Combined Surgical and Endovascular Treatment of an Intraorbital Arteriovenous Fistula Drained Into a Basal Vein of Rosenthal: A Technical Case Report.

BACKGROUND: Intraorbital arteriovenous fistulas (AVFs) are rare lesions, and their treatment is challenging. We have presented a case of an intraorbital AVF treated with endovascular embolization through the surgically accessed basal vein of Rosenthal. CASE DESCRIPTION: A 53-year-old man had been referred to our hospital for an aneurysm-like lesion that was compressing the left optic chiasm. A left internal carotid angiogram demonstrated an orbital AVF fed by a distal segment of the ophthalmic arteries and the anterior branch of the inferolateral trunk that was drained solely into a tortuous basal vein of Rosenthal through a bridging vein of the left optic nerve sheath. Considering the risk of hemorrhagic complications during transvenous manipulation and visual complication in cases of transarterial embolization, combined surgical and transvenous embolization was attempted through the translocated basal vein of Rosenthal to bypass the dangerous path to the fistula. The lesion was directly catheterized through the translocated basal vein of Rosenthal after confirming visual tolerance to brief drainage occlusion using visual evoked potential monitoring, resulting in successful fistula obliteration using detachable coils. CONCLUSION: Direct catheterization of the translocated deep draining vein was useful to bypass the dangerous access to the fistula and could be a feasible alternative strategy for treating selected AVFs.

Ophthalmic Arteries Arising from the Bilateral Anterior Cerebral Arteries Diagnosed by Three-Dimensional Time-of-Flight Magnetic Resonance Angiography.

BACKGROUND: The ophthalmic artery (OA) is a very important branch of the supraclinoid segment of the internal carotid artery. Origination of the OA from the anterior cerebral artery is an extremely rare condition. Being unaware of the origin anomalies of OAs can lead to unfavorable results, especially in cases in which craniofacial surgery or cerebral therapeutic angiography is planned. CASE DESCRIPTION: An anomalous origin of the ipsilateral OAs from the A1 segments of the bilateral anterior cerebral arteries was detected by three-dimensional time-of-flight magnetic resonance angiography. CONCLUSIONS: Anomalous origin of the OA from the anterior cerebral artery may be explained by a failure in the caudal migration of the ventral OA. Because of high soft tissue resolution and absence of radiation, three-dimensional time-of-flight magnetic resonance angiography is preferred for detection of orbital and cerebral vascular anomalies.

Unusual ophthalmic artery origin: Implications for intraarterial chemotherapy of retinoblastoma.

We report two pediatric retinoblastoma patients with unusual ophthalmic artery (OA) origins who were referred to our interventional neuroradiology department for intraarterial chemotherapy. The first patient had double OAs arising from the ipsilateral internal carotid artery and a central retinal artery (CRA) arising from the more cranially located artery, whereas the second patient had an OA originating from the A1 segment of the ipsilateral anterior cerebral artery. In both cases the CRA arose from the artery entering the orbit through the optic foramen. Both patients tolerated the procedures well. To the best of our knowledge, our retinoblastoma patients with unusual OA origins are the first in the literature in which detailed knowledge of the orbital vasculature and especially CRA origin carried immediate clinical implications, altering access strategies. These variations were proved both by flat-panel detector computed tomography findings and superselective injections of OAs with unusual origins. Our results show that in patients with retinoblastoma, a detailed angiographic work-up may be necessary to evaluate the orbital vasculature and possible abnormal origin of the OA. Recognition of possible variations of OA origin and its branching patterns is of the utmost importance for successful treatment and avoidance of adverse events among retinoblastoma patients.

Orbital Arteriovenous Fistula Coexistent with an Arteriovenous Hemangioma: A Rare Occurrence and Review of Literature.

BACKGROUND: Orbital arteriovenous fistula (AVF) is a rare entity with only 17 cases reported so far. An arteriovenous hemangioma is a distinct entity of an angioma that most frequently affects the skin. However, to our knowledge, there has not been a case arising in the orbit. CASE DESCRIPTION: A previously healthy, 73-year-old man had progressive proptosis for 1 month. At presentation, the patient showed considerable proptosis, hyperemia in the lower eyelid, chemosis, and total ophthalmoplegia on the right side. Computed tomography and magnetic resonance imaging revealed a round retro-orbital mass, 23 mm in maximal dimension, and lying in the inferomedial aspect of the orbit with an irregular-shaped lesion in the orbital apex. Cerebral angiography detected an orbital AVF fed by the ipsilateral maxillary and ophthalmic arteries and draining into the inferior ophthalmic vein. Stain of the retro-orbital mass was not identified. A transvenous coil embolization via the facial and superior ophthalmic veins achieved complete isolation of the AVF with satisfactory outcome. Two months later, the orbital mass, well circumscribed and lacking perilesional hemosiderin deposition, was microsurgically resected via a lateral orbitotomy. Histological diagnosis was consistent with an arteriovenous hemangioma. CONCLUSIONS: An AVF and arteriovenous hemangioma may simultaneously develop in the same orbit. In patients with symptomatic orbital AVF and coexisting other orbital pathology, strategies should be carefully planned before setting about the treatment.

Incidental ethmoidal dural arteriovenous fistula coexisting with a pituitary adenoma exacerbating post-transsphenoidal epistaxis.

A 64 year-old man with pituitary adenoma developed massive epistaxis after an uneventful endoscopic transsphenoidal surgery. Angiography showed extravasation from the sphenopalatine artery, to which embolisation was performed. An incidentally coexisting ethmoidal dural arteriovenous fistula supplied by the ophthalmic artery aberrantly originated from the middle meningeal artery caused increased haemorrhage.

Imaging of Cerebral Arteriovenous Malformations and Arteriovenous Fistulas and Occlusion Control Using Intraoperative 3-Dimensional Rotational Fluoroscopy.

BACKGROUND: Intraoperative resection or occlusion control is indispensable in the surgery of vascular anomalies. This can be conducted using local vascular imaging modalities or angiographic techniques. This series was performed to assess whether cerebral arteriovenous malformations (AVMs) and dural arteriovenous fistulae (dAVFs) can be detected in a sufficient quality by intraoperative 3-dimensional (3D) fluoroscopy with intravenous contrast application. MATERIALS AND METHODS: Five patients were included in the analysis (2 AVMs, 3 dAVFs). All patients had preoperative digital subtraction angiography. The head was fixed in a carbon MAYFIELD clamp. After a 3D rotational fluoroscopy scan without contrast agent, a second scan with 50 mL of iodine contrast agent was performed. The Digital Imaging and Communications in Medicine data of both scans were subtracted and reconstructed using the OsiriX imaging software. In 2 patients with dAVF, occlusion control was performed after obliteration of the fistula. RESULTS: In the 2 patients with cerebral AVM, 3D fluoroscopy with intravenous contrast administration resulted in good image quality. Preoperative embolization with Onyx produces significant artifacts that can be largely removed by simple digital subtraction techniques. In dural AVF, occlusion control was well feasible after obliteration of the draining vein at its dural origin. CONCLUSIONS: This technique quickly supplies intraoperative images of adequate quality to locate cerebral AVM and dAVF. However, it does not produce dynamic images. Thus, early draining veins cannot be located unless anatomically identified based on the preoperative DSA. In this case, it can be used for intraoperative obliteration control.

The clinical presentation and collateral pathway development of congenital absence of the internal carotid artery.

OBJECTIVE: The objective of this study was to investigate the clinical presentation, risks, and collateral pathway development of the congenital absence of the internal carotid artery (ICA). METHODS: Sixty-four patients (10 new patients and 54 patients from the relevant literature) were studied. Data on demographic, clinical, and radiologic features were collected, followed by an analysis of the risks associated with ICA agenesis. RESULTS: There were 31 male and 33 female patients whose ages ranged from 5 months to 75 years, with a mean age of 31.1 years. The range of clinical symptoms recorded included transient ischemic attack (17 patients), subarachnoid hemorrhage (12 patients), developmental delay (13 patients), asymptomatic (8 patients), and other symptoms (15 patients). All 64 patients presented with absence of unilateral or bilateral ICAs, as measured by cervical computed tomography angiography or magnetic resonance angiography. The carotid canal was absent in all patients on computed tomography of the base of the skull, and abnormal development of collateral circulation pathways was observed. Five patients presented with basilar artery dilation on angiography. Aneurysms were observed in the angiography results from 16 patients. Ten patients presented with variations in the ophthalmic artery origin (the ophthalmic artery originated from the ipsilateral middle meningeal artery in six patients and from the ipsilateral middle cerebral artery in four patients). CONCLUSIONS: From analysis of our 10 cases of ICA agenesis and our review of the relevant literature, we conclude that young patients with ICA agenesis may present with developmental delay, subarachnoid hemorrhage, or other developmental abnormalities, whereas older patients most commonly present with transient neurologic events. Complications of carotid agenesis are related to specific anatomic subtypes and the resulting collateral circulation development.

Bilateral carotid-anterior cerebral artery anastomoses associated with bilateral ophthalmic arteries arising from the anastomotic arteries diagnosed by magnetic resonance angiography: a case report.

Rarely, the anterior cerebral artery (ACA) arises from the ophthalmic segment of the internal carotid artery, a condition described as carotid-ACA anastomosis or infraoptic course of the ACA that generally demonstrates right-sided predominance. We diagnosed a case of bilateral anastomoses of the carotid and ACA vessels in which bilateral ophthalmic arteries arose from the origins of the anastomotic vessels using magnetic resonance (MR) angiography. Identification of rare arterial variations on MR angiography requires careful scrutiny of source images, and creation of partial volume-rendering images can aid visualization of detailed anatomic structures.

Comprehensive Treatment of Primary Orbital Arteriovenous Malformation.

Primary orbital arteriovenous malformations are a rare kind of vascular malformation lesions. The authors present a 17-year-old man presented with swelling and pulsation in the left upper eyelid. The angiogram of the left internal carotid artery showed that arteriovenous malformations at the left upper eyelid area were supplied with one of the branches of ophthalmic artery. In this report, the authors elaborated the comprehensive treatments of primary arteriovenous malformation.

Transarterial Embolization of a Spontaneous Intraorbital Arteriovenous Fistula With n-BCA Glue.

Arteriovenous fistulae of the orbit are exceedingly rare. They are high-flow vascular malformations involving a fistula from the ophthalmic artery to one of the draining ophthalmic veins. Presenting symptoms can mimic those of carotid cavernous fistulae or of ophthalmic venous varices, and include diplopia, proptosis, chemosis, decreased visual acuity, and retro-orbital pain. Very few case reports are published on the treatment of this aggressive vascular malformation, and they uniformly describe techniques involving a transvenous access for the embolization of the fistula. To the best of authors' knowledge, they report the first case of transarterial embolization of an intraorbital AVF.

Low-Flow Arterialized Venous Malformations of the Orbit.

PURPOSE: Orbital vascular malformations are classified by their hemodynamic properties, either high or low flow. Low-flow lesions may be simple venous, lymphatic, or combined lymphaticovenous malformations. The authors report a series of cases in which predominantly low flow, venous lesions were unexpectedly noted to have arterial feeders. METHODS: A retrospective chart review of patients identified by the authors as having orbital varices with arterial components was conducted. The authors identified 7 such cases. After careful review, 2 cases were excluded due to inconclusive neuroradiographic findings. The authors review the clinical, radiologic, histopathologic, and surgical information from the remaining 5 cases and discuss their clinical significance. RESULTS: All 5 cases were most consistent with variceal lesions: 3 as clinically distensible lesions and 2 as thrombosed lesions. Additional arterial feeder vessels were noted by angiography (3) or intraoperative visualization (2). The arterial contribution varied from faint vessels to distinct branches of the ophthalmic artery. Ages ranged from 13 to 61 years without predilection for gender. Treatments consisted of excision, embolization, and observation. Two poignant cases are highlighted: the first illustrating that an angiogram in isolation of its clinical picture can be misleading and result in treatment intervention with undue risk, and the second illustrating that inadequate treatment of unrecognized arterial components may contribute to recurrences. CONCLUSIONS: Low-flow orbital variceal lesions may have less prominent, arterial components. This type of combined arterialized venous malformation is largely unrecognized in the ophthalmic literature. Correct identification of these lesions is critical in providing safe, effective, and durable treatment.

Arteriovenous Malformation of the Eyelid: Surgical Management and Histologic Study.

A raised erythematous eyelid lesion that appeared in a 31-year-old man was diagnosed as an arteriovenous malformation (AVM), with confirmatory Doppler ultrasound demonstrating high arterial flow. Surgical excision, aided by electrocautery for extensive hemorrhage, resulted in an acceptable cosmetic result. Histopathology of the excised lesion showed collapsed capillary channels lined by endothelium. AVM is rarely encountered in the eyelid.

Immediate Reconstruction After Combined Embolization and Resection of Orbital Arteriovenous Malformation.

Orbital arteriovenous malformations are rare congenital vascular anomalies that can cause eyelid distortion, proptosis, diplopia, and vision loss. The current treatment paradigm involves endovascular embolization followed by resection, with delayed reconstruction. The authors report 2 young adult patients with orbital arteriovenous malformations, who underwent embolization followed days to months later by resection with immediate soft tissue reconstruction. For both patients, the immediate outcome provided good functionality and cosmesis. The authors conclude that immediate reconstruction after embolization-resection of orbital arteriovenous malformations may facilitate early return of form and function for patients, improve quality of life, and potentially reduce the need for further reconstructive procedures.

Clinical and Anatomic Insights From a Series of Ethmoidal Dural Arteriovenous Fistulas at Barrow Neurological Institute.

BACKGROUND: Ethmoidal dural arteriovenous fistulas (dAVFs) have a malignant natural history and an anatomy that make endovascular therapy challenging. Their uniqueness begs for stratified analyses, but this has largely been precluded by their rarity. We sought to summarize the anatomic, presentation, treatment approaches, and clinical outcomes of patients with these lesions. METHODS: We reviewed our prospectively maintained institutional database to identify patients diagnosed with ethmoidal dAVFs from January 1, 2000, to December 31, 2015. We evaluated demographic, presentation, angiographic, treatment, and follow-up data. RESULTS: In total, 27 patients with ethmoidal dAVFs underwent endovascular and/or surgical treatment. Mean patient age was 62 years old and there was a male sex predilection (67% men; 2:1 male-female ratio). All dAVFs exhibited direct cortical venous drainage; venous ectasia was present in 59% of cases. Of the dAVFs, 30% drained posteriorly into the basal vein of Rosenthal or the sylvian veins. Embolization with casting of the draining vein was successful in 2 of 9 cases (22%), including 1 successful transvenous case. There were no clinical or permanent complications from embolization; specifically, no patients experienced visual loss after treatment. Surgical treatment with successful dAVF obliteration was carried out in 24 of 24 patients (100%). One patient declined surgical treatment after attempted endovascular embolization. There were no permanent complications after surgical treatment and no cases of wound infection or cerebrospinal fluid leakage. CONCLUSIONS: Surgical disconnection remains the gold standard in the treatment of ethmoidal dAVFs. Embolization is a consideration for well-selected cases with favorable arterial or venous access anatomy.

A New Anatomic Variation: Coexistence of Both Dandy-Walker Variant and Ophthalmic Artery Originating From Contralateral Internal Carotid Artery.

The authors report on 1 patient of variant origin of right ophthalmic artery (OA) from ophthalmic segment of the left internal carotid artery. A 41-year-old man was performed magnetic resonance (MR) imaging and MR angiography. Cerebral MR imaging revealed a Dandy-Walker variant. In MR angiography the authors observed this unusual variant of origin of OA and a complete occlusion of right internal carotid artery. To the authors' knowledge, this is the first patient who has coincidence of both Dandy-Walker variant and origin of OA from contralateral internal carotid artery. Careful observation of MR angiography images with maximum intensity projection is very important for detecting rare vascular variations.

Unusual origin of the left ophthalmic artery from the basilar trunk.

The formation of the ophthalmic artery (OA) is a complex process with two different proposed embryological steps for its development. Several anatomical variants have been described. We present a very unusual origin of the ophthalmic artery from the basilar trunk, in a 45-year-old male with a history of pontine hemorrhagic stroke. MRI and CTA showed evidence of previous hemorrhage in the pons and several intracranial arterial dysplastic dilatations. DSA confirmed several fusiform dilatations of the basilar trunk. In the left ICA, no ophthalmic artery was seen arising from the carotid siphon. The left ophthalmic artery arises from the basilar trunk and runs lateral to the cavernous sinus through the middle cranial fossa, entering the left orbit at the superior orbital fissure. The patient was treated conservatively. Two main theories for this anomaly are known, one from Lasjaunias and the other from Paget. To our knowledge, this basilar origin of the OA has only been described three times in the literature. For its origin, we propose a partial persistence of the trigeminal artery together with a dominance of the dorsal ophthalmic artery.

Dystrophic hyaloid artery remnants and other abnormalities in a buphthalmic eye with retinoblastoma.

Partial persistence of the hyaloid artery unaccompanied by hyperplastic primary vitreous has not been previously reported in association with retinoblastoma. We describe an 18-month-old child with such a finding who had a retinoblastoma that was undifferentiated, extensively necrotic, heavily calcified, and completely filled the eyeball. The enucleated globe harbored a nonperfused, fossilized remnant of the hyaloid artery due to DNA/calcium deposition in the vascular wall. This structure inserted into a lenticular, extracapsular, fibrous plaque corresponding to a Mittendorf dot. The tumor had induced a placoid cataractous lens, obliterated the anterior and posterior chambers, caused glaucoma leading to buphthalmos, and extended into the optic nerve and extraocularly to involve the orbit. We conclude that the retinoblastoma arose early in ocular morphogenesis, at around 4 months gestation, when the programmed involution of the hyaloid artery begins. This process would typically end at 7-8 months gestation, but was aborted by the tumor. The patient died 6 weeks after surgery without receiving further treatment because of the parents' resistance.

High incidence of ICA anterior wall aneurysms in patients with an anomalous origin of the ophthalmic artery: possible relevance to the pathogenesis of aneurysm formation.

OBJECT: Surgery for aneurysms at the anterior wall of the internal carotid artery (ICA), which are also referred to as ICA anterior wall aneurysms, is often challenging. A treatment strategy needs to be determined according to the pathology of the aneurysm-namely, whether the aneurysm is a saccular aneurysm with firm neck walls that would tolerate clipping or coiling, a dissecting aneurysm, or a blood blister-like aneurysm. However, it is not always possible to properly evaluate the condition of the aneurysm before surgery solely based on angiographic findings. METHODS: The authors focused on the location of the ophthalmic artery (OA) in determining the pathology of ICA anterior wall aneurysms. Between January 2006 and December 2012, diagnostic cerebral angiography, for any reason, was performed on 1643 ICAs in 855 patients at Saitama Medical Center. The authors also investigated the relationship between the origin of the OA and the incidence of ICA anterior wall aneurysms. The pathogenesis was also evaluated for each aneurysm based on findings from both angiography and open surgery to identify any correlation between the location where the OA originated and the conditions of the aneurysm walls. RESULTS: Among 1643 ICAs, 31 arteries (1.89%) were accompanied by an anomalous origin of the OA, including 26 OAs originating from the C3 portion, 3 originating from the C4 portion, and 2 originating from the anterior cerebral artery. The incidence of an anomalous origin of the OA had no relationship to age, sex, or side. Internal carotid artery anterior wall aneurysms were observed in 16 (0.97%) of 1643 ICAs. Female patients had a significantly higher risk of having ICA anterior wall aneurysms (p = 0.026). The risk of ICA anterior wall aneurysm formation was approximately 50 times higher in patients with an anomalous origin of the OA (25.8% [8 of 31]) than in those with a normal OA (0.5% [8 of 1612], p < 0.0001). Based on angiographic classifications, saccular aneurysms were significantly more common in patients with an anomalous origin of the OA than in those with a normal OA (p = 0.041). Ten of 16 patients with ICA anterior wall aneurysms underwent craniotomies. Based on the intraoperative findings, all 6 aneurysms with normal OAs were dissecting or blood blister-like aneurysms, not saccular aneurysms. CONCLUSIONS: There was a close relationship between the location of the OA origin and the predisposition to ICA anterior wall aneurysms. Developmental failure of the OA and subsequent weakness of the vessel wall might account for this phenomenon, as previously reported regarding other aneurysms related to the anomalous development of parent arteries. The data also appear to indicate that ICA anterior wall aneurysms in patients with an anomalous origin of the OA tend to be saccular aneurysms with normal neck walls. These findings provide critical information in determining therapeutic strategies for ICA anterior wall aneurysms.

Persistent vestige of dorsal ophthalmic artery: a case report.

A rare case of a persistent dorsal ophthalmic artery is reported. An elderly woman presented with subarachnoid hemorrhage and hydrocephalus. A CT angiogram revealed findings consistent with a small aneurysm and the presence of a dorsal ophthalmic artery. This was confirmed with catheter diagnostic angiography. The radiological findings, embryology and anatomy of these dual ophthalmic arteries are discussed.