Williams-Campbell Syndrome

Classification according to ICD-10
Q33.3	Lung agenesis, hypoplasia and dysplasia of the lungs
Q33.4	Congenital bronchiectasis
Q33.6	Hypoplasia and dysplasia of the lungs
ICD-10 online (WHO version 2019)

The Williams-Campbell's syndrome (WCS) is a very rare congenital malformation of the lung caused by a generalized aplasia , hypo- or dysplasia of the cartilages of the segment and Subsegmentbronchien is characterized. The syndrome can be viewed as a variant of bronchomalacia .

The name refers to the authors of the first description from 1960 by the Australian pediatricians Howard Williams and Peter Campbell.

distribution

The frequency is not known.

root cause

It has not yet been clarified whether it is an inherited malformation or the result of a virus infection . A combination of both factors, i.e. a genetic disposition in conjunction with a viral infection, may also be the cause.

Clinical manifestations

Clinical criteria are:

• Chronic bronchitis with chronic coughing , wheezing, expectoration with recurrent pneumonia and shortness of breath ( dyspnea ), which usually begins in early childhood
• The trigger is often a measles or adenovirus infection
• In the course of the disease , cyanosis , generalized sac-shaped bronchiectasis , drumstick fingers , short stature , emphysema , pulmonary fibrosis and cor pulmonale as well as chest chest , funnel chest or a barrel chest can develop.

diagnosis

The x-ray shows an overinflation of the lungs with collapse of the bronchi during exhalation. During bronchography or bronchoscopy , fluctuations in the caliber of the 3rd and 4th order bronchi during inhalation and exhalation are noticeable.

Differential diagnosis

The following are to be distinguished:

• Acute interstitial pneumonia (Hamman-Rich syndrome)
• Allergic bronchopulmonary aspergillosis
• bronchial asthma
• Bronchoectasia of a different origin
• Bronchial stenosis syndrome
• Kartagener's syndrome
• Cystic fibrosis
• Mycosis of the lungs
• Wilson-Mikity syndrome (bladder lung syndrome)

therapy

A causal treatment is not possible. Therapy is symptomatic with antibiotics , physiotherapy , massage and positioning drainage . In severe cases, a lung transplant can be considered.

literature

• D. Pauleit, B. Kreft, C. Rothert, H. Schild: Lung changes in the high-resolution spiral CT in Williams-Campbell syndrome. In: RöFo: Advances in the field of X-rays and nuclear medicine . Volume 165, Number 6, December 1996, pp. 589-591, doi : 10.1055 / s-2007-1015818 , PMID 9026105 .
• W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. 2nd edition, Volume 2, p. 60, Springer 1996, ISBN 3-540-60224-0 .

Individual evidence

1. ↑ ^{a b c d} Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
2. ↑ Williams-Campbell Syndrome. In: Orphanet (Rare Disease Database).
3. ↑ Entry on Williams-Campbell Syndrome in Flexikon , a Wiki of the DocCheck company
4. ↑ ^{a b c} Pschyrembel online
5. ↑ H. Williams, P. Campbell: Generalized bronchiectasis associated with deficiency of cartilage in the bronchial tree. In: Archives of Disease in Childhood. Volume 35, April 1960, pp. 182-191, doi : 10.1136 / adc.35.180.182 , PMID 13844857 , PMC 2012546 (free full text).
6. ^ AP Noriega Aldave, D. William Saliski: The clinical manifestations, diagnosis and management of williams-campbell syndrome. In: North American journal of medical sciences. Volume 6, number 9, September 2014, pp. 429-432, doi : 10.4103 / 1947-2714.141620 , PMID 25317385 , PMC 4193147 (free full text) (review).
7. ↑ JB Orens, M. Estenne, S. Arcasoy, JV Conte, P. Corris, JJ Egan, T. Egan, S. Keshavjee, C. Knoop, R. Kotloff, FJ Martinez, S. Nathan, S. Palmer, A Patterson, L. Singer, G. Snell, S. Studer, JL Vachiery, AR Glanville: 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. In: The Journal of Heart and Lung Transplantation . Volume 25, Number 7, July 2006, pp. 745-755, doi : 10.1016 / j.healun.2006.03.011 , PMID 16818116 .

Web links

• BRONCHOMALACIA.  In: Online Mendelian Inheritance in Man . (English)
• Radiopaedia

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !