SESSION TYPE: ILD Cases I
PRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PM
INTRODUCTION: Pulmonary Alveolar Proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates in the alveoli secondary to impaired catabolism of pulmonary surfactant by the macrophage. Three distinct etiologies exist: congenital, secondary and acquired. Surfactant homeostasis is a complex process regulated in part by granulocyte-macrophage colony-stimulating factor (GM-CSF), a 23-kD protein growth facture. In the congenital and acquired forms, the hypothesized mechanism for macrophage dysfunction is insufficient GM-SCF signaling. The natural history of the disease may vary from spontaneous resolution to acute respiratory failure.
CASE PRESENTATION: A 34-year-old female with a history of asthma presented to a rural community hospital with a complaint of insidious shortness-of-breath following an antecedent flu-like illness four months prior. A CT scan was performed which revealed bilateral ground-glass airspace disease, marked interlobular septal thickening and crazy paving consistent with PAP. The patient was transferred to our tertiary care center for definitive diagnosis and treatment. Admission CXR showed diffuse airspace disease (Fig. 1). A diagnostic bronchoscopy was performed and bronchoalveolar lavage (BAL) obtained. BAL results demonstrated proteinaceous periodic acid-Schiff positive material confirming PAP. Following bronchoscopy, the patient developed acute hypoxic respiratory failure requiring endotracheal intubation and mechanical ventilation. Despite increasing support with PEEP and increased FiO2 the patient remained hypoxemic. The patient was started on venovenous extracorporeal membrane oxygenation (VV ECMO) support. As the patient was too unstable to transport to the operating room, bilateral whole lung lavage was performed in the surgical ICU while the patient was oxygenated via VV ECMO. Two days after lung lavage the patient no longer required ECMO support. The patient was successfully liberated from the ventilator four days after lung lavage (Fig. 2 &3). Patient had full recovery and has not developed recurrence of her acquired PAP (Fig. 4).
DISCUSSION: This case describes the novel use of VV ECMO support to facilitate bilateral whole lung lavage for the treatment of PAP outside of the operating room. To our knowledge, this is the first reported case employing the use of VV ECMO for bilateral whole lung lavage at the bedside in an ICU setting for the treatment of PAP.
CONCLUSIONS: Extracorporeal membrane oxygenation support can successfully be used to facilitate bilateral whole lung lavage for the treatment of PAP outside of the operating room.
1) Trapnell, B., M.D., Whitsett, J., M.D. and Nakata, K., M.D., Ph.D. Pulmonary Alveolar Proteinosis. N Engl J Med 2003; 349:2527-39.
2) Trapnell, B., MD, Nakata, K., MD, PhD and Kavuru, M., MD. Pulmonary Alveolar Proteinosis Syndrome. Murray and Nadel's Textbook of Respiratory Medicine, 5th Edition, Expert Consult Online: 1516-36.
DISCLOSURE: The following authors have nothing to disclose: Matthew Hammar, Marvin Balaan
No Product/Research Disclosure InformationAllegheny General Hospital, Pittsburgh, PA