Ann Thorac Surg 2007;84:290-292
© 2007 The Society of Thoracic Surgeons
Case Reports
Surgical Management of Pneumothorax in Pulmonary Alveolar Microlithiasis
Timothy J.P. Batchelor, FRCS(CTh)*,
Dumbor L. Ngaage, FRCS(CTh),
Damian V. McGivern, DM, FRCP,
Michael E. Cowen, FRCS
Department of Cardiothoracic Surgery, Castle Hill Hospital, Cottingham, Hull, United Kingdom
Accepted for publication February 5, 2007.
* Address correspondence to Mr Batchelor, Department of Cardiothoracic Surgery, Castle Hill Hospital, Castle Rd, Cottingham, Hull, HU16 5JQ, United Kingdom (Email: tim.batchelor{at}virgin.net).
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Abstract
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Little is known about pulmonary alveolar microlithiasis, a disease characterized by severe lung calcification. We report only the second case of an associated spontaneous pneumothorax. Bullous disease, which was encountered at the time of surgery, proved impossible to manage using a video-assisted approach. The difficulties related to its management are discussed in this case report.
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Introduction
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Pulmonary alveolar microlithiasis (PAM) is a rare disease. It is characterized by multiple calcium phosphate deposits within the alveolar air spaces known as microliths. Although there is little information on the natural history of PAM, it seems as though disease progression is variable. Even though the condition may remain static in some cases, others may develop progressive lung fibrosis and end-stage respiratory failure [1]. We report only the second case of an associated pneumothorax, and discuss the difficulties related to the surgical treatment of bullous disease in the presence of calcified lungs.
A 45-year-old man presented with a 1-week history of increasing shortness of breath, left-sided pleuritic chest pain and a cough. A chest roentgenogram revealed the typical "sandstorm" appearance of the lungs seen in PAM. In addition, there was a left-sided pneumothorax (Fig 1). The patient had initially been diagnosed with PAM after a lung biopsy as a child. His history included two other ipsilateral pneumothoraces, the last episode occurring 14 years previously. Both episodes were managed with needle aspiration. He also suffered from temporal lobe epilepsy, mild learning difficulties, and asthma. Prior to this admission, his latest respiratory function tests had demonstrated a forced expiratory volume in 1 second of 2.4 L (70% of predicted) with a forced vital capacity of 3.3 L. These had deteriorated during a 12-year period from 3.0 L and 3.95 L, respectively.
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Fig 1. Chest roentgenogram of a left-sided pneumothorax in the presence of pulmonary alveolar microlithiasis. An apical bullous complex is present (arrows) and the trachea is deviated to the contralateral side. The lung fields demonstrate the characteristic "sandstorm" appearance, most marked in the middle and lower zones.
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During the initial management, a size 12-French intercostal drain was inserted percutaneously. However the lung failed to re-expand and an air leak persisted. Therefore the patient was referred for surgical management. At the time of surgery, a video-assisted approach was initially used. The left lung was found to be calcified and noncompliant, and an apical bullous complex was identified (Fig 2). Endoscopic stapling was attempted on three occasions, each time resulting in a broken instrument. Therefore a posterolateral thoracotomy was performed. The apex of the lung was amputated and the lung was repaired with interrupted number 1 polypropylene sutures buttressed with teflon felt. This was complimented by an apical pleurectomy and talc pleurodesis. The patient made an uneventful recovery. At follow-up in the clinic 6 weeks later, a routine chest roentgenogram confirmed full re-expansion of the left lung (Fig 3).
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Fig 2. Apex of the left lung after amputation at the time of thoracotomy. The apical bullous complex is clearly seen.
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Comment
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In the last review of the world literature in 2004, 576 cases of PAM had been reported [2]. However, we found only one previous case of an associated pneumothorax that has been described [3], and at the time of thoracoscopy there were multiple emphysematous bullae found. In our case, an apical bullous complex was encountered that proved to be impossible to excise using conventional endoscopic stapling devices.
There appears to be both a sporadic and a familial form of the disease [1]. The gene responsible has only recently been described and is involved in phosphate metabolism [4]. Inheritance is autosomal recessive. Absence of the protein encoded by the gene results in calcium phosphate deposition within the alveoli. The same mutation may also be responsible for some cases of the more common disease testicular microlithiasis. Treatment options are limited. Systemic therapy with corticosteroids has proven to be ineffective, as has bronchoalveolar lavage. Lung transplantation would seem to be the only effective choice in end-stage disease [1], although not all patients may progress to this point.
A spontaneous pneumothorax in the presence of PAM is unlikely to resolve with conservative management due to the severe lung calcification. Although a video-assisted thoracoscopic approach may be useful in the initial assessment at the time of surgery, attempted resection of bullae using the relatively delicate endoscopic stapling devices available may prove futile. We recommend thoracotomy with excision of the affected areas. The subsequent lung defects should be oversewn and buttressed.
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References
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- Castellana G, Lamorgese V. Pulmonary alveolar microlithiasis Respiration 2003;70:549-555.[Medline]
- Mariotta S, Ricci A, Papale M, et al. Pulmonary alveolar microlithiasis: report on 576 cases published in the literature Sarcoidosis Vasc Diffuse Lung Dis 2004;21:173-181.[Medline]
- Shishido S, Toritani T, Nakano H, Tokushima T. A case of alveolar microlithiasis which developed spontaneous pneumothorax due to progression of emphysematous bullae during 34 years after established diagnosis Nihon Kyobu Shikkan Gakkai Zasshi 1993;31:881-885in Japanese.[Medline]
- Corut A, Senyigit A, Ugur SA, et al. Mutations in SLC34A2 cause pulmonary alveolar microlithiasis and are possibly associated with testicular microlithiasis Am J Hum Genet 2006;79:650-656.[Medline]
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Abstract
Introduction
