title: Lung transplantation for Williams-Campbell syndrome.
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.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Duke University Medical Center, Durham, NC 27710, USA.
authors.forename: S M
authors.initials: SM
authors.lastname: Palmer
authors.forename: D T
authors.initials: DT
authors.lastname: Layish
authors.forename: P S
authors.initials: PS
authors.lastname: Kussin
authors.forename: T
authors.initials: T
authors.lastname: Oury
authors.forename: R D
authors.initials: RD
authors.lastname: Davis
authors.forename: V F
authors.initials: VF
authors.lastname: Tapson
mesh_terms: D000328:Adult; D000402:Airway Obstruction / Q000209:etiology; D001980:Bronchi / Q000098:blood supply / Q000473:pathology; D001982:Bronchial Diseases / Q000151:congenital* / Q000601:surgery; D001987:Bronchiectasis / Q000209:etiology* / Q000601:surgery; D002357:Cartilage Diseases / Q000151:congenital* / Q000601:surgery; D017809:Fatal Outcome; D006801:Humans; D007511:Ischemia / Q000209:etiology; D016040:Lung Transplantation / Q000009:adverse effects; D008297:Male; D018410:Pneumonia, Bacterial / Q000209:etiology; D011183:Postoperative Complications; D011552:Pseudomonas Infections; D011550:Pseudomonas aeruginosa; D011656:Pulmonary Emphysema / Q000209:etiology; D012008:Recurrence; D012131:Respiratory Insufficiency / Q000209:etiology; D013577:Syndrome; D016896:Treatment Outcome