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 present.

· Tell the examiner that you would like to know whether the bronchiectasis is of 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) due to bronchiectasis (aetiology), and is

cyanosed.

QUESTIONS

What do you understand by the term '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, tuberculosis (TB).

· Mechanical bronchial obstruction, as in TB, carcinoma, nodal compression.

· Allergic bronchopulmonary aspergillosis.

· T-Globulin deficiency congenital, acquired.

· Immotile cilia syndrome (Kartagener's syndrome).

· Cystic fibrosis.

· Neuropathic disorders (namely Riley-Day syndrome, Chagas' disease).

· Idiopathic.

What investigations would you perform in such a patient?

FBC, sputum culture, CXR, 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 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 more than 90%, using bronchography as the 'gold standard'.

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 1-2 mm thick and high spatial resolution

algorithms are used to recon-struct images (Radiology 1994; 193:369 74).

What do you know about spiral CT?

This is a rapidly evolving technique to image the chest which has the advantage of truly contiguous sections; consequently

completely seamless reconstructions are possible. This may allow virtual-reality bronchoscopy imaging (Am J Roentgenol 1994;

162: 561-7).

What are the complications of bronchiectasis?

· Pneumonia, pleurisy, pleural effusion, pneumothorax.

· Sinusitis.

· Haemoptysis.

· Brain abscess.

· Amyloidosis.

What are the major respiratory pathogens in bronchiectasis?

Staphylococcus aureus, Haemophilus infiuenzae, Pseudomonas aeruginosa.

How would you treat such patients?

· Postural drainage.

· Antibiotics.

· Bronchodilators.

· Surgery in selected cases.

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's syndrome), said to be due to mercury intoxication. It

was first described from the north of England by Young in 1970.

· Congenital kyphoscoliosis.

· Situs inversus and paranasal sinusitis (Kartagener's syndrome).

· Unilateral absence of pulmonary artery.

What is the indication for surgery in bronchiectasis?

Bronchiectasis localized to a single lobe or segment without clinical, broncho-graphic 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 tuberculosis. 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 Ill 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. All three types can be

present in the same patient.

· Cylindrical bronchiectasis: refers to bronchi that are uniformly dilated and do not taper, but rather end abruptly. This is due to

plugging of smaller bronchi by thick mucus and casts. The bronchi are dilated to greater than 2 mm but can be so large as to

admit a finger.

· Varicose bronchiectasis: refers to dilated bronchi with irregular bulging contours similar to a varicose vein. They do not taper

and terminations are bulbous. Bronchial subdivisions are reduced.

· Cystic or saccular bronchiectasis: the most severe form, characterized by sharply reduced bronchial subdivisions and dilated

bronchi ending in cystic pus-filled cavities.

kaennec was the first to describe bronchiectasis in 1819.

M. Kartagener (b. 1897), a Swiss physician.

C.M. Riley and R.L. Day, both US paediatricians. Riley-Day syndrome consists of dysautonomia and lack of coordination in

swallowing.


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