Bronchiectasis and Marfan's syndrome

Abstract

Marfan's syndrome is a rare hereditary disorder characterized by skeletal, cardiovascular and ocular abnormalities. Pulmonary abnormalities occur in approximately 10%. of patients, the commonest being spontaneous pneumothorax and emphysema. A patient is described who had Marfan's syndrome and bronchiectasis, an association only described on 2 previous occasions in the literature. A 39-year-old factory worker presented with a history of persistent cough and purulent sputum since childhood. He was a non-smoker with no relevant family history. He had marked clubbing of fingers and toes. Examination of the chest revealed bilateral basal crepitations. Chest radiography demonstrated extensive cystic bronchiectasis of the left lower lobe. This was confirmed by bilateral bronchography (Fig. 1) which also showed minor changes on the right side. The patient was tall and thin with a depressed sternum and high arched plate. Examination of the cardiovascular systems was normal whilst ECG showed right axis deviation. Ophthalmoscopy showed no ectopia lentis. Metacarpal index of 9 2 was in the range for Marfan's syndrome (Fig. 2). The patient underwent left lower lobectomy. There was gross bronchiectasis with numerous cystic cavities, confirmed on histological examination. The patient made an uneventful post operative recovery. © 1980 The Fellowship of Postgraduate Medicine.