Whole Lung Lavage of Nine Children with Pulmonary Alveolar Proteinosis: Experience in a Tertiary Lung Center
In a 4.5 year old girl with preoperative prolonged prothrombin time and international normalized ratio (INR) (16.8 seconds and 1.9, respectively), PCPB was established through insertion of RFA and RFV catheters. After seven lavage cycles with a total of 1.8 L of warm normal saline, returning fluid unexpectedly became bloody, the patient desaturated intensely, and hematuria occurred subsequently. So, the lavage was quickly stopped, mechanical ventilation was restored with FiO2 of 1.0, the lavaged lungs were emptied and the patient maintained on CPB to support oxygenation. However, PaO2 was 40mmHg, SaO2 69%, and PaCO2 65 mmHg with a severe metabolic acidosis. After reversal of heparin and separation of the PCPB, the patient was transferred to ICU and controlled mechanical ventilation was maintained while she received vasopressor and inotrope support. Laboratory tests showed a normal platelet count (148×103/µL) and Hb (12gr/dl). Fresh frozen plasma and packed red blood cells were transfused to treat bleeding. However, bright frothy blood continued through endotracheal tube, PaCO2 increased to 160 mmHg, PaO2 decreased to 29 mmHg, metabolic acidosis worsened and the patient died 36 hours after the procedure.