bullous lung disease

Etiology

• small apicial bullae may be present in the lung apex of normal individuals
• congenital
  • Marfan's syndrome
  • Ehlers-Danlos syndrome
• acquired
  • burnt out sarcoidosis
  • cadmium exposure
• panlobular emphysema may appear like bullae
• bullae may occur spontaneously or, more frequently, in the setting of obstructive lung disease (pulmonary emphysema)

Pathology

• lack of air communication with bronchi may cause air trapping

Clinical manifestations

• chronic dyspnea without cough

Diagnostic procedures

• pulmonary function testing
  • obstructive lung disease
    • no response to bronchodilator

Radiology

• chest X-ray
  • sharply demarcated air-containing spaces within the lung, surrounded by a smooth wall (bullae)
    • hyperlucent spaces, & hairline shadows often form their borders & distinguish them from surrounding lung
  • bullae are not always apparent on plain chest radiographs
    • absence of lung markings in a lung zone suggests a bulla
  • hyperinflation, flattened diaphragm, & an increased retrosternal air space suggests bullous lung disease in the setting of pulmonary emphysema

Complications

• pneumothorax
• COPD
• lung abscess
• hemorrhage into bulla
• compression of adjacent normal lung

Differential diagnosis

• bronchiectasis
• pulmonary emphysema

Management

• surgery may improve lung function by 5-10% in 10-15% of patients

More general terms

• lung disease

More specific terms

• congenital cystic adenomatoid malformation; congenital pulmonary airway malformation
• giant bullous emphysema; Vanishing lung syndrome

Additional terms

• bulla; bleb
• Ehlers-Danlos syndrome; cutis elastica (EDS)
• emphysema
• Marfan syndrome
• sarcoidosis