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¹Sheffield Children’s Hospital, Department of Neurosurgery, Sheffield, United Kingdom
²Sheffield Children’s Hospital, Department of Neuroradiology, Sheffield, United Kingdom DOI : 10.5137/1019-5149.JTN.6256-12.1 Blue rubber bleb naevus syndrome is an uncommon disorder whereby patients possess cutaneous vascular malformations. Cranial malformations have been rarely reported in the literature. We report the first ever case of a patient with blue rubber bleb naevus syndrome (BRBNS), who was found to have a symptomatic Chiari malformation and prominent subcutaneous vascular malformations in communication with the intracranial venous sinuses. In our case, a routine operation for Chiari malformation was complicated by significant blood loss. The operative hazards are presented along with suggested peri-operative management strategies when carrying out neurosurgical operations on patients with BRBNS. A brief literature review is included. Keywords : Blue rubber naevus syndrome, Chiari malformation, Foramen magnum decompression, Vascular malformation