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Abstract: Case Reports |

CONGENITAL BRONCHIECTASIS: WILLIAMS-CAMPBELL SYNDROME IN A 26-YEAR-OLD FEMALE FREE TO VIEW

Elie G. Abdallah, MD*; Robert J. Lenox, FRCP, FCCP
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Suny Upstate Medical University, Syracuse, NY



Chest. 2006;130(4_MeetingAbstracts):331S. doi:10.1378/chest.130.4_MeetingAbstracts.331S-a
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INTRODUCTION: Williams-Campbell (WC) syndrome is a rare form of congenital bronchiectasis. It is characterized by the absence or markedly diminished cartilage around the subsegmental bronchi. We present the case of a young woman diagnosed with WC syndrome.

CASE PRESENTATION: A 26-year-old African female patient was referred to the pulmonary clinic for evaluation of chest pain. Her symptoms started 2 years prior to this presentation. The pain was described as sharp and pleuritic in nature, localized to the posterior chest and along the sternal borders, without any radiation, 4/10 in intensity. The patient reported cough productive of a moderate amount of clear sputum as well as mild hemoptysis every month or so. She also reported dyspnea on exertion. She had no fever, chills, night sweats or weight loss. She had had repeatedly negative tuberculin skin tests.She had been given a diagnosis of allergic bronchopulmonary aspergillosis one year prior to this presentation. She did not receive systemic steroids or anti-fungal medication for treatment and consistently denied any history of wheezing. She is a lifelong nonsmoker with no other past medical history. Her allergy testing was positive for cats and she reported a bronchospastic reaction to aspirin. She has 2 brothers with asthma; her mother and father are alive and healthy. She was not using any medications at the time of presentation. Her physical examination revealed a healthy looking 26-year-old female in no acute distress. Vital signs were normal. Head and neck examination was unremarkable. Heart, abdomen and extremities examination was normal. Lung auscultation and percussion did not show any abnormalities.Outpatient laboratory studies including complete blood count with differential, metabolic panel, immunoglobulin levels and alpha-1 anti-trypsin were normal. Serum precipitin for aspergillus was negative. Chest radiograph showed bilateral bronchiectatic changes and a left upper lobe cavitary lesion. CT scan of the thorax revealed diffuse bilateral bronchiectasis involving subsegmental bronchi, forming multiple thick-walled cysts bilaterally. There were large left upper lobe cysts seen, some of which demonstrated air-fluid levels, and the largest one contained a soft tissue density, likely representing mycetoma. A fine needle aspiration of the left upper lobe cavity confirmed the diagnosis of aspergilloma. Pain was managed with narcotics; no other treatment was given. The diagnosis of WC syndrome was made on the basis of the clinical symptoms and the characteristic appearance of the CT images. Bronchial biopsy is not always reliable and usually unnecessary in typical cases.

DISCUSSIONS: WC syndrome was first described in 1960 as a rare form of bronchiectasis. The underlying problem is maldevelopment in the cartilage of the bronchi. It is mainly a disease of childhood, however adult cases have been reported. CT scan characteristics of this syndrome include bilateral and symmetrical cystic dilatation of bronchi on inspiration and collapse on expiration. Unlike in other forms of bronchiectasis, these findings are observed in a uniform manner in the subsegmental bronchi up to the first and second divisions. There is no specific treatment for WC syndrome. Good bronchial hygiene and treatment of acute exacerbations remains the cornerstones of treatment as in any form of bronchiectasis.

CONCLUSION: We present a case of Williams-Campbell syndrome in a young woman. The radiographic findings of bilateral bronchiectasis confined to the subsegmental bronchi are classic for the syndrome.

DISCLOSURE: Elie Abdallah, None.

Wednesday, October 25, 2006

2:00 PM - 3:30 PM

References

Williams H. Campbell P. Generalized bronchiectasis associated with deficiency of cartilage in the bronchial tree.Dis Child1960;35:182-91. [CrossRef]
 
Watanabe Y. Nishiyama Y. Kanayama H. Enomoto K. Kato K. Takeichi M. Congenital bronchiectasis due to cartilage deficiency: CT demonstration.Journal of Computer Assisted Tomography.11(4):701-3,1987Jul-Aug.
 

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References

Williams H. Campbell P. Generalized bronchiectasis associated with deficiency of cartilage in the bronchial tree.Dis Child1960;35:182-91. [CrossRef]
 
Watanabe Y. Nishiyama Y. Kanayama H. Enomoto K. Kato K. Takeichi M. Congenital bronchiectasis due to cartilage deficiency: CT demonstration.Journal of Computer Assisted Tomography.11(4):701-3,1987Jul-Aug.
 
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