Cerebrospinal fluid leak in epidural venous malformations and blue rubber bleb nevus syndrome

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  • 1 Division of Vascular and Interventional Radiology,
  • | 2 Department of Surgery,
  • | 3 Department of Neurosurgery, and
  • | 4 Division of Hematology/Oncology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
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OBJECTIVE

Clinical manifestations of blue rubber bleb nevus syndrome (BRBNS) and multifocal venous malformation (MVM) vary depending on the location of the lesions. The aim of this study was to assess the risk of developing CSF leaks in patients with epidural venous malformations (VMs).

METHODS

The authors retrospectively investigated the relationship between the development of a CSF leak and the presence of epidural VMs.

RESULTS

Nine patients (5 females) had epidural VMs and presentation that was confirmatory or suggestive of a CSF leak: 4 had BRBNS, 4 had MVMs, and 1 had a solitary VM. Of 66 patients with BRBNS, clinical and imaging features of CSF leak were noted in 3 (4.5%) with epidural VMs at the age of 11–44 years. A fourth patient had suggestive symptoms without imaging confirmation. An epidural blood patch was ineffective in 2 patients, both with more than one source of leakage, requiring surgical repair or decompression. Symptomatic downward displacement of the cerebellar tonsils was noted in 3 patients with MVM and 1 with a solitary VM; 3 required surgical decompression.

CONCLUSIONS

These findings suggest an increased risk of CSF leak in patients with epidural VM, including BRBNS, MVMs, and solitary VMs. Awareness of the association between epidural VM and CSF leakage may facilitate earlier diagnosis and therapeutic intervention.

ABBREVIATIONS

BRBNS = blue rubber bleb nevus syndrome; GI = gastrointestinal; KTS = Klippel-Trenaunay syndrome; MVM = multifocal VM; SIH = spontaneous intracranial hypotension; VAC = Vascular Anomalies Center; VM = venous malformation.

Illustration from Dibble et al. (pp 384–394). © Washington University Department of Neurosurgery, published with permission.

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