Tracheobronchomegaly

Johnston and Green (1965) presented 5 cases of which 2 were black brother and sister. Chromosome studies were normal. The parents and 5 other sibs appeared to be unaffected. Two sibs died in early infancy. In one, mongolism was diagnosed. Although bronchopulmonary suppuration largely determines the degree of respiratory disability, infection is not responsible for the underlying lesion of the tracheobronchial tree. The characteristic bronchographic picture led several workers to call the disorder 'trachiectasis with multiple diverticula.' The appearance is created by enlargement of the airways and musculomembranous tissue projecting like corrugations between the cartilaginous rings. The black patient reported by Aaby and Blake (1966) probably suffered from the Ehlers-Danlos syndrome.