoperative day . Recovery was steady but gradual. Pulmonar y funct ions and blood-gas stud ies (Table I) gradually improved as did the chest x-ray picture (Fig. 3). One year after the operation, the woman is able to take care of her home and family.
Discussion Many features of this women's disease seemed to herald a poor prognosis for successful surgical treatment. In general, several investigators report that patients with severe generalized emphysema, hypoxemia," > and chronic sputum production" have not had good results from surgical treatment. This patient's pulmonary function studies were indicative of significant airway and generalized parenchymal disease; she had severe hypoxemia at rest; there was recurrent bronchitis; and pulmonary art ery pressure was markedly elevated. Yet, surgery was accomplished with relatively few complications and
resulted in both subjective and objective improvement. Physiologic improvement following surgery has been demonstrated before.>" 7 , 8 In most patients with extensive disease, such as the one presented here, there is a lack of objective information supporting the subjective improvement. Some workers"- 7 have either failed to measure changes which occurred following surgery or at least have not reported them. Others, such as Benfield and colleagues,' have the general impression that there is no reliable correlation between subjective and objective improvement. The great majority of these patients have localized disease, with the remaining lung tissue essentially normal. In this type of disease, there is little controversy that surgery is beneficial. 2 -5, 8 -1 0 The improvement in pulmonary function which occurred following surgery is summarized in Table 1. Vital capacity increased 70 per cent above the preoperative value. This is reflected in the increase of the one second forced expiratory volumes (FEV 1) ' However, when FEV 1 was expressed as a fraction of the total forced vital capacity, the degree of airway obstruction remained essentially as it was before the operation. The diffusion capacity for carbon monoxide (DLco) was 200 per cent greater than it was prior to surgery. However, the absolute DLco, though significantly improved, continued to indicate severe parenchymal disease . The improvement which did occur in the function suggests there was an increased alveolar area available for gas exchange which in turn resulted in an improved ventilation-perfusion ratio and hence a decreased blood shunt. One individual case does not allow generalizations regarding the outcome of bullectomy in severely ill patients with diffuse obstructive lung disease. However, it does suggest that, even when the reported poor prognostic factors are present, bullectomy should be considered when an experienced thoracic surgeon is available and adequate postoperative respiratory care can be provided.
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Bullae and severe generalized disease
Summary Bullectomy in a woman with severe generalized emphysema, severe hypoxemia at rest, pulmonary hypertension, and chronic bronchitis produced remarkable subjective improvement after 6 months. This was confirmed by objective function tests. Comparison between pre- and postoperative tests demonstrated improvement in vital capacity, pulmonary diffusion capacity, and arterial oxygenation, although obstruction remained unchanged.
REFERENCES Benfield, 1. R., Cree, E. M., Pellett, J. R., Barbee, R., Mendenhall, 1. T., and Hickey, R. c.: Current Approach to the Surgical Management of Emphysema, Arch. Surg. 93: 59, 1966. 2 Baldwin, E., Harden, K. A., Greene, D. G., Cournand, A., and Richards, D. W., Jr.: Pulmonary Insufficiency. IV. A Study of 16 Cases of Large Air Cysts or Bullae, Medicine 29: 169, 1950. 3 Sieben, A. A., Grant, A. R., Kent, D. C., Klopstock, R., and Cincotti, J. J.: Pulmonary
Cystic Disease: Physiologic Studies and Results of Resection, J. THORAC. SURG. 33: 185, 1957. Bates, D. V., Macklem, P. T., and Christie, R. V.: Respiratory Function in Disease, Philadelphia, 1971, W. B. Saunders Company, p. 219. Wesley, J. R., Macleod, W. M., and Mullard, K. S.: Evaluation and Surgery of Bullous Emphysema, J. THORAC. CARDIOVASC. SURG. 63: 945, 1972. Dugan, D. J., and Samson, P. c.. The Surgical Treatment of Giant Emphysematous Blebs and Pulmonary Tension Cysts, J. THORAC. SURG. 20: 729, 1950. Fain, W. R., Conn, J. H., and Campbell, G. 0.: Excision of Giant Pulmonary Emphysematous Cysts: Report of 20 Cases Without Death, Surgery 62: 552, 1967. Warring, F. c., and Lindskog, G. E.: Surgical Management of Giant Air Cysts of the Lungs, Am. Rev. Tuberc. 63: 579, 1951. Head, J. M., Head, L. R., Hudson, T. R., and Head, J. R.: The Surgical Treatment of Emphysematous Blebs and Localized Vesicular and Bullous Emphysema: Analysis of 50 Cases, J. THORAC. CARDIOVASC. SURG. 40: 443, 1960. Laurenzi, G. A., Turino, G. M., and Fishman, A. P.: Bullous Disease of the Lung, Am. J. Med. 32: 361, 1962.