Hemicranial Cough-Induced Headache as a First Symptom of a Carotid-Cavernous Fistula-Case Report.

Background and objectives: Spontaneous carotid-cavernous fistulas (CCFs) are rare, and they may be caused by an aneurysm rupture. Materials and Methods: A case of a man hospitalized for high-intensity hemicranial headache with sudden cough onset as part of an upper respiratory tract infection is presented. The pain was of a pulsating character, localized on the right, behind the eye, followed by nausea and vomiting. Neurological finding registered a wider rima oculi to the right and slight neck rigidity. Laboratory findings detected a mild leukocytosis with neutrophil predominance, while cytobiochemical findings of CSF and a computerized tomography (CT) scan of the endocranium were normal. Results: Magnetic resonance imaging (MRI) angiography indicated the presence of a carotid cavernous fistula with a pseudoaneurysm to the right. Digital subtraction angiography (DSA) was performed to confirm the existence of the fistula. The planned artificial embolization was not performed because a complete occlusion of the fistula occurred during angiographic examination. Patient was discharged without subjective complaints and with normal neurological findings. Conclusions: Hemicranial cough-induced headache may be the first sign of carotid cavernous fistula, which was resolved by a spontaneous thrombosis in preparation for artificial embolization.

XCalibur aneurysm occlusion device for the treatment of direct carotid cavernous fistula: expansion of armamentarium.

We report the first case of a post-traumatic direct carotid cavernous fistula (CCF) treated with the XCalibur aneurysm occlusion device, which is a balloon mounted stent with flow diversion effect. Two devices were deployed across the fistula in an overlapping manner, resulting in complete occlusion of the fistula. Flow diversion with this device can provide a safe and alternative treatment option in direct CCF.

Pathophysiology of carotid-cavernous fistulas in vascular Ehlers-Danlos syndrome: a retrospective cohort and comprehensive review.

BACKGROUND: Vascular Ehlers-Danlos syndrome (vEDS) is a rare condition characterized by connective tissue fragility. Direct spontaneous carotid-cavernous fistula (sCCF) is reportedly pathognomonic of vEDS. We conducted this study to understand the possible mechanisms of occurrence of sCCF in this subset of patients. METHODS: We conducted a retrospective analysis of a monocentric vEDS cohort along with a literature review regarding sCCF in this condition. RESULTS: Of 133 patients regularly followed in our centre between 2000 and 2017, 13 (9.8%) had a diagnosis of direct sCCF (92.3% female, median age 33.0 years, interquartile range (IQR) [26.0-39.5]). There were 7 Glycine missense and 6 splice-site variants but no variant leading to haploinsufficiency. The literature search identified 97 vEDS patients with direct sCCF (79.4% female, 7.2% sex not reported, median age 31.0 years, IQR [24.0-39.0]). Increased carotid circumferential wall stress, higher carotid distensibility and lower carotid intima-media thickness could contribute to a higher risk for direct sCCF in vEDS. There is no predictive factor for the occurrence of sCCF apart from female sex in vEDS. CONCLUSIONS: In vEDS, anatomical and pathophysiological features of the intra-cavernous internal carotid artery make it prone to shunting in the cavernous sinus, due either to a spontaneous rupture or to a spontaneous dissection with pseudoaneurysm formation. Direct sCCF in seemingly healthy young individuals should be highly suggestive of vEDS and prompt further investigation.

Carotid-cavernous fistula: current concepts in aetiology, investigation, and management.

A carotid-cavernous fistula (CCF) is an abnormal communication between arteries and veins within the cavernous sinus and may be classified as either direct or dural. Direct CCFs are characterized by a direct connection between the internal carotid artery (ICA) and the cavernous sinus, whereas dural CCFs result from an indirect connection involving cavernous arterial branches and the cavernous sinus. Direct CCFs frequently are traumatic in origin and also may be caused by rupture of an ICA aneurysm within the cavernous sinus, Ehlers-Danlos syndrome type IV, or iatrogenic intervention. Causes of dural CCFs include hypertension, fibromuscular dysplasia, Ehlers-Danlos type IV, and dissection of the ICA. Evaluation of a suspected CCF often involves non-invasive imaging techniques, including standard tonometry, pneumotonometry, ultrasound, computed tomographic scanning and angiography, and/or magnetic resonance imaging and angiography, but the gold standard for classification and diagnosis remains digital subtraction angiography. When a direct CCF is confirmed, first-line treatment is endovascular intervention, which may be accomplished using detachable balloons, coils, liquid embolic agents, or a combination of these tools. As dural CCFs often resolve spontaneously, low-risk cases may be managed conservatively. When invasive treatment is warranted, endovascular intervention or stereotactic radiosurgery may be performed. Modern endovascular techniques offer the ability to successfully treat CCFs with a low morbidity and virtually no mortality.

Successful treatment of direct carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV without arterial puncture: the transvenous triple-overlay embolization (TAILOREd) technique.

We report successful transvenous treatment of direct carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV using a novel triple-overlay embolization (TAILOREd) technique without the need for arterial puncture, which is known to be highly risky in this patient group. The TAILOREd technique allowed for successful treatment using preoperative MR angiography as a three-dimensional overlay roadmap combined with cone beam CT and live fluoroscopy, precluding the need for an arterial puncture.

Bilateral carotid-cavernous fistula with spontaneous resolution: A case report and literature review.

RATIONALE: Bilateral carotid-cavernous fistula (CCF) is rare and serious extra-ocular disease occurring in clinical which may result in severe complication. Unique manifestations and imaging examinations are important to the diagnosis. PATIENT CONCERNS: A case of bilateral carotid-cavernous fistula in an 60-year-old healthy man caused by a head injury is reported. Further clinical symptoms and signs and imaging examinations lead to the correct diagnosis. DIAGNOSES: Computed tomography angiography of the brain aroused suspicion of bilateral CCF. On physical examination, intraocular pressure in the right eye was 35 mm Hg, while the other eye was 56 mm Hg. INTERVENTIONS: After diagnosis, the patient chose conservative treatment for some reasons. OUTCOMES: The symptom of him had relieved in both eyes but no light perception in the right eye after two months telephone follow-up. LESSONS: Our case study demonstrated that a highly suspicion must be maintained when managing such patients to prevent serious consequences. At the same time, the early diagnosis and treatment of the disease have a critical relationship to the prognosis of patients, which should be paid attention to.

Management of Iatrogenic Direct Carotid Cavernous Fistula Occurring During Endovascular Treatment of Stroke.

Traumatic carotid cavernous fistula may occur as a complication of endovascular treatment of acute stroke. We report 3 cases of such lesions. All patients were initially managed conservatively. Two patients have remained asymptomatic. One patient became symptomatic with right eye proptosis, chemosis, and right lateral gaze diplopia 3 weeks post thrombectomy. He underwent endovascular embolization via transfemoral transvenous approach via the inferior ophthalmic vein.

The enlarged extraocular muscle: to relax, reflect or refer?

PurposeExtraocular muscle enlargement (EOME) is most commonly associated with thyroid eye disease, but there are other causes. We report our outcomes of investigating and managing non-thyroid-related EOME (NTR-EOME).MethodsRetrospective consecutive case series. Sixteen patients identified by clinical features and orbital imaging. Patient demographics, radiological features, and adjuvant tests including biopsy and final diagnosis were recorded.ResultsMean age at presentation 59.3 years (range 24-89 years). Mean follow-up 3.2 years (range 3 months to 5.5 years). Superior rectus (SR) was most commonly involved muscle (8/16 cases) followed by lateral rectus (4/16). Of the 16 cases, 14 were associated with underlying systemic neoplasia (5 lymphoma, 5 metastatic carcinoma, and 4 presumed paraneoplastic syndrome). All SR enlargement was associated with underlying neoplasia. All patients underwent orbital imaging followed by systemic imaging based on clinical index of suspicion (14/16 patients (13 full body CT (FBCT), 1 mammography)). Positive systemic radiological findings were detected in 12/14 cases. Of the remaining 2 patients, 1 underwent full body positron emission tomography-computed tomography (FBPET-CT), which detected thyroid carcinoma, and the second patient underwent FBCT for staging following orbital biopsy showing lymphoma. Four patients (25%) died within 3 years of follow-up due to disseminated systemic malignancy.ConclusionsAll cases of NTR-EOME should be viewed with a high level of clinical suspicion for systemic neoplasia, especially when the SR is involved. FBCT can help to identify a primary systemic cause. FBPET-CT is best reserved for cases negative on FBCT or for staging and monitoring systemic disease. NTR-EOME can be associated with significant mortality (25%), hence warrants prompt and thorough systemic investigation.

Subfoveal choroidal thickness changes in carotid cavernous fistula following spontaneous resolution.

BACKGROUND: We report the enhanced depth imaging optical coherence tomography (EDI-OCT) characteristics and variations in a patient with subretinal fluid secondary to a carotid cavernous fistula. CASE PRESENTATION: A 59-year-old man presented with blurred vision in his right eye. Venous congestion of the epiescleral and retinal vessels were observed. EDI-OCT disclosed macular subretinal fluid with an increase of choroidal thickness up to 341 µm. Brain and orbital computerized tomography showed an enlarged right superior ophthalmic vein. Orbital magnetic resonance imaging and angiography disclosed a decrease in blood flow, an indirect sign of carotid cavernous fistula. After a 3 months follow-up, spontaneous closure of the fistula occurred. Both the dilation of the conjunctiva and retinal veins improved. EDI-OCT showed resolution of the subfoveal fluid and a reduction of the subfoveal choroidal thickness to 271 µm after a 3 months follow-up and 168 µm after a 8 months follow-up. CONCLUSION: Serous retinal detachment has been described as a rare complication of carotid cavernous fistula. In our patient, EDI-OCT examinations revealed a thicker choroidal thickness when subretinal fluid was present as compared to that observed in the contralateral eye or after subretinal fluid resolution.

Multidetector CT angiography in the diagnosis and classification of carotid-cavernous fistula.

AIM: To evaluate the performance of multidetector computed tomography angiography (MDCTA) in identifying and classifying carotid-cavernous fistulas (CCFs). MATERIALS AND METHODS: The neuro-interventional database was searched for patients with CCFs at four different institutions and for normal controls at one of the four institutions. Thirty-four patients were divided into the case group (direct type, n=8; indirect type, n=8) and the control group (n=18). Two readers retrospectively evaluated thin-section CTA images for the presence and laterality of four findings: (1) engorged ophthalmic vein, (2) engorged cavernous sinus, (3) similar enhancement of the cavernous sinus (CS) and internal carotid artery (ICA), and (4) greater enhancement of the CS than the transverse sinus (TS). Dehiscent ICA for the direct type was assessed only in the case group. Sensitivity, specificity, and inter-reader agreement were determined. Attenuation differences between the ICA and CS and between the CS and TS were compared across groups. RESULTS: The sensitivity/specificity for two engorgement and two enhancement findings were 81%/100%, 88%/94%, 100%/100%, and 88%/100%, respectively, for Reader 1, and 75%/100%, 75%/100%, 88%/100%, and 88%/100%, respectively, for Reader 2. Agreement between readers was excellent for all findings (κ>0.80). Dehiscent ICA identified the direct type with a sensitivity/specificity of 100%/75% for Reader 1 and 100%/88% for Reader 2 (κ=0.871). In the case group, the difference between ICA and CS attenuation values was significantly lower (180.1±76.6 versus 7.5±23.7; ⤳<0.001) and that between CS and TS were higher (-31.2±69 versus 102.6±59.3; ⤳<0.001). The area under the curve for the latter values was 0.95. CONCLUSION: The engorgement and enhancement categories in MDCTA may perform comparably in identifying CCFs and measurements of vessel attenuation differences may be of high diagnostic value. Dehiscent ICA can greatly aid in identifying the direct type.

Endovascular Modalities for the Treatment of Cavernous Sinus Arteriovenous Fistulas: A Single-Center Experience.

BACKGROUND: Cavernous sinus (CS) fistulas are classified into traumatic and spontaneous. Traumatic carotid-cavernous fistulas (CCFs) are usually direct internal carotid artery (ICA) high-flow fistulas; whereas spontaneous CCFs are usually dural, low-flow fistulas and generally possess less severe symptoms than direct carotid-cavernous fistulas. METHODS: This study involved 34 patients who were classified into 2 groups: Group A included 26 patients with direct carotid-cavernous fistula; and Group B included 8 patients with indirect dural cavernous fistula. All patients had ocular manifestations. One patient had subarachnoid hemorrhage. Coils were used alone in 19 cases of direct fistula and in 1 case of dural fistulas. Coils and Onyx (Covidien, Mansfield, MA, USA) were used in 7 cases of direct fistula and in 2 cases of dural fistulas. Onyx alone was used to treat 5 cases with dural fistulas but none of the cases with direct fistulas. Covered stents and coils were used in 2 cases of direct fistulas. RESULTS: All patients in both groups showed full recovery of their clinical signs and symptoms. Only 1 procedure-related complication was observed (3%) in which a patient had an embolic event and trigeminal dysesthesia as a result of Onyx reflux through external carotid artery-ICA anastomosis. CONCLUSION: Coils are superior solid embolic agents used for the treatment of direct high-flow fistulas, while Onyx is more valuable in dural low-flow CCF. Onyx shortens the procedure time and decreases procedure cost. Onyx injection inside the CS proper through the transarterial or transvenous route may be safer than Onyx injected inside dural arteries supplying the CS. However, more cases are needed to determine this.

Feasibility of Noninvasive Diagnosis and Treatment Planning in a Case Series with Carotid-Cavernous Fistula using High-Resolution Time-Resolved MR-Angiography with Stochastic Trajectories (TWIST) and Extended Parallel Acquisition Technique (ePAT 6) at 3 T.

PURPOSE: The evaluation of carotid-cavernous fistulas (CCFs) and the intracranial vasculature has been predominantly carried out using conventional digital subtraction angiography (DSA). Recent developments in time-resolved magnetic resonance angiography (MRA) provide the opportunity to assess both multiple arterial and venous phases with high temporal and spatial resolution. Here, we investigated the feasibility of this technique to functionally assess CCF prior to intervention. METHODS: Six consecutive patients with clinical symptoms of a CCF were scheduled for clinically indicated MRA and underwent a protocol that comprised conventional imaging sequences and high resolution time-resolved MRA with interleaved stochastic trajectories (TWIST). The location of the fistulous communication, the flow pattern, and venous drainage were determined by time-resolved MRA and compared with DSA which was available in five out of six patients. RESULTS: Typical morphological findings (including enlargement of the superior ophthalmic vein, exophthalmos) were found in all cases in both conventional MRI and time-resolved MRA source data. The temporal resolution of time-resolved MRA enabled a good separation of the early filling of the cavernous sinus during the arterial phase. Direct fistulous communication was assessed in three patients with good correlation to DSA, whereas indirect CCF could not definitely be visualized. The time-resolved MRA provided information about the flow pattern and the venous drainage of the fistula in all patients, which is essential for therapy planning. CONCLUSION: Time-resolved MRA provides important morphological and functional information in patients with CCF. Although DSA remains the gold standard for diagnosis and exact classification of fistulas, time-resolved MRA can provide the relevant hemodynamic information to plan interventional treatment as a one-step procedure with a focused diagnostic workup.

Imaging anatomy and pathology of extraocular muscles in adults.

The extraocular muscles (EOM) are involved in a variety of disease processes with characteristic findings on imaging. EOM anatomy is described, followed by a review of adult EOM pathology. The imaging characteristics are explained with examples. The pattern of EOM disease on imaging, in corroboration with clinical findings, can often lead the radiologist towards a specific diagnosis.

An unusual cause of peroneal neuropathy.

We discuss the case of a teenage girl who presented with neurologic symptoms suggestive of a peripheral neuropathy, before the development of a central arteriovenous fistula. Electromyography and nerve conduction studies indicated peroneal motor neuropathy, but her comprehensive genetic study results were negative for common Charcot-Marie-Tooth mutations. After 2 years of stable symptoms, she presented with unilateral throbbing headache and tinnitus. Magnetic resonance angiography revealed a carotid cavernous fistula, which was confirmed with conventional angiography. A successful coil embolization of the fistula was performed. Whole exome sequencing demonstrated a de novo heterozygous c.3158G>A (p.G1056D) mutation in the COL31A gene, consistent with Ehlers-Danlos type IV. To our knowledge, this is the first reported case of isolated peroneal motor neuropathy in a patient with Ehlers-Danlos type IV. This case highlights the utility of whole exome sequencing in the diagnosis of patients with neurologic symptoms that do not fit a clear phenotype.