Bronchiectasis in Children
This is a 2-1/2 year old male with a history of recurrent pneumonia who is now being
hospitalized for symptoms of cough, wheezing and hypoxemia.
His past history is significant for dysmorphic features at birth which led to a diagnosis of
a 5p chromosomal deletion consistent with Cri du Chat syndrome. He had difficulty in the
neonatal period due to recurrent choking episodes and a presumed poorly coordinated swallow.
He was initially discharged on oral feedings; however, he developed several episodes of
pneumonia and reactive airway disease, for which he received treatment. At 15 months of age, an
ambulatory evaluation for chronic aspiration was conducted because of recurrent wheezing. A
modified barium swallow revealed mild dysfunction of the oral phase of swallowing with a delay
in bolus transfer, especially evident with liquids. There was no pooling or aspiration noted. A
nuclear scintiscan revealed eight episodes of gastroesophageal reflux (GER) to the hypopharynx,
without evidence of aspiration. He was treated with cisapride and ranitidine. Despite this, he was
hospitalized 4 more times for lower respiratory exacerbations. He is now being hospitalized for
evaluation and treatment of an exacerbation consisting of coughing, wheezing and hypoxemia. A
pediatric pulmonologist is consulted.
His family members are all healthy except for a family history of asthma.
Exam: T 37, P 97, R 40, BP 99/64, oxygen saturation 91% in room air. Weight 11 kg
(<5%ile). He is alert in no acute distress but he is episodically tachypneic. Auscultation of his
chest reveals heterophonous (small airway) and homophonous (large airway) wheezing with
diffuse fine crackles. Examination of his upper and lower extremities reveals moderate digital
clubbing. A chest radiograph shows bibasilar reticulonodular opacities that have been essentially
unchanged for almost one year, with new right middle lobe disease.
A thin section, high-resolution computed tomography (HRCT) scan of his chest shows
bibasilar thickened and dilated bronchi consistent with cylindrical bronchiectasis. CBC,
electrolytes, UA, sweat chloride, ANA, immunoglobulins, alpha-one antitrypsin and fungal
serology are all negative. Bronchoscopy with bronchoalveolar lavage reveals erythematous,
friable airways with no obvious airway anomalies. Lavage revealed a cell count of 750 per
microliter, of which 105 were red blood cells and the remainder white blood cells (70%
neutrophils and 30% macrophages). Gram stain shows no organisms. Cultures are not definitive.
Staining of the lavage fluid with oil red-O reveals numerous lipid-laden macrophages (a marker
of chronic aspiration). A hemosiderin stain (evidence of chronic bleeding) is negative.
A nuclear scintiscan repeated on cisapride and ranitidine shows mid-esophageal GER. He
is placed on cefuroxime for presumed bacterial bronchitis despite inconclusive cultures. More
importantly, he is also started on nasogastric feeds and is not allowed to take anything by mouth.
Within days, his pulmonary signs and symptoms improve markedly. He later undergoes a Nissen
fundoplication with gastrostomy tube placement. After six months of gastrostomy feedings while
remaining NPO, he is clinically improved, having gained weight and tolerated minor upper
respiratory infections without hypoxemia. Seven months post-procedure, he has a follow up HRCT
scan of his chest that shows near resolution of his bronchiectasis. His digital clubbing eventually
resolves and a chest radiograph shows no suggestion of bronchiectasis.
Bronchiectasis is a chronic lung disease whose pathophysiology is poorly understood. It
has traditionally been considered "permanent and irreversible". Traction of airways from collapsed
surrounding structures, bulging of the airways from retained secretions, weakening of the
bronchial wall by infection or inflammation, or combinations of these factors are all suggested
mechanisms. Single or repeated acute infections, chronic obstruction from congenital anomalies,
tumors, cystic fibrosis, chronic asthma or immunodeficiencies may also predispose a patient to
developing the disease. In addition, repeated airway injury from chronic aspiration, with or
without gastroesophageal reflux (GER), has been implicated as another etiologic possibility.
Bronchiectasis has been termed an "orphan disease" which may not always be considered
in the evaluation of children with obstructive pulmonary disease because it has become relatively
uncommon in the antibiotic era. Fifty years ago, Field studied 160 children with bronchiectasis
for almost 2 decades. In that period, she documented a fall in the annual hospitalization rate for
bronchiectasis in five British hospitals (1952-1960) of approximately 48/10,000 to 10/10,000. At
our institution among children of American Military members, there have been 14 pediatric cases
in the past 19 years including the present case, yielding an approximate rate of 0.2/10,000
(unpublished data).
The term "bronchiectasis" has traditionally implied permanent, irreversible alteration in the
anatomy of the airways. It has been classified as cylindrical, varicose or saccular. The diagnosis
should be considered in children with daily, productive cough of longer than 6 weeks duration,
hemoptysis, children with persistent radiographic infiltrates, digital clubbing or isolated, persistent
crackles on auscultation. Traditionally, the diagnosis was made by bronchography (chest
radiograph taken with inhaled contrast), as the plain chest radiograph is relatively insensitive for
the detection of bronchiectasis. Most commonly today, the diagnosis is made by thin-section high
resolution CT scan. The diagnosis is based upon the presence of an internal bronchial diameter
greater than the adjacent pulmonary artery, lack of tapering of the bronchial lumina, and
visualization of the bronchi within 1 cm of the pleura, although the use of the first of these criteria
has been debated.
Bronchiectasis. CT: Markedly dilated bronchi are seen, some with air-fluid levels (yellow arrows), mostly in the right
lung. Chest radiographs: Demonstrate thin-walled, cystic structures in right lower lobe (white arrow), some with air-fluid
levels
(yellow
arrows)
-
The combination of small airway obstruction coupled with chronic inflammation of the
bronchial wall is most likely the mechanism in the development of bronchiectasis. The
inflammation usually results from acute or chronic bacterial infection or "colonization" of the
airways. The majority of cases of bronchiectasis follow severe pneumonia or other lower
respiratory infection. In a series by Field fifty years ago, 24% of cases followed pneumonia, and
33% followed some combination of pertussis and/or measles infections.
Chronic aspiration, either from cricopharyngeal dyscoordination or gastroesophageal
reflux, is a recognized condition that can lead to bronchiectasis in adults and children. It has
probably emerged as a more common cause as antibiotics and vaccinations have diminished the
other infectious etiologies. In addition, bronchiectasis has been shown to be more common in
patients of Polynesian descent. A ciliary defect is thought to be the etiology but has not been
consistently demonstrated. Bronchiectasis has also been reported with increased frequency in
Native Alaskan children, although some theorize that the common thread is a low socioeconomic
level.
Bronchiectasis, particularly the mildest of the pathologic forms, cylindrical bronchiectasis,
may be reversible. When associated with pneumonia, it may resolve with treatment of the acute
process. Resolution of post-obstructive bronchiectasis has even been reported after removal of a
chronic foreign body. Some refer to this as "pseudobronchiectasis".
Clubbing is reported in 37-51% of patients with bronchiectasis. In Field's series of 160
patients with bronchiectasis, clubbing was present in 78 cases (44%). In many cases, the clubbing
cleared after the affected section of the lung was removed surgically. In cases treated medically,
occasionally the clubbing improved and in some cases it disappeared despite persistent
bronchographic evidence of bronchiectasis. Of interest, Field concluded, "clubbing when present,
usually signified irreversible bronchiectasis, providing there was no congenital cardiac lesion".
In the same patient series, Field also described the condition of "reversible bronchiectasis,"
a temporary dilation of airways in areas of the lung that had been collapsed due to infection or
atelectasis. She suggested that duration of cough and pulmonary symptoms was commonly of
three months duration or less in children with reversible bronchiectasis and pulmonary collapse
which reexpanded. In the same series, the majority of children with a history of symptoms for two
years or more generally developed severe bronchiectasis.
The evaluation of a child with bronchiectasis should include an evaluation for any
identifiable cause of the condition. Testing for cystic fibrosis with a sweat chloride test as well as
genotype determination should be performed in select cases. Children in their first two decades
do not usually develop lung disease from alpha-one protease inhibitor deficiency, but a level
should be obtained. Other congenital conditions such as Mounier-Kuhn (congenital absence of
airway muscle) and Williams-Campbell (congenital absence of airway cartilage) can usually be
suspected from the radiograph and CT scan. Marfan's, Ehlers-Danlos, and the Yellow-Nail
syndrome all have other phenotypic findings.
Post infectious causes of bronchiectasis are harder to prove. The child should have a PPD
with anergy panel to assess for TB. Evaluation for allergic aspergillus or allergic fungal disease
should be considered. Serum levels for pertussis, measles and adenovirus are probably not helpful
in a child with bronchiectasis because of immunization and the possibility of previous, unrelated
disease.
Basic aspects of the immune system should be evaluated including serum immunoglobulins
and serum IgE. There are cases of bronchiectasis preceding other symptoms of rheumatic disease,
sometimes by decades, so an anti-nuclear antibody and rheumatoid factor should be obtained.
Finally, a thorough evaluation for gastroesophageal reflux should be undertaken including
extended intraesophageal pH probe monitoring.
Children with bronchiectasis should be treated with antibiotics during symptom
exacerbations based upon sputum culture results. Exacerbations should also be treated with
increased frequency of the daily regimen of chest physiotherapy and postural drainage, usually
conducted twice a day. Intense aerobic exercise is another, excellent means of pulmonary toilet.
Bronchodilators are indicated where there is evidence of bronchial hyper-reactivity. There may
also be a role for inhaled corticosteroids to modulate the host response and curb inflammatory
damage to the lung. Therapy for an identified cause of bronchiectasis should be undertaken,
including aggressive medical and perhaps even surgical therapy for gastroesophageal reflux.
When damage is severe and well localized, pulmonary segmental resection may be
beneficial. However, Field demonstrated a gradual symptomatic improvement of children who
did not have surgical therapy for bronchiectasis, even before the proliferation and availability of
broad spectrum antimicrobials. Lewiston recommended that surgery be delayed unless
symptomatically necessary, until the patient is 6-12 years, because of the possibility of clinical
improvement.
Bronchiectasis has become an uncommon disease in the developed world, but it may often
be unrecognized. It should be suspected in children with chronic respiratory symptoms, since it is
often amenable to long-term medical management. Surgical therapy should be reserved for
situations of recurrent pulmonary sepsis unresponsive to aggressive medical management.
Questions
1. True/False: Causes of bronchiectasis in childhood include cystic fibrosis, asthma and
immunodeficiency.
2. True/False: Bronchiectasis has been traditionally classified as round, cylindrical or
cavitating.
3. True/False: Most commonly today, bronchography is required for the diagnosis.
4. True/False: Chronic aspiration is a recognized cause of bronchiectasis in children.
5. True/False: Children of Polynesian descent are at no increased risk of bronchiectasis.
6. True/False: Therapy for bronchiectasis in children includes early surgical resection.
Answers to questions
1. true
2. false
3. false
4. true
5. false
6. false