williams  campbell syndrome , also known as bronchomalacia , is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi , leading to distal airway collapse and bronchiectasis .
there have been few reports about patients affected by saccular bronchiectasis , paracicatricial emphysema , and diminished cartilage .
this report presents a 57-year - old woman with progressive dyspnea , cough , sputum production , and fever .
the clinical and laboratory examination revealed that the patient had a respiratory infection due to bronchiectasis caused by williams 
although a rare syndrome , when patients signs and symptoms include recurrent respiratory infections , bronchiectasis , productive cough , and dyspnea , williams  campbell syndrome should be included in the differential diagnosis .
williams  campbell syndrome is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi , leading to distal airway collapse and bronchiectasis
campbell syndrome results from the absence of cartilage rings beyond the first and second bronchial divisions with resultant bronchiectasis , which typically affects the fourth- to sixth - order bronchi .
the symptoms and prognosis ultimately depend on the extent of cartilage maldevelopment of the bronchi .
although the syndrome has been best described in children with recurrent pneumonia and broncho - obstructive symptoms such as coughing and wheezing , there have been recent descriptions in adults as well.1,2
a 57-year - old woman presented in the emergency department complaining of progressive dyspnea for the previous 5 days . on clinical examination she had productive cough with clear sputum and intermittent fever .
she was a lifelong nonsmoker but with multiple hospitalizations due to respiratory infections and asthma - like episodes from the age of 8 years . during the past 15 years
the patient was treated symptomatically with antibiotics , nebulizer bronchodilators , and long - term oxygen therapy ( 2 l / min/24 hours ) .
her family past medical history included her 30-year - old sister s sudden death from unknown reasons and her mother s infection from tuberculosis .
at the time of admission her axillary temperature was 38c and blood pressure 120/70 mmhg .
the patient was in respiratory insufficiency with an arterial ph of 7.46 , pco2 of 56 mmhg , po2 of 72 mmhg , and hco3 of 30 mmol /
lung auscultation revealed widespread bilateral crepitating and crackles over the lower part of both lungs .
laboratory data showed an elevated erythrocyte sedimentation rate of 100 mm / h and a white blood count of 9000/l without peripheral blood eosinophilia , and c - reactive protein was 9.3 mg / dl .
her chest x - ray demonstrated increased lung volumes and revealed multiple ring shadows in lungs that were almost symmetrical in distribution and more apparent in the mid and lower zones .
there was the sense of air - fluid levels in some of them corresponding to cystic bronchiectasis . also , there was thickening of the interstitial network .
total serum immunoglobulin ( ig)e concentration was elevated at 2500 u / ml ( normal range 0165 u / ml ) . immediate cutaneous reaction to aspergillus fumigates was not available at the time of the patient s hospitalization ; nevertheless , a. fumigatus - specific serum ige levels were 1.37 u / ml . this is essential for the diagnosis of allergic bronchopulmonary aspergillosis.3 total serum igg , iga , and igm levels were quantified to rule out hypogammaglobulinemia and did not reveal any deficiencies .
antinuclear and antineutrophilic cytoplasmic antibody tests were performed to exclude churg  strauss syndrome , and the results were negative .
there was a reduced forced vital capacity of 1200 ml ( 43.35% pred ) , reduced expiratory volume in 1 second of 720 ml ( 30.7% pred ) with tiffeneau index , reduced expiratory volume in 1 second / forced vital capacity of 76.29 , and carbon monoxide - diffusing capacity of 28% .
a contrast - enhanced computed tomography ( ct ) scan of the chest demonstrated central cylindrical / cystic bronchiectasis involving segmental / subsegmental bronchi , bronchial wall thickening with air - fluid levels , and mucus plugging with predominant localization in the lower lobes .
there was ground glass and tree - in - bud scattered in all lobes , a finding consistent with pneumonitis and acute bronchiolitis .
chest ct also revealed a mosaic picture , which also emerges in all lobe findings consistent with bronchiolitis obliterans .
collapse of bronchi with distal air trapping , the result of an excessively compliant bronchial wall , particularly in the left and right lower lobe , was also present ( figure 2 ) .
the bronchoalveolar lavage fluid obtained from the patient for an acid - fast bacillus was negative on multiple occasions , but the same p. aeruginosa was again isolated .
a diagnosis of saccular bronchiectasis due to williams campbell syndrome was made based on clinicoradiological findings , laboratory testing , and past medical history .
genetic testing was planned , but the patient refused further investigations due to financial constraints .
the patient was treated with intravenous antibiotics ; third - generation cephalosporins , ciprofloxacin , and acetylcysteine ; and chest physiotherapy , and was discharged after 1 month .
written informed consent was obtained from the patient upon discharge for publication of this case report and all accompanying images .
in 1960 , williams and campbell first reported the unusual pattern of bronchiectasis in f i ve patients whose disease presented in infancy with similar clinical features .
they noted markedly compliant , soft central bronchi that ballooned on inspiration and collapsed on expiration . in this syndrome ,
bronchiectasis due to deficiency in cartilage of the third- to sixth - order bronchi generation is observed .
the syndrome is usually presented in childhood , with recurrent pneumonia and broncho - obstructive symptoms such as coughing and wheezing .
nevertheless , adult cases have been reported without pathologic confirmation.2,46 the symptoms and prognosis depend on the extent of cartilage maldevelopment .
ct imaging demonstrates bilateral cylindrical / cystic bronchiectasis distal to the third - generation bronchi ( segmental / subsegmental ) with hyperinflation of the lung.4,7 on expiration , collapse of the bronchi with distal air trapping is observed as the result of an excessively compliant bronchial wall .
the trachea and central bronchi remain normal in caliber , a distinguished feature of this disease .
the mechanism for the deficiency in cartilage is not well understood ; no evidence suggests that cartilage deficiency occurs outside of the bronchi.8,9 in addition , regarding diagnostic exams , bronchoscopy is often unrevealing.10 a genetic background and familial occurrence are also reported.1012 the compliant bronchi collapse during coughing , leading to poor airway drainage .
subsequently , progressive obstructive disease develops , causing hyperinflation of the lung and segmental or lobar collapse .
recurrent destruction of the bronchial tree and inadequate clearance of mucus result in further damage to the lung parenchyma.8,9 the long - term prognosis is variable , with rapid clinical deterioration and death in some children and prolonged survival in others.13 no specific treatment exists for williams campbell syndrome .
prophylaxis from exacerbations remain the basis of treatment.14,15 prophylaxis can be achieved if an oral or intravenous antibiotic is given for 710 days or until sputum production decreases . for severe cases ,
several different antibiotics may be used sequentially in a continuous regimen to minimize bacterial resistance .
transplantation has been reported in a patient with severe respiratory symptoms from williams  campbell syndrome , but the patient died 1 year later . upon postmortem examination
it was observed that the main bronchi had bronchomalacia , which was attributed to a respiratory infection during the postsurgery period.1 moreover , when necessary , based on the patient s symptoms ( bronchospasm and thick , tenacious sputum ) , a bronchodilator , combined with postural drainage and chest percussion , can help remove secretions
. respiratory exercise with free flow and bronchoscopy may be used to help mobilize secretions .
hypoxia requires oxygen therapy.14 noninvasive positive airway pressure can be used when respiratory acidosis exists.16 other acquired and congenital conditions associated with bronchiectasis , including ciliary dyskinesia , cystic fibrosis , allergic bronchopulmonary aspergillosis , and immunoglobin deficiencies , must be excluded .
differential diagnosis of saccular bronchiectasis includes cystic fibrosis , allergic bronchopulmonary aspergillosis , -1 antitrypsin deficiency , ig deficiency , radiation fibrosis , tuberculosis , immotile ( dyskinetic ) cilia syndrome , mounier - kuhn syndrome , and williams  campbell syndrome .
when patients signs and symptoms include recurrent respiratory infections , bronchiectasis , productive cough , and dyspnea , williams  campbell syndrome should be included in the differential diagnosis .
 introductionwilliams  campbell syndrome , also known as bronchomalacia , is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi , leading to distal airway collapse and bronchiectasis . 
 there have been few reports about patients affected by saccular bronchiectasis , paracicatricial emphysema , and diminished cartilage . 
 these are all characteristic of williams  
 campbell syndrome.case presentationthis report presents a 57-year - old woman with progressive dyspnea , cough , sputum production , and fever . 
 the clinical and laboratory examination revealed that the patient had a respiratory infection due to bronchiectasis caused by williams  
 campbell syndrome , which was undiagnosed in the patient until then.conclusionalthough a rare syndrome , when patients signs and symptoms include recurrent respiratory infections , bronchiectasis , productive cough , and dyspnea , williams  campbell syndrome should be included in the differential diagnosis . 
Introduction
Case presentation
Conclusion
Introduction
Case presentation
Discussion
Conclusion