SUCCESSFUL TREATMENT OF LIFE-THREATENING ACUTE CHEST SYNDROME OF SICKLE-CELL DISEASE WITH VENOVENOUS EXTRACORPOREAL MEMBRANE-OXYGENATION

Purpose: We describe a pediatric patient with sickle cell disease and life-threatening acute chest syndrome who was successfully treated wit h venovenous extracorporeal membrane oxygenation (ECMO). Patient and M ethods: An 8-year-old boy with sickle cell disease presented with vaso -occlusive crisis, which progressed to fulminant acute chest syndrome requiring a partial exchange transfusion and mechanical ventilation. D espite very high ventilator settings and significant barotrauma, hypox ia persisted and circulatory failure occurred. He was then successfull y treated with venovenous ECMO for 11 days. One month after decannulat ion he had a seizure associated with abnormalities on magnetic resonan ce images (MRIs). His disease has been managed with a chronic transfus ion program since then. Follow-up after 5 years reveals normal pulmona ry function tests, a normal magnetic resonance angiogram (MRA), and ab ove-average cognitive skills. Conclusion: This is the first report of a pediatric patient with acute chest syndrome successfully managed wit h venovenous ECMO. His course was complicated by a seizure associated with MRI abnormalities, although the outcome has been excellent. This case suggests that treatment with venovenous ECMO should be strongly c onsidered for sickle cell patients with life-threatening acute chest s yndrome, despite maximal conventional support.