Bronchiectasis

Key points

Pathophysiology

Permanent dilatation of segmental airways

Stages/types

Initial - ciliary epithelium is replaced with cuboidal squamous

Fusiform/Varicose - intermediate

Cylindrical - 6-7th order bronchioles - localised damage to the elastic tissue of the airway + oedema and inflammation

Saccular - 3-4th order bronchioles - irreversible - damage involves the muscle and cartilage layers with neovascularisation

Congenital:

A1 antitrypsin

CF

Scoliosis

Marfans

Acquired:

Allergic bronchopulmonary aspergillosis

Foreign body

Chemo/radiotherapy

History and Examination

3 layer sputum

Inspiratory crackles, expiratory wheeze

50% may have digital clubbing - reversible if bronchiectasis treated

Investigations

X-ray: Ring shadows or 'tram track' signs

Hi-res CT gold standard

Management

Indications for resection:

Long standing foreign body

Recurrent local exacerbations

Absence of vascular perfusion

Embolisation for massive haemoptysis

Outcome

Series of 35 patients: After resection 65% asymptomatic, 24% improved

References

Holcomb and Ashcraft’s Pediatric Surgery, 7th edition, 2020, Chapter 23 Acquired lesions of the Lung and Pleura