Williams-Campbell syndrome
Last revised by Subhan Iqbal
on 4 Jul 2024
Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Iqbal S, Yu Y, et al. Williams-Campbell syndrome. Reference article, Radiopaedia.org (Accessed on 25 Mar 2025) https://doi.org/10.53347/rID-9743
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rID:
9743
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Disclosures:
At the time the article was created Yuranga Weerakkody had no recorded disclosures.
View Yuranga Weerakkody's current disclosures
Last revised:
4 Jul 2024,
Subhan Iqbal
Disclosures:
At the time the article was last revised Subhan Iqbal had no financial relationships to ineligible companies to disclose.
View Subhan Iqbal's current disclosures
Revisions:
21 times, by
16 contributors -
see full revision history and disclosures
Systems:
Sections:
Synonyms:
- Williams-Campbell syndrome (WCS)
- Williams-Campbell syndrome
Williams-Campbell syndrome (WCS) is a rare form of congenital cystic bronchiectasis, in which distal bronchial cartilage is defective.
On this page:
Clinical presentation
Williams-Campbell syndrome may present with recurrent pneumonia, wheezing, pulmonary hypertension, barrel-chest deformity, and Harrison sulcus 8,10.
Pathology
It is thought to develop from a deficiency of cartilage formation in the 4th to 6th order sub-segmental bronchi, resulting in distal airways collapse and bronchiectasis 6,7.
Radiographic features
CT
Cystic central bronchiectasis changes, usually symmetric and bilateral, typically involving 4th to 6th order bronchi with preservation of the trachea and main bronchi 2. On expiratory aquisitions, there is characteristic collapse of affected bronchi 9.
History and etymology
The syndrome was first described by Howard Williams and Peter Campbell in 1960 8.
Differential diagnosis
All causes of diffuse and bilateral bronchiectasis including:
References
- 1. McAdams HP, Erasmus J. Chest case of the day. Williams-Campbell syndrome. AJR Am J Roentgenol. 1995;165 (1): 190-1. doi:10.2214/ajr.165.1.7785587 - Pubmed citation
- 2. Marom EM, Goodman PC, McAdams HP. Diffuse abnormalities of the trachea and main bronchi. AJR Am J Roentgenol. 2001;176 (3): 713-7. doi:10.2214/ajr.176.3.1760713 - Pubmed citation
- 3. Webb WR. Radiology of obstructive pulmonary disease. AJR Am J Roentgenol. 1997;169 (3): 637-47. doi:10.2214/ajr.169.3.9275869 - Pubmed citation
- 4. Acar T, Bayraktaroglu S, Ceylan N et-al. Computed tomography findings of tracheobronchial system diseases: a pictorial essay. Jpn J Radiol. 2015;33 (2): 51-8. doi:10.1007/s11604-014-0380-3 - Pubmed citation
- 5. Konoglou M, Porpodis K, Zarogoulidis P et-al. Williams-Campbell syndrome: a case report. Int J Gen Med. 2012;5: 41-4. doi:10.2147/IJGM.S28447 - Free text at pubmed - Pubmed citation
- 6. 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 - Free text at pubmed - Pubmed citation
- 7. Liu M, Liu YQ, Liu ZH, Liu WY, Liu. Williams-Campbell syndrome complicated with pulmonary hypertension and Type 2 respiratory failure: An adult case report. (2019) The clinical respiratory journal. doi:10.1111/crj.13090 - Pubmed
- 8. WILLIAMS H, CAMPBELL P. Generalized bronchiectasis associated with deficiency of cartilage in the bronchial tree. (1960) Archives of disease in childhood. 35: 182-91. doi:10.1136/adc.35.180.182 - Pubmed
- 9. Ghosh S. Williams-Campbell Syndrome. Radiology. 2021. doi:10.1148/radiol.2021211621
- 10. Liu M, Yuan Q, Zhao H, Wang Y. Williams‐Campbell Syndrome Complicated with Pulmonary Hypertension and Type 2 Respiratory Failure: An Adult Case Report. Clinical Respiratory J. 2019;13(12):795-9. doi:10.1111/crj.13090 - Pubmed
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