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.
MullerNL, BerginCJ, OstrowDN, NicholsDM. Role of computed tomography in the recognition of bronchiectasis. AJR Am J Roentgenol, 1984; 143(5):971–976.
11.
Di ScioscioV, ZompatoriM, MisturaI, MontanariP, SantilliL, LuccaroniR, et al. The role of spiral multidetector dynamic CT in the study of Williams-Campbell syndrome. Acta Radiol, 2006; 47(8):798–800.
12.
GeorgeJ, JainR, TariqSM. CT bronchoscopy in the diagnosis of Williams-Campbell syndrome. Respirology, 2006; 11(1):117–119.
13.
BarkerAF. Bronchiectasis. N Engl J Med, 2002; 346(18):1383–1393.
14.
WadaH, SetoR, YamadaH, NagaoT, HajiroT, NakanoY. Respiratory failure of Williams-Campbell syndrome is effectively treated by noninvasive positive pressure ventilation. Intern Med, 2011; 50(16):1729–1732.
15.
FraserRG, MacklemPT, BrownWG. Airway dynamics in bronchiectasis; a combined cinefluorographic-manometric study. Am J Roentgenol Radium Ther Nucl Med, 1965; 93:821–835.
OrensJB, EstenneM, ArcasoyS, ConteJV, CorrisP, EganJJ, et al. International guidelines for the selection of lung transplant candidates: 2006 update: a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant, 2006; 25(7):745–755.
18.
GishenP, SaundersAJ, TobinMJ, HutchisonDC. Alpha 1-antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British Thoracic Association. Clin Radiol, 1982; 33(4):371–377.
19.
HartmanTE, PrimackSL, LeeKS, SwensenSJ, MullerNL. CT of bronchial and bronchiolar diseases. Radiographics, 1994; 14(5):991–1003.
20.
MeyerholzDK, StoltzDA, NamatiE, RamachandranS, PezzuloAA, SmithAR, et al. Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. Am J Respir Crit Care Med, 2010; 182(10):1251–1261.
