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The article presents a clinical long$term follow$up of a young child with the bronchial obstruction syndrome, associated with expectoration of purulent sputum (up to 10 ml for one$time aspiration, ~ 140-200 mL per day). During the differential diagnostics, the congenital anomaly of the tracheobronchial tree was revealed, which turned out to be Williams-Campbell syndrome. Comparison of the malformation combination and course features of Williams-Campbell syndrome, identified in the child, with the literature data, as well as recommendations for monitoring and management of the patients with cystic bronchiectasis are described in the article.

Chronic obstructive pulmonary disease (COPD) patients that have evidence of bronchiectasis demonstrate greater symptom severity, more frequent bronchial infections and exacerbations, lower lung function and increased mortality.1 While the association has been recognized, it is not clear if there is any causal relationship between having COPD and developing bronchiectasis. Certainly, the fact that COPD is associated with a high risk for lower respiratory infection2 and/or dysbiosis3 may be part of the explanation but not all patients with COPD develop bronchiectasis.Bronchiectasis has been defined as a dilation of the airways that persists beyond the acute responses to various insults such as infections, aspiration and toxic inhalations. The most accepted criteria as defined by Naidich include direct signs of a bronchial-arterial ratio >1, lack of bronchial tapering, visualization of peripheral bronchi within 1 cm of the costal pleura or in contact with the mediastinal pleura and indirect signs such as peribronchial thickening, mucus plugging, mosaic pattern, central lobular nodules, tree in bud nodules, focal areas of air trapping and atelectasis/consolidation).4 Bronchiectasis is known to develop and progressively worsen over time secondary to a number of conditions including cystic fibrosis, alpha-1 antitrypsin deficiency, primary ciliary dyskinesia, allergic bronchopulmonary mycosis, immunoglobulin deficiencies and post obstructive pneumonia secondary to tumors, foreign bodies and lymphadenopathy. Other less common causes include rheumatoid arthritis and Sjogren's disease, connective tissue disorders such as tracheobronchomegaly (Mounier Kuhn), Marfan's disease, cartilage deficiency (Williams Campbell syndrome), inflammatory bowel disease, HIV, Job's syndrome, and malignancies such as, chronic lymphocytic lymphoma and graft-versus-host disease, as well as yellow nail syndrome and Young's syndrome.5 It has been proposed that bronchiectasis develops as a result of these various disorders leading to structural and functional changes in the airways that increase susceptibility to chronic bronchial infections secondary to potentially pathogenic microorganisms. The persistent presence of these organisms causes chronic inflammation, airway remodeling and further damage to local defense mechanisms further enabling these potentially pathogenic microorganisms to persist in the airways despite the repeated administration of antibiotics thus creating a vicious cycle as proposed by Cole et al.6 With this backdrop it is not surprising that there has been recognition of a high prevalence of bronchiectasis in the COPD population with rates ranging from 4% to 72%.7 The broad prevalence range reported is likely a result of several factors including differences in patient characteristics, evaluations during times of exacerbation versus clinical stability, and whether the prevalence of bronchiectasis was part of a primary or secondary analysis of data. Suffice it to say that the true prevalence of bronchiectasis/COPD overlap remains unknown. Regardless, similar associations have also been noted in asthma8,9 and it has also been recognized that up to 75% of bronchiectasis patients demonstrate signs of upper airway disease and report severe daily symptoms of nasal congestion, facial pain and/or loss of smell.10 Considering the various combinations and permutations of these overlaps, several COPD phenotypes could be generated but it is debatable as to whether defining such overlap syndromes will lead to particularly unique treatment paradigms. Rather, it may be best to simply look at the various components as treatable traits and optimize treatment for each component as outlined by Polverino and colleagues in an excellent review recently published.1 Polverino and colleagues also point to many questions that need to be addressed regarding the COPD/bronchiectasis overlap. We need a set of updated consensus criteria for defining radiological and clinical bronchiectasis in patients with COPD to better establish prevalence and the potential prognostic value of identifying bronchiectasis in COPD patients. New methods to diagnose bronchiectasis independent of vessel diameter are needed, as the classic definition, airway diameter/blood vessel diameter ratio greater than 1, can be misleading as cardiovascular comorbidities can cause increases in blood vessel diameter. There are also no specific biomarkers linking COPD to bronchiectasis such as possible genetic and epigenetic phenomena that may influence susceptibility to infections and response to treatment that renders only a subgroup of COPD patients to develop bronchiectasis. Other questions remain with regard to alterations of the microbiome, so called dysbiosis,3 that may be related to microaspiration11 and/or the potential impacts of inhaled corticosteroid use and frequent antibiotic use.12-15 Inhaled corticosteroids are often recommended for COPD patients with frequent exacerbations whereas recent guidelines in the Cochrane review have concluded that there is insufficient evidence to recommend the use of inhaled steroids in adults with stable bronchiectasis except in specific conditions where the possible benefits in exacerbation reduction outweigh the risks.16 Other areas for study include interventions that may help prevent progression of disease and exacerbations, including the role of other anti-inflammatory molecules such as phosphodiesterase 4 (PDE4) inhibitors or prophylactic antibiotic treatment either systemically or by inhalation.1 These are important questions to address in the overlap population because treatment recommendations are often conflicting in guideline recommendations for COPD patients versus pure bronchiectasis patients. Conversely, macrolide antibiotics have appeared to be effective in patients with bronchiectasis and frequent exacerbations however there continue to be some concerns about the possible use of macrolides and their ability to induce significant cardiovascular adverse effects,17 particularly in COPD patients who are already at risk of higher cardiovascular comorbidity. Furthermore, there is the concern about the development of multi-antibiotic resistance particularly given the reported prevalence of nontuberculous mycobacterial (NTM) infections in COPD patients.18 In this Journal club we review some recent papers that have addressed some of these issues. Suffice it to say that additional prospective trial research is much required. Note: Abstracts are presented in their original, published format and have not been edited to match JCOPDF style.

Objective: Williams–Campbell syndrome (WCS) is a rare congenital disorder, which leads to bronchiectasis affecting fourth to sixth order of bronchial divisions. Symptoms include cough, sputum, wheeze and recurrent pulmonary infections, classically seen in the paediatric age group with selective bronchiectasis of the mid-order bronchioles. The literature describing diagnosis of Williams–Campbell syndrome in adult population is very sparse. Methods: This report presents a 62-year-old female with cough, fever, dyspnea and generalized body ache. She has had multiple admissions to the hospital since her childhood due to recurrent lower respiratory tract infections. Imaging findings demonstrated multiple cystic thin walled airways, compatible with bronchiectatic changes in the upper, middle and lower lobes bilaterally, bronchial wall thickening with air-fluid levels prominent in the fifth and sixth generation bronchial divisions, with normal calibre trachea and central bronchi. These radiological findings are consistent with diagnosis of Williams–Campbell syndrome, which was diagnosed after ruling out the other common causes of bronchiectasis. Conclusion: Williams–Campbell syndrome is a rare congenital cystic lung disease, the diagnosis of which is made by exclusion of common causes of bronchiectasis such as cystic fibrosis, allergic bronchopulmonary aspergillosis, tuberculosis, dyskinetic cilia syndrome and alpha-1 antitrypsin deficiency. Whenever the clinical picture is consistent with bronchiectasis, especially involving the mid-order bronchioles and recurrent pulmonary infections, it is wise to include WCS in the list of differential diagnoses, even in the adult population.

The article presents a clinical long$term follow$up of a young child with the bronchial obstruction syndrome, associated with expectoration of purulent sputum (up to 10 ml for one$time aspiration, ~ 140-200 mL per day). During the differential diagnostics, the congenital anomaly of the tracheobronchial tree was revealed, which turned out to be Williams-Campbell syndrome. Comparison of the malformation combination and course features of Williams-Campbell syndrome, identified in the child, with the literature data, as well as recommendations for monitoring and management of the patients with cystic bronchiectasis are described in the article., Представлено клиническое наблюдение за ребенком раннего возраста с длительным течением синдрома бронхиальной обструкции, сопровождавшегося отделением большого количества гнойной мокроты (до 10 мл при однократной аспирации, ~ 140–200 мл/сутки). В процессе дифференциальной диагностики выявлена врожденная аномалия трахеобронхиального дерева — синдром Вильямса—Кемпбелла. В статье проведено сравнение комбинации мальформаций и особенностей течения синдрома Вильямса—Кемпбелла, выявленных у ребенка, с данными литературы, а также даны основные рекомендации по мониторингу и менеджменту пациентов с кистозным бронхоэктазисом., Наведено клінічне спостереження за дитиною раннього віку з тривалим перебігом синдрому бронхіальної обструкції, який супроводжувався відділенням великої кількості гнійного мокротиння (до 10 мл при одноразовій аспірації, ~ 140–200 мл/добу). У процесі диференціальної діагностики виявлено вроджену аномалію трахеобронхіального дерева — синдром Вільямса—Кемпбелла. У статті проведено порівняння комбінації мальформацій і особливостей перебігу синдрому Вільямса—Кемпбелла, виявлених у дитини, із даними літератури, а також надані рекомендації щодо моніторингу та менеджменту пацієнтів з кістозним бронхоектазісом.

Williams–Campbell syndrome is a rare congenital syndrome characterized by the absence of cartilage in subsegmental bronchi leading to formation of bronchiectasis distal to the affected bronchi. The syndrome is usually presented in childhood, with recurrent pneumonia and broncho-obstructive symptoms such as coughing and wheezing. When patients’ signs and symptoms include recurrent respiratory infections and diffuse bronchiectasis, Williams–Campbell syndrome should be included in the differential diagnosis. The authors hereby report a case of a 1.5-year-old male child with respiratory distress who was diagnosed as Williams–Campbell syndrome on clinico-radiological grounds.

Williams-Campbell syndrome is a rare disorder characterized by deficiency of subsegmental bronchial cartilage and development of airway collapse and bronchiectasis that may subsequently progress to respiratory failure and death. There are only 2 published reports suggesting a familial association, and only one report of lung transplantation being used as a therapeutic modality. Due to postoperative airway complications, transplantation has not been recommended for this disease. We report the first lung transplant with prolonged survival, approaching 10 years, in a patient with Williams-Campbell syndrome, and provide further evidence to support a familial association.

Williams-Campbell syndrome is a rare disease, characterized by a congenital deficiency of cartilage in the fourth to sixth order bronchi, leading to chronic respiratory failure with recurrent pulmonary infections. An effective and practical treatment has not yet been established. A 31-year-old man who was diagnosed as Williams-Campbell syndrome by inspiratory and expiratory computed tomography findings developed recurrent pulmonary infections and showed progressive deterioration of dyspnea. Domiciliary NPPV was administered, followed by a dramatic improvement of respiratory failure and a decrease in the episodes of pulmonary infections. NPPV may have an advantage in adults with Williams-Campbell syndrome who have severe respiratory failure and recurrent pulmonary infections.

Adenotonsillectomy is a commonly performed procedure that can greatly change airway pressure in patients with obstructive sleep patterns related to enlarged tonsils and adenoids. A case is presented in which a patient with a rare subclinical form of Williams-Campbell syndrome died after outpatient adenotonsillectomy. This case report illustrates how patients with structural abnormalities of the tracheobronchial tree can be at increased risk for complications when undergoing surgical procedures that impact airway dynamics. Arch Otolaryngol Head Neck Surg. 1996;122:1007-1010

Background: Williams-Campbell syndrome is a rare type of bronchiectasis that is due to deficiency or absence of cartilage in the fourth- to sixth-order bronchi. Case Report: The paper presents the case of a patient with large, bilateral bronchiectasis caused by defect of cartilage in the fourth- to sixth-order bronchi referred to as Williams-Campbell syndrome. Conclusions: Williams-Campbell syndrome should be taken into consideration in differential diagnosis of bronchiectasis. Both inspiratory and expiratory high-resolution computed tomography should be performed to establish the diagnosis.

We performed anesthetic management for a patient with severe Williams-Campbell syndrome, which is a congenital deficiency of cartilage in the subsegmental bronchial tree. An 11-year-old girl with this syndrome had labored breathing because of abdominal distension caused by a giant ovarian tumor, and removal of the tumor was scheduled. Because she had been receiving home oxygen therapy for 10 years due to hypoxia, it was possible that positive-pressure ventilation may have increased the risk of perioperative pulmonary complications. So we selected combined spinal and epidural anesthesia with general anesthesia, using a ProSeal (Laryngeal Mask Company, Henley on Thames, UK) laryngeal mask airway. We placed an epidural catheter and induced spinal anesthesia blockade under general anesthesia as the main analgesia technique, in order to maintain spontaneous breathing. The surgery was completed uneventfully and the patient emerged from anesthesia without dyspnea. She had an uneventful recovery and was discharged home.

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results.

Williams-Campbell syndrome, a rare disorder, is characterized by a congenital deficiency of cartilage, typically involving the fourth to the sixth order bronchi, and resulting in expiratory airway collapse and bronchiectasis. The authors report a patient with Williams-Campbell syndrome with type II respiratory failure due to extensive cystic bronchiectasis and secondary emphysema. CT of the thorax showed the affected bronchi had characteristic ballooning on inspiration and collapse on expiration. Three-dimensional images of the tracheobronchial tree were constructed from a volume of data acquired by thin-slice CT scanning. Apart from confirming expiratory collapse of the affected bronchi, these images revealed an absence of the cartilage ring impressions in the bronchial wall, extending bilaterally from the mainstem down to subsegmental bronchi, suggesting cartilage deficiency.

Williams-Campbell syndrome is a cystic bronchiectatic disease secondary to deficiency or defect of cartilaginous plates in the wall of the airways. In the literature, two main forms are suggested: congenital and acquired (post-infectious). The most frequent symptoms are represented by recurrent pulmonary infections from childhood. Multislice spiral dynamic CT has a major role in the study of cystic pulmonary disease and in differentiating Williams-Campbell syndrome from the other causes of cystic bronchiectasis, in which even lung function tests can give deceptive results.

Williams-Campbell syndrome is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi leading to distal airway collapse and bronchiectasis. We report the first case of lung transplantation in a patient with end-stage lung disease secondary to Williams-Campbell syndrome. Although the patient did not have proximal airway collapse prior to transplantation, his posttransplant course was complicated by the development of bronchomalacia of the right and left mainstem bronchi. The patient experienced recurrent pulmonary infections and died of bacterial pneumonia 1 year after transplantation. Autopsy revealed cartilage deficiency in both right and left mainstem bronchi. A hypothesis may be made that a combination of proximal cartilage deficiency and posttransplant airway ischemia led to the development of bronchomalacia after lung transplantation. Thus, in contrast to previous reports, the cartilage deficiency in Williams-Campbell syndrome can involve both proximal and distal airways. Consequently, bilateral sequential lung transplantation may not be an effective therapeutic option in patients with this syndrome.

A 59-year-old man presented with a 4-year history of productive cough, shortness of breath and wheeze. He had been treated for asthma and given several courses of antibiotics which improved his symptoms. Medical history was unremarkable. There was no history respiratory disease in childhood although he was prone to chest infections in adult life. A high-resolution chest CT showed marked proximal cystic bronchiectasis associated with collapse of distal bronchi on expiration. A diagnosis of cystic bronchiectasis due to undiagnosed adult Williams-Campbell syndrome was made on the basis of these characteristic radiological features and the exclusion of other possible causes.

Introduction 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. These are all characteristic of Williams-Campbell syndrome. Case presentation 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-Campbell syndrome, which was undiagnosed in the patient until then. Conclusion 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 was first described in 1960 as a rare form of bronchiectasis. Its pathogenesis is characterized by the absence or markedly diminished cartilage around the bronchi. Although the familial nature was postulated early on, only one possible familial Occurrence has been reported in the literature. We present two family members in whom respiratory symptoms developed within the first year of life and were found to have histopathologic changes consistent with Williams–Campbell syndrome. This would lend further support of a developmental origin for this type of bronchiectasis. Pediatr Pulmonol. 1993; 16:263–267. © 1993 Wiley-Liss, Inc.

The cause of the Williams Campbell syndrome (bronchomalacia with bronchiectasis) is controversial. A boy with bronchomalacia, bifid ribs, and left bronchial isomerism presented with intractable wheeze mimicking asthma. The combination of the abdominal, bronchial, and atrial anatomy seen in this child has been described only once previously. The coexistence of these congenital abnormalities in this boy supports a congenital cause for the Williams Campbell syndrome. The need to assess wheezy children critically is emphasised.

Williams-Campbell syndrome is a rare type of cystic bronchiectasis that is due to defective cartilage of fourth-to sixth-order bronchi. The diagnosis has been made either at autopsy or by bronchographic findings. The article describes an adult case of this syndrome with CT findings that are characteristic of this entity. CT showed areas of emphysematous lung parenchyma distal to the dilated bronchi, suggesting bronchiolitis obliterans secondary to proximal cystic bronchiectasis. These findings are useful in differentiating Williams-Campbell syndrome from other causes of cystic bronchiectasis, thus obviating bronchography.

The CT appearance of bronchiectasis on inspiration and expiration in a patient with congenital bronchiectasis due to cartilage deficiency (Williams-Campbell syndrome) is reported. Computed tomography demonstrated characteristic findings of this syndrome, ballooning of bronchi on inspiration and collapse on expiration.

The clinical, radiological, and physiological findings in 16 patients with extensive bronchiectasis due to deficiency in bronchial cartilage are discussed. These features do not occur in other types of bronchiectasis and comprise a distinctive syndrome. The main clinical features are persistent cough and wheezy breathing. The symptoms are first noticed in infancy often after mild respiratory infection, but at times after measles or pneumonia. Most of the children develop chest deformity and clubbed fingers, and are small in stature. The radiological findings are pulmonary hyperinflation, air-filled dilated bronchi, and, bronchographically, the bronchi balloon during inspiration and collapse with expiration. Physiologically the outstanding features are gross increase in residual lung volume, severe impairment of maximum expiratory flow rates, especially at low lung volumes, a marked decrease in conductance in the upstream segment, poor static elastic recoil, and dynamic compliance which is frequency dependent. It is postulated that this syndrome is due primarily to extensive developmental deficiency of bronchial cartilage, with secondary recurrent respiratory infection.