Safety of Cerebral Digital Subtraction Angiography in Pediatric Patients with Sickle Cell Disease.

Emily Wyse, BS1

Jessica Carpenter, MD2

Suresh Magge, MD2

Ross Fasano, MD2

Monica Pearl, MD1,2

EP – 117

1. Division of Interventional Neuroradiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

2. Department of Interventional Neuroradiology, Children’s National Medical Center, Washington, DC, USA

No disclosure

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.

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 angiographyPerformed 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).

InterventionsPerformed 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%.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.

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.