1. Safety of Cerebral Digital Subtraction Angiography in Pediatric Patients with Sickle Cell Disease. Emily Wyse, BS 1 Jessica Carpenter, MD 2 Suresh Magge, MD 2 Ross Fasano, MD 2 Monica Pearl, MD 1,2 EP – 117 1.Division of Interventional Neuroradiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA 1.Department of Interventional Neuroradiology, Children’s National Medical Center, Washington, DC, USA

2. No disclosure

3. Background Neurologic complications of sickle cell disease (SCD) have an 11% chance of occurring in a patient by age 20. 1 These events most commonly include stroke, hemorrhage, and cognitive and behavioral changes. 1. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-294.

4. Background The evaluation of pediatric patients with SCD and a neurologic condition is routinely performed with non-invasive imaging such as CT, MRI or transcranial doppler. Cerebral digital subtraction angiography (DSA), a minimally invasive procedure, is often not considered due to concerns for intravascular sickling and other complications.

5. Purpose We present a series of pediatric SCD patients who underwent cerebral DSA to evaluate the safety of this procedure.

6. Materials and Methods We reviewed a prospective database from July 2010 to December 2014 of all children with SCD who underwent diagnostic or interventional cerebral DSA at a single institution.

7. Materials and Methods Records were reviewed for age, gender, clinical diagnosis, and intra-procedural or post-procedural complications.

8. Results Total angiograms: 41 Total patients: 28 (17 boys, 11 girls) Age range: 3 to 18 years Median age: 10 years

9. Diagnostic angiography Performed for the following clinical diagnoses: Moyamoya syndrome: n = 6 Stroke: n = 7 Intracranial aneurysm: n = 9 SAH: n = 2 Vasculopathy: n = 4 A, B) Multiple intracranial aneurysms (arrows) are noted in two patients with SCD. The largest is a superiorly projecting ophthalmic segment aneurysm (asterisk).

10. Interventions Performed for the following diagnoses: Intra-arterial nicardipine infusion for vasospasm due to SAH: n = 3 Coil embolization for ruptured aneurysms: n = 1 Coil embolization for unruptured aneurysms: n = 1 Stent-assisted coil embolization for unruptured aneurysm: n = 1 Posteroanterior (C) and lateral (D) views from a right internal carotid artery injection show diffuse vasospasm in a child with SCD and diffuse SAH.

11. Results No intra-procedural complications for diagnostic or interventional cases. Post-procedure, one child suffered from a sickle cell crisis (acute chest syndrome) which resulted in reintubation and an additional night in the PICU. No groin hematomas, strokes, or intracranial hemorrhages.

12. Discussion Pre-procedural preparation includes adequate hydration and optimizing hematologic parameters. Hydration: IV fluids at 1.5 times the maintenance rate. If elective, children were admitted the night prior to the procedure. 1 Hematology: transfusion goal of hemoglobin greater than 10 g/dL and HbS less than 30%. 1 1. Saini S, Speller-Brown B, Wyse E, et al. Unruptured intracranial aneurysms in children with sickle cell disease: Analysis of 18 aneurysms in 5 patients. Neurosurgery. 2015.

13. Conclusion Cerebral DSA can be performed safely in pediatric patients with SCD and should be considered in the evaluation of cerebrovascular pathologies in this population.