Successful treatment of life-threatening acute chest syndrome of sickle cell disease with venovenous extracorporeal membrane oxygenation.
OBJECTIVE
We describe a pediatric patient with sickle cell disease and life-threatening acute chest syndrome who was successfully treated with venovenous extracorporeal membrane oxygenation (ECMO).


METHODS
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. Despite very high ventilator settings and significant barotrauma, hypoxia persisted and circulatory failure occurred. He was then successfully treated with venovenous ECMO for 11 days. One month after decannulation he had a seizure associated with abnormalities on magnetic resonance images (MRIs). His disease has been managed with a chronic transfusion program since then. Follow-up after 5 years reveals normal pulmonary function tests, a normal magnetic resonance angiogram (MRA), and above-average cognitive skills.


CONCLUSIONS
This is the first report of a pediatric patient with acute chest syndrome successfully managed with 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 considered for sickle cell patients with life-threatening acute chest syndrome, despite maximal conventional support.
Pelidis
M A
MA
Division of Pediatric Hematology, Johns Hopkins Hospital, Baltimore, MD 21205, USA.
Kato
G J
GJ
Resar
L M
LM
Dover
G J
GJ
Nichols
D G
DG
Walker
L K
LK
Casella
J F
JF
D000208:Acute Disease; D000755:Anemia, Sickle Cell / Q000150:complications*; D001921:Brain / Q000473:pathology; D002637:Chest Pain / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D002648:Child; D015199:Extracorporeal Membrane Oxygenation; D005334:Fever; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010996:Pleural Effusion; D013902:Radiography, Thoracic; D012640:Seizures / Q000175:diagnosis / Q000209:etiology; D013577:Syndrome