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Williams-Campbell syndrome is a rare disorder characterized by deficiency of subsegmental bronchial cartilage and development of airway collapse and bronchiectasis that may subsequently progress to respiratory failure and death. There are only 2 published reports suggesting a familial association, and only one report of lung transplantation being used as a therapeutic modality. Due to postoperative airway complications, transplantation has not been recommended for this disease. We report the first lung transplant with prolonged survival, approaching 10 years, in a patient with Williams-Campbell syndrome, and provide further evidence to support a familial association., (Copyright 2012 Daedalus Enterprises)

Lung transplantation is now performed in patients with end-stage pulmonary parenchymal or vascular lung disease. The process of evaluating a patient for transplantation, managing the patient during the peri-operative period, and caring for the patient following transplantation is complex. Lung-transplant recipients are prone to unique complications of lung transplantation, as well as general complications of an immunosuppressed host. This article reviews the indications for, expected outcomes of, and management of complications that can develop following lung transplantation. Respiratory therapists play an instrumental role in assisting in the management of this group of patients in the pretransplant and post-transplant periods, and in their long-term management.