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Tracheohronchiomegaly and acquired cutis laxa are described in a 14-year-old boy. Marked dilatation of the tracheobronchial tree associated with recurrent lower respiratory tract infections has been reported chiefly in the adult medical literature as the Mounier-Kuhn syndrome. Cutis laxa is a rare elastic tissue disorder that involves the skin and other systems. While the etiology of tracheobronchiomegaly is unknown, the coexistence of two rare conditions in the same patient suggtsts a common fundamental elastic tissue defect. The investigative studies revealed abnormal elastic fibers in the skin, increased thoracic gas volumes, abnormal pulmonary elastic recoil, and a normal immunologic status except for increased serum and parotid IgA levels.

GROSS dilatation of the tracheobronchial tree associated with chronic recurrent respiratory tract infection was described initially by Mounier-Kuhn1in 1932. Since that time approximately 16 case reports of this rare condition have been documented. The term, tracheobronchiomegaly, has been advocated by Katz and his co-workers2and more recently by Levowitz et al3in describing this condition. Essentially, this anatomic abnormality appears to arise as a congenital defect or atrophy in the tracheobronchial musculature and elastic tissue. Saccular diverticula may or may not be present; the pathogenesis of these diverticula is thought to be herniation of tracheal mucosa between cartilaginous rings. Chronic infection both contributes to and is caused by this alteration in pulmonary structure. Most patients present in early adulthood, and long-standing respiratory symptoms are often elicited.4,5 Bronchoscopy may reveal redundant mucous membrane, cavernous dilatation of the tracheobronchial tree, or no obvious abnormality whatever. Secondary