CT analysis of pulmonary emphysematous and fibrotic changes in patients with von Recklinghausen's disease

Abstract

Five cases of von Recklinghausen's disease were studied with regard to their association with chest diseases, especially pulmonary emphysematous and fibrotic changes. CT analysis revealed that four patients out of five had multiple pulmonary emphysematous lesions and that those lesions were seen in all segments. In two cases out of the four, it was difficult to detect their abnormalities by an ordinary chest roentgenogram, suggesting the possibility of higher incidence of emphysematous changes in von Recklinghausen's disease than has been expected. On the other hand, pulmonary fibrosis was observed in two cases. There were two patients who had no fibrosis but emphysematous lesions. TBLB was performed in one case, in whom no fibrotic changes were observed. Usually pulmonary fibrosis is associated with emphysematous bullae in patients with von Recklinghausen's disease. However, fibrotic and emphysematous changes may not always occur in combination in von Recklinghausen's disease.