Williams-Campbell Syndrome: Case Report
Article Sidebar
Main Article Content
Abstract
Williams-Campbell syndrome (WCS) is a clinical entity characterized by cartilage deficiency in the bronchial wall of the subsegmental bronchi. It is a congenital condition that is usually diagnosed in childhood, but there are case reports in the adult population, and it is believed that this is due to a minor cartilage deficiency. The main symptoms of this condition are the presence of cough, dyspnea and repeated respiratory infections. Radiologically, the presence of thin-walled bronchiectasis is evident. The diagnosis is based on ruling out other more common causes of bronchiectasis, as well as identifying the radiological characteristics and the presence of the congenital component.
We present the case of a young man with respiratory symptoms since childhood requiring multiple hospitalizations due to repeated infectious processes of pulmonary origin, in which the diagnosis of Williams-Campbell syndrome was reached.
Downloads
Article Details
This work is licensed under a Creative Commons Attribution 4.0 International License.
References
Chalmers JD, Chang AB, Chotirmall SH, Dhar R, McShane PJ. Bronchiectasis. Nat Rev Dis Primers 2018;4(1):45.
Athanazio R, Pereira MC, Gramblicka G et al. Latin America validation of FACED score in patients with bronchiectasis: an analysis of six cohorts. BMC Pulm Med 2017;17(1):73. Doi: 10.1186/s12890-017-0417-3.
Watanabe Y, Nishiyama Y, Kanayama H, Enomoto K, Kato K, Takeichi M. Congenital bronchiectasis due to cartilage deficiency: CT demonstration. J Comput Assist Tomogr 1987;11(4):701-703.
Williams H, Campbell P. Generalized bronchiectasis associated with deficiency of cartilage in the bronchial tree. Arch Dis Child 1960;35(180):182-191.
Noriega-Aldave AP, William-Saliski D. The clinical manifestations, diagnosis and management of williams-campbell syndrome. N Am J Med Sci 2014;6(9):429-432. Doi:10.4103/1947-2714.141620.
Jones QC, Wathen CG. Williams-Campbell syndrome presenting in an adult. BMJ Case Rep 2012:bcr2012006775. Doi:10.1136/bcr-2012-006775
Kimoto T, Kawamura T, Nakahara Y, Mochizuki Y. Nihon Kokyuki Gakkai Zasshi 1999;37(5):429-432.
Liu M, Yuan Q, Zhao H, Wang Y. Williams-Campbell syndrome complicated with pulmonary hypertension and Type 2 respiratory failure: An adult case report. Clin Respir J 2019;13(12):795-799. Doi:10.1111/crj.13090
Kaneko K, Kudo S, Tashiro M, Kishikawa T, Nakanishi Y, Yamada H. Case report: computed tomography findings in Williams-Campbell syndrome. J Thorac Imaging 1991;6(2):11-13.
Di Scioscio V, Zompatori M, Mistura I et al. The role of spiral multidetector dynamic CT in the study of Williams-Campbell syndrome. Acta Radiol 2006;47(8):798-800. Doi:10.1080/02841850600849084
Ten Hacken NH, Kerstjens HA. Bronchiectasis. BMJ Clin Evid 2011;2011:1507.
Kelly C, Chalmers JD, Crossingham I, Relph N, Felix LM, Evans DJ, Milan SJ, Spencer S. Macrolide antibiotics for bronchiectasis. Cochrane Database Syst Rev 2018;3(3):CD012406.
Wada H, Seto R, Yamada H, Nagao T, Hajiro T, Nakano Y. Respiratory failure of Williams-Campbell syndrome is effectively treated by noninvasive positive pressure ventilation. Intern Med 2011;50(16):1729-1732. Doi:10.2169/internalmedicine.50.4971
Leard LE, Holm AM, Valapour M et al. Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2021;40(11):1349-1379. Doi:10.1016/j.healun.2021.07.005