J Korean Neurosurg Soc.  2016 May;59(3):296-301. 10.3340/jkns.2016.59.3.296.

Infantile Dural Arteriovenous Fistula of the Transverse Sinus Presenting with Ocular Symptoms, Case Reports and Review of Literature

• Sultan AE
• Hassan T

• KMID: 2192110
• DOI: http://doi.org/10.3340/jkns.2016.59.3.296

Abstract

Dural arteriovenous fistula (DAVF) of the transverse sinus with ophthalmic manifestations in young children are rare. We reviewed two cases of direct AVF of the transverse sinus with ocular manifestations managed at our institution. The first, a 2.5 years old male child presented with left exophthalmos. Angiography revealed AVF between the occipital artery and the transverse sinus. The second, a 2 years old female child, complained of left exophthalmos. Imaging studies showed bilateral direct AVFs of the transverse sinus with bilateral dysmaturation of the sigmoid sinus. Transarterial embolization was done in both cases. Clinical and radiological follow up revealed complete cure.This report suggests that DAVF of the transverse sinus supplied by the external carotid branches can present with ophthalmic manifestations especially if there is distal venous stenosis or obliteration involving sigmoid sinus. Transarterial embolization using coils and liquid embolic agents could be safe and feasible to obliterate the fistula.

Keyword

Dural arteriovenous fistula; Exophthalmos; Transverse sinus; Sigmoid sinus dysmaturation

MeSH Terms

Angiography
Arteries
Central Nervous System Vascular Malformations*
Child
Colon, Sigmoid
Constriction, Pathologic
Exophthalmos
Female
Fistula
Follow-Up Studies
Humans
Male

Figure

• 

  Fig. 1 Imaging study of case 1 before embolization showing direct fistula with the transverse sinus. A : Axial view of brain CTA showing enlarged left superior ophthalmic vein (arrow). B : Axial T1 MRI brain showing enlarged left superior ophthalmic vein (arrow). C and D : 3D CTA showing the fistula between the occipital artery and the transverse sinus enlarged left superior ophthalmic vein (arrow).

• 

  Fig. 2 Imaging study of case 1 before and after embolization showing direct fistula with the transverse sinus. A : Lateral view of the left vertebral artery showing vertebral dural branches supplying the fistula. B : Lateral view of left common carotid injection showing a hugely dilated occipital artery directly communicating with the transverse sinus. C : Common carotid injection after coiling and NBCA injection showing occlusion of the fistula. D : Lateral view of the left CCA angiography after 2 years showing disappearance of the fistula. E and F : anteroposterior and lateral view of the left vertebral artery angiogram showing disappearance of the fistula. NBCA : N-butyl Cyanoacrylate, CCA : common carotid artery.

• 

  Fig. 3 Imaging study of case 2, a 1.5 years old female patient presenting with proptosis. A : Axial T2 MRI of the brain showing dilated ophthalmic veins (white arrow). B : Right CCA angiogram showing direct fistula between the occipital artery and the transverse sinus with the absence of the sigmoid sinus. C : Venous phase of right CCA angiogram showing venous reflux anteriorly through the deep and superficial venous systems including the cavernous sinus (black arrow) and ophthalmic veins. D : Lateral view of left CCA angiogram showing AVF supplied by branches of the ECA. E : Venous phase of left CCA angiogram showing retrograde venous drainage into the superficial and deep venous systems with absent sigmoid sinus. CCA : common carotid artery, ECA : external carotid artery, AVF : arteriovenous fistula.

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  Fig. 4 Cerebral angiography during embolization of the fistula of case 2. A : Lateral view of right ECA angiogram showing AVF supplied by occipital artery. B : Occlusion of the fistula using coils and NBCA. C : Lateral view of left ECA angiogram showing AVF supplied by branches of the ECA. D : Left ECA angiogram after Onyx embolization showing complete disappearance of the fistula. ECA : external carotid artery, AVF : arteriovenous fistula, NBCA : N-butyl Cyanoacrylate.

• 

  Fig. 5 Cerebral angiography 4 years after embolization in case 2. A : Lateral view of left CCA angiogram showing complete disappearance of the fistula. B : Lateral views of the venous phase of left CCA angiogram showing retrograde venous drainage anteriorly into the superficial and deep venous systems with absent sigmoid sinus with complete fistula disappearance (arrow). C : Venous phase of right CCA angiogram showing drainage is passing through the cavernous sinus and ophthalmic with absent sigmoid sinus with complete fistula disappearance (arrow). D : Plain X-ray lateral view of the skull showing shadow of Onyx and coils. CCA : common carotid artery.

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