Palmer S M, Layish D T, Kussin P S, Oury T, Davis R D, Tapson V F
Division of Pulmonary and Critical Care Medicine, Duke University Medical Center, Durham, NC 27710, USA.
Chest. 1998 Feb;113(2):534-7. doi: 10.1378/chest.113.2.534.
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.
威廉姆斯-坎贝尔综合征是一种罕见的疾病，其特征是亚段支气管软骨缺乏，导致远端气道塌陷和支气管扩张。我们报告了首例因威廉姆斯-坎贝尔综合征继发终末期肺病而进行肺移植的患者。尽管该患者在移植前没有近端气道塌陷，但其移植后的病程因左右主支气管发生支气管软化而变得复杂。该患者反复发生肺部感染，移植后1年死于细菌性肺炎。尸检显示左右主支气管均有软骨缺乏。可以提出一个假设，即近端软骨缺乏和移植后气道缺血共同导致了肺移植后支气管软化的发生。因此，与之前的报道不同，威廉姆斯-坎贝尔综合征中的软骨缺乏可累及近端和远端气道。因此，双侧序贯肺移植可能不是该综合征患者的有效治疗选择。
