BRONCHIECTASIS IN CHILDREN: CRACKING THE CODE OF THEORY

Second post in series" cracking the code of theory" ready to go online. The topic of Bronchiectasis is not only usefull in theory but in practicals as well.


By Dr Ranjith kumar CS
He is Currently persuing DM in medical oncology from JIPMER, completed DNB from Kanchi Kamakoti Child Trust Hospital with a GOLD MEDAL in his hand, has great academic interests and contributed about 9 chapters  in scott pediatriks clinical methods in 3rd edition as author and is one of the co-author at dnbpediatrics.com

Bronchiectasis a disease characterized by irreversible abnormal dilatation of the bronchial tree.

In children with bronchiectasis (not due to cystic fibrosis), the male to female ratio is 2 : 1.

Etiology and Pathogenesis:

Infections:
Bordetella pertussis, measles, rubella, togavirus, respiratory syncytial virus, and Mycobacterium tuberculosis

Non –Infectious :
Ciliary dyskinesia, immune deficiency syndromes

Congenital:
1. Williams-Campbell syndrome, in which there is an absence of annular bronchial cartilage

2. Marnier - Kuhn syndrome (congenital tracheo bronchomegaly), in which there is a connective tissue disorder.
3. Right middle lobe syndrome (chronic extrinsic compression of right middle lobe bronchus by
hilar lymph nodes) 
4. yellow nail syndrome (pleural effusion, lymphedema, discolored nails).

Pathology:
Three basic mechanisms are involved in the pathogenesis of bronchiectasis

1.Obstruction can occur because of tumor, foreign body, impacted mucus caused by poor mucociliary clearance, external compression, bronchial webs, and atresia
2.Infections
3.Inflamation

The common thread in the pathogenesis of bronchiectasis is difficulty clearing secretions and recurrent infections with a “vicious cycle” of infection and inflammation resulting in airway injury and remodeling.

Bronchiectasis can present in any combination of three pathologic forms, and defined by high-resolution CT (HRCT) scan( C.V.S)

Cylinder Bronchiectasis: 
The bronchial outlines are regular, but there is diffuse dilatation of the bronchial unit. 
The bronchial lumen ends abruptly because of mucous plugging. 

Varicose Bronchiectasis:
The degree of dilatation is greater and local constrictions cause an irregularity of outline resembling varicose veins. 
There may also be small sacculations.

Saccular Bronchiectasis:
Bronchial dilatation progresses and results in ballooning of bronchi that end in fluid- or mucus-filled sacs. 
This is the most severe form of bronchiectasis
The following definitions have been proposed: 
prebronchiectasis (chronic or recurrent endobronchial infection with nonspecific HRCT changes)
HRCT bronchiectasis (clinical symptoms with HRCT evidence of bronchial dilation—may persist, progress, or improve and resolve)
established bronchiectasis (like the previous but with no resolution within 2 yr)

Clinical Menifestation
1.The most common complaints are cough and copious purulent sputum production. 
2.Younger children may swallow the sputum. 
3.Hemoptysis is seen with some frequency. 
4.Fever can occur with infectious exacerbations. 
5.Anorexia and poor weight gain may occur.

Physical Menifestation:
1.Typically reveals crackles localized to the affected area, but wheezing as well as digital clubbing may also occur.
2.In severe cases, dyspnea and hypoxemia can occur. 
3.Pulmonary function studies may demonstrate an obstructive, restrictive, or mixed pattern. 
4.Impaired diffusion capacity is a late finding

Diagnosis: 

CXR : 
1.Chest radiographs of patients with bronchiectasis tend to be nonspecific.
2.Typical findings can include increase in size and loss of definition of bronchovascular markings,
crowding of bronchi, loss of lung volume. 
3.In more severe forms, cystic spaces, occasionally with air-fluid levels and honeycombing, may occur. 
4.Compensatory overinflation of unaffected lung may be seen

HRCT:
1.Thin-section HRCT scanning is the gold standard, because it has excellent sensitivity and specificity.
2.CT provides further information on disease location, presence of mediastinal lesions, and the extent of segmental involvement.
3.The addition of radiolabeled aerosol inhalation to CT scanning can provide further information.
4.The CT findings in patients with bronchiectasis typically include cylindrical (“tram lines,” “signet ring appearance”), varicose (bronchi with “beaded contour”), cystic (cysts in “strings and clusters”), or mixed forms.
5.The lower lobes are most commonly affected.

Trearment:

1.The initial therapy is medical and aims at decreasing airway obstruction and controlling infection. 
Chest physiotherapy (postural drainage), antibiotics, and bronchodilators are essential. 
2.2 to 4 wk of parenteral antibiotics are often necessary to manage acute exacerbations adequately. 
Antibiotic choice is dictated by the identification and sensitivity of organisms found on deep throat, sputum (induced or spontaneous), or bronchoalveolar lavage fluid cultures. 
3.Chronic prophylactic oral (macrolide) or nebulized antibiotics may be beneficial.
4.Any underlying disorder (immunodeficiency, aspiration) that may be contributing must be addressed.
5.When localized bronchiectasis becomes more severe or resistant to medical management, segmental or lobar resection may be warranted.
6.Lung transplantation can also be performed in patients with bronchiectasis

Prognosis 

Earlier recognition or prevention of predisposing conditions, more powerful and wide-spectrum antibiotics, and improved surgical outcomes are likely reasons

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