A rare condition: Spontaneous subarachnoid haemorrhage due to spinal hemangioblastoma: Report of 2 cases and review of the literature.

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Version: Final published version
License: CC BY 4.0
Serval ID
serval:BIB_14502311A9D7
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
A rare condition: Spontaneous subarachnoid haemorrhage due to spinal hemangioblastoma: Report of 2 cases and review of the literature.
Journal
Neuro-Chirurgie
Author(s)
Tuleasca C., Knafo S., David P., Richard S., Adam C., Aghakhani N., Parker F.
ISSN
1773-0619 (Electronic)
ISSN-L
0028-3770
Publication state
Published
Issued date
11/2020
Peer-reviewed
Oui
Volume
66
Number
5
Pages
359-364
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
Subarachnoid haemorrhage (SAH), secondary to spinal hemangioblastoma (HBL), is extremely rare, with only a few case reports to date. We report the experience of our reference centre for spinal tumours and Von Hippel-Lindau (VHL) disease in patients with spinal HBL presenting with SAH. We further performed a systematic review of the literature.
We report two cases. A systematic search was performed using the PubMed, Embase and Cochrane databases, with no limit for publication date. Inclusion criteria were: patients with HBL presenting with SAH, with or without VHL. The systematic review retrieved only 10 studies, including 16 patients.
In our centre, the first case concerned radicular HBL at D12 level, presenting with spinal and brain SAH. The patient underwent uneventful microsurgical en bloc resection. Postoperative course was normal. The second case concerned HBL with SAH at the cervico-medullary junction, with rapidly fatal course. The systematic review revealed female predominance, at a median age of 40 years, with HBL predominantly located at cervical level, common preoperative symptoms being headache and signs of meningeal irritation.
In conclusion, spinal HBL is an extremely rare cause of SAH. The systematic review found putative risk factors: female gender, age 40-50 years, cervical location, and median size 2cm. Diagnosis can be difficult when presentation mimics intracerebral SAH. We advocate early surgical removal. The risk of rapidly fatal course, in case of major haemorrhage, needs to be borne in mind.
Keywords
hemangioblastoma, hémangioblastomes, hémorragie sous-arachnoïdienne, spinal, spinale, subarachnoid hemorrhage, von Hippel-Lindau, Hemangioblastomas, Spinal, Subarachnoid haemorrhage, Von Hippel–Lindau
Pubmed
Web of science
Open Access
Yes
Funding(s)
University of Lausanne / CRND-31188
Create date
01/09/2020 21:25
Last modification date
08/08/2022 6:08
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