Whole-lung lavage in infants and children with pulmonary alveolar proteinosis.

BACKGROUND

Pulmonary alveolar proteinosis (PAP) is a rare alveolar filling syndrome where the mainstay of treatment is therapeutic whole-lung lavage (WLL). WLL techniques used in adults have to be modified for children because of their small-diameter airways.

AIM

To describe a technique for WLL adapted for small children.

METHODS

We describe a WLL technique that combines safe single-lung ventilation with the use of an age-appropriate endotracheal tube and selective occlusion of the other main bronchus with a balloon catheter through which the lavage is performed. Effectiveness measured by change in oxygen requirements and adverse effects was noted.

RESULTS

We performed 64 WLL procedures in four children (age 13 months to 7 years; body weight 4.7-14 kg). Two children had idiopathic and two had secondary PAP. At referral, all children had dyspnoea at rest and required continuous oxygen supplementation. Two patients showed significantly decreased oxygen demands and radiological improvement after WLL. Two patients showed no significant response. The only adverse effect observed was transient hypoxemia. Complications comprised fluid leak at the balloon (4), balloon rupture (1), and pneumothorax (1).

CONCLUSIONS

This technique for WLL combining single-lung ventilation with an endotracheal tube and lung exclusion for lavage with a balloon catheter can be safely and effectively performed in small children with PAP.