Selective Shunt Occlusion Safe, Effective in vEDS Patient with Abnormal Blood Vessel Communication, Case Report Shows

A patient with vascular Ehlers-Danlos syndrome (vEDS) and a type of abnormal communication between blood vessels called carotid-cavernous fistula (CCF) was successfully treated via selective shunt occlusion, according to a case report.

The study, “Selective Shunt Occlusion of Direct Carotid-Cavernous Fistula with Vascular Ehlers-Danlos Syndrome by Multidevice Technique: A Case Report and Technical Note,” was published in the journal World Neurosurgery.

vEDS, considered the most severe form of EDS, is characterized by systemic complications in blood vessels. CCF, which is the abnormal communication between the internal and external carotid arteries and the cavernous sinus, or the blood vessels taking blood to and from the brain, is the most common neurovascular event that occurs in vEDS.

A team in Japan described the case of a 48-year-old man with vESD and CCF type A — characterized by direct shunting of blood flow from the internal carotid artery (ICA) to the cavernous sinus — treated via selective shunt (or small passage) occlusion using a combination of different approaches.

The man, who had also been diagnosed with bilateral aneurysms — bubble-like overgrowths of weakened blood vessels — in the ICA went to a hospital with head trauma. Though a computed tomography scan revealed no intracranial lesions, he experienced discomfort in his right eye the next day, followed by double vision and protruding eyeball on the right. Right CCF was detected on magnetic resonance imaging (MRI).

Upon referral to Japan’s National Cerebral and Cardiovascular Center in Osaka, the patient also exhibited a drooping eyelid, oculomotor nerve palsy, and murmur in the right eye.

An MRI scan then revealed dilatation of the right superior ophthalmic vein (SOV), a dilated cavernous sinus, and no cortical venous reflux, which occurs when vein valves fail to close properly. Subsequent examinations found intracranial aneurysms in the ICAs.

To avoid vEDS-related vascular complications, the clinicians decided to perform a neck dissection to expose the carotid artery and the jugular vein. The selective shunt occlusion involved transvenous embolization with a double microcatheter technique and transarterial embolization with a balloon-assisted technique. Embolization refers to a minimally invasive procedure that blocks a blood vessel.

Specifically, the approach required the insertion of catheters into the jugular vein and carotid artery, as well as a balloon-guiding catheter into the cervical portion of the right ICA. Then a balloon catheter was introduced, and a microcatheter was advanced into the cavernous sinus through the shunting point.

A guiding catheter was introduced into the inferior petrosal sinus, which runs between the petrous temporal bone and the basilar occipital bone. A microcatheter was then advanced into the SOV and another into the shunt segment, located downstream of the shunting point.

The main drainage to the SOV was closed with coils. Then the shunting point was closed by coil embolization via a transarterial catheter, using the balloon-assisted technique. The CCF was eliminated and normal blood flow in the right ICA was restored. The surgical wound was closed, and the venous and arterial sheaths were removed.

Following surgery, the patient’s symptoms disappeared except for his double vision, which diminished over three months. No wound complications were found in the neck. Post-surgery MRI revealed right CCF occlusion.

“Selective shunt occlusion via a transarterial and transvenous multidevice technique is a useful and safe approach for treating vEDS-associated CCF,” the scientists wrote.