Intraorbital Arteriovenous Malformations Research Articles

Transcranial Orbitotomy for Resection of Orbital Intraconal Arteriovenous Malformation: 2-Dimensional Operative Video

Intraorbital arteriovenous malformations (AVMs) are not common, and their prevalence is unknown. 1,2 They can cause significant morbidity, including vision loss, diplopia, chronic pain, and cosmetic deformity. 3 Treatment can be technically difficult because of the challenging anatomical location with multiple adjacent nerves and ophthalmic vascularity, and it may involve endovascular and open surgical procedures or both. 4-6 We present a video illustrating the case of a 45-year-old man with progressive right-sided proptosis and chemosis with intact vision. Magnetic resonance imaging demonstrated an orbital intraconal lesion in the superior lateral aspect. Angiogram confirmed the presence of an AVM fed by branches of the ophthalmic artery and draining to the superior ophthalmic vein. We performed a transcranial orbitotomy for resection of the AVM. Intraoperative angiogram confirmed complete resection of the AVM. The patient consented to the procedure and the publication of his images. Institutional review board approval is waived for single case reports. The patient did well postoperatively, and his proptosis and chemosis resolved. It is important for the skull base surgeon to be adept in treating orbital pathologies safely and effectively given the overlapping of pathologies that may involve the orbit and skull base such as this.

Extensive severe intraorbital arteriovenous malformations: report of a rare laser complication and successful management.

Arteriovenous malformations (AVMs) are rare congenital errors of vascular morphogenesis, characterised by abnormal direct communications between arteries and veins, bypassing the normal capillary bed. Apart from the central nervous system, typical locations include the head and neck, and less frequently limbs, trunk or internal organs. AVMs of the head and neck often become clinically evident and symptomatic only in later childhood and are characterised by a history of variable growth, sometimes leading to large, deforming, pulsating masses with a propensity to massive haemorrhage. Therapeutic strategy is based on selective embolisation, surgical excision, or a combination of both. Radical surgical excision of local AVMs is the only effective treatment but it may be mutilating, especially for AVMs of the head and neck. Laser therapy represents a good option to treat the cutaneous aspects of AVMs nevertheless, its efficacy is limited. To the date, pharmacological therapy for AVMs is still on debate due to its controversial outcomes as it seems not as effective as other treatments and usually requires a longer course of application. However, pharmacological therapy could be useful in selected patients and for AVMs nonresponsive to traditional treatment, allowing them to obtain acceptable results without serious complications. This paper reports the case of a serious laser complication of extensive intraorbital AVM successfully treated by local reconstruction and topical pharmacological treatment.

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.

Orbital and Cerebral Arteriovenous Malformations

An 8-year-old girl had a 3-month history of progressively impaired vision in the right eye, with paresthesias and spastic paresis of the left arm and leg. Examination of the right eye revealed the tortuous blood vessels of a complex intraorbital arteriovenous malformation.

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.

Intraorbital Arteriovenous Malformation

Intraorbital arteriovenous malformations are infrequent but, when present, typically require complex surgical management. Computed tomography and magnetic resonance imaging help characterize lesion structure, location, and relationship to intraorbital anatomy. Frequently complex, intervention should be prompted by significant proptosis, increased intraocular pressure, pain, bleeding, ulceration, or high-output cardiac complications. For more controlled surgical debridement, angiographic embolization may help occlude the nidus and feeding vessels temporarily. Utmost caution must be taken to avoid injury to the optic nerve and ophthalmic artery. We present a rare case of complex, intraorbital arteriovenous malformation, as well as discuss aspects of management and relevant literature.

Intraorbital Arteriovenous Malformation: Case Report

An intraorbital arteriovenous malformation became symptomatic without exophthalmos but with chemosis and bruit of the left eye. The patient underwent only surgical treatment. The orbital rim was removed through a fronto-orbital approach. The chemosis was corrected and postoperative magnetic resonance imaging and angiography confirmed complete removal of the malformation. Histopathological examination disclosed an arteriovenous malformation in the extrabulbar fatty tissue without invasion of the extraocular muscles or optic nerve. The differences between various types of intraorbital malformations are discussed.

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.

Successful Surgical Treatment of Intraorbital Arteriovenous Malformations: Case Report

OBJECTIVE AND IMPORTANCE: Cases of intraorbital arteriovenous malformations are rare, and their management presents a challenge for multidisciplinary teams. CLINICAL PRESENTATION: A 39-year-old woman developed a pulsating exophthalmos, visual loss, and ocular pain within 1 month, revealing the presence of intraorbital arteriovenous malformations. She underwent only surgical treatment. INTERVENTION: A fronto-orbital approach with removal of the orbital rim was performed. A CO2 laser facilitated all the surgical steps. CONCLUSION: Full recovery of vision and correction of the proptosis have been achieved, and postoperative magnetic resonance imaging and angiography confirmed the total removal of the malformations. The literature is reviewed regarding the management of such lesions. Surgery remains the treatment of choice. The role of embolization is discussed.

Successful Surgical Treatment of Intraorbital Arteriovenous Malformations: Case Report

Successful Surgical Treatment of Intraorbital Arteriovenous Malformations: Case Report

Successful Surgical Treatment of Intraorbital Arteriovenous Malformations: Case Report

Cases of intraorbital arteriovenous malformations are rare, and their management presents a challenge for multidisciplinary teams. A 39-year-old woman developed a pulsating exophathalmos, visual loss, and ocular pain within 1 month, revealing the presence of intraorbital arteriovenous malformations. She underwent only surgical treatment. A fronto-orbital approach with removal of the orbital rim was performed. A CO2 laser facilitated all the surgical steps. Full recovery of vision and correction of the proptosis have been achieved, and postoperative magnetic resonance imaging and angiography confirmed the total removal of the malformations. The literature is reviewed regarding the management of such lesions. Surgery remains the treatment of choice. The role of embolization is discussed.

Intraorbital arteriovenous malformation: Case report

A case of intraorbital arteriovenous malformation presenting with visual loss, exophthalmos, and chemosis of the right eye is reported. Enhanced computer tomography and magnetic resonance imaging showed extraocular muscle enlargement and vascular abnormality in the right retrobulbar space. Angiography revealed an abnormal intraorbital vascular stain with an extremely dilated right ophthalmic artery. Total removal of the intraorbital contents was performed after unsuccessful endovascular and surgical treatment of arteriovenous malformation (AVM). Histopathological examination disclosed an AVM in the retrobulbar fatty tissue with extension into the extraocular muscles and optic nerve.

Vascular anomalies in the orbit: CT and angiographic diagnosis

From a series of 1,615 patients examined radiologically for unilateral exophthalmos, 523 were shown to have a primary mass lesion in the orbit. Of these, 103 were designated as vascular anomalies, exclusive of the haemangiomata which in this series were classified with the benign orbital tumours. Venous malformations were by far the commonest variety of vascular anomalies encountered. They constituted 13.4% of all primary mass lesions in the series and 4.3% of all causes of proptosis. Their typical appearance on venography is described and correlated with the findings on computerized tomography. Other vascular lesions include carotico-cavernous fistulae and arterio-venous shunts in the middle fossa of the skull which cause secondary dilatation of orbital veins and can be diagnosed by a combination of CT scan and venography. Orbital blood cysts in children and intra-orbital arterio-venous malformations are also described in terms of angiography and computerized tomography.

Wyburn-Mason syndrome. Report of two cases without retinal involvement.

Two patients with unilateral optic atrophy had both suprasellar and intraorbital arteriovenous malformations. The first had associated vascular lesions of the ipsilateral mandible and maxilla, while the second had an ipsilateral facial vascular lesion. To our knowledge, these are the first reported cases of the Wyburn-Mason syndrome in which retinal arteriovenous malformations are absent despite extensive intraorbital involvement with the malformation.