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The folded lung
Brit. 3. Dis. Chest (I966) 60, 19.
THE FOLDED LUNG BY A. BLESOVSKY* The Hospitals for Diseases of the Chest
MINOR degrees of folding of the lung are commonly found during decortication of the lung for empyema. The following cases illustrate unusually extensive lung folding due to a fibrous membrane on the costal surface of the visceral pleura of the lower lobe; surprisingly this fibrous reaction was not associated with adhesions between the parietal and visceral layers of the pleura. This condition has not been reported before. On the basis of the findings in the first patient, a correct diagnosis was made in the second and third patients before operation. Case H i s t o r i e s Case z
The patient was a 5i-year-old pipe-fitter admitted on 1.12.56. Dyspncea on exertion had been present for two years, and had become worse 6 months before admission. I n May i956 he suffered what was thought to be influenza, and a dull constant pain across the right lower chest had been present since then. A productive cough had been present for about a year. An industrial history was not obtained at that time, but contact with asbestos could have occurred as the patient was a pipe-fitter. He was a fit, well-built man with no abnormal physical signs in the chest. A rounded opacity in the right lower lobe was seen on the chest radiograph. The lesion was poorly seen on the lateral view. Bronchoscopy was normal, and malignant cells were not found in the sputum. A postero-lateral thoracotomy was performed. The pleura was free, but a thick membrane covered the posterior part of the right lower lobe and there were small plaques on the middle lobe and on the diaphragm. Healed tuberculous disease was present at the apex of the right upper lobe. The membrane was dissected off the lower lobe, which then looked and felt normal. The patient made a good recovery after operation and was discharged on 6.12.56. He was re-admitted on 7.1.57 with thrombophlebitis of the right leg and a small left pulmonary artery embolus. The specimen was made up of laminated, vascular fibrous tissue, with areas of collapsed pulmonary tissue present on the inner surface. * Present address: Department of Cardio-vascular Surgery, Presbyterian Medical Center, Clay and Webster Streets, San Francisco, California (Recelvedfor publication, November i965)
o0
BLESOVSKY
Case 2 The patient was a 33-year-old engineer. Annual chest radiographs were normal until November 1964, when changes were noted at both bases, particularly on the right. Early in 1964 the patient had noticed pains across the chest, without cough or fever. He had been a ship's engineer for IO years, during which time he had come into contact with asbestos from pipe-lagging. Physical signs were normal. The chest radiograph showed pleural shadowing at the right base and the lateral view showed radiating lines quite clearly (Fig. I (a) & (b)). The possibility that a peripheral carcinoma might be present was suggested by the opacities in the right lower lobe seen on the tomograms. The ESR was 27 mm./hour; the blood was otherwise normal. In the differential diagnosis three possibilities were considered: i. Peripheral carcinoma, suggested by the lobulated shadow on the tomograms; ~. in view of the history of contact with asbestos and the peripheral nature of the lesion, mesothelioma of the pleura was considered, but thought to be unlikely because of the absence of effusion and because of the appearance of the lesion on radiograph; 3. the most likely diagnosis was thought to be a fibrous pleural reaction because of the radiographic appearance: a flat, very peripheral lesion with radiating streaks and a small lower lobe. The changes at the left lower lobe were considered to be minor degrees of the same process. Changes similar to those found in the first case were anticipated before operation. A postero-lateral thoracotomy was performed. The pleura was free except for a few adhesions from the lung to the diaphragm. The right lower lobe was covered by a membrane up to i ram. thick. The oblique and transverse fissures were free. Hyaline plaques were present on the diaphragm. As the membrane was being stripped off the lower lobe the folds in the lung created by the fibrous tissue were demonstrated. Once freed, the lobe re-expanded easily and looked and felt normal. Histology of the membrane revealed dense fibrous tissue showing non-specific inflammatory changes. Cas8 3 The patient was a 63-year-old labourer at Tate and Lyle's sugar refinery. At work he had to pass through a room where asbestos pipe-lagging was carried out. A mass miniature radiograph early in 1964 led to further investigation, when progressive changes at the left base of the radiographs were noted. He remained free of symptoms. The radiographs showed shadowing at the left lower lobe which increased between J a n u a r y 1964 and March 1965 (Fig. 3 (a), (b), & (c)). A folded lung was clearly demonstrated in tomograms (Fig. 4)- Bronchograms showed sweeping of the terminal bronchi of the left lower lobe, with no obstruction. Blood examination was normal. Ventilatory function tests were normal. Bronchoscopy was normal, and the sputum was free of neoplastic cells.
PLATE III
FIo. I ( a ) . - - I l l - d e f i n e d shadowing at right base. L i n e a r shadows at left base.
(a)
FIO. I ( b ) . - - L a t e r a l view shows clearly the site of the lesion at the periphery of the lower lobe, a n d the well-marked striations.
(b)
FIG. e . - - T o m o g r a m s : cuts Io a n d I e, same case as Fig. i. Smallness of lower lobe, indicated by site of the oblique fissure a n d r a d i a t i n g strands of fibrous tissue are demonstrated.
2**
To faae p. 2o.
PLATE IV
(a)
(b)
FIG. 3(a) AND (b).--Increasing shadowing at left lower lobe between January 1964 and March 1965 .
FIo. 3 (c).--Lateral view shows peripheral site of lesion. Position of fissure indicates smallness of lower lobe. In this view the lesion shows up less clearly than anticipated from the well-marked changes in the postero-anterior view.
PLATE V
(a), Cut 5
(c), Cut 7
(b), Cut 6
(d), Cut 8
Fro. 4 . - - S a m e case as Fig. 3. Tomograms: cuts 5 to 8. Apparently cavitating lesion in cut 5 and 6, but more medial cuts show that there is no wall inferiorly where the " c a v i t y " is in continuity with the lung tissue. Cut 8 shows clearly that the base of the lung is folded upwards and is separated from the apical lower segment by a thick crescent of tissue.
P L A T E VI
Fro. 5 . - - S a m e case as Fig. 3. Bronchogram: J a n u a r y i965. Note: (i)small left lower lobe; (ii) swept appearance of termination of basal bronchi indicating previous pleurisy; (iii) inferior subsegment of apical lower bronchus crossing posterior basal bronchus; (iv) termination of posterior basal bronchus almost in contact with termination of superior subsegment of apical lower bronchus.
(a)
(b)
FIG. 6 . ~ R i g h t lung of a patient with carcinoma of the middle lobe : early folding as an incidental finding. (a) Surface view: thick smooth m e m b r a n e over right lower lobe, lower margin folded upwards. (b) Cut section : carcinoma in middle lobe. Thick m e m b r a n e overlying infarct in posterior basal segment. To face p. ~I.
T H E FOLDED L U N G
2I
The peripheral site of the lesion, the progressing changes, and the history of contact with asbestos suggested a pleural mesothelioma; but once the tomograms were obtained a firm diagnosis of "folded lung" was made. At operation the pleura was found to be free except for a few adhesions to the diaphragm. Hyaline plaques were present on the parietal pleura in the paravertebral gutter opposite the lesion on the left lower lobe and on the pericardium just above the diaphragm. The left upper lobe was large and filled two-thirds of the left chest. Scattered nodules of healed tuberculosis were palpable in the left upper lobe. The left lower lobe was small and its costal surface covered by a membrane up to 3 mm. thick. The lobe was considerably folded, with the margin between the costal and diaphragmatic surface almost at the apex of the lower lobe. In addition to the infero-superior fold there was a lesser degree of antero-posterior folding. The membrane was cedematous in parts, suggesting continued inflammatory activity. The oblique fissure was free of adhesions. As the membrane was stripped off, the lung unfolded and re-expanded but remained smaller than normal. It looked and felt otherwise normal. Histology of the membrane showed it to be made up of thick strands of fibrous tissue with non-specific inflammatory changes.
Discussion Folded Lung is very uncommon, and I have been unable to find any reference to it in the literature. The diagnosis can be made from the radiograph: the lesion is peripheral, it is laminar, and there are radiations from it. In the more advanced case, as in the third patient, the fold of the lung can be seen. Though the findings at operation varied in severity they were strikingly similar in each case: there were no pleural adhesions at the site of the lesion, a thick membrane enfolded an otherwise apparently normal lower lobe and there were hyaline plaques on the parietal pleura. In the third patient the oedema of parts of the membrane and the progressing changes on the X-ray suggested that the lesion was active, but we were unable to find a causative agent.
./Etiology The cause of the condition is unknown, but may be related to infection, infarction or asbestos. Infection and infarction as a cause can only be conjectured, because the underlying lung at operation was normal: if these conditions were responsible it must be assumed that the pulmonary lesion had resolved completely. There is circumstantial evidence that the lesion is a form of asbestosis, because contact with asbestos was certain in two of the patients and probable in the third, and because hyaline plaques, commonly associated with asbestosis (Harmon, 1964) were present in all three. Benign pleurisy due to asbestos has been described (Eisenstadt, I964) , but this was characterized by dense pleural
22
BLESOVSKY
adhesions, a feature strikingly absent in the present cases. If the "folded l u n g " is due to asbestosis, it is a reaction not previously described in that condition. Pathogenesis The absence of adhesions at the site of the membrane over the lower lobe is unusual, but can be explained as follows. Occasionally at thoracotomy for mitral stenosis infarcts are found in the lower lobe unassociated with adhesions but associated with a small pleural effusion. If the primary lesion in the lung causes an effusion, this will keep the affected lobe away from the chest wall; during this time fibrin is deposited on the lower lobe, and by the time the effusion absorbs the membrane on the lower lobe has become organized and smooth and will not adhere to the parietal pleura. As the membrane organizes it contracts and causes the lung to fold. That this is one way in which the membrane forms is illustrated by the case of a patient with carcinoma of the middle lobe. At thoracotomy there was a small effusion associated with an infarct in the lower lobe. There were no adhesions, and the lower lobe was covered b y a thick, shiny membrane. The lower margin of the lobe was beginning to fold upwards (Fig. 6). Though a correct diagnosis is possible from the radiographic appearances, because of the rarity of the condition a thoracotomy is advisable to confirm the diagnosis. Removal of the membrane allows the lobe to re-expand, but it is doubtful if any improvement in lung function results.
Summary Unusual degree of folding of the lung is described in three cases. Diagnosis was made in the second and third patients before operation. The pathogenesis is discussed, and the possibility that these three cases were related to asbestos is suggested but unproved. ACKNOWLEDGEMENTS I wish to thank J. A. Aylwin, W. P. Cleland and Sir Thomas H. Sellors for permission to report these cases° REFERENCES EISv.NST.4a~T,H. B. (1964). Dis. Chest, 46, 78. HAt,NON,J. W. G. (I964). Dis. Ghest, 45, xo7. WAONER,J. C., SL~OGS,C. A., & MARCHAND,P. (I96o). Brit. J. indust. Med., I7, 26o.
THE FOLDED LUNG BY A. BLESOVSKY* The Hospitals for Diseases of the Chest
MINOR degrees of folding of the lung are commonly found during decortication of the lung for empyema. The following cases illustrate unusually extensive lung folding due to a fibrous membrane on the costal surface of the visceral pleura of the lower lobe; surprisingly this fibrous reaction was not associated with adhesions between the parietal and visceral layers of the pleura. This condition has not been reported before. On the basis of the findings in the first patient, a correct diagnosis was made in the second and third patients before operation. Case H i s t o r i e s Case z
The patient was a 5i-year-old pipe-fitter admitted on 1.12.56. Dyspncea on exertion had been present for two years, and had become worse 6 months before admission. I n May i956 he suffered what was thought to be influenza, and a dull constant pain across the right lower chest had been present since then. A productive cough had been present for about a year. An industrial history was not obtained at that time, but contact with asbestos could have occurred as the patient was a pipe-fitter. He was a fit, well-built man with no abnormal physical signs in the chest. A rounded opacity in the right lower lobe was seen on the chest radiograph. The lesion was poorly seen on the lateral view. Bronchoscopy was normal, and malignant cells were not found in the sputum. A postero-lateral thoracotomy was performed. The pleura was free, but a thick membrane covered the posterior part of the right lower lobe and there were small plaques on the middle lobe and on the diaphragm. Healed tuberculous disease was present at the apex of the right upper lobe. The membrane was dissected off the lower lobe, which then looked and felt normal. The patient made a good recovery after operation and was discharged on 6.12.56. He was re-admitted on 7.1.57 with thrombophlebitis of the right leg and a small left pulmonary artery embolus. The specimen was made up of laminated, vascular fibrous tissue, with areas of collapsed pulmonary tissue present on the inner surface. * Present address: Department of Cardio-vascular Surgery, Presbyterian Medical Center, Clay and Webster Streets, San Francisco, California (Recelvedfor publication, November i965)
o0
BLESOVSKY
Case 2 The patient was a 33-year-old engineer. Annual chest radiographs were normal until November 1964, when changes were noted at both bases, particularly on the right. Early in 1964 the patient had noticed pains across the chest, without cough or fever. He had been a ship's engineer for IO years, during which time he had come into contact with asbestos from pipe-lagging. Physical signs were normal. The chest radiograph showed pleural shadowing at the right base and the lateral view showed radiating lines quite clearly (Fig. I (a) & (b)). The possibility that a peripheral carcinoma might be present was suggested by the opacities in the right lower lobe seen on the tomograms. The ESR was 27 mm./hour; the blood was otherwise normal. In the differential diagnosis three possibilities were considered: i. Peripheral carcinoma, suggested by the lobulated shadow on the tomograms; ~. in view of the history of contact with asbestos and the peripheral nature of the lesion, mesothelioma of the pleura was considered, but thought to be unlikely because of the absence of effusion and because of the appearance of the lesion on radiograph; 3. the most likely diagnosis was thought to be a fibrous pleural reaction because of the radiographic appearance: a flat, very peripheral lesion with radiating streaks and a small lower lobe. The changes at the left lower lobe were considered to be minor degrees of the same process. Changes similar to those found in the first case were anticipated before operation. A postero-lateral thoracotomy was performed. The pleura was free except for a few adhesions from the lung to the diaphragm. The right lower lobe was covered by a membrane up to i ram. thick. The oblique and transverse fissures were free. Hyaline plaques were present on the diaphragm. As the membrane was being stripped off the lower lobe the folds in the lung created by the fibrous tissue were demonstrated. Once freed, the lobe re-expanded easily and looked and felt normal. Histology of the membrane revealed dense fibrous tissue showing non-specific inflammatory changes. Cas8 3 The patient was a 63-year-old labourer at Tate and Lyle's sugar refinery. At work he had to pass through a room where asbestos pipe-lagging was carried out. A mass miniature radiograph early in 1964 led to further investigation, when progressive changes at the left base of the radiographs were noted. He remained free of symptoms. The radiographs showed shadowing at the left lower lobe which increased between J a n u a r y 1964 and March 1965 (Fig. 3 (a), (b), & (c)). A folded lung was clearly demonstrated in tomograms (Fig. 4)- Bronchograms showed sweeping of the terminal bronchi of the left lower lobe, with no obstruction. Blood examination was normal. Ventilatory function tests were normal. Bronchoscopy was normal, and the sputum was free of neoplastic cells.
PLATE III
FIo. I ( a ) . - - I l l - d e f i n e d shadowing at right base. L i n e a r shadows at left base.
(a)
FIO. I ( b ) . - - L a t e r a l view shows clearly the site of the lesion at the periphery of the lower lobe, a n d the well-marked striations.
(b)
FIG. e . - - T o m o g r a m s : cuts Io a n d I e, same case as Fig. i. Smallness of lower lobe, indicated by site of the oblique fissure a n d r a d i a t i n g strands of fibrous tissue are demonstrated.
2**
To faae p. 2o.
PLATE IV
(a)
(b)
FIG. 3(a) AND (b).--Increasing shadowing at left lower lobe between January 1964 and March 1965 .
FIo. 3 (c).--Lateral view shows peripheral site of lesion. Position of fissure indicates smallness of lower lobe. In this view the lesion shows up less clearly than anticipated from the well-marked changes in the postero-anterior view.
PLATE V
(a), Cut 5
(c), Cut 7
(b), Cut 6
(d), Cut 8
Fro. 4 . - - S a m e case as Fig. 3. Tomograms: cuts 5 to 8. Apparently cavitating lesion in cut 5 and 6, but more medial cuts show that there is no wall inferiorly where the " c a v i t y " is in continuity with the lung tissue. Cut 8 shows clearly that the base of the lung is folded upwards and is separated from the apical lower segment by a thick crescent of tissue.
P L A T E VI
Fro. 5 . - - S a m e case as Fig. 3. Bronchogram: J a n u a r y i965. Note: (i)small left lower lobe; (ii) swept appearance of termination of basal bronchi indicating previous pleurisy; (iii) inferior subsegment of apical lower bronchus crossing posterior basal bronchus; (iv) termination of posterior basal bronchus almost in contact with termination of superior subsegment of apical lower bronchus.
(a)
(b)
FIG. 6 . ~ R i g h t lung of a patient with carcinoma of the middle lobe : early folding as an incidental finding. (a) Surface view: thick smooth m e m b r a n e over right lower lobe, lower margin folded upwards. (b) Cut section : carcinoma in middle lobe. Thick m e m b r a n e overlying infarct in posterior basal segment. To face p. ~I.
T H E FOLDED L U N G
2I
The peripheral site of the lesion, the progressing changes, and the history of contact with asbestos suggested a pleural mesothelioma; but once the tomograms were obtained a firm diagnosis of "folded lung" was made. At operation the pleura was found to be free except for a few adhesions to the diaphragm. Hyaline plaques were present on the parietal pleura in the paravertebral gutter opposite the lesion on the left lower lobe and on the pericardium just above the diaphragm. The left upper lobe was large and filled two-thirds of the left chest. Scattered nodules of healed tuberculosis were palpable in the left upper lobe. The left lower lobe was small and its costal surface covered by a membrane up to 3 mm. thick. The lobe was considerably folded, with the margin between the costal and diaphragmatic surface almost at the apex of the lower lobe. In addition to the infero-superior fold there was a lesser degree of antero-posterior folding. The membrane was cedematous in parts, suggesting continued inflammatory activity. The oblique fissure was free of adhesions. As the membrane was stripped off, the lung unfolded and re-expanded but remained smaller than normal. It looked and felt otherwise normal. Histology of the membrane showed it to be made up of thick strands of fibrous tissue with non-specific inflammatory changes.
Discussion Folded Lung is very uncommon, and I have been unable to find any reference to it in the literature. The diagnosis can be made from the radiograph: the lesion is peripheral, it is laminar, and there are radiations from it. In the more advanced case, as in the third patient, the fold of the lung can be seen. Though the findings at operation varied in severity they were strikingly similar in each case: there were no pleural adhesions at the site of the lesion, a thick membrane enfolded an otherwise apparently normal lower lobe and there were hyaline plaques on the parietal pleura. In the third patient the oedema of parts of the membrane and the progressing changes on the X-ray suggested that the lesion was active, but we were unable to find a causative agent.
./Etiology The cause of the condition is unknown, but may be related to infection, infarction or asbestos. Infection and infarction as a cause can only be conjectured, because the underlying lung at operation was normal: if these conditions were responsible it must be assumed that the pulmonary lesion had resolved completely. There is circumstantial evidence that the lesion is a form of asbestosis, because contact with asbestos was certain in two of the patients and probable in the third, and because hyaline plaques, commonly associated with asbestosis (Harmon, 1964) were present in all three. Benign pleurisy due to asbestos has been described (Eisenstadt, I964) , but this was characterized by dense pleural
22
BLESOVSKY
adhesions, a feature strikingly absent in the present cases. If the "folded l u n g " is due to asbestosis, it is a reaction not previously described in that condition. Pathogenesis The absence of adhesions at the site of the membrane over the lower lobe is unusual, but can be explained as follows. Occasionally at thoracotomy for mitral stenosis infarcts are found in the lower lobe unassociated with adhesions but associated with a small pleural effusion. If the primary lesion in the lung causes an effusion, this will keep the affected lobe away from the chest wall; during this time fibrin is deposited on the lower lobe, and by the time the effusion absorbs the membrane on the lower lobe has become organized and smooth and will not adhere to the parietal pleura. As the membrane organizes it contracts and causes the lung to fold. That this is one way in which the membrane forms is illustrated by the case of a patient with carcinoma of the middle lobe. At thoracotomy there was a small effusion associated with an infarct in the lower lobe. There were no adhesions, and the lower lobe was covered b y a thick, shiny membrane. The lower margin of the lobe was beginning to fold upwards (Fig. 6). Though a correct diagnosis is possible from the radiographic appearances, because of the rarity of the condition a thoracotomy is advisable to confirm the diagnosis. Removal of the membrane allows the lobe to re-expand, but it is doubtful if any improvement in lung function results.
Summary Unusual degree of folding of the lung is described in three cases. Diagnosis was made in the second and third patients before operation. The pathogenesis is discussed, and the possibility that these three cases were related to asbestos is suggested but unproved. ACKNOWLEDGEMENTS I wish to thank J. A. Aylwin, W. P. Cleland and Sir Thomas H. Sellors for permission to report these cases° REFERENCES EISv.NST.4a~T,H. B. (1964). Dis. Chest, 46, 78. HAt,NON,J. W. G. (I964). Dis. Ghest, 45, xo7. WAONER,J. C., SL~OGS,C. A., & MARCHAND,P. (I96o). Brit. J. indust. Med., I7, 26o.