Ann Thorac Surg 2005;80:342-344
© 2005 The Society of Thoracic Surgeons
Case report
Lung Volume Reduction Surgery for a Professional Athlete With Swyer-James Syndrome
Akira Tasaki, MD,
Ryoichi Nakanishi, MD, PhD*
Department of Thoracic Surgery, Shin-Kokura Hospital, Federation of National Public Service Personnel Mutual Aid Associations, Kitakyushu, Japan
Accepted for publication December 10, 2003.
* Address reprint requests to Dr Nakanishi, Department of Thoracic Surgery, Shin-Kokura Hospital, Federation of National Public Service Personnel Mutual Aid Associations, 1-3-1 Kanada, Kokurakita-ku, Kitakyushu 803-8505, Japan (Email: ryoichi{at}med.uoeh-u.ac.jp).
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Abstract
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Swyer-James syndrome is an uncommon, emphysematous disease characterized by roentgenographic hyperlucency of one lung or lobe due to loss of the pulmonary vascular structure and to alveolar overdistension. We here describe a 28-year-old professional bicycle racer with Swyer-James syndrome who had dyspnea on exertion, especially during a bicycle race. Lung volume reduction surgery was performed with video-assisted thoracoscopic surgery. This treatment improved his pulmonary function, and he is active as a bicycle racer now.
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Introduction
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Swyer-James syndrome was first described in 1953 as unilateral pulmonary emphysema in a 6-year old child [1]. In 1954, MacLeod [2] reported 9 cases of one lung transradiancy and analyzed clinical, radiologic, and bronchographic features of the disease in detail. Almost all the cases of Swyer-James syndrome were followed by conservative therapy, and surgical treatment was rarely performed. We report a patient with Swyer-James syndrome who underwent video-assisted thoracoscopic lung volume reduction surgery (VAT-LVRS) with progressive improvement of subjective symptoms and pulmonary function.
A 28-year-old man who is a "Keirin" professional bicycle racer was referred to our department for evaluation of dyspnea on exertion. He was asymptomatic in daily life, but he suffered from shortness of breath when he exerted his full energy to his last spurt, dashing toward the finish line. No matter how hard he tried to build up his strength, he could not improve his respiratory function. He was admitted to our department for further evaluation and medical treatment of his persistent respiratory distress on exertion.
On admission, pulmonary function test showed a mild restrictive pattern (Table 1). Chest radiographs showed overinflation of the left lung (Fig 1) and computed tomography scan revealed emphysematous alternation (Fig 2). Perfusion scan demonstrated the decreased perfusion in the damaged lung (Fig 3). These clinical and radiologic findings suggested the diagnosis of Swyer-James syndrome. In order to improve his respiratory function and to relieve his symptoms, surgical treatment was considered after informed consent was obtained.
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Fig 1. Chest radiograph at admission, showing hyperlucency of the left lower lung field, rightward shift of the mediastinum, and flattened diaphragm.
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Fig 2. Computed tomography scan at admission, showing hyperlucency of the left lower lobe with decreased bronchovascular markings.
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Fig 3. Radionuclide perfusion scan showing decreased pulmonary blood flow in the left lower lung field.
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Under general anesthesia using one-lung ventilation, a VAT-LVRS was carried out through four access ports that were 5.5-, 5.5-, 12-, and 12-mm in diameter. The emphysematous change was mainly located in S8 and S9 in the left lower lobe. The affected site was resected using four stapling devices with absorbable polyglycolic acid felt (Neoveil; Gunze Corp, Kyoto, Japan). Histopathologic findings of the surgical specimen showed emphysematous pulmonary tissue with dilated bronchioles and cystic dilatation of alveoli and constrictive bronchiolitis (Fig 4).
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Fig 4. Pathologic findings showing constrictive bronchiolitis, suggested by the feature of bronchioles surrounded by fibrosis (hematoxylin & eosin staining; magnification x200).
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The postoperative recovery was uneventful. He recovered well enough to be discharged on postoperative day 5 and remained in the hospital for the purpose of strict implementation of pulmonary rehabilitation. He was discharged on postoperative day 27 after the rehabilitation program was completely finished. He has been followed up regularly since discharge. One year after the operation, he had progressive improvement in respiratory function (Table 1) and became completely asymptomatic even when he raced. Twenty-eight months after the operation, he is still active as a professional bicycle racer, and he can exert his full energy to his last spurt without the shortness of breath he felt previously.
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Comment
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Swyer-James syndrome is morphologically characterized by the presence of constrictive bronchiolitis with dilatation and destruction of alveolar structures, resulting in significant air trapping and lung hypoperfusion [3]. Although classically involving an entire lung, the disorder can be lobar or segmental. The main pathogenetic event seems to be acute bronchiolitis with obliteration of small airway in infancy. In the present case, histopathologic examination revealed pulmonary emphysema and constrictive bronchiolitis. Those findings were compatible with Swyer-James syndrome.
Patients with this syndrome are usually treated conservatively, but surgical treatment should be considered when infections can not be controlled [4]. There are few reports on surgical treatment. Most patients underwent pneumonectomy, and some were treated with lobectomy [5] or segmentectomy [6]. The patient in the present case had no symptoms in daily life and no history of chronic or recurrent infection of the affected lung. He suffered from shortness of breath when he exerted his full energy to his last spurt, however, and his pulmonary function was slightly disturbed. The affected site potentially causes a shunt effect during effort. We considered that these findings were due to emphysematous alternation of his left lung and conducted unilateral LVRS.
Lung volume reduction surgery is predicated upon the principle that a reduction in lung volume can favorably affect the mechanics of diaphragm, chest wall, and airways. It is generally believed that LVRS is a therapeutic option for patients with severe emphysema after all conservative measures have been exhausted. Lung volume reduction surgery should theoretically improve lung function irrespective of the type of disease causing hyperinflation [7]. Bloch and coworkers [8] reported a case of LVRS successfully performed in a 14-year-old boy with disabling airflow obstruction and hyperinflation secondary to postinfectious bronchiolitis. Hence, patients with irreversible pulmonary hyperinflation of etiology other than pulmonary emphysema may profit by LVRS as well. We also concluded that the LVRS treatment could improve not only our patient’s pulmonary function but also his quality of life as an athlete. The operative procedure was performed with a video-assisted thoracic surgerytechnique that is less invasive than conventional thoracotomy.
We successfully performed VAT-LVRS in this patient. He had progressive improvement in respiratory function and became completely asymptomatic during a bicycle race. We believe that VAT-LVRS can be used for managing Swyer-James syndrome. It is effective for improving the pulmonary function and clinical symptoms of patients with this disease.
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Acknowledgments
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The authors would like to thank Hiroyuki Miyazaki, MD, for pathology evaluation and Masataka Irie, PT, for physical therapy support.
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References
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- Swyer PR, James GCW. A case of unilateral pulmonary emphysema Thorax 1953;8:133-136.
- MacLeod WM. Abnormal transradiancy of one lung Thorax 1954;9:147-153.
- Fregonese L, Girosi D, Battistini E, et al. Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with Macleod/Swyer-James syndrome and bronchiectasis Pediatr Pulmonol 2002;34:412-416.[Medline]
- Vishnevsky AA, Nikoladze GD. New approach to the surgical treatment of Swyer-James-Macleod syndrome Ann Thorac Surg 1990;50:103-104.[Abstract]
- Morita K, Shimizu J, Kamesui T, Watanabe Y, Nonomura A, Kita K. A case of surgical treatment of Swyer-James syndrome [English abstract] J Jpn Assoc Thorac Surg 1994;42:1949-1952.
- Ohri SK, Rutty G, Fountain SW. Acquired segmental emphysemathe enlarging spectrum of Swyer-James/Macleod’s syndrome. Ann Thorac Surg 1993;56:120-124.[Abstract]
- Fessler HE, Permutt S. Lung volume reduction surgery and airflow limitation Am J Respir Crit Care Med 1998;157:715-722.[Abstract/Free Full Text]
- Bloch KE, Weder W, Boehler A, Zalunardo MP, Russi EW. Successful lung volume reduction surgery in a child with severe airflow obstruction and hyperinflation due to constrictive bronchiolitis Chest 2002;122:747-750.[Abstract/Free Full Text]
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N. Barbetakis, D. Paliouras, C. Asteriou, and G. Samanidis
eComment: Diagnostic and surgical considerations in Swyer-James-MacLeod syndrome
Interactive CardioVascular and Thoracic Surgery,
April 1, 2009;
8(4):
484 - 484.
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Abstract
Introduction
