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Prepublished online as a Blood First Edition Paper on September 26, 2002; DOI 10.1182/blood-2002-04-1183.
Submitted April 19, 2002
Accepted July 24, 2002
Reversible Posterior Leukoencephalopathy Syndrome and silent cerebral infarcts are associated with severe Acute Chest Syndrome in children with Sickle Cell Disease
Jessica N Henderson, Michael J Noetzel, Robert C McKinstry, Desiree A White, Melissa Armstrong, and Michael R DeBaun*
Pediatrics, Division of Hematology/ Onclolgy, Washington University School of Medicine, St. Louis, MO, USA
Neurology, Pediatrics, Washington University School of Medicine, St. Louis, MO, USA
Mallinkrodt Institute of Radiology, Neuroradiology, Washington University School of Medicine, St. Louis, MO, USA
Department of Psychology, Washington University, St. Louis, MO, USA
* Corresponding author; email: debaun_m{at}kids.wustl.edu.
Patients with severe Acute Chest Syndrome (ACS) requiring endotracheal intubation and erythrocytapheresis may be at an increased risk of neurologic morbidity. This study examines patients with sickle cell disease who developed severe episodes of ACS leading to endotracheal intubation, ventilatory support for respiratory failure and erythrocytapheresis. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) studies, a neurologic examination by a pediatric neurologist and cognitive testing were done in all patients. Five consecutive patients, ages 3 to 9 years, were identified with severe ACS. All patients developed neurologic complications resulting from ACS episodes including seizures (n=2), silent cerebral infarcts (n=3), cerebral hemorrhage (n=2), and Reversible Posterior Leukoencephalopathy Syndrome (n=3). Children with severe ACS should have a MRI of the brain, neurologic examination by a neurologist, and cognitive testing to detect the presence of neurologic morbidity.
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