TRACHEOBRONCHIOMEGALY AND ACQUIRED CUTIS LAXA IN A CHILD

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.