Neuropediatrics 2018; 49(S 02): S1-S69
DOI: 10.1055/s-0038-1675946
Oral Presentation
Stroke and Inflammatory Diseases
Georg Thieme Verlag KG Stuttgart · New York

FV 820. Pediatric Moyamoya Angiopathy: A Rapid Progressive Disease in Children < 5 Years of Age

Annette Hackenberg
1   Department of Pediatric Neurology, University Children’s Hospital Zurich, Zurich, Switzerland
,
Gerasimos Baltsavias
2   Department of Neuroradiology, University Hospital Zurich, Zurich, Switzerland
,
Nadia Khan
3   Division of Pediatric Neurosurgery, Department of Surgery, Moyamoya Center, University Children’s Hospital Zurich, Zurich, Switzerland
› Author Affiliations
Further Information

Publication History

Publication Date:
30 October 2018 (online)

 

Background: Intracranial stenosis/occlusion of the internal carotid artery (ICA) and circle of Willis in pediatric moyamoya angiopathy leads to early and repetitive strokes.

Aims and Objectives: We evaluate the clinical and neuroradiological presentation at the time of diagnosis and follow-up in newly diagnosed children <5 years of age referred to our Moyamoya Center, University Children’s Hospital Zurich.

Methods: Seventy-nine children with newly diagnosed moyamoya angiopathy at a mean age of 6 years (range: 5 months–17 years) underwent preoperative work-up and cerebral revascularization between 2011 and 2017. Thirty-five children (44%) were < 5 years of age. Neurological evaluation along with a preoperative MRI and MRA, angiography, and H2 15O-PET with acetazolamide challenge was performed in all children. Depending on these results, cerebral revascularization was performed in multiple vascular territories involved. All children were followed up from 6 months to 5 years to date after surgery.

Results: Thirty-five children were < 5 years of age (23 less than 2 years of age; 12 between 2 and 5 years of age). F:M ratio was 15:20.

Bilateral disease was seen in 70%. Posterior cerebral artery (PCA) involvement was seen in 42% of children. Stroke (67%) and TIAs (75%) were the main symptoms at presentation. The majority of children (94%) presented with cortical territorial strokes. Middle cerebral artery (MCA) territory was always involved. Multiterritorial strokes were seen in 31% with MCA and anterior cerebral artery (ACA) involvement. In 25% ACA, MCA, and PCA territories were involved. Rapid progression of disease with repetitive strokes was seen within 2 to 8 weeks in nine children, all were less than 2 years of age. Progression of disease to the contralateral side was seen within 1 year in two patients who initially presented with unilateral MCA angiopathy.

All children underwent multiple territorial staged cerebral revascularization and remain stroke free at 5 years after surgery.

Conclusion: Moyamoya is a rapidly progressive angiopathy in children < 5 years of age involving both anterior and posterior cerebral circulation causing territorial cortical strokes. Early diagnosis, treatment, and strict follow-up are imperative.