Role of CT in the Management of Pneumothorax in Patients with Complex Cystic Lung Disease

Role of CT in the Management of Pneumothorax in Patients with Complex Cystic Lung Disease

Role of CT in the Management of Pneumothorax in Patients with Complex Cystic Lung Disease* Gerrard D. Phillips, MA , DM; Beatrice Trotman-Dickenson, M...

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Role of CT in the Management of Pneumothorax in Patients with Complex Cystic Lung Disease* Gerrard D. Phillips, MA , DM; Beatrice Trotman-Dickenson, MBBS; Margaret E. Hodson, MD; and Duncan M. Geddes, MSc, MD

The diagnosis and treatment of pneumothorax in patients with complex cystic lung disease may be difficult when relying on plain chest radiography alone . We report four cases in which management was greatly facilitated by the use of CT scanning of (CHEST 1997; 112:275-78) the chest. Key words: computed tomography; cystic lung disease; pne umothorax

ediagnosis and treatment of pne umothorax in paTh tients with cystic lung disease may be difficult when

relying on plain chest radiography alone. The complex appearance of the lungs themselves or partial adherence of the lung to the chest wall due to p!ior surge1y or inflammation , or both of these factors , may result in an unusual configuration of the pneumothorax or may mask it altogether. Selecting an appropriate and safe site for intercos tal tube drainage may thus be difficult or impossible . CT scanning allows examination of the cross-sectional anatomy of the thorax and can provide information that greatly enh ances the management of pn eumothorax in such patients . We describe four cases th at demonstrate the role of CT scanning in such circums tances. All CT scans were obtained on an ultrafast electron beam scann er (! matron ; South San Francisco, Calif) . REPORT OF CASES CASE

FIGURE 1. Case 1. Posteroanterior chest radiograph showi ng a widespread coarse reticular patte rn consistent with advanced Lange rhans' cell histiocytosis. The lung volumes are increased l basal and right paramediastinal pneumothoand th ere is a arge rax. Two right chest drains are in situ; th e upper drain lies in th e obli que fissure.

cystic lung destruction and a large right tension pneumothorax (Fig 2) . The pneumothorax appeared partially loculated due to adhesions from the previous pleu rectomies. However, th e anterior and posterior air collections were seen to be in communication on th e more superior sections (not shown) . The right chest drains were demonstrated, with one lyi ng in the oblique fissure and not in continuity with the pneumothorax. This latter drain

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A 23-year-old woman with severe lung disease (FEV1 , 31% predicted; FVC, 33% predicted; diffusion of carbon monoxide, 25% predicted ) due to Langerhan s' cell histiocytosis presented with acute breathlessness. In the past she had had bilateral pleurectomi es for recurrent pneumothorax. A plain chest radiograph demonstrated a right apical pneumothorax. Simple tube drainage failed, and the patient unde1went th oracotomy and oversewing of th e air leak. Postoperatively, the air leak recurred, infection supervened, and the patient required a period of mechanical ventilation. Two light chest tubes were inserted to drain th e loculated pneumothorax during this time. It subsequently proved possible to wean the patient from the ventilator with th e two right-sided chest drains still in situ. Three weeks later, she us ddenly became more breathless . A chest radiograph showed no apparent change in the size of th e right pneumothorax (Fig 1). A CT scan obtained several hours late r revealed severe *From the Department of Thoracic Medicine, Royal Brompton National Heart and Lung Hospital, Sydney Street, London, UK. Manuscript received October 3, 1996; revision accepted ovember 15. Reprint requests: Dr Phillips, Dorset County Hospital, Princes Street, Dorchester, Dorset DTl lTS, UK

FIGURE 2. Case 1. CT scan at the level of th e lw1g bases with 6-mm collimation, revealing a large right tension pneumothorax. The collapsed light lung is ad he rent to the la teral chest wall , separating the hemithorax into anterior and posterior compartments. The supelior light chest drain lies in the oblique fissure. The left lung s hows extensive cystic destruction. CHEST I 112 I 1 I JULY, 1997

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was removed, and a new posterobasal drain was inse1ted under CT guidance with immediate symptomatic relief. Over a period of weeks, th e pneumothorax slowly resolved, and the chest drains we re removed without recurrence of th e problem. CASE 2 A 2.5-year-old woman with severe bronchiectasis due to cystic fibrosis presented to her local hospital with acute shortness of breath. She suffered from fi·equent chest infections requiring intravenous antibiotics and a sputum culture was positive for Pseudomonas aerugin.osa. He r FEV 1 was normally only 0.46 L and her FVC was 1.15 L. In th e past, she had had two small instances of pneumothorax in the left lung treated by aspiration. Two months previously, a right apical pneumothorax had required thoracoscopy with oversewing of th e air leak. On presentation, the chest radiograph showed no evidence of a pneumothorax. On transfe r to the H.oyal Brompton Hospital, another radiograph de monstrated a curvi li near opacity adjacent to th e right lateral chest wall (Fig 3). This appearance was suggestive of a pneumothorax. While breathing 60% oxygen, she was extre mely distressed and hypoxic, with a Po 2 value of 65 mm Ilg and a Pco 2 of 41 mm Hg. Although the posteroanterior radiograph demonstrated a small right pneu mothorax, th e size appea red disproportionately small compared with her symptoms. Moreove r, neither the view on this film nor a lateral vi ew clarified th e optimum site for chest drain inse1tion. There fore, aCT scan was performed. This demonstrated a surprisingly large 1ight te nsion pneumothorax with a complex configuration due to adhesions (Fig 4). A chest drain was immediately inserted, with

FIG UHE 4. Case 2. CT scan obtained through th e lung bases with 6-mm collimation , re~ealing a co~p l ex right tension pneumothorax. The size and configuration of th e pneumothorax could not be determined on the plain radiograph. Widespread bronch ial wall th ickening with peribronchial inflammation is shown in the left lung.

minimal symptomatic relief. A subsequent limited scan demonstrated the drain lying within th e 1ight oblique fissure. The fissure, which had previously been expanded by the pneumothorax, had now collapsed around the drain, occluding it. A second drain was positioned supe1iorly and anteriorly with relie f of the patient's acute breathlessness. Assisted ventilation was th erefore avoided. Although a subsequent scan demonstrated reexpansion of the right lung, th ere was continued air leak. An elective repair with surgical oversewing and pleurodesis was performed. Subsequently, the patient's condition worsened, and although the pneumothorax did not recu r, she died of respiratmy failure 3 months late r. CASE 3 A 24-year-old woman with cystic fibrosis was admitted with an infective exacerbation of her bronchi ectasis. She suffered approximately three such episodes p eryear. Usually, her FEV1 and FVC were 0.65 Land 1.3 L, respectively. Ten years previously, she had had limited bi lateral pleurectomi es for instances of nonsimu ltaneous apical pneumothorax. She was treated with intravenous antipseudomonal antibiotics. Two weeks later, she expe ri enced sudden severe right chest pain with mi ldly increased breathlessness. With the patient in th e e re ct position, a plain chest radiograph revealed chronic bullous changes at the right apex and a new area of hyperlucency con touring the 1ight hemidiaphragm (Fig 5). This suggested a small loculated ri ght basal pneu mothorax. ACT scan (Fig 6) was performed because her chest pain was di sproportionately severe in relation to th e chest radiographic appearance. The scan demonstrated a multiloculated and predominantly subpulmona1y pneumothorax which , du e to its location , may have been under t ension and thu s may have caused her symptoms. Since chest pain rather than breathlessness was the problem, a drain was not inserted. The pneumothorax and her symptoms gradually resolved spontan eously.

FIG UHE 3. Case 2. Posteroanterior chest radiograph demonstrating diffuse bronchial wall thickening, mu cus plugging, and patchy areas of consolidation. The lungs are hype1inflated with evidence of volume loss in the upper lobe of th e right lung. There is mediastinal shift to the left. In th e right midzone, there is a subtle vilinear opacity area of hyperlucency bounded by a fin e c u1 (arrow) representing a pneumothorax. 276

CASE 4 A 26-year-old wo man with cystic fibrosis prese nted with a left pne umothorax that r equired intercostal tube drainage. H.esolution was prolonged and required intermittent suction, but it subsequently proved possible to remove the drain 2 weeks later, s all residual pneumothorax. One week later, th e leaving a m Selected Reports

DISCUSSION

FIGURE 5. Case 3. Posteroanterior chest radi ograph revealing bilateral upper lobe volume loss with patchy inhomogeneous consolidation and bullous formation within th e ri ght apex. At the ea of hyperright base, contouring the hemidiaphragm, is a n ar lucency (arrow) not seen on prior racliographs, suggesting a loculated right basal pneumothorax.

patient became very breathless. A plain radiograph revealed a recurrent multiloculated l eft pneum oth orax. A CT scan revealed multiple air collecti ons partially s eparated b yfibrous bands but de monstrated that th ey were in continui ty. A single chest drain was inserted with immecliate symptomatic relie f and reexpansion of the lung. However, due to acontinued air leak, an elective surgical repair and limited p leurodesis were subsequently perform ed.

FIGURE 6. Case 3. CT scan through the lung bases demonstrating a right anterior pneumothorax compressi ng the middle lobe. Note the subpleural cysts.

Patients with cystic lung disease are particularly likely to have pneumothorax. However, the reticular pattern , bullous formation, and partial adherence of the lung to the chest wall that are so prevalent in such patients make identification of pneumothorax diffi cult. In addition, instances of pneumothorax in such p atients are rarely simple so that while comparison with prior radiographs or the taking of a l ateral radiograph 1 may be useful, plain chest radiographs may fail to identifY the presence of an air collection, may underestimate its size, or may fail to identifY the safest site for drainage. In patients who are severely breathless, the consequences of misdiagnosis or injudicial placement of a chest drain may be grave. We describe fou r patients with severe cystic lung disease whose management was greatly enhanced b y CT scanning. CT confirmed the diagnosis, demonstrated the site and size of the pneumothorax, identified adhesions and loculations, and indicated the optimum site for drainage. In two patients, fu rther surgery was a voided; in one of these, it was possible to manage the situation without even requiring recourse to intercostal intubation. In the other two cases, CT -guided chest drain placement provided symptomatic relief and allowed stabilization of the patient plior to elective surge1y . Previous studies have shown that CT scanning is no more sensitive than a lateral d cubitus e plain film in the detection of a simple pneumothorax 2 but is much more accurate in correctly determining the size of one.3 Studies vary as to whether d ete ction of multiple lung blebs by CT does4 or does not5 predict a g reater likelihood of recurrence of simple s pontaneous pneumothorax. A number of reports also suggest that CT is very useful in the dete ction of occult pneumothorax in trauma patients. 6 ·7 Although one previous reportH addressed the use of CT in the management of complex pneumothorax, only one of the three patients mentioned had interstitial lung disease. Bourgouin et al9 discuss the use of CT in two patients with bullous lung disease, while Chon and coworkers 10 describe the use of the te chnique in the management of air collections in ventilated p atients with ARDS . We believe that the present report is the first to document the various ways in which CT scanning can aid in the diagnosis and management of pneumothorax in a group of patients who all have complex cystic lung disease. Because of the existence of multiple cystic areas and adhesions to the lateral chest wall, the plain chest radiograph did not provide sufficient information for therapeutic decision making in a nyof our patients. CT scanning, however, demonstrated the presence of one or more a reas of pneumothorax, revealed the position and anatomy of these, allowed d ecisions to be made on whether or not to insert an intercostal chest drain, and also indicated the best position for doing so. CT thus enabled the stabilization of acutely ill patients either as a prelude to surgery or definitively. W e therefore advocate the use of CT in such patients when they become acutely breathless and the plain radiograph either fails to reveal the presence of a CHEST I 112 I 1 I JULY, 1997

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pneumothorax, although one is suspected, or fails to provide sufficient information to allow management decisions to be made. REFERENCES 1 Morgan RA, Owen CM , Collins CD, et al. Detection of pne umothorax with lateral shoot through digital radiography. Clin Radiol 1993; 48:249-52 2 Carr JJ, Read JC, Choplin RH , e t la. Plain and computed radiography for detecting experimentally induced pneumothorax in cadavers. Radiology 1992; 183:193-99 3 Engdahl 0 , Toft T, Boe J.Chest radiography-a poor method for determining the size of a pne umothorax. Chest 1993; 103:26-29 4 Warner BW, Bailey WW, Shipley H.T. Value of computed tomography of the lung in th e management of p1immy spontaneous pneumothorax. Am J Surg 1991; 162:39-42 5 Mitlehner W, Friedrich M, Dissman W. Value of compu ted tomography in the detection of bullae and blebs in patients with primary spontaneous pneumothorax. H.espiration 1992; 59:221-27 6 Wall SD, Federle MP, Jeffrey RB , e t al. CT diagnosis of unsuspected pneumothorax after blunt abdominal trauma. AJR Am J Roentgenol 1984; 141:919-21 7 Tocino JM , Miller MH, Frederick PR, et al. CT detection of occult pneumothorax in head trauma. AJR Am J Roentogenol 1984; 143:987-90 8 Crabbe MM, Mappin FG, Fontene lle LJ. The use of computed tomography to assess and treat complex pneumothorax. Milit Med 1993; 158:193-96 9 Bourgouin P, Cousineau G, Lamire P, et al. Computed tomography used to exclude pneumothorax in bullous lung disease. J Can Assoc Radiol 1985; 36:341-42 10 Chon KS , van Sonnenberg E, D 'Agostino HB, et al. CT guided percutaneous drainage of loculated thoracic air collections in patients with severe pulmonary compromise [abstract] . Soc Thorac Radio! 1994; 11:11

Pneumothorax as a First Manifestation of Sarcoidosis* Marias E. Froudarakis, MD, FCCP; Demosthenes Bouros, MD, FCCP; Argyro Voloudaki, MD; Spyros Papiris, MD; Yiannis Kottakis , MD; Stavros H. Constantopoulos, MD, FCCP; and Nikolaos M. Siafakas, MD, PhD, FCCP

Pneumothorax is a rare manifestation of sarcoidosis, occurring usually late in the course of the disease. We report five cases of pneumothorax as a presenting manifestation of sarcoidosis. In two patients, *From th e Departments of Thoracic Medicine (Drs. Froudarakis, Bouros, Kottakis, and Siafakas) and Radiology (Dr. Voloudaki), Medical School, University of Crete, and th e Department of Thoracic Medicine (Drs. Papiris and Constantopoulos), Medical School, University of Ioannina, Greece. Manuscript received October 10, 1996; revision accepted Dece mber 17, 1996. Reprint requests: Marias E. Froudarakis, MD, Department of Thoracic Medicine, University Hospital Heraklion, Crete, 71110 Greece; email: [email protected]

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thoracotomy showed extensive pleural infiltration by noncaseating granulomas. High-resolution CT scans showed cavitated subpleural nodules and subpleural bullae in one case. These findings support that necrosis of subpleural granulomas or rupture of a subpleural bullae, or both, are the mechanisms of pneumothorax in sarcoidosis. Three patients with a lung function impairment were treated with oral corticosteroids. One nontreated patient died due to progression of the disease. (CHEST 1997; 112:278-80) Key words: corticosteroids; diffuse interstitial lung disease; granuloma; granulomatous disease; pneumothorax; sarcoidosis Abbreviations: CCT=conventional CT; HRCT = high-resolution CT

pneumothorax occurs in 2 to 4% of patients with sarcoidosis. 1·2 It is suggested that this rare manifestation of sarcoidosis is related to either rupture of a subpleural bleb or necrosis of a subpleural granuloma. 2 Although pneumothorax is observed in the late evolution of sarcoidosis , it seems that it could occur in the early stages as welJ.3 We report five patients with pneumothorax as a presenting manifestation of sarcoidosis. CASE REPORTS We reviewed the cases of 193 patients who had sarcoidosis from 1981 to 1995. The cases of five patients were reported with pneumothorax noted as a revealing manifestation of sarcoidosis. Diagnosis was confirmed by transbronchial biopsy in three patients and by open-lung biopsy in two patie nts showing noncaseating granulomas. Absence of acid-fast bacilli and fungi was noted in sputum cultures, biopsy specimens, and body fluids. The Mantoux test was negative in all patients. The serum angiotensinconverting enzyme was elevated in all cases (mean, 92.4± 17.7 IU/mL; normal range, 15 to 50 IU/mL). Urine and serum calcium values were normal in 4 and elevated in 1 patient (case 4). In all patients, lung function (spirometry and transfer factor) were measured 3 months after the resolution of pneumothorax. In two pati ents, lung function was norm al, wh ile in three patients a restrictive impairment was noted. CASE

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A 46-year-old man with a history of insulin-dependent diabetes was admitted to the hospital in July 1982 for a complete pneumothorax on the right side. After resolution of the pneumothorax, the chest x-ray film and the conventional CT (CCT) scan of the thorax showed diffuse reticular lesions with bilateral hilar and subcarinal lymphadenopathy. Because lung function was normal, it was decided only to do follow-up examinations on the patient. Three years later, the patient's condition deteriorated. An attempt to introduce c01ticosteroid therapy failed because of the disregulation of his diabetes mellitus. The patient finally died 12 years later due to progression of sarcoidosis; however, no relapse of the pneumothorax was noted during the follow-up period. CASE

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A 33-year-old man was admitted to th e hospital in April 1986 for a co mplete pneumothorax on the right side. After resolution Selected Reports