664: A RARE CASE OF NEUROPSYCHIATRIC LUPUS VASCULITIS: TREATING A DIAGNOSTIC DILEMMA

Abstract

Introduction: Fat emboli syndrome (FES) in the setting of sickle cell disease (SCD) occurs secondary to bone ischemia, releasing fatty acids into the blood, and leading to infarct and microhemorrhages to the brain through either pulmonary or cardiac shunt. The characteristic MRI findings of cerebral FES are a “starfield” appearance of scattered micro-infarcts. Cerebral FES is uncommon in pediatrics, in patients without evidence of a shunt and in patients without inciting bone injury. Description: 16 year old male with HbSS presented with low back pain concerning for acute vaso-occlusive pain crisis that progressed to acute chest syndrome, respiratory failure and ARDS requiring intubation. He had respiratory acidosis and prolonged hypoxia on arrival. His initial HgbS fraction was 76%, requiring multiple exchange transfusions. He developed multiorgan dysfunction including acute renal failure, bilateral cortical renal infarcts, and biventricular cardiac dysfunction with ejection fraction of 35-40% and no shunt. He received therapeutic plasma exchange (TPE) in the setting of thrombocytopenia with schistocytes. A week later, his respiratory failure had improved and sedation was lifted for extubation when he was found to have increased tone, myoclonic movements, clonus, extensor posturing, gaze deviation and bilateral Hoffman sign. EEG showed no evidence of seizures. MRI demonstrated diffuse microhemorrhages and diffusion restriction in the white matter of the brain, localized heavily in the corpus callosum, as well as extensive bone ischemia of cervical vertebrae consistent with a microvascular occlusive process. The bony changes and starfield pattern seen on MRI were suggestive of cerebral FES. He tested positive for parvovirus B19, commonly seen in FES. He was extubated 6 days after diagnosis of FES and transferred to rehab facility after a month. Discussion: Although well described in adults, we found only a single other case describing FES with SCD in a child. Also notable are his delayed development of FES after recovery from other organ injuries and in the absence of cardiac shunt, and unknown intrapulmonary shunt. Prognosis for this disease, particularly in pediatrics, is unclear and treatment is mostly supportive aside from exchange transfusions.