SESSION 11: New technologies in Pediatric Neurosurgery Management strategy in pediatric moyamoya angiopathy: the Zurich
Moyamoya Center experience
Nadia Khan1, Dubravka Deanovic2, Martin Hoelzle2, Martina Hug3, Annette Hackenberg4,
Alfred Buck5, Gerasimos Baltsavias6
1 Moyamoya Center, University Children’s Hospital Zurich, Switzerland
2 Department of Anesthesiology, University Children’s Hospital Zurich, Switzerland
3 Center for Child Development, University Children’s Hospital Zurich, Switzerland
4 Department of Pediatric Neurology, University Children’s Hospital Zurich, Switzerland
5 Department of Nuclear Medicine, University Hospital Zurich, Switzerland
6 Department of Neuroradiology, University Hospital Zurich, Switzerland

Introduction and Objectives: Moyamoya is a dynamic and progressive steno-occlusive angiopathy
of the circle of Willis presenting with repetitive ischemic strokes In the pediatric age group. The
management strategy of newly diagnosed children referred to our moyamoya center is presented.
Method / Patient Selection: Twenty-six newly diagnosed moyamoya children, referred from within
Europe and internationally, were managed at our moyamoya center between March 2011 - December
2013. Mean age was 8 years (range 1-17 years). Female to male ratio was 15:11.
Results: Diagnostic workup consisted of clinical-neurological evaluation including child
development/cognition testing, diagnostic 6-vessel cerebral angiography, MRI and H215O-PET scan
with Diamox challenge.
Headache and transient ischemic attacks were common presenting symptoms. Moyamoya syndrome
was seen in half of the children (13/26). Two of the syndromic moyamoya children were asymptomatic
clinically. Bilateral cortical/subcortical infarcts in distribution territories of anterior cerebral artery (ACA),
middle cerebral artery (MCA) and/or posterior cerebral artery (PCA) were seen mainly in children of
age < 5 years. Unilateral angiopathy was seen in 4 children. Twenty-four children showed bilaterally
decreased perfusion reserves on Diamox-H215O-PET in ACA, MCA and /or PCA territories.
Depending on symptomatology, extent of angiopathy and territorial perfusion reserve deficits, multiple
1 or 2 staged cerebral revascularization surgeries were performed. Clinical-neuroradiological follow up
at 6 months and 1-3 years after last surgery is ongoing and to be continued into adulthood.The first 15
children at average post-op follow up of 9 months (6 months-1 year) remain stroke free.
Conclusions: Moyamoya in Europe and internationally is highlighted. It is not only limited to the Asian
population. Accurate diagnostic work-up and thorough pre surgical planning is necessary for a good
clinical outcome. A dedicated center i.e. an interdisciplinary medical and neurosurgical team with an
infrastructure appropriate for the pediatric age group is pivotal for optimal management.

24th ESPN Congress (Rome-Italy • May 4-7, 2014)


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