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Case Ninety Nine - Bronchiectasis

(Figs. 1a and 1b)

(Figs. 2a and 2b)

Click on Images for Enlarged View

Clinical History:

Patient #1 is an 81 year old woman with persistent cough and hemoptysis.
Patient #2 is a 60 year old female with cough and hemoptysis. Patient states a history of a pneumonia and empyema within the right lung as a child.

Findings:

Patient 1: There is diffuse, predominantly central dilation of the bronchi, consistent with bronchiectasis (Fig 1a ) There is marked enlargement of the trachea and central bronchi (Fig 1b).

Patient 2: There is marked cystic bronchiectasis involving the right middle and right lower lobe (Fig 2a). The left lung is clear of bronchiectatic change.
There is subtle loss of volume in the right lung, with right sided deviation of the anterior junction line. There is subtle hyperlucency of the right lower lobe (Fig 2b.) The right lower lobe pulmonary small is small.

Diagnosis:

Patient 1: Central bronchiectasis, secondary to tracheobronchmegaly (Mounier Kuhn).

Patient 2: Right lung cystic bronchiectasis, c/w Swyer-James syndrome.

Discussion: Bronchiectasis is defined by abnormal dilation of the bronchial tree. This was fist described by Laennec in 1801, who suggested that bronchial dilation was secondary to accumulation and stagnation of endobronchial secretions. Bronchiectasis is defined by cylindrical, varicose or cystic types, and corresponds to the degree of severity of the bronchiectasis.

The distribution of bronchiectasis can help suggest its etiology. Central bronchiectasis is caused by cystic fibrosis, ABPA, Williams Campbell, or congenital tracheobronchomegaly, otherwise termed Mounier Kuhn. Upper lobe bronchiectasis is seen in patients with cystic fibrosis or TB, while lower lobe bronchiectasis may be secondary to childhood infection, aspirations, or immunodeficiency.

These two cases represent unusual causes of bronchiectasis. The first case is an example of Mounier Kuhn, in which the bronchiectasis is accompanied by tracheobronchomegaly. This abnormality may extend to the larynx, and is characterized by lack of the cartilaginous and membranous cartilage. The second case is a case of Swyer-James, in which a unilateral bronchiectasis is associated with a small, hyperlucent lung and a small pulmonary artery. While in most cases this abnormality most often accompanies an early viral pneumonia, this patient’s history of early childhood bacterial pneumonia could produce the same result.

References:
Aronchick JM and Miller WT. Journal of Thoracic Imaging 19954;(10):255-267.

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R.C.Gilkeson, M.D.