Bronchiectasis

Published on 02/04/2015 by admin

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101 Bronchiectasis

Instruction

Examine this patient’s chest.

Listen to this patient’s chest.

Salient features

History

• Cough with copious purulent sputum, recurrent haemoptysis

• Intermittent fever and night sweats

• History of recurrent chest infections

• Weight loss.

Examination

• Copious purulent expectoration (remember to check the sputum cup in a chest case)

• Finger clubbing

• Bilateral coarse, late, inspiratory crackles.

Proceed as follows:

• Comment on kyphoscoliosis if any

• Tell the examiner that you would like to know whether the bronchiectasis is long standing; if so, you would like to examine the abdomen for splenomegaly (amyloidosis).

In addition there may be signs of collapse, fibrosis or pneumonia.

Diagnosis

This patient has bilateral, coarse, late inspiratory crackles with purulent sputum (lesion) caused by bronchiectasis (aetiology), and is cyanosed.

Questions

What do you understand by bronchiectasis?

It is a chronic necrotizing infection of the bronchi and bronchioles leading to abnormal, permanent dilatation of the airways.

Mention the causes of bronchiectasis

• Postpneumonic, measles, pertussis, TB

• Mechanical bronchial obstruction, as in TB, carcinoma, nodal compression

• Allergic bronchopulmonary aspergillosis

• Gammaglobulin deficiency, congenital, acquired

• Immotile cilia syndrome (Kartagener syndrome)

• Cystic fibrosis

• Neuropathic disorders (namely Riley–Day syndrome, Chagas’ disease)

• Inflammatory bowel disease, rheumatoid arthritis

• Idiopathic.

What investigations would you do in such a patient?

FBC

Sputum culture

Chest radiography

Bronchography

CT scan of the chest.

Advanced-level questions

How can CT assess bronchiectasis?

High-resolution CT performed at the end of expiration suggests that small airways disease may be an early feature of bronchiectasis, which then leads to more progressive injury and bronchiolar distortion. Larger studies with long-term follow-up are required to confirm this. Conventional CT has a sensitivity of 60–80% for detecting bronchiectasis, whereas high-resolution CT has a sensitivity of >90%, using bronchography as the ‘gold standard’.

Specific abnormalities found on high-resolution CT (Fig. 101.1) include:

• dilatation of an airway lumen (rendering it >1.5 times as wide as a nearby vessel)

• lack of tapering of an airway toward the periphery

• varicose constrictions along airways

• ballooned cysts at the end of a bronchus (the bullae found in patients with emphysema have thinner walls and are away from an airway).

image

Fig. 101.1 Severe bronchiectasis in the right lower lobe with plugging of the dilated bronchi. Mild, cylindric bronchiectasis in the left lower lobe showing the signet ring sign.

(With permission from Albert et al. 2008.)

Non-specific findings include:

• consolidation or infiltration of a lobe with dilatation of the airways

• thickening of the bronchial walls, mucous plugs

• enlarged lymph nodes

• a reduction in vascular markings similar to that seen in emphysema (probably as a result of the inflammatory destruction of smaller airways and vessels).

Note: Cystic fibrosis and allergic bronchopulmonary aspergillosis involve an upper-lobe distribution, and Mycobacterium avium complex infection often involves the middle lobe or lingula. Bronchiectasis usually affects the lower lobe.

What is the difference between standard and high-resolution CT?

In standard CT, the resolution is 10 mm thick whereas with high-resolution CT the slices are 1–1.5 mm thick and high spatial resolution algorithms are used to reconstruct images (Radiology 1994;193:369–74).

What do you know about spiral CT?

This is a rapidly evolving technique to image the chest and has the advantage of creating truly contiguous sections; consequently totally seamless reconstructions are possible. This may allow virtual-reality bronchoscopy imaging. Spiral CT may elucidate additional subtle changes, because it reduces motion artefact, but it requires a larger dose of radiation.

What are the complications of bronchiectasis?

• Pneumonia, pleurisy, pleural effusion, pneumothorax

• Sinusitis

• Haemoptysis (life-threatening haemoptysis is >600 ml/day)

• Brain abscess

• Amyloidosis.

What are the major respiratory pathogens in bronchiectasis?

Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa.

How would you treat such patients?

• Identification of acute exacerbations and administration of antibiotics

• Suppression of the microbial load

• Treatment of underlying conditions

• Reduction of the excessive inflammatory response

• Promotion of bronchial hygiene:

• Postural drainage
• Bronchodilators.

• Control of bronchial haemorrhage

• Surgery in selected cases: surgical removal of extremely damaged segments or lobes that may be a nidus for infection or bleeding.

What abnormalities may be associated with bronchiectasis?

• Congenital absence of bronchial cartilage (Williams–Campbell syndrome)

• Tracheobronchomegaly (Mounier–Kuhn syndrome)

• Obstructive azoospermia and chronic sinopulmonary infection (Young syndrome), said to be caused by mercury intoxication. It was first described in the north of England by Young in 1970

• Congenital kyphoscoliosis

• Situs inversus and paranasal sinusitis (Kartagener syndrome) (p. 117)

• Unilateral absence of pulmonary artery.

What is the indication for surgery in bronchiectasis?

Bronchiectasis localized to a single lobe or a segment without clinical, bronchographic or CT evidence of bronchiectasis or bronchitis affecting other parts of the lung.

What are the common sites for localized disease?

Left lower lobe and lingula.

What do you understand by the term bronchiectasis sicca?

Bronchiectasis or ‘dry’ bronchiectasis is that which presents with recurrent dry cough associated with intermittent episodes (months or years apart) of haemoptysis. The haemoptysis can be life threatening as bleeding is from bronchial vessels with systemic pressures. There is usually a past history of granulomatous infection, particularly TB. The upper lobes are often primarily affected, allowing good drainage.

What do you know about bronchiectasis in allergic bronchopulmonary aspergillosis?

The bronchial dilatation occurs in more proximal bronchi as a result of type III immune complex reactions.

What do you know about Reid’s classification of bronchiectasis?

In 1950, Reid correlated pathological changes with bronchography and described three different appearances:

• Cylindrical bronchiectasis: bronchi that are uniformly dilated (to >2 mm but can be so large as to admit a finger) and do not taper but rather end abruptly; caused by plugging of smaller bronchi by thick mucus and casts

• Varicose bronchiectasis: dilated bronchi with irregular bulging contours similar to a varicose vein and no tapering; terminations are bulbous and bronchial subdivisions are reduced

• Cystic or saccular bronchiectasis is the most severe form and is characterized by sharply reduced bronchial subdivisions and dilated bronchi ending in cystic pus-filled cavities.

All the three types can be present in the same patient.

What are the modes of presentation of the bronchiectasis?

Bronchiectasis may be diffuse or focal disease. There are three types of focal airway obstruction that may lead to bronchiectasis:

• Luminal blockage by a foreign body, broncholith or slowly growing beinign tumour

• Extrinsic narrowing caused by enlarged lymph nodes (e.g. in middle lobe syndrome, which involves a small angulated orifice surrounded by a collar of lymph nodes that may enlarge and encroach on the main airway after granulomatous disease caused by infection with mycobacteria or fungi

• Twisting or displacement of the airways after a lobar resection (e.g. the occasional cephalad displacement of a lower lobe after surgery for the resection of the upper lobe).

Note: Recurrent or persistent lobar pneumonia is a key distinguishing feature of the first two types of focal bronchiectasis and is important to recognize because interventional bronchoscopy or surgery may result in palliation and sometimes cure.

Laennec was the first to describe bronchiectasis in 1819.

M Kartagener (1897–1975), a Swiss physician.

CM Riley and RL Day, both US paediatricians. Riley–Day syndrome consists of dysautonomia and lack of coordination in swallowing.

CJR Chagas (1879–1934) a Brazilian physician.

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