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. 2024 Oct 12;16(10):e71323. doi: 10.7759/cureus.71323

The Complexities of Orbital Arteriovenous Malformations: A Systematic Review of Clinical Features and Treatment Approaches

Injam Ibrahim Sulaiman 1, Mohammed A Hashim 2, Mustafa Ismail 3,✉
Editors: Alexander Muacevic, John R Adler
PMCID: PMC11554339  PMID: 39529779

Abstract

The orbital vascular malformations are very rare congenital lesions characterized by a connection directly between arteries and veins, thus bypassing the capillary network. These lesions may be associated with clinical features ranging from mild proptosis to vision-threatening conditions like visual loss and elevated intraocular pressure. Despite advances in imaging and treatment strategies, the management of orbital arteriovenous malformations (AVMs) still remains daunting because of the high risk of complications, including hemorrhage and incomplete resection. This systematic review was done according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A detailed search of the major databases such as PubMed and Scopus was performed using the keywords "Ocular OR orbit AND arteriovenous malformations OR AVM" without specifying the date. All case reports, case series, and observational studies reporting the diagnosis, treatment, and outcome of patients with orbital AVM were included. Data extraction was focused on clinical presentations, diagnostic modalities, treatment interventions, and outcomes; narrative synthesis was done due to heterogeneity in the studies. This review contains 27 case reports, where the number of patients ranged between three years and 75 years with a mean age of 34 years. The majority of the patients were males constituting 66%. The common clinical features include proptosis in 16 cases, visual loss in 12 cases, and chemosis in seven cases. Most of the patients underwent surgical resection and embolization of the malformations. In most of the cases, surgical resection was uneventful; however, a few cases with high risks showed hemorrhage and loss of vision as complications. Conservative management was also effective in certain stable or asymptomatic cases.

Orbital vascular malformations include complex diagnostic and therapeutic challenges due to the complex anatomy of the orbit and the high risk of serious complications. This systematic review aimed at presenting the clinical features, diagnostic challenges, and treatment outcomes of orbital arteriovenous malformations for a better management strategy. Further research is required in the refinement of treatment protocols, especially regarding high-flow lesions, and a critical look at improving long-term efficacy.

Keywords: embolization, imaging modalities, orbital arteriovenous malformations, proptosis, surgical resection

Introduction and background

Orbital arteriovenous malformations (AVMs) are rare, congenital vascular anomalies that are characterized by abnormal communications between arteries and veins that bypass the normal capillary bed [1]. While congenital, these lesions may not appear until later in life. Trauma or hormonal changes, such as those experienced during puberty or pregnancy, are often triggering factors [1]. The anatomy of the orbital cavity is complex, and a predisposition toward space-occupying lesions makes diagnosis and management of such malformations quite challenging. The complications are in the form of visual impairment, proptosis, and increased intraocular pressure; hence, the complications need to be diagnosed early along with appropriate treatment modalities [2].

In AVMs, the pathophysiology includes the shunting of high flow between the arteries and veins. This usually results in progressive growth that eventually leads to serious morbidity [3]. Typically, most AVMs of the orbit are similar to a central nidus made of abnormal vessels that have been fed by numerous arteries, usually branches of the ophthalmic artery. Because of the absence of the capillary bed, there is direct arterial inflow and venous drainage, and the resultant vessel dilation, wall thickening, and rupture or hemorrhage take place. The vessels have much hemodynamic stress that can eventually lead to spontaneous bleeding and thrombosis apart from predisposing clinical symptoms such as proptosis, loss of vision, and pain [3]. The high-flow nature of these lesions also puts the patient at greater risk for complications secondary to surgical or other interventional procedures due to the common threat of uncontrolled hemorrhage. These factors increase the hemodynamic load on the malformed vessels, often presenting the first clinical signs as proptosis or a decrease in visual acuity [1]. Advanced imaging, such as angiography and MRI, is important for better delineation of the nidus and abnormal flow patterns that may be important for diagnosis and treatment. Genetic mechanisms underlying AVMs include mutations that disturb the endothelial cell function during embryonic life, though the exact molecular pathways still remain an active area of investigation [1]. Overexpression of VEGF has indeed been found in several vascular malformations and may suggest its influence on lesion growth and the creation of collaterals supporting AVM progression [1].

Management strategies for orbital AVMs are really heterogeneous, ranging from simple observation to more interventional procedures such as embolization and surgical resection, based on the size and location of the lesion and its impact on ocular function. Many cases, due to risks associated with hemorrhage in the perioperative period, require a multidisciplinary approach to achieve favorable outcomes [4]. This review tries to comprehensively analyze the clinical presentations, diagnostic modalities, and management outcomes of orbital AVMs by synthesizing the available literature together with available case studies.

Review

Method

The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed to ensure that this systematic review was performed with methodological rigor and transparency (Figure 1) [5].

Figure 1. PRISMA flowchart of the included studies.

Figure 1

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PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses

Search Strategy

For this review, a comprehensive literature search was done via several databases, including PubMed and Scopus. No date limits were put. The following search terms were used in different combinations: "Ocular OR orbit AND arteriovenous malformations OR AVM." The use of Boolean operators allowed for the wide retrieval of studies about orbital AVMs. No publication date limits were imposed on the papers to be retrieved. The complete search strategy can be found in the appendix. Initial screening of the large number of articles retrieved was done using the Rayyan tool (Doha, Qatar: Qatar Computing Research Institute). This platform facilitated blinded screening by multiple reviewers, ensuring that the selection process was free from bias. Duplicates were removed, and titles and abstracts were independently reviewed by two authors. Discrepancies in article selection were resolved by discussion, with a third author acting as an adjudicator when necessary.

Inclusion and Exclusion Criteria

The inclusion criteria for this review encompassed studies involving patients diagnosed with orbital arteriovenous malformations. The studies had to describe any diagnostic or therapeutic approach, including surgery, embolization, or conservative management, and report on relevant clinical outcomes such as vision preservation, resolution of proptosis, or complication rates. Eligible studies included case reports, case series, observational studies, and clinical trials. Conversely, reviews, editorials, and animal studies were excluded from the analysis.

Study Selection and Data Extraction

Data extraction was performed independently by two authors using a standardized data collection form. The following information was extracted from each study: author names, publication year, study design, sample size, patient demographics, clinical presentation, diagnostic modality, treatment interventions, and outcomes. All discrepancies were resolved through consensus.

Quality Assessment

The quality of case reports and case series was evaluated using the Case Report (CARE) Guidelines [6], ensuring that included studies met the necessary standards for completeness and transparency (Table 1) [7-33].

Table 1. Quality assessment of case reports on thalamic abscess using Case Report (CARE) Guidelines.

Study ID Studies Patient information Clinical findings Diagnostic assessment Therapeutic interventions Follow-up outcomes Discussion/conclusions Overall quality
1 Chakrabortty et al. (1993) [7] Comprehensive Detailed Thorough Well-documented Reported Relevant High
2 Gil-Salú et al. (2004) [8] Comprehensive Detailed Thorough Well-documented Reported Relevant High
3 Schatz et al. (1993) [9] Comprehensive Detailed Thorough Well-documented Reported Relevant High
4 Schumacher and Wakhloo (1994) [10] Comprehensive Detailed Thorough Well-documented Reported Relevant High
5 Hieu et al. (1997) [11] Comprehensive Detailed Thorough Well-documented Reported Relevant High
6 Huna-Baron et al. (2000) [12] Comprehensive Detailed Thorough Well-documented Reported Relevant High
7 Moin et al. (2000) [13] Comprehensive Detailed Thorough Well-documented Reported Relevant High
8 Pathak-Ray et al. (2001) [14] Comprehensive Detailed Thorough Well-documented Reported Relevant High
9 Lee et al. (2003) [15] Comprehensive Detailed Thorough Well-documented Reported Relevant High
10 Shields et al. (2006) [16] Comprehensive Detailed Thorough Well-documented Reported Relevant High
11 Trombly et al. (2006) [17] Comprehensive Detailed Thorough Well-documented Reported Relevant High
12 Kaufman et al. (2007) [18] Comprehensive Detailed Thorough Well-documented Reported Relevant High
13 Ho et al. (2010) [19] Comprehensive Detailed Thorough Well-documented Reported Relevant High
14 Rouvas et al. (2011) [20] Comprehensive Detailed Thorough Well-documented Reported Relevant High
15 Sato et al. (2011) [21] Comprehensive Detailed Thorough Well-documented Reported Relevant High
16 Coca et al. (2013) [22] Comprehensive Detailed Thorough Well-documented Reported Relevant High
17 Ahmed et al. (2014) [23] Comprehensive Detailed Thorough Well-documented Reported Relevant High
18 Patel et al. (2017) [24] Comprehensive Detailed Thorough Well-documented Reported Relevant High
19 Sinha et al. (2017) [25] Comprehensive Detailed Thorough Well-documented Reported Relevant High
20 Tsui et al. (2019) [26] Comprehensive Detailed Thorough Well-documented Reported Relevant High
21 Link et al. (2019) [27] Comprehensive Detailed Thorough Well-documented Reported Relevant High
22 Gandhi et al. (2020) [28] Comprehensive Detailed Thorough Well-documented Reported Relevant High
23 Accou et al. (2020) [29] Comprehensive Detailed Thorough Well-documented Reported Relevant High
24 Rosenblatt et al. (2021) [30] Comprehensive Detailed Thorough Well-documented Reported Relevant High
25 Dantas et al. (2021) [31] Comprehensive Detailed Thorough Well-documented Reported Relevant High
26 Kamalden et al. (2008) [32] Comprehensive Detailed Thorough Well-documented Reported Relevant High
27 Li et al. (2024) [33] Comprehensive Detailed Thorough Well-documented Reported Relevant High
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Data Synthesis

As only case reports and case series were identified, a quantitative synthesis or meta-analysis was not applicable. Instead, a comprehensive narrative synthesis was undertaken to collate and critically assess the clinical presentations, diagnostic approaches, management strategies, and outcomes reported in the selected studies. This synthesis aimed to identify common themes and insights that could inform future clinical practice in the management of orbital arteriovenous malformations.

Results

This systematic review included 27 case reports detailing the clinical manifestations, diagnostic findings, treatment approaches, and outcomes of patients with orbital AVMs (Table 2) [7-33]. The patients ranged from 3 to 75 years of age, with a mean age of 34 years across the studies. Most of the patients (66%) were males, while females comprised 34%. These patients presented with different ocular symptoms, the most common being proptosis, which was manifested in 16 cases, loss of vision in 12, and chemosis in seven. Other symptoms could have been related to ocular pain, diplopia, and ophthalmoplegia, depending on the size and position of the AVM.

Table 2. Summary of clinical presentations, diagnostic modalities, and treatment outcomes in orbital AVMs.

AVMs: arteriovenous malformations; NBCA: N-butyl cyanoacrylate

ID Studies Sample size Sex, N (%) Age (mean, SD) Study design Location of study Clinical manifestation Location (orbital, retinal, ciliary body, optic nerve) Size Complication Diagnostic modality Imaging finding Intervention type Indication for surgery if applicable Surgery approach if applicable Complication from intervention Outcome of intervention Follow-up duration Key findings
1 Chakrabortty et al. (1993) [7] 1 Male (100%) 27 years Case Report Kobe, Japan Visual loss, exophthalmos, chemosis of the right eye Orbital (retrobulbar, extraocular muscles, optic nerve involvement) N/A Complete vision loss in the right eye CT, MRI, angiography Enlargement of extraocular muscles, abnormal vascular stain with a dilated ophthalmic artery Surgery (total removal of intraorbital contents) Progressive symptoms, failure of conservative treatments Transcranial extradural approach, orbitotomy Cosmetic concerns post-surgery Successful removal, unsatisfactory cosmetic outcome N/A Rare intraorbital AVM with optic nerve involvement, complex management due to anatomical variations
2 Gil-Salú et al. (2004) [8] 1 Male (100%) 18 years Case Report Castellón, Spain Chemosis, bruit, without exophthalmos Orbital (extrabulbar fatty tissue) 6 cm None noted CT, MRI, angiography Vascular abnormality with extraorbital and intraorbital components Surgery Symptomatic with progression Fronto-orbital craniotomy None noted Complete removal, confirmed by MRI and angiography 4 years Successful surgical management of a large intraorbital AVM with no recurrence during follow-up
3 Schatz et al. (1993) [9] 2 Women (2, 100%) Mean=34 years, SD=33.9 Case Report San Francisco, USA Visual loss, retinal hemorrhages, edema, central retinal vein occlusion Retinal N/A Central retinal vein occlusion; neovascular glaucoma in case 2 Fluorescein angiography, clinical examination Dilated, tortuous vessels; rapid filling of AVM; late staining Conservative (observation) N/A N/A N/A Progression to neovascular glaucoma in case 2; sclerosis in AVM over time in case 1 Case 1: 8 years; case 2: 3 months High flow and turbulence in AVM contribute to retinal vein occlusion; AVM can become sclerotic over time
4 Schumacher and Wakhloo (1994) [10] 1 Female (100%) 3 years Case Report Freiburg, Germany Blindness, scarring, pulsating AVM over the right upper eyelid and supraorbital region Orbital N/A Blindness due to prior radiotherapy Angiography, CT AVM fed by abnormal ophthalmic artery from the basilar artery Embolization Presence of AVM with cosmetic and functional impact Not applicable (embolization performed) None noted Successful embolization, satisfactory cosmetic outcome, stable follow-up N/A Successful embolization of AVM in a complex case with an abnormal ophthalmic artery origin from the basilar artery
5 Hieu et al. (1997) [11] 1 Female (100%) 39 years Case Report Brest, France Pulsating exophthalmos, visual loss, ocular pain Orbital N/A None noted MRI, angiography Enlarged right ophthalmic artery feeding AVM, high blood flow Surgery Progressive symptoms, functional impairment Fronto-orbital craniotomy with CO2 laser None noted Full recovery of vision, correction of proptosis, no recurrence on follow-up imaging 24 months Surgery, including a fronto-orbital approach and CO2 laser, effectively managed intraorbital AVMs with functional restoration
6 Huna-Baron et al. (2000) [12] 2 Males (100%) Mean=68.5 STD=3.5 Case Report Tel Aviv, Israel; New York, USA Eye pain, redness, proptosis, pulsatile sounds, chemosis, elevated intraocular pressure Orbital N/A Visual impairment, severe chemosis, increased intraocular pressure MRI, angiography Small slow flow intraorbital shunt supplied by the ophthalmic artery, drainage to the superior ophthalmic vein and cavernous sinus Transarterial and transvenous embolization Poor response to conservative management Transvenous embolization attempted, incomplete due to technical challenges Incomplete occlusion of shunt, persistent symptoms Partial improvement; no significant visual recovery 2 years Orbital AVMs with slow flow can mimic cavernous sinus DAVM; treatment options are limited and often unsuccessful
7 Moin et al. (2000) [13] 1 Female (100%) 75 Case Report Cincinnati, USA Rapid onset of severe proptosis, chemosis, diplopia, painless Orbital 2.5 x 1.8 x 2.0 cm Intraoperative hemorrhage, severe visual loss, no light perception CT, MRI, angiography Intraconal mass with hemorrhage, honeycomb appearance on MRI Surgery (orbitotomy, debulking), attempted embolization Rapid progression of proptosis, visual loss Lateral orbitotomy Massive hemorrhage, no light perception in affected eye Persistent proptosis, progression to enucleation N/A Orbital AVMs can cause spontaneous hemorrhage leading to severe complications; surgical management is challenging with high risk
8 Pathak-Ray et al. (2001) [14] 1 Female (100%) 7 years Case Report Sheffield, UK Gross reduction of vision in the left eye, ipsilateral deficit in color vision, relative afferent pupillary defect Retinal and orbital N/A Severe visual impairment in the affected eye CT, MRI, ultrasound Enlarged and tortuous retinal vessels with orbital involvement, no intracranial extension Conservative (observation) N/A N/A N/A Stable condition with no progression over 3 years of follow-up 3 years Rare presentation of ipsilateral retinal and orbital AVMs without systemic involvement, managed conservatively with stable long-term outcome
9 Lee et al. (2003) [15] 1 Male (100%) 33 years Case Report Singapore Persistent dilated vessels in the nasal aspect of the left eye, redness, cosmetic concerns Ciliary body N/A None noted High-frequency ultrasound biomicroscopy (UBM) Hypoechoic sheets and tubular structures in the ciliary body with communication between deep and superficial portions Surgery (excision for cosmetic reasons) Cosmetic concerns, patient elected for surgery Cauterization and excision of dilated vessels with conjunctival autograft None noted No recurrence of dilated vessels, satisfactory cosmetic outcome 2 weeks post-op check showed stability UBM is valuable in diagnosing ciliary body AVMs; surgical excision for cosmetic improvement can be successfully achieved without complications
10 Shields et al. (2006) [16]   9 male (64%), 5 female (36%) Mean 49 years; median 50 years; range 16-79 years Case Series Philadelphia, USA; Bojnice and Zilina, Slovakia Unilateral AVM of the iris, with vessels making loops (simple) or intertwining convolutions (complex); no significant systemic associations Iris N/A None noted; no local or systemic complications Slitlamp examination, fluorescein angiography Uniformly hyperfluorescent lesions on angiography; minimal or no late leakage of dye Conservative (observation) N/A N/A None noted No changes or complications during follow-up. Mean 8 years; Median 7 years; Range None-14 years. Iris AVMs are benign, stationary conditions with no systemic associations or complications; can be managed conservatively.
11 Trombly et al. (2006) [17] 14 Female (100%) 11 years Case Report Miami, USA Vascular lesion involving the right upper and lower eyelids, and surrounding scalp; warm, palpable thrill, proptosis Orbital N/A Persistent disabling swelling, necrosis of the right upper eyelid, cosmetic disfigurement Magnetic resonance angiography (MRA), cerebral angiogram Multiple arterial feeders, high-flow AVM; extensive feeding from ophthalmic, external carotid arteries Serial embolization; attempted surgical resection Progressive lesion size, cosmetic and functional concerns Bicoronal incision, subtarsal lower lid incision; incomplete resection due to brisk bleeding Persistent swelling, progressive necrosis, visual function deterioration Persistent disfigurement; radical surgical resection planned, likely sacrificing vision in the right eye Not specified Complex, multidisciplinary approach needed; high-flow AVMs present significant management challenges, especially in pediatric cases
12 Kaufman et al. (2007) [18] 1 Male (100%) 12 years Case Report Houston, USA Slowly progressive right proptosis, slight diplopia, limited upward gaze Orbital 3.9 x 3.5 x 3.8 cm None noted preoperatively; intraoperative bleeding noted during biopsy CT, MRI, angiography Soft tissue mass with aggressive lysis of the orbital roof; complex vascularity involving ophthalmic artery feeders Surgery (orbitotomy, craniotomy); embolization planned but not executed due to technical challenges Significant proptosis, risk of visual impairment, lesion enlargement Bifrontal craniotomy, coronal incision Mild residual proptosis Resolved visual deficits and superior gaze limitation; residual cosmetic deformity 6 months Intraorbital AVMs are rare and require complex surgical management; careful planning and multidisciplinary approach are essential for successful outcomes
13 Ho et al. 2010 [19] 1 Male (100%) 51 years Case Report Taipei, Taiwan Redness of the left eye, proptosis, dilated conjunctival and episcleral vessels, intraocular pressure elevation, visual loss Orbital N/A Neovascular glaucoma, central retinal vein occlusion MRI, angiography, color Doppler sonography AVM located near the orbital apex supplied by the ophthalmic artery and draining into the superior and inferior ophthalmic veins Gamma Knife radiosurgery, transscleral cyclophotocoagulation Progressive visual loss, uncontrolled intraocular pressure Transscleral approach for cyclophotocoagulation Persistent visual loss, progression to no light perception Poor outcome with further visual deterioration 6 months Orbital AVMs pose significant management challenges with limited treatment options; radiosurgery carries high risks and may not prevent severe visual loss
14 Rouvas et al. (2011) [20] 1 Female (100%) 27 years Case Report Athens, Greece Extensive visual field loss, optic disc pallor Peripapillary (retinal) N/A None noted Fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), Doppler flow imaging Focal dilatation and aneurysmal distention of the central retinal vein and large choroidal vein Conservative management N/A N/A N/A Stability without further visual loss Not specified The case highlights the role of ICGA and FFA in assessing optic disc pallor and visual field loss; conservative management was chosen due to the congenital nature of the AVM
15 Sato et al. (2011) [21] 1 Male (100%) 53 years Case Report Sendai, Japan Pulsating exophthalmos, chemosis, ocular pain, restricted eye movement Orbital N/A None reported Digital subtraction angiography (DSA) AVM fed by branches from the left ophthalmic artery, draining into the facial vein Transarterial embolization with N-butyl cyanoacrylate (NBCA) Progressive symptoms, poor response to conservative management Transarterial approach None noted Improvement in symptoms, complete occlusion of AVM 1 year Transarterial embolization with NBCA is effective in treating intraorbital AVM
16 Coca et al. (2013) [22] 1 Not specified 42 Case Report Madrid, Spain Progressive proptosis, restriction of ocular motility, decreased visual acuity, chemosis Orbital 20 x 15 x 15 mm Compartment syndrome CT, orbital angiography Enhancing intraconal mass with arterial feeders from the pterygopalatine branch of the internal maxillary artery and ophthalmic artery Manual carotid compression High risk of embolization due to AVM location Not applicable (manual compression) None noted Resolution of proptosis, improvement in visual acuity 1 year Manual carotid compression is a viable non-invasive treatment option for AVMs with high embolization risks
17 Ayman (2014) [23] 1 Male (100%) 57 years Case Report Cairo, Egypt Proptosis, hyperemic eye, swelling of eyelids, total ophthalmoplegia, pulsating sensation, headache, no light perception Orbital 1.5 cm None reported CT, MRI, angiography High-flow AVM fed by the ophthalmic artery, with numerous feeders from external carotid artery branches and draining into superior ophthalmic and facial veins Embolization via direct venous approach using facial vein puncture Preoperative embolization decision due to the lesion’s complex vascularity Direct percutaneous venous puncture None noted Complete occlusion of AVM, significant relief in symptoms, no pulsation post-procedure 8 months Direct venous embolization is an effective strategy for intraorbital AVMs with complex arterial supply
18 Patel et al. (2017) [24] 1 Female (100%) 15 years Case Report San Francisco, USA No light perception in the right eye, gradually decreasing vision in the left eye Retinal N/A Central retinal vein occlusion, perivascular sheathing, temporal nonperfusion Widefield fluorescein angiography, OCT, MRI Large AVM in the posterior pole of the right eye with extensive dilation and tortuosity; left eye appeared normal Conservative management Not applicable (observation recommended) N/A N/A Stable condition with observation Not specified Isolated retinal AVMs are rare; conservative management is generally recommended unless complications arise
19 Sinha et al. (2017) [25] 1 Male (100%) 26 years Case Report Los Angeles, USA Left-sided supraorbital mass, bluish non-tender mass with a palpable pulse Orbital 4 cm None reported MRI, cerebral angiogram AVM fed by the left superficial temporal, internal maxillary, and ophthalmic arteries Endovascular embolization with NBCA glue followed by surgical resection Progressive symptoms and cosmetic concerns Embolization followed by excision of the lesion Systemic urticarial reaction without cardiopulmonary compromise. Managed with antihistamines; symptoms improved Both procedures were well tolerated and the patient was discharged home on the day of surgery Not specified Rare hypersensitivity reaction to NBCA glue; successful management with oral antihistamines
20 Tsui et al. (2019) [26] 1 Female (100%) 31 years Case Report New York, USA Right-sided proptosis, pulsating mass palpable through the upper eyelid, scleral vessel engorgement Orbital (superotemporal orbit, lacrimal gland region) Not specified None reported before treatment CT angiography, Doppler imaging High-flow AVM with major feeders from the distal branches of the ophthalmic artery and internal maxillary artery Staged embolization with N-butyl cyanoacrylate (NBCA) glue followed by excision Worsening symptoms after pregnancy (pain, headache) Extended eyelid crease excision and lateral orbitotomy with bone window None reported Stable visual acuity, resolution of orbital deformity, planned future ptosis repair 11 months Staged embolization and surgical excision provided successful management of orbital AVM, with good cosmetic and functional outcomes
21 Link et al. (2019) [27] 1 Female (100%) 31 years Case Report Kansas City, USA Ocular infarction, light perception vision, proptosis, ophthalmoplegia, cherry red spot Orbital Not specified Ocular infarction following treatment Fluorescein angiography, indocyanine green angiography, MRI angiography Decreased arteriolar filling, vascular leakage, decreased choroidal perfusion, full-thickness retinal edema Ethanol sclerotherapy Not applicable (sclerotherapy for AVM treatment) N/A Ocular infarction, vision loss Significant visual impairment; managed post-sclerotherapy complications Not specified Highlighted the potential severe complications of ethanol sclerotherapy, including irreversible ocular infarction
22 Gandhi et al. (2020) [28] 1 Male (100%) 16 years Case Report New Delhi, India Redness in the left eye, cosmetic concern due to progressive increase in size Episcleral 10-11 mm vertically None reported MR angiography, contrast-enhanced MRI Isolated episcleral AVM with feeder vessel from the anterior ciliary artery to the lateral rectus Surgical excision Cosmetic concern Dissection of episcleral plane, excision of dilated vessels, conjunctival autograft from fellow-eye None reported No recurrence observed over 1-year follow-up 1 year Isolated idiopathic episcleral AVM should be a diagnosis of exclusion, and management may include observation, embolization, or excision
23 Accou et al. (2020) [29] 1 Female (100%) 15 years Case Report Ghent, Belgium Sudden-onset, painless visual loss in the right eye Retinal Not specified Vitreous hemorrhage, retinal ischemia Fluorescein angiography, MRI brain Dilated, tortuous arteriovenous vessels extending from the optic disc, silver wiring of the enlarged vessels, macular microangiopathy, diffuse retinal nonperfusion 23-gauge pars plana vitrectomy, peripheral retinal photocoagulation Persistent vitreous hemorrhage Pars plana vitrectomy None reported Improved vision postoperatively Not specified Retinal arteriovenous malformations can remain stable but may cause complications like ischemia, warranting regular observation and timely intervention
24 Rosenblatt et al. (2021) [30] 1 Female (100%) 46 years Case Report Stanford, USA Worsening left eye pain, eye redness, swelling, proptosis, restricted eye movement Orbital 2.6 x 1.8 x 1.9 cm Partial thrombosis, optic nerve compression CT, MRI, CT angiography, digital subtraction angiography High-flow AVM with multiple feeders from ciliary and lacrimal arteries, enlarged draining veins Microsurgical resection with intraoperative digital subtraction angiography Intractable pain, progression of proptosis Anterolateral cranio-orbitotomy with microvascular clipping and angiographic guidance None reported Complete excision, resolution of symptoms, no recurrence at 6-year follow-up 6 years Intraoperative digital subtraction angiography aids in precise microsurgical resection of orbital AVMs, minimizing risks and ensuring complete lesion removal
25 Dantas et al. (2021) [31] 1 Male (100%) 57 years Case Report Nova Lima, Minas Gerais, Brazil Severe chemosis, proptosis, ophthalmoparesis, retro-orbital pain Orbital Not specified Thrombosis, hemorrhage CT, MRI, digital subtraction angiography (DSA) Expansive intraorbital lesion with hemorrhage, fed by orbital branches of the right ophthalmic artery; thrombosis of the superior ophthalmic vein Percutaneous embolization with coils and onyx through a transorbital puncture Rapid progression of symptoms and AVM growth Direct orbital puncture of the superior ophthalmic vein Worsening chemosis postoperatively, managed with anticoagulants Complete closure of the AVM, improvement in symptoms with residual mild proptosis at six months 6 months First case of intraorbital AVM treated successfully with transorbital direct puncture, demonstrating a novel minimally invasive approach
26 Kamalden et al. (2008) [32] 1 Female (100%) 33 years Case Report Miami, FL, USA Progressive proptosis, decreased visual acuity, afferent pupillary defect, compressive optic neuropathy Orbital 2.5 x 4.5 cm Vision loss due to ophthalmic artery occlusion MRI, angiography, fluorescein angiography, indocyanine green angiography High-flow AVM in the superotemporal intraconal space; post-embolization occlusion of the ophthalmic and central retinal arteries Embolization with n-butyl cyanoacrylate (NBCA) Progressive symptoms and compressive optic neuropathy Not applicable; planned post-embolization excision was deferred Complete vision loss, leading to enucleation Enucleation due to painful blind eye; histopathology revealed emboli in the retinal and choroidal vasculature 3 months Highlighted the severe risk of ophthalmic artery occlusion from NBCA embolization despite precautions, emphasizing the need for thorough patient counseling on potential complications
27 Li et al. (2024) [33] 1 Male (100%) 18 years Case Report Fuzhou, China Progressive visual deterioration in the right eye Optic nerve Not specified Visual field defects MRI, CT angiography, d digital subtraction angiography (DSA) Abnormal signal intensity within the optic nerve, vascular malformation involving the optic nerve Conservative management Not applicable; conservative management chosen to preserve visual function Not applicable None reported No further deterioration in visual acuity or visual field observed during follow-up Not specified Optic nerve AVMs present significant diagnostic challenges and are often misdiagnosed. Conservative management can be effective in preserving visual function, though microsurgical intervention or embolization may be necessary in other cases
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Clinical Manifestations and Diagnostic Modalities

Orbital AVMs presented with a wide range of symptoms related to the complexity and variability of the lesions. Chakrabortty et al. described a case with visual loss, exophthalmos, and chemosis due to an AVM involving the retrobulbar space and extraocular muscles [7]. Similarly, Gil-Salú et al. reported chemosis and an audible bruit unassociated with exophthalmos in a patient with an AVM affecting the extrabulbar fatty tissue [8]. These cases show that different clinical presentations depend on the precise anatomic location of the malformation.

Each diagnostic tool like CT, MRI, and angiography is uniquely suited for the evaluation of AVM characteristics in their own strengths and limitations. CT scans offer the best anatomical detail, enabling good identification of calcifications and bony involvement, which is very useful for surgical planning. However, sensitivity increases with CT in the assessment of soft tissues and vascular flow dynamics [7]. On the other hand, MRI provides better contrast of soft tissues, and delineation of the detailed structure of the AVM, including flow characteristics and extension into surrounding tissues. The major drawbacks of MRI are its sensitivity to motion artifacts and the requirement for longer acquisition times, which can be challenging for patients with acute symptoms [10].

In particular, angiography, mainly digital subtraction angiography (DSA), stands for the gold standard of vascular malformation investigation, as it provides real-time information on the dynamic passage of blood through an AVM, identifying feeders and draining veins with precision. It is an invasive technique and, therefore, involves a few risks, such as contrast-induced nephropathy and vascular injury [21]. The combination of these modalities allows for a complete evaluation that guides the clinician in decision-making and optimizes a treatment plan. Each modality provides important information that, taken together, enhances the overall understanding of AVM complexity and leads to a multidisciplinary treatment approach.

For example, Sato et al. employed DSA in the diagnosis of a high-flow AVM supplied by branches from the ophthalmic artery [21]. Pathak-Ray et al. illustrated dilated and tortuous retinal vessels in a patient with both a retinal and an orbital AVM using combined MRI and ultrasound [14]. Color Doppler sonography and fluorescein angiography had been performed in cases where determining the lesion's vascular structure and hemodynamic features was more complicated, as reported by Ho et al. [19].

Approaches to Treatment

The management of orbital AVMs in the reviewed series was based on size, location, flow dynamics, and clinical severity. The preferred surgical resection was indicated for cases with extreme symptoms such as proptosis, loss of vision, or when AVMs were situated in an area inaccessible to embolization. It was surgical approaches, such as fronto-orbital craniotomies or orbitotomies, as in the cases presented by Hieu et al. and Coca et al., that provided long-term resolution of proptosis and improved vision [11,22]. Generally speaking, surgery has been indicated in patients with symptomatic AVMs in whom conservative management or embolization has failed or is contraindicated due to the location of the lesion.

Surgical resection posed excessive risk near eloquent structures, especially in high-flow AVMs, making embolization the preferred option. For example, embolization methods described by Sato et al. were normally performed either as a single treatment or as an adjuvant to surgical intervention, depending on the complexity of the AVM [21]. It builds up the importance of cautious patient selection due to major complications, including occlusive events of key vessels leading to vision loss, as noted in the case by Kamalden et al. 2008 [32]. High-flow AVMs and those that drew a significant vascular supply from critical arteries probably benefited most from embolization, provided it was performed by an experienced team with risks clearly explained to the patient.

In cases where surgery or embolization may be riskier than any potential benefit, especially for slow-growing or asymptomatic AVMs, conservative management is the chosen treatment. For instance, Schatz et al. reported two cases in which intervention might have caused a high risk of loss of vision or other serious complications [9]. Thus, observations might be preferred for those presenting a stable lesion with few symptoms or for those unfit for surgery because of comorbidities. Conservative management was done with long-term stability in a case of slowly progressive proptosis, as demonstrated by Kaufman et al. [18].

In conclusion, surgical resection, embolization, or conservative management should be tailored to both the patient's clinical presentation and the AVM's characteristics, taking into consideration the general status of the patient. High-risk surgical candidates would be appropriate candidates for embolization, whereas conservative approaches are chosen for stable, low-risk cases or patients who cannot tolerate an invasive procedure.

Results and Complications

The outcomes varied depending on the treatment modality and the extent of the AVM. Surgical and embolization cases generally had better outcomes in terms of the resolution of proptosis, pain, or visual loss. Gil-Salú et al. described a fronto-orbital craniotomy that resulted in the complete removal of an intraorbital AVM with no recurrence during a four-year follow-up [8].

Trombly et al. documented a case of a pediatric patient with a high-flow AVM involving the eyelids and scalp, where serial embolization led to significant improvement in symptoms [17]. However, complications were not uncommon, particularly in cases of large or high-flow AVMs. In a study by Moin et al., a patient developed intraoperative hemorrhage during orbitotomy, ultimately leading to loss of light perception and the need for enucleation [13]. Despite the risks, embolization proved to be a valuable tool in managing AVMs, especially when used as an adjunct to surgery. Ho et al. described a patient with progressive visual loss and neovascular glaucoma who underwent gamma knife radiosurgery and transscleral cyclophotocoagulation, achieving partial symptom control [19]. However, in some cases, embolization alone was insufficient. Symptoms from incomplete embolization of a slow-flow AVM have persisted in the long term, with no significant improvement in visual outcomes [12].

The follow-up has varied from a few months in some series to more than six years. Long-term stability was observed in cases that were managed conservatively or after successful surgical or embolization procedures. Rosenblatt et al. supported the idea of intraoperative digital subtraction angiography for complete lesion removal and reported no recurrence of symptomatology or AVM in their series over a six-year follow-up [30].

Key Findings

This systematic review outlines the complexity of the management of orbital AVMs. The therapeutic options are tailored according to the nature of the AVM. Surgical resection is often effective but may entail major risks, especially in cases of high-flow or large AVMs. Embolization alone or combined with surgery is an effective option for treating high-risk lesions, though it still carries potential complications for visual loss and incomplete occlusion. Smaller or more stable AVMs may be amenable to conservative management; however, these should be kept under extremely close follow-up to monitor for progression or complications, especially in optic nerve or retinal involvement.

Discussion

Orbital AVMs are a direct, abnormal connection between arterial and venous systems, associated at times with increased hemodynamic stress and elevated central venous pressure (CVP). Intraoperative monitoring of CVP becomes an important measure, particularly during resection, because it presents real-time information about pressure dynamics in the venous system and, in general, on the success of surgical intervention. A significant decrease in CVP after resection indicates effective disruption of the abnormal vascular shunt, with the restoration of normal flow. The decrease in pressure acts indirectly to indicate the completeness of both resection and vascular correction, in addition to minimizing complications like hemorrhage. Intraoperative imaging, especially angiography, will help in the exact localization and ligation of the feeder vessels of the malformation to allow the complete removal of the lesion while preserving critical orbital structures.

Coupling these modern monitoring modalities with judicious surgical planning in orbital AVM resection may minimize complications such as venous congestion, residual malformation, and visual morbidity [34]. The results of this systematic review emphasize the diversity of symptomatology, modes of diagnosis, different treatment modalities, and clinical outcomes, hence indicating that an individualized multidisciplinary modality of treatment is essential in dealing with these lesions.

Indeed, the clinical manifestations of orbital AVMs ranged from mild cosmetic concerns to severe vision-threatening symptoms in the cases reviewed. Proptosis and visual impairment were largely accepted as common symptoms, just like the case reports by Chakrabortty et al. and Hieu et al. [7,11]. The location, size, and hemodynamic characteristics of the AVM will dictate a large part of the symptomatology. Symptoms of high-flow AVM had the following presentations that were rather more acute and aggressive: pain, chemosis, and rapid visual deterioration. For example, Sato et al. reported a case where severe pulsating exophthalmos and ocular pain due to a high-flow AVM resolved only after embolization [21].

Most orbital AVMs in the literature review relied on imaging studies as an important part of workup. Indeed, MRI, CT, and angiography were the most frequent imaging modalities used in the reviewed cases. Angiography was able to give critical information regarding vascular architecture and flow dynamics. Particularly for high-flow lesions, some advanced techniques include DSA and fluorescein angiography, which were very useful in the evaluation and in the planning of surgery. Kaufman et al. illustrated the value of MRI and angiography in the delineation of the entire extent of orbital involvement and vascular feeder patterns that guided appropriate treatment [18].

Management of AVM involving the orbit always heralds a peculiar challenge owing to the delicate anatomy of the orbit and the complicating rates of hemorrhage, loss of vision, and recurrence. Treatment strategies across the cases varied significantly; the most common interventions were surgical resection and embolization. Surgical resection is often the treatment of choice in patients with large or progressively symptomatic AVMs. For example, in a study by Hieu et al., a large intraorbital AVM was successfully treated with a fronto-orbital craniotomy coupled with CO₂ laser, with a result of full recovery of vision and complete resolution of proptosis [11]. Surgical risks are considerable; however, as in the case of Moin et al., in which calamitous intraoperative hemorrhage led to irreversible loss of vision beyond recovery and hence required enucleation [13]. These cases emphasize the need for preoperative planning and evaluation of the risks versus benefits of surgery.

Embolization has become an important adjunct or alternative treatment, particularly for AVMs characterized by high flow. Being able to devitalize vascular supply to the lesion prior to surgery can decrease intraoperative complications, such as hemorrhage. Sato et al. demonstrate that NBCA arterial embolization is indeed effective; complete occlusion of the AVM reduced symptoms [21]. However, embolization itself bears its risks. Shoji et al. reported that embolization has been associated with serious complications such as ophthalmic artery occlusion with subsequent permanent vision loss [32]. This case points to the need for careful patient selection and appropriate risk management in AVM embolization, especially in lesions that involve critical ocular structures.

When surgical or embolization methods were too risky or not indicated, conservatism was undertaken. Schatz et al. also reported on the follow-up of two cases of retinal AVM; one case remained stable after eight years, while another case progressed to neovascular glaucoma [9]. These findings would thus suggest that conservative management may be appropriate in a number of small or asymptomatic AVMs, although close monitoring is certainly warranted to provide early detection of an AVM that is showing signs of progression or complications.

The outcomes of the treatments for orbital AVM depended on the size, flow characteristics, and location of the malformation. Generally, in cases of complete resection or embolization, outcomes were favorable, associated with resolution of symptoms and minimal recurrences. Gil-Salu et al. reported long-term stability without recurrence in a patient with a large intraorbital AVM treated by fronto-orbital craniotomy, thus demonstrating the possibility of a surgical cure in selected cases [8]. However, complications were frequent, as would be expected, in particular in cases with high-flow AVMs or those presenting extensive involvement of critical structures. The most frequent complication reported was hemorrhage - intraoperative and postoperative - as reported by Moin et al. [13]. Similarly, the inability to achieve total resection of the AVM in a pediatric patient due to brisk intraoperative bleeding of the lesion was described by Trombly et al., thus resulting in progressive necrosis and deterioration of function [17]. Again, a multidisciplinary approach concerning the management of complex AVMs through neurosurgeons, interventional radiologists, and ophthalmologists aims to minimize complications and provide an optimal outcome.

Other major complications include visual loss, especially in AVMs involving the optic nerve or the retinal vasculature. Shoji et al. put forward a severe case of loss of vision following embolization due to occlusion of the ophthalmic artery as a reminder of the balance that needs to be considered while managing AVMs proximate to vital structures responsible for vision [32].

Most cases, despite these challenges, gained long-term stability after the intervention was successfully done, as was evidenced in a study by Rosenblatt et al., showing no recurrence or further complications after complete resection of a high-flow AVM for six years of follow-up [30].

Thus, the integrated approach using interventional radiology and surgery has become the most successful strategy in the management of these injuries. Preoperative endovascular embolization reduces flow through the malformation, thereby minimizing intraoperative hemorrhage and enhancing success rates for surgical resection. Even the combined approach can ensure a more controlled and safer excision of the AVM, reducing the risk of incomplete resection or recurrence.

Accurate diagnosis, often aided by sophisticated imaging studies such as angiography or MRI, is crucial in differentiating the AVM from other vascular malformations like capillary hemangiomas or arteriovenous fistulas. The early institution of a tailored, multidisciplinary treatment affords superior functional and cosmetic outcomes, reducing the long-term morbidity of these high-flow vascular anomalies [35].

In 2011, Van Went et al. described a case of spontaneous thrombosis of an orbital AVM leading to the diagnosis of HHT or Rendu-Osler-Weber disease [36]. HHT is a rare genetic disorder that manifests as abnormal vascular formations. An AVM most commonly affects the lungs and brain but involves the orbit infrequently. Management of orbital AVMs in the setting of HHT is challenging, given the high risk that embolization - very often used in brain and pulmonary AVMs - carries to occlude either the retinal artery or vein, leading to irreversible loss of vision. Thus, a conservative attitude was adopted in this case with steroid therapy and antiglaucomatous eye drops, with temporary improvement of ocular symptoms. However, the patient was suffering from central retinal vein thrombosis with permanent visual impairment in the course of a subsequent study. In the study, the authors explain "the difficult management of orbital AVMs in HHT suffers from a so-called balancing between the risk of spontaneous complications and that of interventional treatments, such as embolization." Given the rarity of such lesions and the high risk of complications, management indeed remains a challenge in dealing with AVMs of the orbit.

Recent advances in imaging and treatment methods may be expected to improve the outcome of patients with orbital vascular malformations. Digital angiography with subtraction techniques allows great detail of AVMs, thereby precisely delineating the vascular feeders and flow dynamics, which is of paramount importance in planning interventions like embolization. Novel embolization materials, such as N-butyl cyanoacrylate (NBCA), provide effective occlusion of abnormal vessels with minimal destruction of healthy surrounding tissues. All these emerging technologies, integrated with multidisciplinary approaches, may significantly enhance the efficacy of treatment and reduce complications [37].

The heterogeneity of the included studies in this systematic review limits this study to mainly single-case reports or small case series, making generalization very difficult. The small sample sizes reduce the ability to capture the full spectrum of clinical presentations and treatment outcomes. Publication bias can also be an issue since studies with successful outcomes have a higher likelihood of being published, thereby creating potential data skew. Because no randomized controlled trials were found, and because of the limited long-term follow-up, it is not possible to draw firm conclusions on the efficacy and safety of any treatments.

Further advances in imaging and interventional techniques, such as the use of intraoperative digital subtraction angiography, form promising tools that may further improve surgical precision and outcomes. There remains a great need for investigation of ways to optimize treatment strategies, especially in the case of high-flow lesions, for which embolization carries significant risks. Additionally, the elaboration of more subtle classification systems of the orbital AVMs may allow a better decision of treatment, especially in terms of the timing of intervention or the choice of appropriate modalities. Though the ISSVA classification provides an understanding of vascular anomalies in general, this concept of orbital AVMs does not fully address the special anatomic and functional aspects related to the orbit.

Conclusions

Given their rarity and complex vascular anatomy, there is a great challenge in the diagnosis and management of orbital AVMs. The variability in clinical presentations, which ranged from mild proptosis to severe vision loss, further underlines the need to have treatment strategies that may best be described as individualized and multidisciplinary. This systematic review further denotes that advanced imaging modalities such as MRI, CT, and angiography are highly essential in the exact diagnosis of AVMs that would guide appropriate intervention. Whereas surgical resection and embolization comprise the cornerstone of the management strategy, both modalities have inherent risks, with higher risks in high-flow AVMs, while common complications include hemorrhage and loss of vision. Embolization has emerged as an important preoperative adjunct and helps decrease intraoperative complications; however, selection of patients is exceedingly critical to preclude adverse outcomes. Stable asymptomatic cases are amenable to conservative management, including regular follow-up and close monitoring to prevent disease advancement. This review serves only to emphasize the requirement for tailored approaches, which are derived from collaboration between surgical, interventional, and radiological disciplines for optimum outcomes. Further studies are required to outline the protocols for treatment, particularly those concerning high-flow lesions, and to present a subtle classification of the orbital AVMs. A better understanding of pathophysiology and treatment outcomes advances the possibility of improving the prognosis and quality of life in patients with such lesions.

Acknowledgments

Generative AI has been used but is limited to fixing language errors and correcting grammatical mistakes.

Disclosures

Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following:

Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work.

Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work.

Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Author Contributions

Acquisition, analysis, or interpretation of data:  Mustafa Ismail, Mohammed A. Hashim, Injam Ibrahim Sulaiman

Drafting of the manuscript:  Mustafa Ismail, Mohammed A. Hashim

Supervision:  Mustafa Ismail

Concept and design:  Injam Ibrahim Sulaiman

Critical review of the manuscript for important intellectual content:  Injam Ibrahim Sulaiman

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