Turkish Neurosurgery 2013 , Vol 23 , Num 6
Blue Rubber Bleb Naevus Syndrome and Chiari Malformation: High Risk of Peroperative Haemorrhage
Anuj BAHL1, Ashok RAGHAVAN2, Saurabh SINHA1
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
Corresponding author : Anuj Bahl, anujbahl3@yahoo.co.uk