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Lung Transplantation for Williams-Campbell Syndrome

Scott M. Palmer, Jr.; Daniel T. Layish; Peter S. Kussin; Victor F. Tapson; Tim Oury; Robert D. Davis
Author and Funding Information

Affiliations: From the Division of Pulmonary and Critical Care Medicine, Department of Medicine, Duke University Medical Center, Durham, NC.,  From the Department of Pathology, Duke University Medical Center, Durham, NC.,  From the Division of Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, NC.

Affiliations: From the Division of Pulmonary and Critical Care Medicine, Department of Medicine, Duke University Medical Center, Durham, NC.,  From the Department of Pathology, Duke University Medical Center, Durham, NC.,  From the Division of Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, NC.

Affiliations: From the Division of Pulmonary and Critical Care Medicine, Department of Medicine, Duke University Medical Center, Durham, NC.,  From the Department of Pathology, Duke University Medical Center, Durham, NC.,  From the Division of Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, NC.


1998 by the American College of Chest Physicians


Chest. 1998;113(2):534-537. doi:10.1378/chest.113.2.534
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Abstract

Williams-Campbell syndrome is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi leading to distal airway collapse and bronchiectasis. We report the first case of lung transplantation in a patient with end-stage lung disease secondary to Williams-Campbell syndrome. Although the patient did not have proximal airway collapse prior to transplantation, his posttransplant course was complicated by the development of bronchomalacia of the right and left mainstem bronchi. The patient experienced recurrent pulmonary infections and died of bacterial pneumonia 1 year after transplantation. Autopsy revealed cartilage deficiency in both right and left mainstem bronchi. A hypothesis may be made that a combination of proximal cartilage deficiency and posttransplant airway ischemia led to the development of bronchomalacia after lung transplantation. Thus, in contrast to previous reports, the cartilage deficiency in Williams-Campbell syndrome can involve both proximal and distal airways. Consequently, bilateral sequential lung transplantation may not be an effective therapeutic option in patients with this syndrome.


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