Respiratory Strategies and Airway Management in Patients with Pulmonary Alveolar Proteinosis: A Review

Abstract

Background. Pulmonary alveolar proteinosis is a rare disorder characterized by a large accumulation of lipoproteinaceous material within the alveoli. This causes respiratory failure due to a restriction of gas exchange and changes in the ventilation/perfusion ratio. Treatment methods include noninvasive pharmacological approaches and invasive procedures, such as whole-lung lavage under general anesthesia. Methods. Based on the literature search using free-term key words, we have analyzed published articles concerning the perioperative management of adult and pediatric patients with pulmonary alveolar proteinosis. Results and Discussion. In total, 184 publications were analyzed. Only a few manuscripts were related to anesthetic, respiratory, and airway management in patients suffering from pulmonary alveolar proteinosis. Airway should be strictly separated using a double-lumen tube. Respiratory strategies involve the use of manual clapping, continuous positive airway pressure, high-frequency jet ventilation of the affected lung, and employment of venovenous extracorporeal membrane oxygenation in the most serious of cases. Conclusion. The goal of this review is to summarize the current published information about an anesthetic management strategy with a focus on airway management, ventilation, and oxygenation techniques in PAP patients.

Therapeutic Whole Lung Lavage for Alveolar Proteinosis

“…The disease is often complicated by superimposed bacterial and opportunistic infections. 4 The disease course is variable, with spontaneous recovery to terminal cardiorespiratory failure. Diagnosis is made from bronchoalveolar fluid lavage, histology, immune-histochemical tests, and computed tomography (CT).…”

“…The appropriate ventilation strategy has not been described for these patients. Although there are reports of patients being ventilated with pressure control mode, with Pplat targeted to 30 cms of H 2 O, 4 this may often be difficult because the PIP and Pplat may be severely elevated. In this patient, with twolung ventilation and a set Vt of 400 mL, the Pplat was in the range of 45 cms, thus the compliance was <10 mL/cms of H 2 O (normal compliance in ventilated patients is around 50-100 mL/cms of H 2 O).…”

“…Severe hypoxemia may require extracorporeal membrane oxygenation (ECMO). 4 During the filling phase of the lavage, the pulmonary vasculature of the lavaged lung tends to get compressed, thus minimizing shunt and improving oxygenation, whereas during the drainage phase, the pulmonary vasculature to the lavaged lung tends to expand, resulting in re-establishment of shunt and hypoxemia. The usual practice of monitoring end-tidal carbon dioxide to monitor the adequacy of ventilation may be unreliable because the arterial-to-alveolar carbon dioxide gradient may be large.…”

Lavado pulmonar total bilateral en una sola sesión bajo ECMO veno-venoso en un caso proteinosis alveolar severa: cinco años de seguimiento

“…Formas severas de proteinosis alveolar, aunque bien descriptas, son infrecuentes 3 . El LPT ha sido bien descripto bajo soporte vital con circulación extracorpórea y más recientemente ECMO 2,5,6 . La decisión de realizar LPT bajo ECMO veno-venoso en lugar de venoarterial puede ser controversial por el potencial fallo del ventrículo derecho, pero en este caso fue bien tolerado.…”

“…Raramente es diagnosticada en etapas tan avanzadas que requieran soporte vital extracorpóreo para sostener el LPT 3 . El LPT bajo circulación extracorpórea y más recientemente con oxigenación bajo membrana extracorpórea (ECMO) ha sido descripto, pero excepcionalmente realizado en modalidad de ECMO veno-venoso por potencial claudicación del ventrículo derecho 3,4,5 . La evolución posterior de esos casos graves es escasamente conocida 2 .…”