Reversible bronchiectasis caused by influenza virus mimicking Williams-Campbell syndrome.

The term bronchiectasis refers to permanent enlargement of the bronchi. It is increasingly diagnosed because of high-resolution computed investigations. It can be congenital or acquired, the latter mostly following infection. Williams-Campbell syndrome is a rare form of congenital non-cystic fibrosis bronchiectasis. Here we report a 5-month-old girl with reversible bronchiectasis treated with extracorporeal membrane oxygenation for acute respiratory distress syndrome (ARDS) caused by influenza virus following surgery for congenital heart disease. Chest CT showed an abnormally large bronchial tree mimicking Williams-Campbell syndrome. At 9 months later, chest CT showed regression of bronchiectasis and normalized caliber of previously collapsed segments in both lungs. This atypical course illustrates that influenza virus can cause reversible bronchiectasis in infants and mimic congenital disease such as Williams-Campbell syndrome.

Airway Malformations and Bronchiectasis: A Pediatric Study.

Congenital airway malformations are most often identified in early childhood. The development of bronchiectasis in association with malformations of the lower airway has been described, particularly among adult patients. The coexistence in a pediatric population of these conditions is not well described. This study was conducted to identify whether younger patients with airway malformations commonly develop bronchiectasis. International Classification of Diseases, Ninth revision (ICD-9 codes) were defined for airway anomalies and bronchiectasis. The electronic medical records system of a children's hospital was then searched for the number of patients with upper airway anomalies with or without bronchiectasis. The airway database was then cross referenced with the ICD codes for bronchiectasis to identify patients with both conditions. There were 844 patients with airway anomalies and 117 with bronchiectasis in the electronic system during the time period of August 1, 2009 to September 30, 2014. There was only 3 patients identified with both bronchiectasis and airway anomalies. The coexistence of bronchiectasis is low among the pediatric population with upper airway anomalies studied. This would suggest that the children with airway anomalies have been treated with strategies that are effective in the prevention of recurrent lower respiratory tract infection. Further study may be done to define the effectiveness of various strategies in preventing aspiration and lower respiratory tract infection. In addition, this methodologic technique utilizing database integrative platforms is useful in the identification of patients for further study and to identify the coexistence of pediatric conditions.

Traqueobroncomegalia y bronquiectasias en el síndrome de Mouniere-Kuhn. Colonización por Pseudomonas aeruginosa y antibioterapia inhalada / Tracheobronchomalacia and bronchiectasis in the Mouniere-Kuhn syndrome. Pseudomonas aeruginosa colonization and inhaled antibiotic therapy

El síndrome de Mouniere-Kuhn se caracteriza por bronquiectasias (BQ) congénitas asociadas a traqueobroncomegalia. Es una enfermedad poco prevalente en la población general (1-4,5%), siendo infrecuente el diagnóstico en la edad adulta. Las infecciones respiratorias constituyen una complicación frecuente en su evolución y entre ellas, la infección y colonización por Pseudomona aeruginosa. La terapia antibiótica inhalada con colistina, tobramicina, etc. ha demostrado su eficacia en pacientes con BQ asociadas a fibrosis quística. La instauración de este tratamiento en nuestro caso se asoció con una mejoría en la sintomatología clínica y reducción de las exacerbaciones (AU)

Mounier-Kuhn syndrome is characterized by congenital bronchiectasis (BC) associated to tracheobronchomegaly. It is a disease of little prevalence in the general population (1-4.5%), its diagnosis being uncommon in the adult age. Respiratory infections are a frequent complication in its course and, among them, infection and Pseudomona aeruginosa colonization. Inhaled antibiotic therapy with colistin, tobramycin, etc. has demonstrated its efficacy in patients with cystic fibrosis bronchiectasis. The establishment of this treatment in our case was associated with an improvement in the clinical symptoms and reduction of the deteriorations (AU)

The role of spiral multidetector dynamic CT in the study of Williams-Campbell syndrome.

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.

CT bronchoscopy in the diagnosis of Williams-Campbell syndrome.

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.

Malformaciones broncopulmonares / Bronchopulmonary malformations

La malformación congénita broncopulmonar es una entidad clínica poco frecuente y de difícil diagnóstico, destacan entre los métodos de apoyo la radiología, la tomografía axial computada, la angiografía, el Doppler color y la ultrasonografía. Se presenta una revisión retrospectiva de 10 años con 21 casos de los cuales 13 corresponden a niños menores de 15 años y 8 adultos. Los quistes broncógenos son los más frecuentes (6-28,5 por ciento). En segundo lugar la bronquiectasia quística congénita (5-23,8 por ciento), la malformación adenomatoide quística (4-19 por ciento), el secuestro pulmonar (3-14,2 por ciento), la aplasia pulmonar (2-9,5 por ciento) y la fístula arteriovenosa (1-5 por ciento). Todos fueron intervenidos a excepción de los 2 pacientes portadores de aplasia pulmonar. Se realizaron 12 lobectomías y 7 segmentectomías. Sólo un caso debió reintervenirse por un hemotórax y no hubo mortalidad. El seguimiento ha mostrado excelentes resultados alejados, habiéndose intervenido uno de ellos posteriormente de un ductus persistente

[An adult case of Williams-Campbell syndrome associated with pulmonary hypertension and a severe decrease in ventilatory response].

Williams-Campbell syndrome is a unique form of bronchiectasis caused by a congenital defect in bronchial cartilage, and is rare in Japan. A 34-year-old man was admitted to our hospital with a fever, and a productive cough. Arterial blood gas analysis revealed severe type II-respiratory failure. Many thin-walled cystic shadows (5-60 mm in diameter) were present in the entire lung field. Pulmonary function tests revealed obstructive impairment. Bronchograms demonstrated cystic bronchiectasis, with ballooning on inspiration and collapse on expiration, characteristic of Williams-Campbell syndrome. Despite severe hypoxia, he did not suffer from dyspnea. We examined ventilatory response to hypercapnea (HCVR) and hypoxia (HVR), and both HCVR and HVR were abnormal. In addition, the mean pulmonary artery pressure was 26 mmHg, indicating pulmonary hypertension.

Mortality following adenotonsillectomy in a patient with Williams-Campbell syndrome.

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.

[Left pneumonectomy in a child with congenital bronchiectasis]. / Pneumonectomia sinistra in un bambino affetto da bronchiectasie congenite.

A case of diffuse bronchiectasis concerning an entire lung in an eight-year-old boy is described. The disease in this case was widespread in the entire left lung and was considered as consequent to a defect in the development of the left bronchial wall. Because of the frequent respiratory infections, occurring since birth, and given the good anatomical and functional conditions of the right lung, we recommended the surgical excision of the sick lung. After one year, the operation showed good results with regard to respiratory functions. The functioning of the remaining pulmonary parenchyma is quite satisfactory and allows the boy an excellent quality of life.

Multiple endobronchial chondromatous hamartoma in a child.

The multiple endobronchial chondromatous hamartoma is a very rare tumour. The authors report a case of a 5-year-old girl treated by pneumonectomy.

Lobar emphysema and atelectasis syndrome, a nosological unity.

Shrinking and atelectatic pulmonary processes--atelectasis syndrome--and lobar emphysema show morphological similarities: congenital malformations of the bronchial structure, external compression of the bronchi as well as inflammatory processes. Ventilation mechanics determine whether in the described processes an atelectasis or an emphysema manifests itself clinically. Usually, one finds over-expansions in connection with atelectasis syndrome and atelectases together with lobar emphysema. Both processes can be reversible, if we are not dealing with bronchial malformations. Longer lasting processes as well as acute, life threatening forms require operation. The belief that atelectasis syndrome and lobar emphysema constitute a nosological unity is illustrated by our cases of 9 patients with atelectasis syndrome and 16 patients with lobar emphysema.

Familial congenital bronchiectasis: Williams-Campbell syndrome.

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 world lend further support of a developmental origin for this type of bronchiectasis.

Surgery for congenital malformations of the lung.

In the course of a survey conducted in 59 hospitals performing thoracic surgery, 14 hospitals supplied data that could be used for the study. Out of 1347 anomalies diagnosed 1343 were surgically treated, with a 30-day mortality rate of 0.3% (5 patients). In a retrospective study over a period of 10 years (1978-1988) we identified 198 anomalies out of a total of 6350 thoracotomies; so our percentage grading of pulmonary anomalies is supported by the data of the above-mentioned survey according to which cystic pulmonary malformations such as inhibition malformations, excess malformation and lobar emphysema represent a majority with 72.2% (survey 83%). Congenital anomalies of lung formation occurred in 23% of the patients of the survey and in 15% of our own patients. Therapy consisted of parenchyma-saving surgery, i.e. enucleation (n = 87), segmental resection (n = 65) and lobectomy (n = 63) with bronchoplastic reconstruction; there was no 30-day mortality. Adenomatoid-cystic malformation, lymphangiectasis, congenital lobar emphysema and stenosis of the tracheobronchial tree are often an indication for immediate surgical treatment in neonates. Solitary cysts, bronchiectasis, sequestration of the lung, an AV-fistula present with symptoms mostly between the ages of 20-40 and therefore were surgically treated secondarily.