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 Table of Contents  
PHOTO ESSAY
Year : 2019  |  Volume : 31  |  Issue : 2  |  Page : 145-149

Cracking the mysteries: Arteriovenous malformation presenting as proptosis


Department of Ophthalmology, Government Medical College, Thrissur, Kerala, India

Date of Web Publication27-Aug-2019

Correspondence Address:
Dr. P Ramya Menon
Vrindavan, Sunder Iyer Road, Ottapalam, Palakkad - 679 101, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_32_19

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  Abstract 


Arteriovenous malformations (AVMs) are developmental anomalies that occur when the embryonic vascular network fails to differentiate. Proptosis can be caused by both intraorbital and extraorbital AVMs. Digital subtraction angiography (DSA) is essential for the diagnosis and for planning the management of orbital AVMs. In this article, we are discussing two cases of proptosis with AVM as the underlying pathology, but managed by different modes of treatment. Decision on the mode of treatment of AVMs should be taken carefully after weighing the pros and cons.

Keywords: Arteriovenous malformation, digital subtraction angiography, proptosis


How to cite this article:
Menon P R, Thulaseedharan S, Sudha V, Rajini K C. Cracking the mysteries: Arteriovenous malformation presenting as proptosis. Kerala J Ophthalmol 2019;31:145-9

How to cite this URL:
Menon P R, Thulaseedharan S, Sudha V, Rajini K C. Cracking the mysteries: Arteriovenous malformation presenting as proptosis. Kerala J Ophthalmol [serial online] 2019 [cited 2019 Dec 13];31:145-9. Available from: http://www.kjophthal.com/text.asp?2019/31/2/145/265495




  Introduction Top


Arteriovenous malformations (AVMs) of the orbit are developmental hamartomas consisting of an abnormal nidus of dysplastic vascular channels with feeding arteries and draining veins and the absence of intervening capillary network.[1] Intraorbital AVMs are rare, and their management represents a challenge for multidisciplinary teams. Unilateral proptosis can be caused by both intraorbital and extraorbital vascular malformations.


  Case Reports Top


Case 1

A 53-year-old hypertensive female with a history of gradual painless progressive protrusion of the right eye of 8 years' duration presented with sudden worsening of symptoms over 2 weeks. She complained of associated headache [Figure 1].
Figure 1: (Case1 ) A 53year old female who presented with axial proptosis right eye

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On examination, the best-corrected visual acuity in right eye was 6/12 and in left eye was 6/6. Color vision was normal in both eyes. External eye examination of the right eye showed severe axial proptosis (26 mm), chemosis, and dilated and tortuous episcleral vessels [Figure 2]. Pupillary reactions were brisk. Fundus was normal except for minimal tortuosity of vessels. Examination of the left eye was normal.
Figure 2: RE Severe axial proptosis(26mm) with chemosis & dilated episcleral vessels

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Routine investigations and thyroid function tests (TFTs) were normal.

USG ORBIT showed an irregular heterogeneous soft-tissue mass in the retrobulbar and suprabulbar regions with peripheral vascularity and enlarged thrombosed vein in the right orbit-suspicious of cavernous hemangioma [Figure 3] and [Figure 4].
Figure 3: USG orbit showing irregular ill-defined heterogeneous mass in the retrobulbar & suprabulbar region

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Figure 4: Colour doppler showing peripheral vascularity

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CT HEAD and ORBIT (PLAIN and CONTRAST): Lobulated hyperdense soft-tissue density with intense linear peripheral enhancement, central nonenhancing areas, and tiny calcification noted in the right retrobulbar region with proptosis [Figure 5] and [Figure 6]. Possibility of vascular malformation-with thrombosis.
Figure 5: CT Head and Orbit(Plain and Contrast) showing axial proptosis right eye

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Figure 6: CT HEAD and ORBIT (PLAIN and CONTRAST): Lobulated hyperdense soft-tissue density with intense linear peripheral enhancement, central nonenhancing areas. Possibility of vascular malformation

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MRI BRAIN (PLAIN and CONTRAST): Well-defined, intraconal, heterogeneously enhancing lesion in the superomedial aspect of the right orbit. Multiple flow voids noted within the lesion. [Figure 7] and [Figure 8] show patchy areas of T1 hyperintensity within the lesion. Suspicious of orbital varix with thrombosis/arteriovenous (AV) malformation.
Figure 7: MRI BRAIN (PLAIN and CONTRAST) showing intraconal, heterogeneously enhancing lesion in the superomedial aspect of the right orbit and suspicious of orbital varix with thrombosis/AV malformation

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Figure 8: MRI coronal view

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The case was discussed with neurosurgeon and radiologist and as suggested by them, digital subtraction angiography (DSA) was done.

DSA report showed right orbital vascular blush suggestive of vascular malformation with feeder vessel from the right ophthalmic artery and from the right external carotid artery through meningo lacrimal branches. Superior and inferior ophthalmic veins were partially opacified [Figure 9], [Figure 10], [Figure 11].
Figure 9: DSA showing right orbital vascular malformation

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Figure 10: Right orbital vascular blush suggestive of vascular malformation

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Figure 11: Orbital vascular malformation with feeder vessel from the right ophthalmic artery

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The possible treatment was embolization. However, as the feeder vessel was from the ophthalmic artery, embolization was deferred due to potential risk of visual loss. Hence patient was managed consevatively and is on regular follow up to check vision, IOP and visual field. She is maintaining good vision in that eye [Figure 12].
Figure 12: The patient on conservative management

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Case 2

A 12-year-old girl presented with headache for 6 months and forward protrusion of the right eye for 4 months [Figure 13]. On examination, best-corrected visual acuity OU: 6/6. External eye examination showed mild axial proptosis (22 mm) of the right eye with dilated episcleral vessels [Figure 14]. Fundus showed normal disc with dilated and tortuous vessels in both eyes.
Figure 13:( Case2 ) A 12 yr old girl presenting with mild axial proptosis RE

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Figure 14: Dilated episcleral vessels RE

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Blood routine and TFT were normal.

MRI BRAIN (PLAIN and CONTRAST): AVM in medial extraconal space of the right orbit, right cavernous sinus, right cerebellopontine cistern with an intraparenchymal component in the anterior aspect of right cerebellar peduncle and cerebellar hemisphere, associated with prominence of cortical veins and dural sinuses. Chiari I malformation [Figure 15].
Figure 15: MRI BRAIN (PLAIN and CONTRAST): Arteriovenous malformation in medial extraconal space of the right orbit, right cavernous sinus, right CP cistern with an intraparenchymal component in the right cerebellar peduncle and cerebellar hemisphere. Chiari I malformation

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She was referred to a higher center for DSA.

DSA report: Extensive high-flow dural arteriovenous fistula (DAVF) involving right transverse sinus, torcula of herophill, and right jugular foramen (Cognard Type IIB) with significant cerebral venous hypertension and prolonged transit time.

She underwent coil and squid embolization of predominant right middle meningeal artery (MMA) feeders with near complete obliteration of the fistulous flow from right MMA. Residual fistulous from other dural arteries persistent (DAVF Type 2b). She is symptomatically better. Now she is having 6/6 vision in that eye and is on regular follow-up to assess visual acuity, color vision, visual field, and intraocular pressure. Future plan is staged embolization of rest of dural feeders.


  Discussion Top


Diagnosis and management of periorbital AVM remain a challenge requiring a multidisciplinary approach involving interventional radiologist.[2] Angiography is essential for diagnosis and for planning the management of orbital AVMs.[1] The management of AVMs is challenging because of the close relationship of these malformations with central retinal artery. Intraorbital AVMs are exceptions to endovascular treatment because embolization of the central retinal artery, a distal branch of the ophthalmic artery, can complicate the procedure and devastate a patient's vision.[3],[4] The small caliber and tortuous course of the ophthalmic artery make selective catheterization difficult. True AVMs involving only the orbit are rare.

The extraorbital component of the AVMs can produce ophthalmological manifestations when draining into the orbital veins. Embolization is possible only in a few extraorbital AVMs.[5],[6] AVMs fed primarily by the ECA system are most amenable to embolization, but the nidus still should be resected.

Orbital AV fistula (AVF) is the only condition that may be confused with orbital AVMs on radiology and angiography. Orbital AVFs may be traumatic or spontaneous and are limited to orbit, with no connection to the cavernous sinus. These lesions can be differentiated from orbital AVMs by angiography. AVFs demonstrate a direct AV connection without the intervening nidus that is characteristic of AVMs.[7]

Squid is a nonadhesive liquid embolic agent for embolization of brain AVMs. It is composed of ethylene-vinyl alcohol copolymer with suspended micronized tantalum powder for radiopacity and dimethyl sulfoxide solvent.[8]


  Conclusion Top


A thorough evaluation of proptosis cases must be done before deciding on treatment. Decision on the mode of treatment of AVMs should be taken carefully after weighing the pros and cons.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Warrier S, Prabhakaran VC, Valenzuela A, Sullivan TJ, Davis G, Selva D. Orbital arteriovenous malformations. Arch Ophthalmol 2008;126:1669-75.  Back to cited text no. 1
    
2.
Mukherjee B, Vijay V, Halbe S. Combined approach to management of periocular arteriovenous malformation by interventional radiology and surgical excision. Indian J Ophthalmol 2018;66:151-4.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Kim AW, Kosmorsky GS. Arteriovenous communication in the orbit. J Neuroophthalmol 2000;20:17-9.  Back to cited text no. 3
    
4.
Huna-Baron R, Setton A, Kupersmith MJ, Berenstein A. Orbital arteriovenous malformation mimicking cavernous sinus dural arteriovenous malformation. Br J Ophthalmol 2000;84:771-4.  Back to cited text no. 4
    
5.
Peeters FL. Vascular malformations as the cause of unilateral exophthalmus: Diagnostic and therapeutic considerations. Diagn Imaging 1982;51:1-7.  Back to cited text no. 5
    
6.
Rootman J, Kao SC, Graeb DA. Multidisciplinary approaches to complicated vascular lesions of the orbit. Ophthalmology 1992;99:1440-6.  Back to cited text no. 6
    
7.
Yazici B, Yazici Z, Erdogan C, Rootman J. Intraorbital arteriovenous fistula secondary to penetrating injury. Ophthalmic Plast Reconstr Surg 2007;23:275-8.  Back to cited text no. 7
    
8.
Akmangit I, Daglioglu E, Kaya T, Alagoz F, Sahinoglu M, Peker A, et al. Preliminary experience with squid: A new liquid embolizing agent for AVM, AV fistulas and tumors. Turk Neurosurg 2014;24:565-70.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15]



 

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