Bronchiectasis is a disease in which there is permanent enlargement of parts of the airways of the lung.[5] Symptoms typically include a chronic cough with mucus production.[3] Other symptoms include shortness of breath, coughing up blood, and chest pain.[2] Wheezing and nail clubbing may also occur.[2] Those with the disease often get frequent lung infections.[8]
Bronchiectasis may result from a number of infectious and acquired causes, including pneumonia, tuberculosis, immune system problems, as well as the genetic disorder cystic fibrosis.[11][3][12] Cystic fibrosis eventually results in severe bronchiectasis in nearly all cases.[13] The cause in 10–50% of those without cystic fibrosis is unknown.[3] The mechanism of disease is breakdown of the airways due to an excessive inflammatory response.[3] Involved airways (bronchi) become enlarged and thus less able to clear secretions.[3] These secretions increase the amount of bacteria in the lungs, resulting in airway blockage and further breakdown of the airways.[3] It is classified as an obstructive lung disease, along with chronic obstructive pulmonary disease and asthma.[14] The diagnosis is suspected based on symptoms and confirmed using computed tomography.[7] Cultures of the mucus produced may be useful to determine treatment in those who have acute worsening and at least once a year
8. • Saccular bronchiectasis follows a major pulmonary
infection or results from a foreign body or bronchial
stricture and is the main type requiring surgical
attention.
• Cylindrical bronchiectasis consists of bronchi
that do not end blindly but communicate with lung
parenchyma.
• Hood,2 noted a third type referred to as
varicose, a mixture of the former two, distinguished by
alternating areas of cylindrical and saccular
disease.
9. • Pseudobronchiectasis, a term coined by Blades and
Dugan,6 is a cylindrical dilatation of a
bronchus that is temporary and disappears in several
weeks or months and develops after an acute
pneumonic process. This type has no surgical
implications and should be taken into consideration
before
diagnosing a bronchiectasis with single CT finding
especially in a patient without an acceptably long
history of bronchopulmonary symptoms
12. • Certain genetic syndromes may be associated with
some form of bronchiectasis. These include
cysticfibrosis, alpha1-antitrypsin deficiency,
immunoglobulin A (IgA), and, IgG deficiency
14. • The distribution to
some extent is characteristic of the etiology. For
example, in patients with Kartagener syndrome,
hypogammaglobulinemia, and cystic fibrosis, the
areas of involvement are generally diffuse and
bilateral
and involve multiple cystic segments of both upper
and lower lobes. Tuberculosis is either unilateral or
bilateral and generally involves the upper lobes or
superior segment of the lower lobes
16. • PATHOPHYSIOLOGY
The healthy lung protects itself from the continuously inhaled
pathogens with a complex defense system
that consists of antibodies and mucociliary clearance. Any
condition that interferes with the protection
mechanism such as low antibody levels, connective tissue
disorders, or impaired clearance of the
bronchial airway could lead to colonization of pathogens in
bronchopulmonary tissues resulting in further
inflammation. As a result, abnormal dilation of the bronchus
develops with the destruction of the elastic
and muscle layers of the small-sized bronchial airways. In
addition, lack of ciliated cells and collection
of mucus in the dilated airways serve as perfect media for the
bacteria to colonize. Eventually, the vicious
cycle of bronchiectasis develops
17. • (Fig. 83.3). As seen on the CT scan of a patient with
left lower lobe
disease in Figure 83.4, the lobar bronchus itself may
also be severely destructed together with the smaller
bronchial branches. However, whether the destruction
of the lobe bronchus is a part of the initiation of
bronchiectasis, or it is only a consequence of the
inflammatory process in small-sized bronchi remains a
question to be answered by further studies.
18.
19. • causes of bronchiectasis. Recurrent pulmonary infections,
immunodeficiency
disorders, tuberculosis, allergic bronchopulmonary aspergillosis, slowly
growing tumors that obstruct the
bronchus, congenital mucociliary disorders, and childhood diseases like
measles or pertussis can all
induce the disease. However, in almost half of the patients, no initiating
event could be identified. In fact,
not all patients with a history of measles, whooping cough, or recurrent
pneumonia develop
bronchiectasis. The exact mechanism of bronchiectasis and the reason
why some people with similar
history develop bronchiectasis while others do not are not clear.
Nevertheless, impaired immunity,
connective tissue disorders, cystic fibrosis, ciliary defects as well as
previous bronchopulmonary
bacterial or viral infections are thought to have a role in the
development of the disease.
20. • Frequency of Distribution of Bronchiectasis: Area of
Involvement
Left lung more often than right lung7,9
Left lower lobe, most frequently involved
Lingula and middle lobe next most frequently involved
Total left bronchiectasis, fourth most commonly
involved
Right lower and total right are less often involved
Right upper lobe is involved more often than left
upper lobe
22. • DIAGNOSIS OF BRONCHIECTASIS
Clinical Features
The diagnosis of bronchiectasis is generally clinical. A mild
degree of disease that involves one or two
lung segments may be associated with either none or minor
symptoms except for periods of infectious
exacerbations. A typical patient, however, has classical
symptoms of daily purulent, mucopurulent or
mucoid sputum discharge, cough, fatigue, low exercise
tolerance, and occasional hemoptysis. Patients
generally have a history of frequent bronchopulmonary
infections necessitating antibiotics as well as
hospitalizations for the treatment of infectious recurrences.
23. • The requirement for three or more prolonged
treatment sessions yearly is not rare. An important
proportion of patients have a history of long-
standing
medical treatment due to the diagnosis of chronic
bronchitis, sinusitis, or asthma before the correct
diagnosis is made by radiological investigations.
24. • Patients’ relatives may give information about bad
breath odor, or if specifically asked, a foreign body
aspiration during childhood. Finger clubbing may
also be seen in severe cases. Children with
bronchiectases are likely to be in a stage of
developmental
deficiency when compared to their age group.
Chronic respiratory symptoms in children should
alert the physician to the possibility of
bronchiectasis.
25. • The British Thoracic Society,16 recommends that
bronchiectasis should be considered in
adults who have persistent productive cough with the following
features: young age at presentation,
history of symptoms over many years, absence of smoking
history, daily expectoration of large volumes of very purulent
sputum, haemoptysis, sputum colonization by Pseudomonas
aeruginosa, and nonproductive cough.
28. Chest X-ray and CT scan showing bronchiectasis with air-fluid
levels, and bronchial wall thickening with right lower lobe
predominance.
29. • HRCT confirms the diagnosis (Fig. 83.5B) in all patients. Early
bronchiectases that cannot be
identified on x-ray can indeed be detected by HRCT.
Bronchial wall dilation, which is assessed as
internal lumen diameter greater than the adjacent
pulmonary artery, is the distinguishing feature.
Additionally, bronchial wall thickening, lobar collapse, mucus
in the bronchus, the mediastinum and
fissure displacements may also be seen. HRCT must be
evaluated for the presence of features suggestive
of cystic fibrosis, tuberculosis, tracheobronchomegaly as in
Mounier–Kuhn syndrome (Fig. 83.6) or a
foreign body in the bronchial obstruction
36. • Immunological Tests
As mentioned above, primary antibody deficiency
is one of the major underlying conditions of
bronchiectasis. Structural lung destruction will also
cause a secondary antibody defect. Thus, it is
important to screen newly diagnosed patients for
IgG, IgA, and IgM antibody deficiencies
37. • The aim of treatment is to manage underlying disorders,
decrease the frequency of infectious episodes,
control bronchopulmonary infections, increase the quality of life
by reducing daily symptoms, achieve the
normal development of the child, and prevent possible
complications. Various treatment approaches
become necessary during the course of the disease including
physiotherapy, pharmacotherapy,
rehabilitation, bronchoscopic aspiration, and surgery. Thoracic
surgeon must always be a part of the
multidisciplinary team along with a chest physician, pediatrician
for children, experienced
physiotherapist, and immunology consultant. Radiology and
microbiology departments should also
provide input
38. • https://www.bronchiectasis.scot.nhs.uk/physiotherapy
• Physiotherapy
Physiotherapy constitutes an important part of the management and starts with the
education of the patients
and relatives about the disease, along with the mechanisms and the importance of airway
clearance
techniques.16 Commonly used physiotherapy maneuvers are postural drainage, active
breath cycle, and
manual techniques (i.e., chest clapping).10,16 Patients should be encouraged to remove
excessive mucoid
bronchial secretions especially in the morning. Postural drainage is performed according to
the location
of the disease in the lung, necessitating either a left or right side, or head-down position.
While a right or
left lateral side down position is needed in upper lobe disease, head down is used when
the lower lobes
are involved. Humidification with nebulized saline before
postural drainage is helpful to activate ciliary
function and provide more sputum discharge.
39. • Medical Treatment
Presence of sputum production alone or isolation of a pathogen without
clinical signs of active infection
is not always an indication for antibiotic treatment.16 Antibiotics are used
when the clinical picture
deteriorates as demonstrated by an increased cough or sputum production,
high fever, shortness of breath,
and hemoptysis. Treatment should be started empirically (immediately after
the sputum sample is sent for
microbiological analysis) based on the previous isolation and continue for
14 days. Because the
responsible organism is H. influenza in most cases, treatment with a β-
lactam antibiotic (amoxicillin) is
rewarding. However, P. aeruginosa responds best to treatment with
ciprofloxacin. To avoid the
development of antibiotic resistance, it is important to prevent its use
without bacterial culture and
sensitivity analysis
40. • Bronchodilators with β2 agonist and anticholinergic
drugs may be initiated and continued as long as
lung function or symptoms improve. There is no
evidence for a role of inhaled or oral
corticosteroids.16
Allergic bronchopulmonary aspergillosis may occur as
a result of a hypersensitivity reaction to
Aspergillus and is treated with oral corticosteroids and
azole antifungal agents. Immunodeficiency
situations require intravenous administration of
immunoglobulin.
42. • A long course
of conservative management is a must before proceeding
with a surgical resection. Periodic hemoptysis
warrants surgical treatment if the areas of bronchiectasis are
amenable to surgical removal. Otherwise,
intensive hospital care and bronchial artery embolization
should be considered. In the event of bleeding
due to tuberculous sequelae, the source may be a lacerated
pulmonary artery. Hence, in these patients,
even minor hemoptysis should be monitored closely and
surgery considered early
43. • One of the main indications has always been the “localized
disease.” It is important to make it clear
that the term “localized” does not necessarily refer to the
disease being localized in a single segment or a
lobe. As an example, the association of bronchiectasis both
in left lower lobe and the lingular segment is
not rare. In this situation, a lower lobectomy combined to
lingular segmentectomy is warranted (Videos
83.2 and 83.3). In a similar fashion, a bilobectomy or a
lobectomy together with segmentectomy may be
undertaken in patients with bronchiectasis in the right lung
44. • In this context, “bilateral disease” is not an absolute surgical
contraindication unless it is disseminated
to all lobes.22 As an example, the choice of bilateral lower
lobectomy, in the presence of an appropriate
cardiopulmonary function, represents a good option in patients
with the disease extent seen in the chest
CT view on Figure 83.8. In contrast, Figure 83.9 is an example of a
disseminated disease where surgical
resection is contraindicated. In this setting, the morbidity of
bilateral thoracotomy represents a major
concern when evaluating patients with bilateral bronchiectasis.
However, in the era of video-assisted
thoracic surgery (VATS), it is imperative to strike a balance
between the morbidity of bilateral sequential
operations and the risk of inefficient conservative management
48. • While some patients with bronchiectasis tolerate several infectious
episodes every year, others are
disturbed by the symptoms caused by a localized segmental disease.
Therefore, in elective situations, the
decision for and the timing of surgery should also take patient’s
preference into consideration.23
As a rule, neither preoperative pulmonary function measurement nor
surgical intervention should be
undertaken during an infectious episode. Sensitivity-oriented antibiotics
should be used immediately
before the operation to decrease sputum production for 7 to 10 days.
Postural drainage and chest
physiotherapy should also be ordered for 10 days before the operation to
lower the probability of
atelectasis and/or pneumonia that might occur due to diminished
expectoration of sputum in the early
postoperative period
49. • Contraindications for Surgery in Non-CF
Bronchiectasis
Experienced chest physicians or pediatricians can
manage successfully the patients with bronchiectasis
who have mild symptoms. For this reason, patients
who have not been subjected to an appropriate
longterm medical treatment should not be considered
for surgery. Other contraindications include
disseminated disease that does not allow the target
area to be removed successfully by surgery (Fig.
83.9), primary ciliary dyskinesia, or conditions
characterized by immunodeficiency, and severe COPD
50. • Surgical Techniques
As Dogan and colleagues reported,24 lobectomy is the
most frequent procedure followed by
segmentectomy. Pneumonectomy can be necessary
only in the rare patients who develop a destroyed
lung.25 Additionally, disseminated non-CF
bronchiectasis can be treated successfully with lung
transplantation.26
Currently, there are three approaches available for
pulmonary resection for bronchiectasis;
thoracotomy, standard 3-port VATS, and uniportal or
single incision VATS.
51. • Thoracotomy
The lateral thoracotomy approach is the same as the one used for other
purposes. At thoracotomy, the
diseased lobe may be found atelectatic and firmly attached to the
surrounding structures like the
diaphragm, chest wall, pericardium, aorta, and other lobe(s). It is easier to
start releasing the
bronchiectatic lobe from the mediastinal surface after the chest wall
adhesions are separated by blunt and
sharp dissections. When there is a firm symphysis between the lobes due
to chronic inflammation, the
sharp dissection needed in the fissure may cause tears in the healthy lung
resulting in prolonged
postoperative air leaks.23 Therefore, careful fissural division, preferentially
using a stapling device, is
advisable
52. • RESULTS OF SURGERY
Following lung resection for bronchiectasis, the postoperative
mortality figures should be similar to those
performed for other indications. However, as in all other
operations for inflammatory lung diseases,
postoperative morbidity, mainly resulting from postoperative
atelectasis and pneumonia caused by mucus
plugging, is higher in patients undergoing
thoracotomy.21,22,25,26 Nevertheless, the success rate of
surgery
is excellent, ranging from 74% to 94% in large series.31–35 Table
83.8 summarizes the results of surgery
through thoracotomy in recent publications. It is important to
note that more favorable recovery has been
reported after thoracoscopy compared to thoracotomy