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Cerebral vasculopathy in childhood neurofibromatosis type 2: cause for concern?
Author(s) -
Lascelles Karine,
Afridi Shazia,
Siddiqui Ata,
Hemingway Cheryl,
Ferner Rosalie,
Ganesan Vijeya
Publication year - 2018
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.13920
Subject(s) - neurofibromatosis type 2 , medicine , bevacizumab , neurofibromatosis , magnetic resonance imaging , stroke (engine) , magnetic resonance angiography , pediatrics , radiology , chemotherapy , mechanical engineering , engineering
Unlike adult neurofibromatosis type 2 ( NF 2), which presents with symptoms related to bilateral vestibular schwannomas, children with NF2 most frequently present with ocular, dermatological, and neurological symptoms. Arteriopathy, a well‐established feature in neurofibromatosis type 1, is not a widely recognized feature of NF 2. Here we report three children with NF 2 with cerebral arteriopathy and/or arterial ischaemic stroke. Bevacizumab, a vascular endothethial growth factor inhibitor, is an established treatment for rapidly growing vestibular schwannomas; however, it carries a risk of both ischaemic and haemorrhagic stroke. Thus, the role of screening and risk to benefit ratio of bevacizumab in NF 2 merit further consideration. What this paper adds Children with neurofibromatosis type 2 (NF2) may be at increased risk of cerebral vasculopathy and arterial ischaemic stroke. Targeted magnetic resonance angiography should be performed in children with NF2 who are being considered for bevacizumab therapy.

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