A 61-year-old woman who is otherwise healthy presented to her PCP with progressive right-sided pulsatile tinnitus for 4-6 weeks. Her PCP and ENT evaluated her extensively and prescribed multiple courses of steroids and subsequent unremarkable MRI and MRA imaging. She developed acute onset diplopia secondary to a new partial right VI nerve palsy several days prior to our evaluation. A cerebral angiogram confirmed the presence of a high flow direct fistula of the right internal carotid artery–right cavernous sinus. Collateral venous drainage into the orbit and skull base was observed (Fig. A). After successful cannulation of the direct aperture between the right carotid artery and the cavernous sinus, balloon assisted coil embolization of the posterolateral compartment was performed until the fistula was closed (Fig. B and C). She experienced immediate cessation of her pulsatile tinnitus with early improvement of the right VI nerve palsy and near complete recovery of her vision within 6 weeks.
Figure: (A) Right ICA Cavernous Fistula with Venous Shunting (B) Balloon Assisted Coil Embolization (C) Fistula Cured Post Embolization
Key Learning Points:

  1. Carotid-Cavernous Sinus Fistula (CCF) may occur because of traumatic or spontaneous communication in the walls of the intra-cavernous ICA or its branches directly to the cavernous sinus resulting in short-circuiting or shunting of high-pressure arterial blood into the venous system of the cavernous sinuses.
  2. Intra-cavernous aneurysms are often felt to predispose to a Direct CCF which have high rates of arterialized blood flow and can result in rapid progression of clinical symptoms including cranial nerve injury and vision loss. 
  3. Early evaluation by a neurovascular specialist is recommended for patients presenting with new onset or progressive pulsatile tinnitus, especially in patients with associated visual symptoms or new neurologic deficits.
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Brain Stem Cavernous Malformation
A 13 year old boy fell while playing soccer and had a head ache. He was taken to an outside ER and was sent home.

Over the next week his mother noted a facial weakness and brought him to another ER where a CT scan was done which showed a bleed in his brainstem from a Cavernous malformation unrelated to his fall.
Before Treatment
He was transferred to our care and over the next week or so the bleed increased and he developed worsening weakness and slow heart rate. We took him to the OR and, operating under the temporal lobe to approach the brainstem from in front, removed the clot and the cavernous malformation.

Now, 2 months post operatively, he is normal.
After Treatment
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Dural Arterial - Venous Malformation
(Dural AVF/AVM)
A 55-year-old woman presented with acute onset of severe headache, and possible witnessed seizure. Imaging confirmed intraventricular hemorrhage primarily focused within the fourth ventricle with diffuse subarachnoid hemorrhage within the posterior fossa and along the tentorium (Fig. 1 A). No severe neurologic deficits were observed initially. Her cerebral angiogram revealed a high flow dural arterial venous fistulous malformation of the tentorium supplied by the bilateral external carotid and left vertebral arteries into a large venous varix within the fourth ventricle at the site of the hemorrhage (Fig. 1 B, C). Deep venous drainage is observed into the Galenic and Straight Sinus of the posterior fossa. Successful trans-arterial embolization with liquid embolics (N-butyl cyanoacrylate, NBCA) of the bilateral external and left vertebral posterior meningeal artery feeders was successful at reducing >90% of these high-pressure indirect shunts (Fig. 1 D), with a small residual shunts from the distal occipital arteries that were subsequently targeted for cure with Gamma Knife Radiosurgery.
Figure 1. [A] Ruptured aneurysm of the Deep Venous Posterior Fossa drainage and AV shunting [B] Posterior Meningeal Artery Fistula [C] Right Middle Meningeal Artery Fistula [D] Post embolization Left Vertebral Angiogram demonstrates resolution of the high flow and pressure A-V Shunting following successful embolization with liquid embolics (NBCA).
Key Learning Points:

  1. Indirect Dural AV Fistula Vascular Malformations represent high risk lesions that result from acquired and/or congenital indirect fistula and A-V shunts that can arise within many of the deep venous structures of the brain (Superficial and Deep Venous Drainage systems). 
  2. Many patients may present with initially mild symptoms of pulsatile tinnitus or headaches but may progress over time to more severe high-risk symptomatology including hemorrhagic stroke, permanent neurologic deficits, and even mortality. 
  3. Early evaluation by an expert multi-disciplinary team is critical to diagnosis, management, and successful treatment and recovery with a variety of medical, endovascular, and surgical approaches.
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