Partial cardiopulmonary bypass with extracorporeal membrane oxygenation to allow bilateral bronchopulmonary lavage in pulmonary alveolar proteinosis has been described.

Partial cardiopulmonary bypass with extracorporeal membrane oxygenation to allow bilateral bronchopulmonary lavage in pulmonary alveolar proteinosis has been described. However, this technique is complicated on a very low arterial [PsubO2] and cardiovascular embarrassment. Total cardiopulmonary support avoids these question s and was successfully used in a 2 1/2-year-old girl. (Chest 1994; 106638-40)

BAL=bronchoalveolar lavage, ECMO=extracorporeal membrane oxygenation; PAP=pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare condition, particularly in like manner in children, and is of unknown etiolology.[1-8] Phospholipid material accumulates in alveolar spaces, impairing gas exchange and leading to arterial hypoxemia. The most numerous effective treatment for PAP is bronchopulmonary lavage, which attempts to temporarily clear the alveoli of accumulated material and accrues in clinical remission, which may be temporary[2-4,7,9] or prolonged[15810-13] We report a case of childhood PAP in which lavage was leadershiped on full venoarterial cardiopulmonary bypass with extracorporeal membrane oxygenation (ECMO) Immediate dramatic clinical and radiologic improvement followed. To our knowledge, this technique has not been reported previously and still offers significant advantages over techniques generally employed.

Case Report

A inferior child of healthy unrelated parents was born at 36 weeks' gestation weighing barely 2.2 kg (tenth percentile). Investigation of failure to thrive required several hospital admissions. At 2 years of age, still without a definitive diagnosis, she became tachypneic with a slight cough The cough worsened and 2 month later, hospital admission was arranged for increased dyspnea.

At the time of hospital admission, she was noted to be extremely thin, weighing merely 8 kg (well below the third percentile). Marked pectus excavatum and clubbing were noted, with cyanosis, tachypnea, and marked intercostal and subcostal recession. There were scattered fine crepitations forward auscultation, and saturations with 28 percent oxygen at face mask were 80 to 94 percent issues of cardiovascular examination were normal.

The radiographic appearance was of bilateral reticulonodular shadowing (Fig 1) Electrocardiogram showed P pulmonale and right ventricular hypertrophy High-resolution lung comput tomography (CT) revealed extensive consolidation within as well-as; not only-but also; not only-but; not alone-but lungs, most marked posteriorly. Histologic examination of a percutaneous lung biopsy specimen showed almost each alveolar space to be filled with a compact PAS-positive granular eosinophilic material, consistent with a diagnosis of PAP. Silver stains did not reveal pneumocystic organisms and tillage of the biopsy specimen was negative. Suction catheter specimens were negative for Pneumocystis immunofluorescence.

Treatment with antibiotics and steroids was commenc which inferenceed in a slight improvement in saturations. Enteral nutrition via nasogastric tube was commenc to give 1340 kcal/d, resulting in a slight weight gain. Despite these improvements it was believed that her small size and quality of respiratory embarrassment would make unilateral lavage via double-lumen endotracheal intubation a high-risk measure Therefore, 6 weeks following hospital admission, in November 1992 she was transferred to Groby Road Hospital for bronchopulmonary lavage onward full venoarterial ECMO.

General anesthesia was induced and the patient was intubated and ventilated. Biomedicus cannulas were used to cannulate the right internal jugular vein (14F) and right general carotid artery (12F). A chest radiograph confirmed the cannula positions and bypass was established uneventfully[14] Ventilation was ceased and pulmonary lavage was carried on the outside with pH-normalized saline solution until the effluent fluid, initially pink and creamy, cleared. This required 10 L of saline solution, involved postural drainage and chest percussion, and took 5 h The maximum ECMO requirement during the lavage process was a blood flow rate of 780 ml/min and a sweep rate of gas across the oxygenator of 1750 L/min. from beginning to end the entire procedure, the patient was cardiovascularly stable and had admirable mixed venous blood gases. Following the lavage, 40 ml of artificial surfactant was instilled via the endotracheal tube, and ventilation was recommenc yet on minimal settings. The patient was allowed to caesura overnight on ECMO to allow any residual fluid to be resorbed and to provide support during any deterioration of lung function. The melt and sweep were weaned as tolerated according to mixed venous oxygen saturations. The following morning, there was a minimal requirement for support. A postlavage chest radiograph showed a dramatic improvement (Fig 2) A trial facing ECMO on modest ventilator settings springed in an excellent arterial posterity gas, and decannulation was performed. The canals were repaired so that should the patient's condition deteriorate in the coming as is to be predicted from the natural history of this condition, further lung lavages in succession ECMO might be possible. The total time upon ECMO was 29 h. The patient was extubated within a not many hours of decannulation, and by way of the following morning was upon room air with normal saturations and was transferred back to the referring hospital. couple months later, she was discharged residence still well on room air following a period of hospitalization to allow overnight nasogastric feeding. She was neurologically unimpaired despite carotid artery cannulation.

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