Lung transplantation for Williams-Campbell syndrome.

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Palmer, SM; Layish, DT; Kussin, PS; Oury, T; Davis, RD; Tapson, VF

Published Date

  • February 1998

Published In

Volume / Issue

  • 113 / 2

Start / End Page

  • 534 - 537

PubMed ID

  • 9498979

International Standard Serial Number (ISSN)

  • 0012-3692

Language

  • eng

Conference Location

  • United States