Indian J Pediatr (October 2015) 82(10):938–944
DOI 10.1007/s12098-015-1866-4
REVIEW ARTICLE
Non Cystic Fibrosis Bronchiectasis
Anand K. Gupta 1 & Rakesh Lodha 1 & Sushil K. Kabra 1
Received: 11 May 2015 / Accepted: 28 July 2015 / Published online: 27 August 2015
# Dr. K C Chaudhuri Foundation 2015
Abstract Bronchiectasis is a pathological abnormality of the
airways in which there is permanent dilatation and thickening
of the airways. Precise incidence/prevalence in India is not
known. Recent data suggests that about 1 % young children
admitted in a hospital with pneumonia may develop bronchi-
ectasis. Due to significant burden of pneumonia in young
children in developing countries including India, it may be a
significant problem that is possibly under recognized. Causes
of bronchiectasis depend on the burden of respiratory infec-
tions and availability of the investigations for identification of
the underlying cause. Post infectious causes are common in
countries where infections are more common; however, since
these countries are usually resource constrained and therefore,
are not able to appropriately diagnose the other causes, leading
to more than real overrepresentation of infections as a cause.
In countries with less of infectious illnesses and good diag-
nostic facilities, malformations of airways, immune deficiency
disorders and primary ciliary dyskinesia are common causes
of bronchiectasis. High resolution CT scan of chest confirms
the diagnosis. Treatment is supportive care and consists of
maintenance of nutrition, airway clearance and antibiotics
for exacerbations. Medical treatment is successful in the
majority.
Keywords Aspiration syndrome . Bronchiectasis . FENO .
Primary ciliary dyskinesia
* Sushil K. Kabra
skkabra@hotmail.com
1
Department of Pediatrics, All India Institute of Medical Sciences,
New Delhi 110029, India
Introduction
Bronchiectasis is a pathological term which denotes perma-
nent dilated and thickened airways as a result of chronic in-
flammation and recurrent infection due to multiple underlying
illnesses.
Precise burden of bronchiectasis is not known. Various esti-
mates indicate variation in incidence and underlying illness in
different geographic regions. Data from England and Wales
suggests that between 2001 and 2007, mortality rates due to
bronchiectasis in adults increased at 3 %/year [1]. Reports from
New Zealand and Australia suggest an increasing health burden
of chronic suppurative lung disease in children as well as adults.
A survey in New Zealand estimated an overall incidence of 3.7
per 100 000 in under 15-y-old children per year [2]. In Central
Australian indigenous population, prevalence in children below
15 y was estimated to be 1470/100,000 [3].
There is no data on magnitude of problem of bronchiectasis
from the developing countries. General impression is that it is a
common cause of morbidity and mortality in the developing
countries.
A systematic review on long term sequal of pneumonia es-
timated bronchiectasis in 0.9 % (0.7–8.7 %) children hospital-
ized with pneumonia [4]. In a systematic review in India, num-
ber of children between 0 and 4 y were calculated as 12,79,60,
004 and a total of 3,53,61,230 episodes of pneumonia were
estimated per year. Out of this about 11 % progressed to severe
pneumonia [5]. It may have resulted in severe pneumonia in 38,
89,735 children. The incidence of bronchiectasis may vary
from 27,22,814 to 3,38,40,694 per year due to pneumonia
alone. It gives a figure of 212 to 2646 children developing
bronchiectasis per one million children below 4 y of age.
These calculations are based on estimates and assumptions,
may not indicate real life scenario, but suggest that bronchiec-
tasis is not uncommon and is possibly underdiagnosed.
Indian J Pediatr (October 2015) 82(10):938–944
Pathophysiology
Bronchiectasis is dilatation and thickening of the airways. In
pre-existing abnormalities (congenital or acquired), recurrent
infection and inflammation leads to bronchial wall destruc-
tion. Recurrent infection/inflammation may result in impaired
mucociliary clearance of purulent and inflammatory material.
Bronchoscopic biopsies and bronchoalveolar lavage analysis
has helped in understanding the mechanisms of development
of bronchiectasis [6, 7]. It has been suggested that the key
factor in the development of bronchiectasis is bronchial infec-
tion and inflammation. It has been identified that infection
leads to mucosal infiltration by neutrophils and T lympho-
cytes; which in turn results in increased concentrations of
inflammatory elements such as neutrophil elastase, interleu-
kin-8, tumor necrosis factor-alpha, and prostanoids. A com-
plex interaction between these mediators results in permanent
dilatation and thickening of the airways i.e., bronchiectasis
[6–8]. It is suggested that underrecognition and
undertreatment of protracted bacterial bronchitis (PBB) which
is persistence of bacterial infection of airways in children for
more than 4 wk may be responsible for the development of
bronchiectasis in the developing countries as well as vulnera-
ble children in the developed countries [9].
Etiology/Underlying Illnesses in Children
There are many causes underlying the development of bron-
chiectasis. They can be classified into two major groups: con-
genital problems and acquired problems [10–15]. A list of
underlying illnesses is given in Table 1 and comparison of
various underlying causes from different parts of the world
is described in Table 2.
Clinical Manifestations
Clinical manifestations of bronchiectasis are variable and de-
pend on the causes and extent of the illness. There is no uni-
form reporting of the clinical symptoms. A comparison of
presenting clinical symptoms is given in Table 3. A recent
study from India [15] reported 80 children with bronchiecta-
sis. Common manifestations were: Cough (96 %), breathless-
ness (81 %), expectoration (66 %), fever (62 %), wheezing
(52 %), repeated pneumonia (46 %) hemoptysis (16 %)
and failure to thrive (70 %). Common manifestations
include: Chronic cough, wheezing, exercise intolerance
and recurrent chest infections. Advanced disease may
manifest with failure to thrive, clubbing and pulmonary
arterial hypertension.
939
Diagnosis
Diagnosis of bronchiectasis should be suspected in children
presenting with any of the following clinical symptoms:
Persistent wet/moist cough lasting for more than 8 wk and
persisting in between colds, asthma not responding to appro-
priate treatment, persistence or recurrence of symptoms of
pneumonia, pertussoid cough not resolving even after 6 mo,
persistent crepitations in the chest without obvious explanation,
recurrent respiratory problems with associated esophagus and
upper respiratory illnesses and unexplained hemoptysis [16].
Imaging has remained a diagnostic tool for bronchiectasis.
A few decades ago, bronchiectasis was suspected on abnormal
findings in X-ray film and confirmed by bronchography. X-
ray film of chest (CXR) lacks specificity. Abnormalities on X-
ray film suggestive of bronchiectasis include: prominent
bronchovascular markings, dilated bronchus, loss of lung vol-
ume and peribronchial thickening. Now high resolution CT
(HRCT) has replaced bronchography and is considered gold
standard [17, 18]. Contrast enhanced chest CT (CECT) may
be considered if reactivation of tuberculosis or mass lesion in
mediastinum, including lymphnodes are suspected.
Findings in HRCT suggesting bronchiectasis include: dilata-
tion of airway lumen more than nearby vessel; lack of tapering
towards periphery; bronchial wall thickening; cylindrical, vari-
cose, and/or saccular changes with local or diffuse disease [19,
20]. Findings on HRCT may give clue about underlying illness.
Central bronchiectasis suggests allergic bronchopulmonary as-
pergillosis (ABPA), cystic fibrosis or recurrent aspirations.
With advanced technology, HRCT may detect subtle ab-
normalities, including dilatation of airways during acute pneu-
monia, aspiration or foreign body aspiration. These changes
may resolve in repeat HRCT after 6–8 wk [21, 22]. In some
patients bronchial dilatation may persist static or may show
resolution; [11, 23] though it is difficult to differentiate natural
resolution or resolution due to aggressive treatment of bron-
chiectasis. It is suggested that the term prebronchiectasis may
be used in those which resolve over the period of time [24].
Performance of Magnetic resonance imaging (MRI) of
chest and HRCT chest in picking up findings like
peribronchial wall thickening, mucous plugging and bronchi-
ectasis was assessed to be similar [25]. MRI is not associated
with exposure to radiation but needs longer duration of seda-
tion or general anesthesia. HRCT can be done quickly with
minimal or no sedation but is associated with radiation expo-
sure. With more experience and improvement in technology,
MRI may find a place in imaging of pediatric chest diseases.
Investigations for Identification of Underlying Cause
The aim of laboratory investigations for confirmation of the
underlying cause is to administer specific treatment.
940 Indian J Pediatr (October 2015) 82(10):938–944

Table 1 Underlying causes of

bronchiectasis in children Congenital causes Acquired causes

Malformations Post infectious following

Airway abnormalities: Severe pneumonia
Laryngotracheobronchomalacia Measles
H-type tracheoesophageal fistula Pertussis
Laryngeal cleft Tuberculosis
Congenital cartilage abnormalities
Pulmonary sequestration Obstruction of airways
Foreign body aspiration
Problems of airway clearance Intra or extra luminal compression
Primary ciliary dyskinesia Lymphnode
Cystic fibrosis Tumor mass
Alpha-1 antitrypsin deficiency Cysts
Vascular ring
Primary immune deficiency
B cell deficiencies Allergy and inflammation
Pan hypogammaglobulinemia Allergic bronchopulmonary aspergillosis (ABPA)
Common variable immune deficiency Rheumatic and autoimmune disorders
Phagocytic defects: CGD
Hyper IgE syndrome Environmental causes
Ataxia telengiectasia Toxic inhalation

Acquired immune deficiency disorders

HIV infection
Chemotherapy
Long term steroids

CGD Chronic granulomatous disease

Bronchoscopy: 3–15 % of underlying causes are
malformations of airways. Fiber optic bronchoscopy helps in
identification of airway abnormalities and obtaining samples
from the airways. It can identify airway abnormalities like
laryngotracheomalacia very well [26].
Work-up for immune deficiency disorders: 6–34 % of un-
derlying causes are immune deficiency. Common causes are
pan hypogammaglobulinemia (common variable immune de-
ficiency, X-linked hypogammaglobulinemia or isolated IgA
deficiency). Therefore, serum immunoglobulin profile (IgG,

Table 2 Underlying illnesses of non Cystic fibrosis bronchiectasis

Li et al. 2005 [10] Karakoc Eastham Nikolaizik Karadag Kumar

et al. 2001 [11] et al. 2004 [12] et al. 1994 [13] et al. 2005 [14] et al. 2015 [15]

Total numbers studied 138 23 93 41 111 80

Location UK Turkey UK London Turkey India
Post infectious 3.7 35 30 29.7 23.8
Primary ciliary dyskinesia 14 13 1 17 6.3 15
Allergic bronchopulmonary - - - - - 7.5
aspergillosis
Immune deficiency 33.8 17.4 21 27 15.3 6.2
Aspiration syndrome 18.4 3 5
Congenital malformations 3.7 5 15 3.7
Others - 17.41 92 3.63 4.84
Idiopathic 25.7 18 37 37.8 36.2
1
Asthma, 2 Obliterative bronchiolitis, 3 Foreign body aspiration, 4 Includes asthma, HIV, foreign body (FB) aspiration
Indian J Pediatr (October 2015) 82(10):938–944 941

Table 3 Clinical manifestations of children with bronchiectasis Diagnosis of PCD is confirmed by demonstration of
Li et al. Karadag Kumar abnormal ciliary structure on electron microscopy [28].
2005 [10] et al. et al. Identification of genetic mutation may be alternative di-
2005 [14] 2015 [15] agnostic test in future. At present, it is expensive as
there are a high number of PCD genes and only 60 %
Total numbers studied 138 111 80
of cases can be identified by genetic testing. Diagnostic
Geographic region UK Turkey India
facilities may not be available at all the centers, there-
Chronic cough 34.6 96.9 96 fore it is advisable to refer a suspected patient to cen-
Recurrent wheeze 10.3 46.9 52 ters that have diagnostic facilities [28].
GERD 8.1 - - For diagnosis of allergic bronchopulmonary aspergillosis,
Rhinitis 5.1 - - recently a combination of clinical and lab parameters has been
Recurrent otitis media 5.1 - - suggested [30].
Failure to thrive 4.4 - -
Exercise intolerence 1.5 49 81
Recurrent chest infection 77.2 - 46
Hemoptysis - 10 16 Investigations for Monitoring of Bronchiectasis
Expectoration - 80.6 66
It is important to monitor children with bronchiectasis to as-
GERD Gastroesophageal reflux disease
sess progression of the illness and effect of the treatment. In
All figures are in percentages
older children, spirometry gives objective evidence of airways
and may be repeated every 3–6 mo. In younger children, clin-
IgM, IgA) should be estimated in suspected immune deficien- ical monitoring including symptom relief and weight gain
cy disorders. Other investigations may be considered depend- may be used. Pulmonary function test in pre- school children
ing on the suspicion of underlying illness. Hyper IgE syn- are emerging methods and may be available in future.
drome can be confirmed by documenting high levels of serum Periodic sputum examination may give clear idea about
IgE. Human immunodeficiency virus infection can be con- colonization of the airways. It may also help in deciding about
firmed by ELISA test in children above 18 mo of age. If antibiotic regimen for pulmonary exacerbations.
combined immunodeficiency or chronic granulomatous dis- Imaging such as x-ray film of the chest and computerized
ease is suspected, flowcytometry based investigations may tomography (CT) scan is not required routinely. These may be
confirm diagnosis. obtained in pulmonary exacerbations that are not responding
Cystic fibrosis (CF), once considered a disease of to the treatment. If patient has pulmonary hemorrhage that
Caucasian population, is being diagnosed more frequently does not respond to supportive care, a CT angiography for
from other parts of the world including India [27]. It is impor- demonstration of systemic to pulmonary collaterals may be
tant to suspect CF on clinical features and confirm by sweat considered.
test or mutation studies.
Primary ciliary dyskinesia (PCD) is reported to be 1–17 %
in different studies [10–15]. Diagnostic tests for PCD include
screening test and confirmation of the diagnosis. Screening Treatment
test includes saccharine clearance test, rate of clearance of
radiolabelled aerosol from the airways and nasal NO [28]. In Majority of children with bronchiectasis can be managed with
saccharine clearance test, a crystal of saccharine is placed medical treatment. The aim of the treatment includes nutri-
below inferior turbinate bone and the time taken for appreci- tional support, keeping airways clear and treatment of individ-
ation of sweet taste is recorded. It requires cooperation from ual episode of exacerbation with antibiotics.
the child and normal value for children is not available. It is no
more being used. Delayed clearance of radiolabelled aerosol
from the airways has been used in children above 5 y of age General Measures
and results may not be reliable in the presence of cough [29].
The test is associated with exposure to radiation and reference These include maintaining good hydration, adequate nu-
values for children are not available. trition, vaccination against common respiratory patho-
Nasal NO measurement is standard practice in older chil- gens like pertussis, measles, influenza, pneumococcal
dren and adults. Disorders of Mucociliary Clearance infection and HiB infection. Children with bronchiecta-
Consortium (GDMCC) in North America recommend nasal sis should be protected against smoke (biomass burning,
NO measurement as a screening tool for PCD in children [28]. tobacco) and irritants.
942
Medical Treatment
Medical treatment is mainstay of management and consists of
airway clearance, use of bronchodilators and inhaled steroids,
mucolytic agents and administration of systemic and inhaled
antibiotics as indicated.
Airway Clearance
Airway clearance is mainstay of non pharmacologic treatment
and is underutilized. To keep secretions thin, child should be
encouraged to drink plenty of liquids. If there is reactive air-
ways disease, bronchodilators before chest physiotherapy
may help to keep the airways clean.
Mucolytic agents to improve airway clearance has been
advocated for CF as well as Non CF bronchiectasis.
However, there is not much data about their beneficial role
in children. A systematic review including children and adults
with bronchiectasis looked into the efficacy of various inter-
ventions and concluded that there is inconclusive evidence on
the use of long-term antibiotics and nebulised hypertonic sa-
line for reducing exacerbation frequency and similarly no con-
clusive evidence to prove that human deoxyribonuclease
(RhDNase) increases exacerbation frequency [31]. Similarly,
there was no conclusive evidence to support role of hypertonic
saline or mannitol for non CF bronchiectasis. In the same
review, a small (clinically not relevant) beneficial effect was
observed with inhaled steroids [31].
Traditional chest physiotherapy has four components:
Postural drainage, percussion, vibration of the chest wall, and
coughing. A systematic review comparing various techniques
of physiotherapy including active cycle of breathing techniques
(ACBT), forced expiration techniques (FET), autogenic drain-
age, postural drainage, oscillating positive expiratory pressure
(OPep) and high frequency chest wall oscillation (HFCWO)
did not find any randomized controlled trial (RCT) on the
subject. They observed that all the methods worked well
to improve pulmonary function test and health related
quality of life. There is need for RCTs to compare these
techniques in children with non CF bronchiectasis [32].
In view of current evidence, parents of children diagnosed
with bronchiectasis should be encouraged to maintain good
hydration of children, perform daily chest physiotherapy, use
bronchodilators and inhaled steroids if there is an evidence of
reactive airway disease. As of now, there is no conclusive
evidence to support use of mucolytic agents (bromhexin),
hypertonic saline or other osmotic agents.
Antibiotics
Antibiotics should be used with an evidence of pulmonary
exacerbations as indicated by increased expectoration, fever,
weight loss, decrease in FEV1 (if available). Long term inhaled
Indian J Pediatr (October 2015) 82(10):938–944
antibiotics is part of treatment regimen in Cystic Fibrosis. There
are not many reports of use of inhaled antibiotics in non CF
bronchiectasis. Preliminary report suggests beneficial role of
inhaled tobramycin in non CF bronchiectasis [33].
Prophylactic systemic antibiotics, specifically cotrimoxazole
has been used in immune deficiency disorders with benefit. A
recent review on use of prophylactic antibiotics in antibody
deficiency disorder conclude that well-designed multicenter
prospective trials are required to compare benefit of antibiotics
with other management options [34]. Observational studies
using long term oral macrolides (erythromycin, azithromycin)
in patients with frequent pulmonary exacerbations have docu-
mented beneficial effects [35, 36]; there is a need for random-
ized controlled trials to confirm their utility.
Chronic respiratory problems may be associated with gas-
troesophageal reflux disease and may benefit with H2 blockers
or proton pump inhibitors.
Surgical Treatment
Majority of the patients respond well to medical treatment.
Localized disease that is uncontrolled with medical treatment
or localized bleed may be an indication for resection of a lobe
or whole lung. In a recent report from India, 17.7 % children
with bronchiectasis required surgery [15].
Specific Treatment
Children in whom cause is not identified or there is no specific
treatment (post infectious, malformations etc) should be treat-
ed with supportive care. Children with aspiration syndrome
may benefit with surgical intervention (fundal plication in
gross gastroesophageal reflux unresponsive to medical treat-
ment). Those with hypogammaglobulinemia may be treated
with intravenous gamma globulin every 3–4 wk.
Prognosis
With better understanding of pathophysiology and diagnostic
workup, prognosis of bronchiectasis has improved. In a recent
report from India [15], 11 % patients died of pulmonary in-
fections during follow up. Significant proportion of children
had morbidity such as poor growth in 61 (76.3 %), pulmonary
hemorrhage in 13 (16.2 %), chronic hypoxemia in 8 (10 %),
and pulmonary hypertension in 3 (3.8 %). Surgical interven-
tion was needed in 14 (17.5 %) children.
Conclusions
Bronchiectasis is relatively a common cause of respira-
tory morbidity. Causes are varied and depend on the
Indian J Pediatr (October 2015) 82(10):938–944
geographic region and availability of diagnostic work up
of underlying illness. In developing countries, post in-
fectious causes are common while in countries with less
burden of infection, other causes like malformations,
immune deficiency disorders and primary ciliary dyski-
nesia are more common. Bronchiectasis should be
suspected in children presenting with long term wet
cough. Clinical presentation depends on the severity of
illness and underlying cause. Diagnosis is established by
HRCT chest. Treatment is supportive and medical treat-
ment is successful in majority. Localized disease with
uncontrolled bleeding due to systemic to pulmonary col-
laterals is an indication for surgery.
Contributions AKG: Involved in literature search, manuscript writing;
RL: Involved in manuscript writing; SKK: Involved in literature search,
manuscript writing and will act as guarantor for manuscript.
Conflict of Interest None.
Source of Funding None.
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