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Evaluation and medical management of giant bullae

Author
Fernando J Martinez, MD, MS
Section Editor
James K Stoller, MD, MS
Deputy Editor
Helen Hollingsworth, MD

INTRODUCTION

A bulla is defined as an air space in the lung measuring more than one centimeter in diameter in the distended state. The term giant bulla is used for bullae that occupy at least 30 percent of a hemithorax [1-4]. A single giant bulla may be present, or a giant bulla may be accompanied by a number of smaller adjacent bullae.

The evaluation and management of giant bullae in patients with emphysema will be reviewed here. The general management of chronic obstructive pulmonary disease and the roles of bullectomy, lung volume reduction surgery, and lung transplantation in the management of advanced emphysema are discussed separately. (See "Management of stable chronic obstructive pulmonary disease" and "Bullectomy for giant bullae" and "Lung volume reduction surgery in COPD" and "Lung transplantation: General guidelines for recipient selection".)

ETIOLOGY

Giant bullae typically develop as a consequence of cigarette smoking, although some are idiopathic and others have less common associations. Marijuana smoking has been associated with giant bullae in a few patients, although this observation may be confounded by concomitant cigarette smoke exposure [5].

Bullous lung disease has also been described in association with HIV infection [6-8] and in intravenous drug users, particularly following injection of methadone, methylphenidate, or talc-containing drugs, although these relationships are not clearly established. In methylphenidate injectors, the pattern of emphysema is basilar, similar to that seen in patients with alpha-1 antitrypsin deficiency. (See "Overview of pulmonary disease in injection drug users", section on 'Bullous lung disease and emphysema'.)

Patients with Marfan's syndrome occasionally develop apical bullae, but these generally do not meet criteria for giant bullae. Similarly, bullae have been described in Ehlers-Danlos type IV, polyangiitis with granulomatosis, Sjögren's syndrome and sarcoidosis, but usually not giant bullae [9-12]. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders".)

                    

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Literature review current through: Nov 2016. | This topic last updated: Fri Oct 30 00:00:00 GMT+00:00 2015.
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