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 – 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

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Background Neurologic complications of sickle cell disease (SCD) have an 11% chance of occurring in a patient by age 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):

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.

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

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.

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

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

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).

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.

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.

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% 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

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.