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Caroticocavernous fistula
Dr Yuranga Weerakkody and Dr Donna D'Souza et al.
Carotico-cavernous fistulas represent abnormal communication between the carotid circulation and the cavernous
sinus. They can be classified as direct or indirect which are separate conditions with different aetiologies.
Demographics and clinical presentation
Direct CCFs are often secondary to trauma, and as such the demographics reflect the distribution of head trauma, most
commonly seen in the young male patients. Presentation is acute and symptoms develop rapidly.
In contrast, indirect CCFs have a predilection for the postmenopausal female patient and the onset of symptoms is often
insiduous.
Other conditions that predispose to increased risk include
Ehlers-Danlos syndrome
fibromuscular dysplasia
Clinical findings include:
pulsatile exophthalmos / proptosis : ~ 75%
3
chemosis and subconjunctival haemorrhage
progressive visual loss : 25-32%
3
pulsatile tinnitus (usually objective)
raised intracranial pressure
subarachnoid haemorrhage, intracerebral haemorrhage, otorrhagia, epistaxis : ~ 2.5-8.5%
3
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Pathology
Classification
It can be broadly classified into two main types
direct : direct communication between intra-cavernous ICA and cavernous sinus.
indirect : communication exists via branches of the carotid circulation (ICA or ECA)
Another method is to classify according to four main types
type A - direct connection between the intracavernous ICA and CS
type B - dural shunt between intracavernous branches of the ICA and CS
type C - dural shunt between meningeal branches of the ECA and CS
type D - B + C
Di rect : type A
A direct fistula is due to a direct communication between the intracavernous ICA and the cavernous sinus. There are a
number of causes, however aneurysm rupture and trauma are by far the most common.
ruptured intracavernous carotid artery aneurysm
trauma (including surgery / angiography)
other causes include:
collagen deficiency syndromes
fibromuscular dysplasia
arterial dissection
Indi rect : types B, C, D
Indirect fistulas are due to communication by multiple branches between the ICA / ECA and CS. The are most
frequent are type C, with meningeal branches of the ECA forming the fistula
3
.
They are postulated to occur secondary to cavernous sinus thrombosis with revascularisation. Other predisposing
factors appear to be pregnancy, surgical procedures in the region, sinusitis
3
.
They should be considered as special cases of dural arteriovenous fistulas.
Radiographic features
CT
proptosis
enlarged superior ophthalmic veins
extra ocular muscles may be enlarged
orbital oedema
may show SAH / ICH from ruptured cortical vein
DSA - Angiography
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rapid shunting from ICA to CS
enlarged draining veins
retrograde flow from CS, most commonly into the opthalmic veins
Ultrasound
arterialised ophthalmic veins may be seen on US-Doppler
Treatment and prognosis
The natural history of CCF is highly varied, ranging from spontaneous closure to rapidly progressive symptoms. Poor
treatment outcome indicators include feeding vessel aneurysms (indirect CCF) and retrograde filling of cortical veins
(increased risk of haemorrhage).
Direct fistulas have a relatively high spontaneous rate of haemorrhage (8.4%). This can be in the form of subarachnoid,
intracerebral or external haemorrhage (epistaxis, otorrhagia). Subconjunctival haemorrhage is also common but does not
carry the same poor prognosis
3
.
Carotid compression therapy is most useful in the treatment of indirect fistulas resulting in spontaneous closure in up
to 30% of cases. It is only successful in 17% of direct fistulas, presumably due to the higher flow through larger defect
3
.
Trans arterial balloon embolisation is the treatment of choice for symptomatic direct CCF. If not possible
detachable coils may be used. Both arterial and venous access (including superior ophthalmic vein) may be employed.
Indirect fistulas typically require a combined trans arterial (closure of feeders) and trans venous (closure of cavernous
sinus) approach. They are more difficult to treat, and have a higher rate of spontaneous closure.
Surgical treatment with ligation or trapping of involved segments has also been used
3
References
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Article Information:
Systems: Vascular, Central Nervous System, Head & Neck, Interventional
Tags: vascular, emergencymedicine
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Case 1: CTA
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Case 2: indirect (ICA)
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Case 3: indirect (ECA)
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Case 4: direct (ICA)
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Case 5
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Case 6: with enlarged opthalmic veins
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Case 7
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