Orbital Arteriovenous Malformation Research Articles

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

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

Combined Surgical and Endovascular Embolization of an Orbital Arteriovenous Malformation

Combined Surgical and Endovascular Embolization of an Orbital Arteriovenous Malformation

Orbital Arteriovenous Malformations (AVM): A Review

AbstractOrbital arteriovenous malformations (AVM) are identified by abnormal high-flow connections between the veins and the arteries, which are often supplied by the branches of internal and external carotid arteries, also with a nidus of cellular stroma distributed between the vessels. Orbital arteriovenous malformations are rare among all congenital malformations. Pain, glaucoma, optic nerve ischemia and compression and retinal ischemia are some of the causes of enlarging orbital AVMs. Because of the rare nature of these lesions, they are usually misdiagnosed and treated improperly. For the diagnosis of such orbital AVMs, angiography becomes an essential tool. The primary method of diagnosing orbital AV malformations are non-invasive cross-sectional imaging and catheter angiography. Treatment for the same includes reducing the blood flow to the arteriovenous malformation (AVM) via preoperative embolization. And surgical resection. Complete excision is challenging because of the increased risk of involvement of the ophthalmic artery which can cause vision loss, preoperative embolization is difficult in some patients. An effective way of treating arteriovenous malformation is ethanol sclerotherapy. Diagnosing and managing Orbital AV malformations is important as these are rare and can cause permanent vision loss.

Pituitary macroadenoma causing vision loss in Wyburn-Mason syndrome: illustrative case.

Wyburn-Mason syndrome (WMS) is a neurocutaneous disorder consisting of vascular malformations of the brain, eye, and skin. These include characteristically high-flow intracranial and intraorbital arteriovenous malformations (AVMs) that present commonly with visual deterioration, headache, and hemiplegia. Complete removal of these lesions is challenging. Most patients are followed closely, and intervention occurs only in the setting of worsening symptoms secondary to AVM growth or hemorrhage. Here the authors present the first known case of a patient with WMS and a pituitary macroadenoma. A 62-year-old man with a 30-year history of WMS with right basal ganglia and orbital AVMs and right eye blindness presented for new-onset left-sided vision loss. A pituitary adenoma was identified compressing the optic chiasm and left optic nerve. Magnetic resonance imaging and digital subtraction angiography studies were obtained for surgical planning, and the patient underwent an endoscopic transnasal transsphenoidal resection, with significant postoperative vision improvement. Given the variable presentation and poor characterization of this rare syndrome, patients with WMS presenting with new symptoms must undergo evaluation for growth and hemorrhage of known AVMs, as well as new lesions. Further, in patients undergoing intracranial surgery, extensive preoperative imaging and planning are crucial for safe and successful procedures.

Microsurgical Resection of an Orbital Arteriovenous Malformation With Intraoperative Digital Subtraction Angiography.

Microsurgical Resection of an Orbital Arteriovenous Malformation With Intraoperative Digital Subtraction Angiography.

Cracking the mysteries: Arteriovenous malformation presenting as proptosis

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.

Effect of Resection of an Orbital Arteriovenous Malformation on Central Venous Pressure

We report the first utilization of intraoperative central venous pressure (CVP) monitoring in the resection of an orbital arteriovenous malformation. A 24-year-old woman with a history of a left orbital mass who had previously undergone resection of a cranio-orbital arteriovenous malformation presented with gradual recurrence in the left orbit. She visited the emergency department with sudden vision loss, which resolved over several hours. This transient vision loss was thought to be due to a steal phenomenon from the ophthalmic artery due to the residual vascular malformation. Further surgical resection was undertaken. A preoperative angiogram identified residual feeding vessels, and the larger vessels were embolized. At the start of the procedure, her CVP was elevated (29 mm Hg), as measured by a central venous line. The remaining feeding vessels were surgically ligated, and an intraoperative arteriogram confirmed their successful ablation. At the conclusion of the procedure, the CVP had decreased to 9 mm Hg.

Challenges in the management of massive intraorbital and hemifacial arteriovenous malformation as causing life-threatening epistaxis

Arteriovenous malformations are congenital lesions that may evolve with time and manifest in a plethora of presentations. They can occur as torrential epistaxis when it extensively involves the facial region. Multi-imaging modalities are available to assist in characterizing the structure of the lesion as well as its location and extent. This complex disease requires a multidisciplinary team approach with preoperative embolization and surgery. We present a rare cause of life-threatening epistaxis in a gentleman with a longstanding orbital and hemifacial arteriovenous malformation and discuss the complexities involved in its management.

Spontaneous Thrombosis of an Orbital Arteriovenous Malformation Revealing Hereditary Haemorrhagic Telangiectasia (Rendu-Osler-Weber Disease)

Hereditary Haemorrhagic Telangiectasia (HHT) is a genetic disorder responsible for cutaneous or mucosal telangiectasia and arteriovenous malformations (AVMs). The most frequent locations are lung and brain. In contrast, orbital AVMs are very rare. We describe a case of symptomatic orbital arteriovenous malformation due to spontaneous thrombosis. A 65-year-old woman was referred for chronic right eye proptosis associated with dilation of conjunctival vessels with a jellyfish pattern. Right visual acuity was 20/40 and intraocular pressure was 40 mmHg. Personal and familial history of recurrent epistaxis, associated with multiple telangiectasia within lips and palate, led to the diagnosis of HHT. Magnetic resonance imaging (MRI) completed with cerebral angiography found a giant and occluded AVM within the right orbit. Other AVMs were also found in brain and chest, confirming the diagnosis. Antiglaucomatous eyedrops were added to reduce intraocular pressure and a steroid therapy was begun. Two months later, visual acuity decreased in the right eye, due to a central retinal vein thrombosis. In conclusion, Most brain or pulmonary AVM can be treated by embolization. By contrast, this treatment in case of orbital location can lead to central retinal artery and/or central retinal vein occlusion, which may also appear as a spontaneous complication of the orbital AVM. Therapeutic management of orbital AVM is thus not standardized, and the balance between spontaneous and iatrogenic risk of visual loss has to be taken into account.

Intraorbital Arteriovenous Malformation Treated by Transarterial Embolization: Technical Case Report

Intraorbital arteriovenous malformations (AVMs) are rare, and their clinical management is controversial. A case of orbital AVM successfully treated by transarterial embolization with diluted glue is reported. A 53-year-old man developed a left pulsating exophthalmos, chemosis, and ocular pain, revealing the presence of a left intraorbital AVM. A microcatheter was selectively placed into the feeders arising from the left ophthalmic artery. Diluted glue was injected toward the nidus through the microcatheter, with monitoring by digital subtraction angiogram. These procedures were performed for 2 individual proper feeders. Superselective angiography for the central retinal artery was also performed instead of a retinal Wada test. His orbital symptoms improved immediately after the procedure without further visual disturbance. Follow-up digital subtraction angiogram revealed the disappearance of the AVM. Transarterial embolization can be considered one of the radical treatments for orbital AVMs.

Orbital Arteriovenous Malformations

To present the clinical features, management, and outcomes in a series of patients with orbital arteriovenous malformations (AVMs). Clinical records of patients with orbital AVMs confirmed using angiography were reviewed as a retrospective, noncomparative, interventional case series. Eight patients (3 women and 5 men) with unilateral AVMs and a mean age of 39 years (median, 36.5 years; range, 26-70 years) were reviewed. Findings existed for an average of 11.2 years before diagnosis and included periocular mass (7 patients, 88%); periocular edema, pulsation/bruit, proptosis, episcleral congestion, and previous trauma (4 patients each, 50%); elevated intraocular pressure (3 patients, 38%); pain and reduced visual acuity (2 patients each, 25%); and restriction of extraocular movements, and diplopia (1 patient each, 12%). All of the patients except 1 underwent surgical resection, with 3 (38%) receiving preoperative embolization of feeder vessels; all of the patients had initial resolution of manifestations after treatment. Angiography is essential for diagnosis and for planning the management of orbital AVMs. Treatment depends on patient-specific features and includes observation, embolization, and surgical excision or combined preoperative embolization/excision. Given their vascular nature, the main cause of poor management outcomes is perioperative hemorrhage. Outcomes after a multidisciplinary approach are good, with few recurrences reported at follow-up.

Arterial Occlusion following Embolization of an Orbital Arteriovenous Malformation

Orbital arteriovenous malformation is a rare condition which poses a management problem in view of the complexity of the vessels involved, and the potentially blinding and life-threatening complications from the modes of treatment. Treatment requires obliteration of the abnormal vascular communication. Vision may be adversely affected by the condition itself, as well as a result of invasive treatment modalities. This case reports such a situation where the visual outcome was severely affected following embolization of the feeding vessels.

Orbital exenteration after transarterial embolization in a patient with Wyburn-Mason syndrome: Pathological findings

We present the pathological findings at orbital exenteration in a patient with Wyburn-Mason syndrome who underwent transarterial embolization. A 31-year-old man with a 10-year history of gradual exacerbation of left exophthalmos and left cheek swelling was found to have facial and orbital arteriovenous malformations on the left side. There was no vascular malformation in the brain. The feeding arteries derived from the left internal maxillary artery, facial artery, and ophthalmic artery. He underwent several courses of transarterial embolization of the feeding arteries from the left internal maxillary artery and then from the facial artery, resulting in no reduction of the arteriovenous malformation. He finally elected to undergo ophthalmic artery embolization in the expectation of a reduction and with the understanding that he would lose sight in his left eye. Two years later, he requested lid-sparing orbital exenteration and reconstruction with cutaneous flap transfer and prosthesis for cosmetic reasons. Pathologically, orbital vascular channels of varying sizes were filled with embolizing glue and had degenerating vascular wall cells surrounded by inflammatory cell infiltration. The central retinal artery in the optic nerve was also filled with the embolizing glue, and the retina lost the ganglion cell layer and inner nuclear layer but maintained the outer nuclear layer and outer segments. Marked anastomoses and hence incomplete embolization among the feeding arteries of facial and orbital vascular malformations in Wyburn-Mason syndrome do not respond well to attempts at feeding vessel embolization, which result in unsuccessful closure of the malformation.

Diagnosis and Treatment of a Superficial Upper Eyelid Arteriovenous Malformation

Combined embolization and surgical excision as a safe treatment for a high flow superficial upper eyelid anteriovenous malformation. Introduction: Orbital arteriovenous malformations (AVM) may cause significant morbidity including chronic pain and cosmetic disfigurement. These rare lesions require a multidisciplinary approach. A case of an orbital high-flow AVM treated subsequently by an mterventional radiologist and oculoplastic surgeon is presented. Materials and methods: Clinical assessment, computed tomography and digital substruction angiography revealed a high-flow AVM. Transvenous and percutaneous embolization was followed by surgical excision. Results: A 1.5 cm, slowly growing, compressible pulsating lesion in the left upper eyelid of a 23 year old man was found. Eye motility and ophthalmologic examination were normal. Valsava manoeuvre didn't increase the size. Imaging revealed a high-flow AVM feeded by the arteria temporalis superficialis (itself feeded by the a. supraorbitalis and supratrochlearis). Draining vessels were the superior ophthalmic vein and a superficial lateral orbital vein. This malformation was first embolized. To prevent iatrogenic embolization of the sinus cavernous, the vein draining into the superior ophthalmic was occluded by a transvenous approach, followed by a percutaneous injection with diluted glue (equal amounts of glubran-2 and lipiodol). The remaining low-flow lesion was then safely excised. Conclusion: High-flow orbital AVM represent a considerable treatment challenge. Good cosmetical and functional outcome was obtained safely by subsequent embolization and surgical excision. When treating these rare malformations it is of the highest importance to coordinate the interdisciplinary efforts.

A case report of Wyburn-Mason syndrome and review of the literature

Wyburn-Mason syndrome is a distinct congenital neurocutaneous entity comprised of ipsilateral arteriovenous malformations (AVMs) of the midbrain, vascular abnormalities affecting the visual pathway, and facial nevi. We report a case and review of the literature of all other reported cases of Wyburn-Mason syndrome (n = 26) in the English literature since 1973. In this review, we report on a 4(1/2)-year-old boy with Wyburn-Mason syndrome who presented with left retinal and orbital AVMs and a ruptured thalamic AVM. The patient did not respond to light in the left eye and demonstrated a left afferent pupillary defect. He did not have any cutaneous lesions. We also characterize other reported cases of Wyburn-Mason syndrome. The presentation of patients with Wyburn-Mason syndrome can vary greatly according to the site and the extent of vascular lesions. Intracranial AVMs occasionally hemorrhage with significant morbidity. Treatment is controversial, and patients are typically managed conservatively by observation.

High-flow Orbital Arteriovenous Malformation in a Child

Vascular malformations of the orbit cause significant morbidity such as chronic pain, diplopia, amblyopia, and cosmetic disfigurement. They are rare lesions which require multidisciplinary care, and in the modern era results of treatment have been greatly improved with the assistance of endovascular therapy. Other treatment options include laser therapy, percutaneous embolization, open surgery, or a combination of these modalities. Nevertheless some patients suffer poor results despite modern medical advances. A case of an orbital arteriovenous malformation (AVM) initially treated independently by a dermatologist, a plastic surgeon, and a neuroendovascular interventionalist is presented. When treating patients with these rare but disabling lesions it is of the highest importance to coordinate efforts between all pertinent specialists in order to promote the best possible result.

Commentary

This article describes a unique presentation of a large intraorbital arteriovenous malformation (AVM) and reviews orbital and ocular adnexal AVMs. Although not exhaustive, the concise summary provides enough background to cover the differential diagnoses. The surgical description is a fine review of a cautious approach to dealing with large lateral intraorbital AVMs involving the superior lateral orbit. As alluded to in this article, smaller AVMs may be considered for intravenous embolization. The success rate of embolizing selected smaller AVMs varies from 10 to 40%. These embolization techniques are associated with the same risk profile as surgical approaches to the same lesions. Grade I AVMs are associated with a 5% surgical risk and their surgical success rate is almost 100%. In contrast, embolization techniques are associated, at best, with a 40% chance of therapeutic control whereas the risk is equivalent to that of surgery. Consequently, most authorities would agree that a surgical approach to orbital AVMs appears to be warranted. The literature and personal experience testify to the efficacy of microembolizing spontaneous and traumatic cavernous-sinus fistulas. Access for embolization can be attained through an upper lid crease cut-down, exposing the superior ophthalmic vein. Gil-Salú, González-Darder, and Vera-Román have presented a case report that highlights a rare but important orbital challenge. They should be congratulated for their endeavors and presentation.

Commentary

This article describes a unique presentation of a large intraorbital arteriovenous malformation (AVM) and reviews orbital and ocular adnexal AVMs. Although not exhaustive, the concise summary provides enough background to cover the differential diagnoses. The surgical description is a fine review of a cautious approach to dealing with large lateral intraorbital AVMs involving the superior lateral orbit. As alluded to in this article, smaller AVMs may be considered for intravenous embolization. The success rate of embolizing selected smaller AVMs varies from 10 to 40%. These embolization techniques are associated with the same risk profile as surgical approaches to the same lesions. Grade I AVMs are associated with a 5% surgical risk and their surgical success rate is almost 100%. In contrast, embolization techniques are associated, at best, with a 40% chance of therapeutic control whereas the risk is equivalent to that of surgery. Consequently, most authorities would agree that a surgical approach to orbital AVMs appears to be warranted. The literature and personal experience testify to the efficacy of microembolizing spontaneous and traumatic cavernous-sinus fistulas. Access for embolization can be attained through an upper lid crease cut-down, exposing the superior ophthalmic vein. Gil-Salú, González-Darder, and Vera-Román have presented a case report that highlights a rare but important orbital challenge. They should be congratulated for their endeavors and presentation.

Spontaneous hemorrhage in an intraorbital arteriovenous malformation

Objective To report the clinical findings and management of spontaneous hemorrhage in an unsuspected intraorbital arteriovenous malformation. Design Interventional case report. Methods Review of clinical findings, radiologic studies, and treatment of the patient. Results A 75-year-old woman sought treatment for the rapid onset of severe proptosis. Orbital exploration of a “mass” imaged on computed tomography scan and magnetic resonance imaging resulted in massive intraoperative hemorrhage. Subsequent arteriographic and histopathologic analysis confirmed an underlying orbital arteriovenous malformation. Conclusions Spontaneous intraorbital hemorrhage from an arteriovenous malformation is extremely rare, but should be considered in the differential diagnosis of rapidly progressive proptosis.

Clinical findings in a patient with spontaneous arteriovenous fistulas of the orbit

PURPOSE: To report clinical and radiologic findings of a patient with spontaneous arteriovenous fistulas of the orbit. METHOD: Case report. RESULTS: A 73-year-old woman was initially examined with a 1-year history of mild proptosis of the right eye. She had no history of trauma. Neuro-ophthalmologic examination disclosed dilatation of conjunctival vessels, increased intraocular pressure, mild proptosis and bruit in the right eye, and ocular signs suggestive of carotid-cavernous sinus fistulas or orbital arteriovenous malformations. The patient exhibited dilation of the superior ophthalmic vein in enhanced computed tomography of the orbit. Selective cerebral angiography disclosed communications between branches of both ophthalmic and facial arteries and the superior ophthalmic vein in the orbit. CONCLUSION: Arteriovenous fistulas of the orbit must be considered in the differential diagnosis of carotid-cavernous sinus fistulas and arteriovenous malformations, although they are quite rare.