To the Editors:
I read with interest the article by Beccaria et al. 1 and the accompanying editorial by Morgan 2, relating to the benefit of lung lavage in the treatment of adult patients with pulmonary alveolar proteinosis. Although pulmonary alveolar proteinosis occurs rarely in paediatric patients and is usually fatal, I would like to put forward data concerning the successful long-term benefit of lung lavage in paediatric patients.
My colleagues and I have successfully used multiple total lung lavages in infants and paediatric patients with biopsy-proven pulmonary alveolar proteinosis 3–7. Two unrelated infants experienced symptoms at 1 month of age, and, at 7 months, a diagnosis of pulmonary alveolar proteinosis was made. Before lavage, our patients only weighed 5 kg and manifested significant hypoxaemia.
At 9 months of age, both infants had successful total bilateral lung lavage using the single-lumen endotracheal tube technique 8, 9. A brief description of this technique follows. In a hyperbaric chamber with ketamine anaesthesia and pancuronium, a 4-mm uncuffed nasotracheal tube was secured in the horizontal left lateral decubitus position. The lavage was performed by repeated infusion and drainage of 30 mL tidal volume of saline during apnoea, followed by ventilation of both lungs with oxygen. Apnoea never exceeded 1 min and ventilation was ≥2 min. The patient was then placed in a supine position and both lungs were ventilated with 100% oxygen. Subsequently, the patient was secured in a horizontal right lateral decubitus position and the lavage procedure was repeated. Lavage was discontinued when the effluent was clear or when the residual fluid was equal to the total lung capacity of the lavaged lung. The procedure was completed in 3 h using 1,500 mL of saline. The use of a hyperbaric chamber prevented significant hypoxaemia during the therapeutic lung lavage.
The two infants tolerated the initial and repeat lavages without sequelae, and are living and well at the ages of 23 and 30 yrs. The first patient who was treated with this procedure at 8 yrs old has required additional lavages, with the last lavage in 1984. She is currently asymptomatic at aged 40 yrs. Recent analysis of the lavage fluid from these three patients revealed normal surfactant B.
Under hyperbaric conditions, the single-lumen endotracheal lung lavage technique provides a safe and effective long-term mode of therapy for infants and children with pulmonary alveolar proteinosis.
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