Bilateral Low-Flow Type-D Dural Carotid-Cavernous Fistula: Diagnosis and Treatment with 3D Time-of-Flight Magnetic Resonance Angiography.

BACKGROUND Carotid-cavernous fistula (CCF) is a rare, atypical vascular shunt between the carotid arterial system and the venous channels of the cavernous sinus, classified according to the shunt's anatomy, by etiology (resulting from trauma or occurring spontaneously), or by hemodynamic characteristics (such as low- or high-flow fistulas). CASE REPORT A 62-year-old female patient with poorly controlled arterial hypertension presented with bilateral periorbital edema, conjunctival chemosis, ophthalmoplegia, diplopia, and diminished visual acuity. On magnetic resonance angiography (MRA), abnormal arterial flow along the cavernous sinuses was noted, suggestive of bilateral CCF. The diagnosis of indirect dural low-flow CCF (Barrow Type D) was later confirmed by digital subtraction angiography, with feeding arteries from intracavernous internal carotid artery branches, and meningeal branches of the external carotid artery, draining bilaterally to ophthalmic veins, the intracavernous sinus, and the inferior petrosal sinus. The patient was successfully treated with endovascular embolization. At 7-month follow-up, no residual arteriovenous shunting was detected. This case highlights the importance of non-invasive radiological methods for CCF, and presents rarely published radiological findings of bilateral Type-D dural CCFs on 3-dimensional time-of-flight MRA with post-treatment MRA follow-up. CONCLUSIONS Regardless of the patient's history of possible trauma, a patient presenting with bilateral periorbital edema, conjunctival chemosis, ophthalmoplegia, diplopia, and diminished visual acuity should have a spontaneous bilateral CCF investigated to prevent delayed treatment. Experienced neuroradiologists are needed to accurately detect indirect CCF, since this condition often does not demonstrate classic symptoms.

Bilateral vertebral artery injury leads to brain death following traumatic brain injury: a case report.

BACKGROUND: Vertebral artery injury is a rare condition in trauma settings. In the advanced stages, it causes death. CASE: A 31-year-old Sundanese woman with cerebral edema, C2-C3 anterolisthesis, and Le Fort III fracture after a motorcycle accident was admitted to the emergency room. On the fifth day, she underwent arch bar maxillomandibular application and debridement in general anesthesia with a hyperextended neck position. Unfortunately, her rigid neck collar was removed in the high care unit before surgery. Her condition deteriorated 72 hours after surgery. Digital subtraction angiography revealed a grade 5 bilateral vertebral artery injury due to cervical spine displacement and a grade 4 left internal carotid artery injury with a carotid cavernous fistula (CCF). The patient was declared brain death as not improved cerebral perfusion after CCF coiling. CONCLUSIONS: Brain death due to cerebral hypoperfusion following cerebrovascular injury in this patient could be prevented by early endovascular intervention and cervical immobilisation.

Long-term outcome of endovascular treatment for indirect carotid-cavernous fistulas.

OBJECTIVE: Endovascular treatment (EVT) is the primary approach used to treat indirect carotid-cavernous fistulas (CCFs). In this study, the authors evaluated the immediate and long-term efficacy and safety of different endovascular techniques for indirect CCFs. METHODS: The databases of two endovascular centers were retrospectively reviewed to collect the patients with indirect CCFs treated using endovascular techniques between 2013 and 2023. Demographics, clinical presentation, CCF features, EVT characteristics, and clinical and radiological outcomes were evaluated and analyzed. The analysis was performed to compare the clinical and radiological data between different endovascular approaches and different embolic materials. RESULTS: Ninety-eight patients were included in the study. EVT was successful in 95 patients (96.9%). Immediate complete obliteration of the CCF was achieved in 93.9% of patients, with 98% undergoing embolization with liquid embolic agents (LEAs) and 95.6% undergoing coiling alone. Complete CCF obliteration was higher in the transvenous than in the transarterial approach (94.3% vs 75%, p = 0.010). At ≥ 6 months follow-up, complete CCF obliteration was achieved in all patients (100%). The rate of procedure-related complications was higher following LEAs than with coiling alone (32.0% vs 15.6%). New cranial nerve (CN) palsy was diagnosed in 26.0% and 2.2% after embolization with LEAs and coiling alone, respectively (p = 0.001), with complete CN palsy recovery in 78.6%. Procedure-related intracranial hemorrhage occurred in 3 patients (3.1%). Two patients experienced an ischemic stroke following Onyx migration into the internal carotid artery. Ocular symptoms improved in 93% (83/89) of the patients who were followed. CONCLUSIONS: In this study, complete obliteration of an indirect CCF was achieved in more than 90% of patients. Despite the occurrence of some new postprocedural ocular CN palsy, ocular symptoms improved in most patients in long-term follow-up. The transvenous approach was the most effective method for treating the indirect CCF. Coiling was safer than LEAs for the embolization of the indirect CCF.

Comparison of the transarterial, transvenous, and superior ophthalmic vein approaches in the treatment of indirect carotid-cavernous fistulas.

OBJECTIVE: Indirect carotid-cavernous fistulas (CCFs) are abnormal arteriovenous shunting lesions with a highly variable clinical presentation that depends on the drainage pattern. Based on venous drainage, treatment can be either transarterial (TA) or transvenous (TV). The aim of this study was to compare the outcomes of indirect CCF embolization via the TA, TV, and direct superior ophthalmic vein (SOV) approaches. METHODS: The authors conducted a retrospective analysis of 74 patients admitted to their institution from 2010 to 2023 with the diagnosis of 77 indirect CCFs as confirmed on digital subtraction angiography. RESULTS: A total of 74 patients with 77 indirect CCFs were included in this study. Embolization was performed via the TA approach in 4 cases, the TV approach in 50 cases, and the SOV in 23 cases. At the end of the procedure, complete occlusion was achieved in 76 (98.7%) cases. The rate of complete occlusion at the end of the procedure and at last radiological follow-up was significantly higher in the SOV and TV cohorts than in the TA cohort. The rate of recurrence was highest in the TA cohort (25% for TA vs 5.3% for TV vs 0% for SOV, p = 0.68). CONCLUSIONS: The rate of immediate complete occlusion was higher in the TV and SOV cohorts than in the TA cohort while the rate of complete occlusion at final follow-up was highest in the SOV cohort. The SOV approach was significantly associated with higher rates of postoperative complications. Indirect CCFs require careful examination of the fistulous point and the venous drainage to provide the most effective patient-tailored approach.

Carotid-cavernous fistula: A potential treatable cause of bilateral abducens palsy and conjunctival hyperemia.

OBJECTIVE: Carotid cavernous fistulas (CCFs) represent uncommon and anomalous communications between the carotid artery and the cavernous sinus. MATERIALS AND METHODS: Case report RESULTS: We present the clinical details and successful management of a previously healthy 44-year-old patient who presented with one-month worsening headache, bilateral abducens palsy and conjunctival injection. Imaging modalities including magnetic resonance imaging (MRI) with contrast and digital subtraction angiography (DSA) facilitated the diagnosis of CCF. The patient underwent endovascular coiling of the CCF, leading to neurological recovery and symptom remission. CONCLUSION: This case highlights the importance of promptly CCF diagnosis in patients with multiple cranial nerve palsies and conjunctival hyperemia. Moreover, it emphasizes the efficacy of endovascular coiling in achieving symptom remission.

Off-label use of large diameter Concerto fibered coils through a 0.017 inch microcatheter for transvenous embolization of indirect carotid-cavernous fistulas: two case reports.

BACKGROUND: A carotid-cavernous fistula is an abnormal communication between the arteries and veins within the cavernous sinus. While conservative management may be prudent in low risk cases, many patients require intervention and endovascular embolization has evolved as the preferred method of treatment. Embolization can be performed via either the transarterial or transvenous approach. One major challenge of the transvenous approach is the complex and variable compartmentation of the cavernous sinus, which often requires the use of low profile microcatheters to navigate and reach the fistulous point. Fibered coils are also preferred when performing transvenous embolization of carotid-cavernous fistula, as they are of higher thrombogenicity and allow for faster occlusion of the fistula. However, most low profile (0.017-inch) microcatheters are not able to deploy fibered coils based on the manufacturer's instructions. CASE PRESENTATION: We present two successful cases of off-label use of Medtronic Concerto fibered coils via a 0.017-inch microcatheter during transvenous embolization of carotid-cavernous fistula in a 60-year-old and an 80-year-old Chinese female, respectively. CONCLUSION: Our case series highlight the possibility of deploying large diameter (up to 10 mm) Concerto fibered coils through a low profile (0.017-inch) microcatheter in an off-label manner for transvenous embolization of indirect carotid-cavernous fistula.

[Carotid-cavernous fistulas]. / Sinus-cavernosus-Fisteln.

BACKGROUND: Carotid cavernous fistulas (CCFs) are rare but clinically significant vascular anomalies characterized by abnormal connections between the cavernous sinus and arteries. This overview presents a comprehensive analysis of anatomy, classification, clinical presentation, diagnosis, imaging, and therapy of CCFs. The cavernous sinus, a central venous structure in the brain, is of critical importance for understanding CCFs due to its proximity to key structures such as the internal carotid artery and cranial nerves. CLASSIFICATION: CCFs are classified into direct and dural types, with direct fistulas typically being high-flow and dural fistulas being low-flow. The symptomatology varies greatly and can range from noises in the head, diplopia, red eye, tearing, to blurred vision and headaches. The diagnostic assessment requires a combination of detailed medical history, neurological and ophthalmological examination, and the use of imaging techniques. METHODS: In imaging, computed tomography (CT) and magnetic resonance imaging (MRI) are crucial for depicting the anatomical structures and blood vessels, while digital subtraction angiography (DSA) is considered the gold standard for accurate representation of the fistula. The treatment of CCFs is complex and depends on the type of fistula, location, and clinical condition of the patient. CONCLUSION: This overview emphasizes the importance of precise diagnosis and individualized therapy to achieve optimal results and avoid complications. Ongoing developments in medical imaging and treatment techniques will continuously improve the treatment outcomes of patients with CCFs.

Post-traumatic carotid-cavernous fistula.

A 28-year-old woman who suffered a head injury 2 months before presented with a history of progressive right eye proptosis, ophthalmoplegia, and conjunctival injection. The radiological workup with a magnetic resonance imaging of the brain and cerebral angiography revealed a direct, high-flow, right post-traumatic carotid-cavernous fistula. An endovascular procedure was performed, and the carotid-cavernous fistula was successfully occluded with a progressive resolution of the complained symptoms.

Pressure-Lowering Effect of Interventional Fistula Occlusion in Secondary Glaucoma due to Orbital Draining Intracerebral Fistulae. / Drucksenkender Effekt der interventionellen Fistelokklusion bei Sekundärglaukom infolge orbital drainierender intrazerebraler Fisteln.

BACKGROUND: Carotid cavernous sinus fistulas (CSCF) are pathological connections of the internal and/or external carotid artery (and/or its branches) to the cavernous sinus. Ophthalmological symptoms and problems occur particularly when drainage is via the superior ophthalmic vein. MATERIAL AND METHODS: Seven eyes of six patients with a high-grade suspicion of CSCF were included in this retrospective monocentric study. Digital subtraction angiography (DSA) was performed in the included patients, where an interventional fistula closure was performed in the case of CSCF. Four of the six patients received a pre- and post-interventional day-night intraocular pressure profile. Furthermore, medical history, symptoms, visual acuity, slit lamp microscopic findings, and DSA findings were evaluated. RESULTS: The most common symptoms reported by patients were red eyes, diplopia, and exophthalmos. When the intraocular pressure (IOP) was measured, 83.33% of the patients showed increased values. The mean IOP in the day-night intraocular pressure profile in the affected eye before intervention was 23.5 (± 2.7) mmHg compared to 14.1 (± 2.3) mmHg in the healthy eye. A significant difference could thus be demonstrated in side comparison (p = 0.0047). The post-interventional measurement showed a mean IOP of 15.3 (± 1.0) mmHg in the affected eye and thus a significant difference to the pre-interventional measurement in the affected eye (p = 0.0018). Four of the six patients with CSCF were taking antiglaucomatous eye drops before the intervention, and two patients after the intervention. The number of antiglaucoma drugs used could also be reduced. CONCLUSION: Interventional fistula closure is an effective method for treating the secondary increase of IOP in CSCF. Successful closure of the fistula showed a significant reduction in IOP, which was not possible with the sole administration of antiglaucoma drugs. Radius-Maumenee syndrome should be considered as a differential diagnosis.

Vascular Ehlers-Danlos syndrome with a Novel missense COL3A1 gene mutation present with bilateral spontaneous carotid-cavernous fistula: a case report.

This report describes a unique case of vascular Ehlers-Danlos syndrome (vEDS) characterized by multiple spontaneous direct carotid-cavernous sinus fistulas (CCF). The patient initially presented with ocular symptoms and was effectively treated with transarterial coil embolization. Five years later, the patient developed recurrent contralateral CCF that required complex endovascular techniques. Genetic testing identified a novel mutation in the COL3A1 gene, confirming the diagnosis of vEDS. This case report provides a near-term perspective on the identification of structural abnormalities in the COL3A1 protein to ensure the safety of endovascular therapy for patients with vEDS.

The importance of the oral and maxillofacial surgery and traumatology team in the diagnosis of a cavernous carotid fistula: Case report.

Due to the anatomical proximity between the skull and the face, maxillofacial trauma can often cause injuries to cranial structures. Among these complications are carotid-cavernous fistulas (CCF), which are rare in maxillofacial trauma, although their etiology is usually traumatic. They are described as an atypical communication between the internal carotid artery and the cavernous sinus, which can generate a sudden change in the direction and distribution of blood flow between the brain and orbit. This paper aims to report a case of craniomaxillofacial trauma in which the patient evolved with diplopia, palpebral ptosis, and ophthalmoplegia of the left eye, diagnosed as traumatic CCF. The oral and maxillofacial surgery and traumatology team of the University Hospital of Western Paraná was observed these symptoms for the first time during post-operative follow-up after one day of surgery to repair mandibular fractures of the patient in question. With the identifying the signs and symptoms, a neurosurgery was requested for assessment and management. After clinical evaluation and imaging tests, they diagnosed the condition as CCF 5 days after the mandibular fractures and the patient was referred for treatment. Surgery was performed, in the same day, to resolve the CCF with the endovascularly by embolization with micromoles. The CCF was resolved and the patient continued on outpatient follow-up, progressing without sequelae from the CCF or mandibular fractures.

Carotid cavernous fistula masquerading as orbital inflammation on noninvasive imaging in a peripartum patient.

A carotid cavernous fistula (CCF) is a pathological connection between the internal or external carotid artery and the cavernous sinus venous system. Pregnancy has been proposed as a risk factor for spontaneous CCF, but the exact mechanism of risk is unknown. Overall, there are few published reports describing CCF in pregnancy. Study of this risk factor relationship is further complicated by potential ambiguity in diagnosing CCF, which may present as subtle, nonspecific findings on initial workup. We report a case of CCF during pregnancy in which the diagnostic process was notable for misleading, noninvasive, imaging studies.

Direct Transorbital Approach for Treatment of Carotid Cavernous Fistula: An Illustrative Case Series.

BACKGROUND AND OBJECTIVES: Endovascular techniques have become the first-line treatment for carotid-cavernous fistulas (CCFs). Direct transorbital venous access may be used if anatomic constraints limit standard transarterial or transvenous access. We describe our institutional experience with the transorbital approach for Barrow Type A-D CCFs. METHODS: Patients with CCFs undergoing transorbital endovascular treatment at our institution between 2017 and 2019 were retrospectively reviewed. Demographic, treatment, and outcome data were collected. RESULTS: Eight patients met inclusion criteria, 4 female and 4 male patients. The mean age was 43 years, with 6 right-sided CCF and 2 left-sided CCFs. Symptoms were present for an average of 1.5 months before treatment. All patients presented with eye pain and subjective visual changes. Seven (87.5%) patients presented with proptosis, 6 (75%) patients had elevated intraocular pressure (IOP), and 3 (37.5%) patients had ophthalmoplegia. Six CCFs (75%) were spontaneous, and 2 CCFs (25%) were traumatic. Barrow types were A (n = 1), B (n = 1), C (n = 1), and D (n = 5). All patients underwent direct percutaneous transorbital embolization with coils followed by Onyx. Three patients had undergone prior transarterial and/or transvenous treatment. A radiographic cure was obtained in all patients after direct transorbital embolization. After CCF cure, cranial nerve palsies resolved in 66.7% of patients, visual acuity in the affected eye was improved or stable in 75% of patients, and IOP had normalized in 85.7% of patients. Proptosis improved in all patients, with complete resolution in 75%. CONCLUSION: Direct transorbital embolization is a safe and potentially curative treatment for all 4 Barrow types of CCFs.

CAROTID CAVERNOUS SINUS FISTULA FINDINGS IN OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY BEFORE AND AFTER PERCUTANEOUS EMBOLIZATION.

BACKGROUND/PURPOSE: Optical coherence tomography angiography has been used to evaluate the posterior segment in a wide variety of pathologies because it is a noninvasive image technique, but its role in the evaluation of the retina in a case of carotid cavernous sinus fistula has not been described yet. METHODS RESULTS: We present a patient who consulted with spontaneous left superior eyelid hematoma and was diagnosed with left indirect carotid cavernous sinus fistula. In this study, optical coherence tomography angiography was used to evaluate the different macular capillary plexuses in a patient with carotid cavernous sinus fistula and a clinical situation of secondary local venous stasis before and after percutaneous embolization. Augmented vessel density was seen in superficial and deep capillary plexuses and in choriocapillaris before the percutaneous embolization, and a decrease of the parameters was seen after the treatment. CONCLUSION: All macular capillary plexuses presented with augmented vessel density levels that normalized after treatment. These findings were previously undescribed, and they suggest that optical coherence tomography angiography may be useful to initially evaluate patients with carotid cavernous sinus fistula who are planned to undergo embolization and to follow them up until normalization of the vascular structures is reported.

Patch Technique for Primary Treatment of Type A Carotid Cavernous Fistula: A Case Series and Technical Notes.

BACKGROUND: The utilization of flow diverters (FDs) in the treatment of high-flow Type A carotid cavernous fistulas (CCFs) has been described before mainly as an adjunct to the traditional endovascular techniques and rarely as a stand-alone treatment. In this study, we retrospectively evaluated our experience with FDs as the solo nonadjunctive treatment of Type A CCF with severe cortical venous reflux (CVR). METHODS: A retrospective review was performed of patients with Type A CCFs who were treated using FDs' patch technique (PT). Patients' demographics, clinical data, and preoperative and postoperative ocular examination were recorded. The procedure technique, pipeline embolization device (PED) diameters, and immediate and late procedure outcomes were described. RESULTS: Three patients were included in this case series. All patients had history of trauma and presented with decreased visual acuity, cranial nerve II deficit, limited extraocular muscles' movement, and increased intraocular pressure (IOP). Diagnostic angiography was performed, which confirmed high-flow Type A CCF. Endovascular treatment was performed through distal radial access in 2 patients and femoral access in 1 patient by deploying 4 sequentially enlarging PEDs with immediate resolution of the ocular symptoms. Follow-up angiography confirmed complete resolution of CCF in 2 patients. One patient was lost to follow-up; however, angiogram at 4 months demonstrated residual small CCF with significant improvement from postprocedure angiogram. CONCLUSIONS: The patch technique using sequentially enlarging FDs is a reasonable alternative solo technique for the treatment of direct CCF symptoms and results in immediate resolution of CVR while preserving the cavernous sinus anatomy.