Hospital Practice
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
A Patient with a Chronic Cough and One Hyperlucent Lung Patrick P. Litam & Robert Lenox To cite this article: Patrick P. Litam & Robert Lenox (1992) A Patient with a Chronic Cough and One Hyperlucent Lung, Hospital Practice, 27:3, 129-132, DOI: 10.1080/21548331.1992.11705384 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705384
Published online: 17 May 2016.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [RMIT University Library]
Date: 18 June 2016, At: 12:42
The Problem Patient
A Patient with a Chronic Cough and One Hyperlucent Lung PATRICK P. LITAM and ROBERT LENOX St. Elizabeth Hospital Medtcal Center, Youngstown, Ohto, and Guthrte Cltntc. Sayre, Pa.
Hospital Practice 1992.27:129-132.
Case Presentation A 43-year-old man was first seen in the pulmonary clinic for chronic cough and recurrent respiratory infections with Hemophilus trifluenzae and, more recently, Pseudomonas aerugtnosa. At that time, he was admitted for parenteral antibiotic therapy after failing to respond to oral quinolone treatment. His medical problem started with a near-fatal bout of Asian flu at the age of 12. After that. he had wheezing and dyspnea on exertion. He also had a cough productive of 7 to 8 tablespoons of white to yellow sputum. At the age of21, he was hospitalized for hemoptysis. Chest roentgenography showed decreased vascularity in the left lung field. Bronchoscopy was normal. Bronchography, however, showed bilateral bronchiectasis, with the left side worse than the right. A lung scan showed little or no circulation to the left lung. Since then, the patient continued to have chronic cough with repeated pulmonary infections, hemoptysis, and decreased exercise tolerance. When he presented to us, a pulmonary function test revealed moderate airflow obstruction (FEV /FVC ratio of 53% of predicted value) with borderline restrictive lung disease. On physical examination, de-
creased breath sounds were heard over the left lung field, as well as bilateral expiratory wheezes and coarse rales at the bases. Laboratory studies (including CBC with differential. urinalysis, and SMA 20) were normal. Blood cultures were
negative. Arterial blood gases on room air were Po 2 73 mm Hg and Pco2 32 mm Hg; and the pH was 7.46. Plethoric appearance of the right pulmonary vasculture was seen on a chest roentgenogram (Figure 1). There was a reduction in size and branches
Figure 1. In a 43-year-old man with a chronic cough and recurrent respiratory infections, a chest x-ray showed plethoric right-sided pulmonary vascularity. In addition, vessels in the left lung were reduced in size and number, and the left lung was mildly hyperventilated and hyperlucent. Hospital Practice March 15. 1992
129
of vessels within the left lung. with mild hyperinflation and hyperlucency. Peribronchial thickening, involving the right lower lobe and the lingula of the left base, was compatible with bronchiectasis.
A ventilation scan with xenon133 showed a significant reduction in left lung ventilation, with trapping of air. The rightlung ventilation was inhomogeneous. A technetium-99m-labeled macroaggregated albumin scan re-
vealed asymmetrically decreased perfusion of the entire left lung. The right lung showed segmental and subsegmental defects involving the middle lobe, posterior base of the right lower lobe, and posterior segment of the right upper lobe. ACT scan of the chest showed generalized diminished attenuation of the left lung, with cylindrical and circular lucencies compatible with bronchiectasis involving both lungs (Figure 2). The patient had a prompt resolution of his respiratory infection after administration of parenteral antibiotics.
Hospital Practice 1992.27:129-132.
The Case in Context
Figure 2. Thoracic CT scans showed diminished attenuation of vasculature in the patient's left lung (above, arrow), and cylindrical or circular lucencies in both lungs that were compatible with bronchiectasis (below, arrows).
Swyer-James syndrome was first described in 1953 in a nineyear-old boy who had recurrent bronchitis associated with unilateral hyper lucent lung, small ipsilateral hemithorax, and angtographically proven small ipsilateral pulmonary artery and bronchiectasis in the involved lung. A year later, W. M. Macleod reported similar conditions in adults. The pathologic process in Swyer-James syndrome is usually that of bronchiolitis obliterans, defined by the presence of granulomatous tissue plugs within the lumen of the small airway, and occasionally alveolar ducts with obliterative scarring. The affected region usually shows panacinar emphysema distal to the obstruction. Most patients remain asymptomatic until the underlying pathology is exacerbated by an acute respiratory illness. Some patients, however, become symptomatic-with chronic cough. decreased exercise tolerance, and repeated episodes of bacterial infection with bronchiectasis-as did our patient. Although the etiology is not (continues)
130
Hospital Practice March 15. 1992
Hospital Practice 1992.27:129-132.
CHRONIC COUGH
(conttnued)
clear, there are two theories regarding this illness. One theory supports the view that the initial abnormality occurs in the peripheral bronchial tree following infection at an early age, with secondary hypoplasia of the pulmonary artery. Early in the pathologic process, the obliterated bronchial tissue produces a functional decrease in blood flow; subsequently, the compromised ventilation causes hypoplasia of the alveoli and of the pulmonary vasculature. The volume of affected lung is related to the age at which bronchiolar damage occurred-i.e., before the age of eight, by which time a full complement of alveoli has developed. If bronchiolitis obliterans occurs later in life. the lungs and ipsilateral hemithorax may remain normal in size, and the chance of acquired hypoplasia of the pulmonary artery may be slight. Another view holds that the presence of a primary unilateral pulmonary vascular abnormality predisposes to bronchial and alveolar changes. It is well known that the pulmonary artery is important in the proper
growth of the pulmonary parenchyma. Congenital hypoplasia of the artery could, therefore, cause the parenchymal change ofSwyer-James syndrome. The radiographic features of Swyer-James syndrome include the hallmark oflobar or unilateral hyper lucent lung with normal or reduced volume on inspiration, severe airway obstruction or air-trapping during expiration, and a greatly reduced peripheral and central pulmonary circulation. The syndrome has also been characterized on radionuclide imaging by diminished perfusion of the affected lung, with relatively normal distribution of the radioactive xenon at equilibrium and delayed xenon washout in a normal-sized or small hyperlucent lung. Computed tomography has also been used to visualize and characterize the pulmonary pathology. Although SwyerJames syndrome was originally described as a unilateral condition on the basis of plain chest radiographs, radionuclide imaging and computed tomography are capable of detecting bilateral lung involvement and have the advantage of being noninvasive, compared with bronchography.
Selected Reading Swyer PR. James GLW: A case of unilateral pulmonary emphysema. Thorax8: 133. 1953 Macleod WM: Abnormal translucency of one lung. Thorax 9: 14 7, 1954 Epler GR. Colby TV: The spectrum ofbronchlolltls obllterans. Chest 83:161,1983 Reid L, Simon G: Unilateral lung translucency. Thorax 17:230, 1962 Elder JC et al: Unilateral pulmonary artery absence of hypoplasia. Circulation 17:557,1958 Belcher JR. Pattinson JN: Hypoplasia of the lobar pulmonary arteries. J Thorac Cardlovasc Surg 34 : 35 7, 1957 O'Dell CW et al: Ventilation-perfusion lung Images In the Swyer-James syndrome. Radiology 121: 423, 1976 Martl-Bonmatu Let al: CT ftndtngs In Swyer-James syndrome. Radiology 172:477,1989
132
Hospital Practice March 15, 1992
In the differential diagnosis of Swyer-James syndrome, two entities should be ruled out radiographically: 1) an endobronchial lesion incompletely obstructing the lumen of a lobar bronchus and 2) pulmonary artery agenesis. The reduced distal parenchymal ventilation in the former results in local hypoxia, which leads to reflex vasoconstriction and reduced perfusion of the affected bronchopulmonary segment. Since this condition produces air-trapping on expiration, it must be excluded by bronchoscopy. In the latter entity, the pulmonary artery is interrupted in the region of the hilum so that the lung is devoid of pulmonary artery perfusion. Treatment of Swyer-James syndrome is usually conservative and often guided by the patient's symptomatology. It consists of chest percussion and postural drainage. antibiotic therapy for pulmonary bacterial infections, and sometimes surgical resection. The patient's bronchiectasis must be predominantly unilateral if surgery is to help. In our patient, clinical and radiographic follow-up showed relative stability of his symptoms. Moreover, with prompt antibiotic treatment of recurrent respiratory infections, his pulmonary function has not deteriorated. In this case, CT scanning documented all of the findings necessary to diagnose Swyer-James syndrome. D
When the patient was seen, Dr. Litam was an Intern at Robert Packer Hospital, Sayre, Pa. He is now a Senior Resident in Medicine, St. Elizabeth Hospital Medical Center, Youngstown, Ohio. Dr. Lenox is an Associate in the Pulmonary Section, Guthrie Clinic, Sayre.
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
A Patient with a Chronic Cough and One Hyperlucent Lung Patrick P. Litam & Robert Lenox To cite this article: Patrick P. Litam & Robert Lenox (1992) A Patient with a Chronic Cough and One Hyperlucent Lung, Hospital Practice, 27:3, 129-132, DOI: 10.1080/21548331.1992.11705384 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705384
Published online: 17 May 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [RMIT University Library]
Date: 18 June 2016, At: 12:42
The Problem Patient
A Patient with a Chronic Cough and One Hyperlucent Lung PATRICK P. LITAM and ROBERT LENOX St. Elizabeth Hospital Medtcal Center, Youngstown, Ohto, and Guthrte Cltntc. Sayre, Pa.
Hospital Practice 1992.27:129-132.
Case Presentation A 43-year-old man was first seen in the pulmonary clinic for chronic cough and recurrent respiratory infections with Hemophilus trifluenzae and, more recently, Pseudomonas aerugtnosa. At that time, he was admitted for parenteral antibiotic therapy after failing to respond to oral quinolone treatment. His medical problem started with a near-fatal bout of Asian flu at the age of 12. After that. he had wheezing and dyspnea on exertion. He also had a cough productive of 7 to 8 tablespoons of white to yellow sputum. At the age of21, he was hospitalized for hemoptysis. Chest roentgenography showed decreased vascularity in the left lung field. Bronchoscopy was normal. Bronchography, however, showed bilateral bronchiectasis, with the left side worse than the right. A lung scan showed little or no circulation to the left lung. Since then, the patient continued to have chronic cough with repeated pulmonary infections, hemoptysis, and decreased exercise tolerance. When he presented to us, a pulmonary function test revealed moderate airflow obstruction (FEV /FVC ratio of 53% of predicted value) with borderline restrictive lung disease. On physical examination, de-
creased breath sounds were heard over the left lung field, as well as bilateral expiratory wheezes and coarse rales at the bases. Laboratory studies (including CBC with differential. urinalysis, and SMA 20) were normal. Blood cultures were
negative. Arterial blood gases on room air were Po 2 73 mm Hg and Pco2 32 mm Hg; and the pH was 7.46. Plethoric appearance of the right pulmonary vasculture was seen on a chest roentgenogram (Figure 1). There was a reduction in size and branches
Figure 1. In a 43-year-old man with a chronic cough and recurrent respiratory infections, a chest x-ray showed plethoric right-sided pulmonary vascularity. In addition, vessels in the left lung were reduced in size and number, and the left lung was mildly hyperventilated and hyperlucent. Hospital Practice March 15. 1992
129
of vessels within the left lung. with mild hyperinflation and hyperlucency. Peribronchial thickening, involving the right lower lobe and the lingula of the left base, was compatible with bronchiectasis.
A ventilation scan with xenon133 showed a significant reduction in left lung ventilation, with trapping of air. The rightlung ventilation was inhomogeneous. A technetium-99m-labeled macroaggregated albumin scan re-
vealed asymmetrically decreased perfusion of the entire left lung. The right lung showed segmental and subsegmental defects involving the middle lobe, posterior base of the right lower lobe, and posterior segment of the right upper lobe. ACT scan of the chest showed generalized diminished attenuation of the left lung, with cylindrical and circular lucencies compatible with bronchiectasis involving both lungs (Figure 2). The patient had a prompt resolution of his respiratory infection after administration of parenteral antibiotics.
Hospital Practice 1992.27:129-132.
The Case in Context
Figure 2. Thoracic CT scans showed diminished attenuation of vasculature in the patient's left lung (above, arrow), and cylindrical or circular lucencies in both lungs that were compatible with bronchiectasis (below, arrows).
Swyer-James syndrome was first described in 1953 in a nineyear-old boy who had recurrent bronchitis associated with unilateral hyper lucent lung, small ipsilateral hemithorax, and angtographically proven small ipsilateral pulmonary artery and bronchiectasis in the involved lung. A year later, W. M. Macleod reported similar conditions in adults. The pathologic process in Swyer-James syndrome is usually that of bronchiolitis obliterans, defined by the presence of granulomatous tissue plugs within the lumen of the small airway, and occasionally alveolar ducts with obliterative scarring. The affected region usually shows panacinar emphysema distal to the obstruction. Most patients remain asymptomatic until the underlying pathology is exacerbated by an acute respiratory illness. Some patients, however, become symptomatic-with chronic cough. decreased exercise tolerance, and repeated episodes of bacterial infection with bronchiectasis-as did our patient. Although the etiology is not (continues)
130
Hospital Practice March 15. 1992
Hospital Practice 1992.27:129-132.
CHRONIC COUGH
(conttnued)
clear, there are two theories regarding this illness. One theory supports the view that the initial abnormality occurs in the peripheral bronchial tree following infection at an early age, with secondary hypoplasia of the pulmonary artery. Early in the pathologic process, the obliterated bronchial tissue produces a functional decrease in blood flow; subsequently, the compromised ventilation causes hypoplasia of the alveoli and of the pulmonary vasculature. The volume of affected lung is related to the age at which bronchiolar damage occurred-i.e., before the age of eight, by which time a full complement of alveoli has developed. If bronchiolitis obliterans occurs later in life. the lungs and ipsilateral hemithorax may remain normal in size, and the chance of acquired hypoplasia of the pulmonary artery may be slight. Another view holds that the presence of a primary unilateral pulmonary vascular abnormality predisposes to bronchial and alveolar changes. It is well known that the pulmonary artery is important in the proper
growth of the pulmonary parenchyma. Congenital hypoplasia of the artery could, therefore, cause the parenchymal change ofSwyer-James syndrome. The radiographic features of Swyer-James syndrome include the hallmark oflobar or unilateral hyper lucent lung with normal or reduced volume on inspiration, severe airway obstruction or air-trapping during expiration, and a greatly reduced peripheral and central pulmonary circulation. The syndrome has also been characterized on radionuclide imaging by diminished perfusion of the affected lung, with relatively normal distribution of the radioactive xenon at equilibrium and delayed xenon washout in a normal-sized or small hyperlucent lung. Computed tomography has also been used to visualize and characterize the pulmonary pathology. Although SwyerJames syndrome was originally described as a unilateral condition on the basis of plain chest radiographs, radionuclide imaging and computed tomography are capable of detecting bilateral lung involvement and have the advantage of being noninvasive, compared with bronchography.
Selected Reading Swyer PR. James GLW: A case of unilateral pulmonary emphysema. Thorax8: 133. 1953 Macleod WM: Abnormal translucency of one lung. Thorax 9: 14 7, 1954 Epler GR. Colby TV: The spectrum ofbronchlolltls obllterans. Chest 83:161,1983 Reid L, Simon G: Unilateral lung translucency. Thorax 17:230, 1962 Elder JC et al: Unilateral pulmonary artery absence of hypoplasia. Circulation 17:557,1958 Belcher JR. Pattinson JN: Hypoplasia of the lobar pulmonary arteries. J Thorac Cardlovasc Surg 34 : 35 7, 1957 O'Dell CW et al: Ventilation-perfusion lung Images In the Swyer-James syndrome. Radiology 121: 423, 1976 Martl-Bonmatu Let al: CT ftndtngs In Swyer-James syndrome. Radiology 172:477,1989
132
Hospital Practice March 15, 1992
In the differential diagnosis of Swyer-James syndrome, two entities should be ruled out radiographically: 1) an endobronchial lesion incompletely obstructing the lumen of a lobar bronchus and 2) pulmonary artery agenesis. The reduced distal parenchymal ventilation in the former results in local hypoxia, which leads to reflex vasoconstriction and reduced perfusion of the affected bronchopulmonary segment. Since this condition produces air-trapping on expiration, it must be excluded by bronchoscopy. In the latter entity, the pulmonary artery is interrupted in the region of the hilum so that the lung is devoid of pulmonary artery perfusion. Treatment of Swyer-James syndrome is usually conservative and often guided by the patient's symptomatology. It consists of chest percussion and postural drainage. antibiotic therapy for pulmonary bacterial infections, and sometimes surgical resection. The patient's bronchiectasis must be predominantly unilateral if surgery is to help. In our patient, clinical and radiographic follow-up showed relative stability of his symptoms. Moreover, with prompt antibiotic treatment of recurrent respiratory infections, his pulmonary function has not deteriorated. In this case, CT scanning documented all of the findings necessary to diagnose Swyer-James syndrome. D
When the patient was seen, Dr. Litam was an Intern at Robert Packer Hospital, Sayre, Pa. He is now a Senior Resident in Medicine, St. Elizabeth Hospital Medical Center, Youngstown, Ohio. Dr. Lenox is an Associate in the Pulmonary Section, Guthrie Clinic, Sayre.
