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Adrian W. Laxton, Patrick Shannon, Sukriti Nag, Richard I. Farb and Mark Bernstein

✓ This 39-year-old man presented with a 6-month history of occipital headaches. Magnetic resonance imaging revealed an irregularly shaped fourth ventricle mass. One month after his initial presentation, he was admitted to the hospital with significant tumor expansion and clinical deterioration. A posterior fossa craniectomy was performed and the mass was resected. Histopathological analysis of this tumor showed central necrosis with associated edema in an otherwise typical and benign-appearing subependymoma. To the authors' knowledge, this is the first reported case of rapid, nonhemorrhagic expansion associated with necrosis in a previously asymptomatic subependymoma.

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Wouter I. Schievink, M. Marcel Maya, Franklin G. Moser, Ravi S. Prasad, Rachelle B. Cruz, Miriam Nuño and Richard I. Farb

OBJECTIVE

Spontaneous spinal CSF–venous fistulas are a distinct type of spinal CSF leak recently described in patients with spontaneous intracranial hypotension (SIH). Using digital subtraction myelography (DSM) with the patient in the prone position, the authors have been able to demonstrate such fistulas in about one-fifth of patients with SIH in whom conventional spinal imaging (MRI or CT myelography) showed no evidence for a CSF leak (i.e., the presence of extradural CSF). The authors compared findings of DSM with patients in the lateral decubitus position versus the prone position and now report a significantly increased yield of identifying spinal CSF–venous fistulas with this modification of their imaging protocol.

METHODS

The population consisted of 23 patients with SIH who underwent DSM in the lateral decubitus position and 26 patients with SIH who underwent DSM in the prone position. None of the patients had evidence of a CSF leak on conventional spinal imaging.

RESULTS

A CSF–venous fistula was demonstrated in 17 (74%) of the 23 patients who underwent DSM in the lateral decubitus position compared to 4 (15%) of the 26 patients who underwent DSM in the prone position (p < 0.0001). The mean age of these 16 women and 5 men was 52.5 years (range 36–66 years).

CONCLUSIONS

Among SIH patients in whom conventional spinal imaging showed no evidence of a CSF leak, DSM in the lateral decubitus position demonstrated a CSF–venous fistula in about three-fourths of patients compared to only 15% of patients when the DSM was performed in the prone position, an approximately five-fold increase in the detection rate. Spinal CSF–venous fistulas are not rare among patients with SIH.

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Wouter I. Schievink, Marcel M. Maya, Franklin G. Moser, Alexander Tuchman, Rachelle B. Cruz, Richard I. Farb, Ryan Rebello, Kesava Reddy and Ravi S. Prasad

Spontaneous CSF–venous fistulas may be present in up to one-fourth of patients with spontaneous intracranial hypotension. This is a recently discovered type of CSF leak, and much remains unknown about these fistulas. Spinal CSF–venous fistulas are usually seen in coexistence with a spinal meningeal diverticulum, suggesting the presence of an underlying structural dural weakness at the proximal portion of the fistula. The authors now report the presence of soft-tissue venous/venolymphatic malformations associated with spontaneous spinal CSF–venous fistulas in 2 patients with spontaneous intracranial hypotension, suggesting a role for distal venous pathology. In a third patient with spontaneous intracranial hypotension and a venolymphatic malformation, such a CSF–venous fistula is strongly suspected.