SHM Converge Scientific Abstract ePoster Gallery-A Severe Presentation of Sickle Cell Crisis in a Patient With Moyamoya

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A 28-year-old man with sickle cell disease, prior ischemic stroke, chronic kidney disease, and hypertension was admitted for a vaso-occlusive pain crisis. On day 2, he developed fever, dyspnea, and hypoxia, with imaging consistent with acute chest syndrome. He improved over the next few days with IV antibiotics and blood transfusions.<br /><br />Despite ongoing pain treatment, he remained persistently hypertensive, refractory to three home antihypertensive medications; this was initially attributed to pain. On day 9, he became acutely altered and had a witnessed seizure. CT of the head showed multifocal cortical hypodensities in all lobes, concerning for infarcts. By day 11, he had a second seizure and severe headache. CTA revealed bilateral internal carotid artery occlusions with moyamoya, and MRI demonstrated posterior reversible encephalopathy syndrome (PRES). A nicardipine infusion was started.<br /><br />Over the following days, his headache improved as blood pressure normalized, and his pain improved with opioid therapy. He was discharged in stable condition on day 17.<br /><br />The case highlights PRES as a rare but serious neurologic complication of sickle cell vaso-occlusive crisis. The authors suggest that hypertension may be an independent risk factor for neurologic complications during sickle cell pain crises, and that early blood pressure control may reduce morbidity and improve long-term outcomes.

Asset Subtitle

Maggie Rempe

Meta Tag

Author List Chelsea R. Navarrette, Maggie Rempe, Marilee Schaner

Category Clinical Vignettes

Concept Posterior Reversible Encephalopathy Syndrome

Concept Vaso-occlusive Pain

Concept Severe Hypertension

Concept Seizure

Concept Altered Mental Status

Distinguished Non-Finalist

Presenter Organization University of Nebraska Medical Center

Presenting Author Maggie Rempe

Track Adult

Keywords

sickle cell disease

vaso-occlusive crisis

acute chest syndrome

hypertension

seizure

posterior reversible encephalopathy syndrome

PRES

moyamoya

ischemic stroke

blood pressure control

Posterior Reversible Encephalopathy Syndrome

Vaso-occlusive Pain

Severe Hypertension

Seizure

Altered Mental Status