LUNG-TRANSPLANTATION FOR WILLIAMS-CAMPBELL-SYNDROME

Williams-Campbell syndrome is a rare disorder characterized by a defic iency of cartilage in subsegmental bronchi leading to distal airway co llapse and bronchiectasis. We report the first case of lung transplant ation in a patient with end-stage lung disease secondary to Williams-C ampbell syndrome. Although the patient did not have proximal airway co llapse prior to transplantation, his posttransplant course was complic ated by the development of bronchomalacia of the right and left mainst em bronchi. The patient experienced recurrent pulmonary infections and died of bacterial pneumonia 1 year after transplantation, Autopsy rev ealed cartilage deficiency in both right and left mainstem bronchi. A hypothesis may be made that a combination of proximal cartilage defici ency and posttransplant ail-way ischemia led to the development of bro nchomalacia after lung transplantation. Thus, in contrast to previous reports, the cartilage deficiency in Williams-Campbell syndrome can in volve both proximal and distal airways. Consequently, bilateral sequen tial lung transplantation may not be an effective therapeutic option i n patients with this syndrome.