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Dural Arteriovenous Fistulas

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Handbook of Cerebrovascular Disease and Neurointerventional Technique

Part of the book series: Contemporary Medical Imaging ((CMI))

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Abstract

Dural arteriovenous fistulas (dAVF) are abnormal arteriovenous shunts between meningeal arteries and intracranial dural sinuses or veins. This chapter discusses the anatomy, classification schemes, pathophysiology, clinical presentation, pediatric dAVFs, imaging characteristics, natural history, and management. Nuances of presentation and management based on location of the fistula are also discussed. The Appendix 14.1 covers carotid-cavernous fistulas. Spinal dural AV fistulas are covered in Chap. 20, Spinal Vascular Lesions.

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Authors and Affiliations

  1. Departments of Neurosurgery, Neurology and Radiology, University of Alabama at Birmingham, Birmingham, Alabama, USA

    Mark R. Harrigan

  2. Department of Neurosurgery and Radiology, University of Alabama at Birmingham, Birmingham, Alabama, USA

    John P. Deveikis

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Appendix 14.1: Direct Carotid-Cavernous Fistulas

Appendix 14.1: Direct Carotid-Cavernous Fistulas

1.1 Direct CC Fistulas: Clinical Features

  1. 1.

    Causes

    1. (a)

      Trauma (most common cause)

      1. (i)

        CC fistula was found in 3.8% of patients with skull base fracture [155].

    2. (b)

      Ruptured cavernous segment ICA aneurysm (cause of ≤20% of direct CC fistulas [156,157,158])

    3. (c)

      Ehlers-Danlos Type IV [159] (see separate discussion below)

    4. (d)

      Fibromuscular dysplasia [160]

    5. (e)

      Pseudoxanthoma elasticum

    6. (f)

      Iatrogenic causes: CCF following:

      1. (i)

        Endoscopic sinus surgery.

      2. (ii)

        Transsphenoidal pituitary surgery [161].

      3. (iii)

        Trigeminal balloon microcompression gangliolysis [162].

      4. (iv)

        Perforation of the meningohypophyseal trunk during embolization of a meningioma [163].

      5. (v)

        Pipeline flow-diversion for cavernous aneurysm [164, 165].

    7. (g)

      Fungal arteritis associated with:

      1. (i)

        Osteogenesis imperfecta [166].

  2. 2.

    Anatomy

    1. (a)

      ICA defect:

      1. (i)

        Most direct fistulas are a single hole measuring 2–6 mm in diameter [167].

      2. (ii)

        Most common location the defect in traumatic cases is the horizontal cavernous segment [167].

      3. (iii)

        May consist of more than one defect in the ICA (e.g., “double-hole” fistula) or a complete transection of the ICA.

      4. (iv)

        Bilateral traumatic CC fistulas are present in 1–2% of cases [157, 168]

    2. (b)

      Venous drainage:

      1. (i)

        Cavernous sinus.

        • Retrograde intracranial venous flow is present in 9% of patients [169].

  3. 3.

    Presentation

    1. (a)

      Classic description: Pulsating exophthalmos.

    2. (b)

      Most frequent presentation: Exophthalmia with pulsating conjunctival hyperemia and vascular murmur [156].

    3. (c)

      Severity of symptoms depends on the cause of fistula and the severity of the ICA lesion.

    4. (d)

      Common findings include injection and chemosis, proptosis, elevated intraocular pressure, ophthalmoplegia, and a periorbital bruit.

      1. (i)

        The sixth cranial nerve is most commonly affected [117].

      2. (ii)

        A third cranial nerve palsy, when present, may or may not be pupil-sparing (in contrast to third cranial nerve palsies due to a posterior communicating artery aneurysm, which almost invariably has pupillary involvement).

      3. (iii)

        Rarely, seventh cranial nerve dysfunction is present [170].

    5. (e)

      A bruit is present in 80% of cases [157].

    6. (f)

      Cerebral ischemia of the ipsilateral hemisphere can occur if the ICA flow is diverted into the cavernous sinus (i.e., a functional ICA occlusion is present, even if the vessel is physically patent) and collateral circulation is insufficient. A direct CC fistula may also act as a sump and divert flow from major intracranial arteries such as the p-comm [169].

  4. 4.

    Imaging

    1. (a)

      CT

      1. (i)

        In traumatic cases, a fine-cut head CT should be obtained to assess for skull fractures.

      2. (ii)

        Skull fractures are present in 7–17% of traumatic CC fistula cases.

      3. (iii)

        CTA may show occlusion of the ICA or engorgement of the cavernous sinus.

    2. (b)

      MRA: Elliptical centric time-resolved imaging of contrast kinetics (EC-TRICKS) has provided good imaging of a direct CC fistula [171].

    3. (c)

      Angiography

      1. (i)

        A catheter angiogram remains the gold standard for a complete evaluation of a direct CC fistula [101].

      2. (ii)

        Contralateral carotid and vertebral injections should be done to assess collateral circulation.

      3. (iii)

        The anatomy of high-flow fistulas are difficult to discern, particularly if all of the contrast is diverted into the cavernous sinus. Helpful angiographic techniques:

        • Mehringer–Hieshima maneuver [172]: Low-rate injection (2–3 mL/s) into the ipsilateral ICA while manual compression is applied to the ipsilateral CCA. Flow through the fistula is reduced to make it easier to see.

        • Huber maneuver [173] (aka Allcock’s test): Selective injection of the dominant vertebral artery while manual compression is applied to the ipsilateral CCA. The fistula will opacify by retrograde flow.

1.2 Direct CC Fistulas: Management

Spontaneous closure of direct CC fistulas occurs, but it is rare [158]. Some small asymptomatic direct fistulas can be left untreated, but most do require intervention. Further discussion of the management of cavernous segment ICA aneurysms is in Chap. 13.

  1. 1.

    Manual compression

    1. (a)

      Technique: The patient is instructed to use the opposite hand to locate the pulse of the carotid artery in the mid-neck region just lateral to the trachea. Gradually increasing pressure is applied until the palpable pulse is stopped. The opposite hand is used in case hemispheric ischemia develops. Compression is maintained for 10–15 s at a time, 2–3 times an hour.

      1. (i)

        Contraindications: Cervical carotid artery disease (atherosclerosis, dissection), sick sinus syndrome, poor patient compliance.

      2. (ii)

        Results: 17% of patients with a direct CC fistula had complete closure of the fistula with no recurrence either clinically or at angiography done 1 year later [103].

  2. 2.

    Cavernous sinus embolization

    1. (a)

      Transarterial

      1. (i)

        Detachable balloons

        • Cavernous sinus embolization with detachable balloons is associated with a fistula occlusion rate of ~90% with preservation of the ICA in 60–88% of cases [61, 168, 174]. Detachable balloons are not currently available in the United States, but they are still in use in other countries [175, 176].

      2. (ii)

        Coils [177, 178]

        • Coil embolization is most useful for the treatment of direct CC fistulas due to a ruptured cavernous segment aneurysm [176]. Transarterial embolization of traumatic CC fistulas can be problematic, particularly when the defect in the wall of the ICA is large. An initial closure of the fistula may be obtained, but the coils may migrate within the cavernous sinus over time, leading to a recurrence of the fistula. The authors of this handbook have witnessed this scenario repeatedly.

        • Coils or balloons + liquid embolics

          • Transarterial embolization of the cavernous sinus with coils, combined with glue (NBCA) or Onyx, can be effective in select cases [175].

    2. (b)

      Access to the cavernous sinus can even be reached through the vertebral artery [179].

    3. (c)

      Transvenous

      1. (i)

        Transvenous embolization with coils alone seems to be more effective than transarterial embolization with coils alone, likely because better microcatheter positioning is possible and tighter packing of the cavernous sinus can be achieved [177, 178].

    4. (d)

      Superior ophthalmic vein approach [67].

  3. 3.

    Stenting

    1. (a)

      Stent-assisted embolization [180].

    2. (b)

      Covered stent repair of the ICA [181, 182].

    3. (c)

      Pipeline flow-diversion is being used to augment coil embolization, particularly in the case of fistulas caused by ruptured cavernous carotid aneurysm [183].

  4. 4.

    Carotid sacrifice

    1. (a)

      Parent vessel occlusion is a valid option, but should be preceded by balloon test occlusion.

1.3 Ehlers-Danlos Type IV

Ehlers-Danlos Type IV (aka vascular type) is a rare autosomal dominant collagen-vascular disorder. Type IV accounts for 4% of all Ehlers-Danlos cases and is the most severe form of the disease.

  1. 1.

    Diagnosis

    1. (a)

      Four clinical criteria [184]:

      1. (i)

        Easy bruising

      2. (ii)

        Thin skin with visible veins

      3. (iii)

        Characteristic facial features

      4. (iv)

        Rupture of arteries, uterus, or intestines

    2. (b)

      Confirmation of diagnosis:

      1. (i)

        Cultured fibroblasts synthesize abnormal type III procollagen molecules or identification of a mutation in the gene for type III procollagen [185].

  2. 2.

    Pathophysiology

    1. (a)

      Type III collagen is decreased or absent.

    2. (b)

      The vessels in affected patients have reduced total collagen content and have thin walls with irregular elastic fibrils and reduced cross-sectional area.

  3. 3.

    Epidemiology

    1. (a)

      Very rare. Prevalence is unknown.

  4. 4.

    Clinical features

    1. (a)

      Hypermobility of large joints and hyperextensibility of the skin, features of the more common forms of Ehlers-Danlos syndrome are unusual in Type IV.

    2. (b)

      Median life expectancy is 48 years [185].

      1. (i)

        Complications are rare in childhood; 25% of patients have a first complication by the age of 20 years and more than 80% have at least one complication by the age of 40 [185].

      2. (ii)

        Most deaths result from arterial dissection or rupture [185].

    3. (c)

      Cerebrovascular manifestations

      1. (i)

        About 10% of patients have an arterial problem affecting the central nervous system [185].

      2. (ii)

        Direct CC fistulas

        • Most common cerebrovascular complication.

        • Patients are predominantly female [186].

        • May be bilateral [187].

        • Endovascular treatment of direct CC fistulas in patients with Ehlers Danlos IV is very hazardous because of the fragility of the vessels [159, 187, 188].

        • A review of published reports of diagnostic cerebral angiography in patients with Elhers-Danlos IV, the overall morbidity was 36% and mortality was 12% [189]. In another report, two of four patients died due to remote vascular injuries around the time of their neurointerventional procedures [188].

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Harrigan, M.R., Deveikis, J.P. (2018). Dural Arteriovenous Fistulas. In: Handbook of Cerebrovascular Disease and Neurointerventional Technique. Contemporary Medical Imaging. Humana, Cham. https://doi.org/10.1007/978-3-319-66779-9_14

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  • DOI: https://doi.org/10.1007/978-3-319-66779-9_14

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