Cerebrovascular Evaluation INTERNAL CAROTID ARTERY THROMBUS Sickle Cell Disease Watson Smith, RDMS, RVT From the D. E. Strandness, Jr. Vascular Laboratory and The Division of Vascular Surgery, Department of Surgery, University of Washington, Seattle, WA

Background- What is Sickle Cell Disease? Inherited blood disorderInherited blood disorder Abnormal hemoglobin S- RBCs become crescent shapedAbnormal hemoglobin S- RBCs become crescent shaped RBCs carry less O2- get stuck in small vesselsRBCs carry less O2- get stuck in small vessels Pain crisisPain crisis Treated with RBC exchange transfusionsTreated with RBC exchange transfusions Bone marrow Tx.Bone marrow Tx.

Sickle Cell and Thrombus- Poorly Understood Can lead to large vessel intimal hyperplasiaCan lead to large vessel intimal hyperplasia Sickle cells can adhere to endotheliumSickle cells can adhere to endothelium Superimposed thrombosis- most frequent cause of stroke in Sickle cell patientsSuperimposed thrombosis- most frequent cause of stroke in Sickle cell patients

Day 1 31 year old male with Sickle Cell Anemia and Hx. of Renal Tx. presents to ED Recently hospitalized for sickle cell crisisRecently hospitalized for sickle cell crisis Right side weakness and decreased sensationRight side weakness and decreased sensation Head CT- No acute intracranial abnormality.Head CT- No acute intracranial abnormality.

Day 1 MRA Non Contrast MRA- Left lacunar infarcts Non Contrast MRA- Left lacunar infarcts >50% stenosis left ICA- atherosclerotic plaque vs. artifact.

Day 1 Initial Carotid Duplex Exam

Day 1 Other Duplex Findings Everything else normal in carotid and vertebral arteriesEverything else normal in carotid and vertebral arteries TCD exam normal- no collaterals, stenosis or Doppler emboliTCD exam normal- no collaterals, stenosis or Doppler emboli

Day 1 Therapies and Strategies Heparin drip, ASAHeparin drip, ASA Vascular consult- 2 nd MRA with Gadolinium to confirm diagnosesVascular consult- 2 nd MRA with Gadolinium to confirm diagnoses Hematology consult- possible transfusionHematology consult- possible transfusion

Day 2 No new Sxs. Improved strength in right upper extremityNo new Sxs. Improved strength in right upper extremity 3rd MRA- findings concerning for ongoing embolic phenomena.3rd MRA- findings concerning for ongoing embolic phenomena. No Doppler emboli on repeat TCDNo Doppler emboli on repeat TCD No intervention or surgery. Given the location and nature of the thrombus, the operation itself presents a significant risk of generating emboliNo intervention or surgery. Given the location and nature of the thrombus, the operation itself presents a significant risk of generating emboli

Day 2 2 nd Duplex exam Carotid Images Day 2 carotid imageDay 2 carotid image

Days 3 and 4 Sxs. continue to improveSxs. continue to improve TCD exams remain normalTCD exams remain normal anticoagulatedanticoagulated RBC Exchange transfusion plannedRBC Exchange transfusion planned

Day 5 New onset of right upper extremity weakness, dysarthria. AphasiaNew onset of right upper extremity weakness, dysarthria. Aphasia CT no hemorrhage and no obvious new infarctCT no hemorrhage and no obvious new infarct MRI New foci of acute infarcts in the left temporal and left frontal lobeMRI New foci of acute infarcts in the left temporal and left frontal lobe Now scheduled for surgical explorationNow scheduled for surgical exploration pre op Duplex exam was orderedpre op Duplex exam was ordered

Day 5 Duplex Exam The thrombus was gone! The surgery was cancelledThe thrombus was gone! The surgery was cancelled

The patient did OK! RBC Exchange transfusion completedRBC Exchange transfusion completed Speech therapy- Sxs. slowly improvingSpeech therapy- Sxs. slowly improving AnticoagualtedAnticoagualted Follow ups planned with hematology and vascular surgeryFollow ups planned with hematology and vascular surgery

1 Year Later Seen in ED with Neck painSeen in ED with Neck pain Carotid imageCarotid image

Discussion, Conclusions Duplex US gold standard for carotid bifurcations lesionsDuplex US gold standard for carotid bifurcations lesions Importance of extracranial evaluations for TCD examsImportance of extracranial evaluations for TCD exams Emboli monitoring with TCD had limitationsEmboli monitoring with TCD had limitations Sickle cell disease is a unique processSickle cell disease is a unique process