Familial congenital bronchiectasis: Williams-Campbell syndrome

Williams-Campbell syndrome was first described in 1960 as a rare form of bronchiectasis. Its pathogenesis is characterized by the absence or markedly diminished cartilage around the bronchi. Although the familial nature was postulated early on, only one possible familial occurrence has been reported in the literature. We present two family members in whom respiratory symptoms developed within the first year of life and were found to have histopathologic changes consistent with Williams-Campbell syndrome. This world lend further support of a developmental origin for this type of bronchiectasis.