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Findings: Chest x-ray reveals extensive bullous changes, predominantly in the apices. Increased density is identified in more normal lung which is compressed secondary to the expansive bullae. Corresponding CT images (Figures 2 and 3 above) again demonstrate bilateral hyperinflation with multiple bullae. Compression of more lung is again noted. Figure 4 is a chest x-ray of a different patient demonstrating an air-fluid level representing an infection in a large bulla.
Pathology: Due to worsening dyspnea, patient underwent right-sided bullectomy. Pathology demonstrated bullae with normal surrounding lung tissue.
Diagnosis: Primary bullous lung disease.
Discussion: Bullae are air-filled, thin-walled spaces in the lung resulting from destruction of alveolar tissue. They have a tendency to enlarge over time. Primary bullous disease is seen without evidence of emphysema or interstitial lung disease. It may be familial or in association with Marfan’s Syndrome, Ehlers-Danlos Syndrome, IVDA, neurofibromatosis, or HIV disease. It may also be seen in Vanishing Lung Syndrome.
It is often asymptomatic unless large bullae result in dyspnea. Other complications include spontaneous pneumothorax or bullae that develop secondary infection or hemorrhage. Figure 4 (above) is a different patient with an air-fluid level in a large bullae representing an infectious process. Symptomatic patients may require bullectomy procedures.
Differential diagnosis might include secondary bullous disease due to COPD or interstitial pulmonary fibrosis. Alpha 1 - antitrypsin deficiency results in emphysema predominantly involving the lower lobes.
References:
Fraser GF, Paré, JAP, Paré PD, Fraser RS and Genereaux GP:
Diagnosis of Diseases of the Chest, 3rd Edition. WB Saunders, 1990.
Brant WE and Helms CA: Fundamentals of Diagnostic Radiology, Williams & Wilkins, 1994.
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