LIQUID VENTILATION IN AN INFANT WITH PULMONARY ALVEOLAR PROTEINOSIS

Partial liquid ventilation (PLV) has been applied in various pulmonary diseases. We describe the use of partial liquid ventilation as a lava ge method following normal saline (NS) lavage in an infant with pulmon ary alveolar proteinosis (PAP) and severe hypoxemia. A 6 weeks old 3.4 kg former 36 weeks gestation boy on supplemental oxygen was transferr ed to our NICU with persistent tachypnea, dry cough, and increasing ox ygen requirements. A lingular open lung biopsy revealed PAP. He develo ped progressive respiratory failure requiring ventilatory support, nec essitating conventional NS ravage, followed by lung lavage with perflu bron (LiquiVent; Alliance Pharmaceutical Corp. and Hoechst Marion Rous sel) while on venovenous extracorporeal life support (ECLS), Lung lava ge with NS and perflubron yielded minimal cloudy effluent. Gas exchang e and pulmonary function deteriorated following NS ravage and attempts to discontinue ECLS were poorly tolerated. In contrast, tidal volume, PaO2, and pulmonary compliance increased after PLV, while the (A-a) D -O2 decreased to a point where ECLS was no longer required. Once perfl ubron was added repeatedly to the ventilator circuit to correct for ev aporation over the 4 days of PLV. Cardiovascular status remained stabl e for several days; however, eventually he required reinitiation of EC LS and more mechanical ventilatory support with each trial,off ECLS. H e was maintained on high pressures and FiO2 without any possibility to wean him from mechanical ventilation. Life support was withdrawn 1 mo nth after admission. The survival from PAP in infants remains dismal, even with total lung NS lavage. While both NS and perflubron ravage in this patient were not effective in removing the proteinaceous alveola r debris, PLV following NS lavage was associated with an improvement i n gas exchange and lung compliance. Pediatr Pulmonol, 1998; 26:283-286 , (C) 1998 Wiley-Liss, Inc.