Cavernous-Carotid Fistula Presenting with Intracerebral Hemorrhage in the Absence of Ocular Symptoms.

BACKGROUND: Cavernous-carotid fistulas (CCFs) can present with a variety of symptoms depending on the anatomy of the fistula and its venous drainage. Patients most commonly present with scleral injection, pulsatile exophthalmos, and/or chemosis. CASE DESCRIPTION: We report a patient who presented with intraparenchymal hemorrhage in the absence of any of the commonly associated ocular symptoms and signs. After multiple imaging studies, the CCF was diagnosed and treated with endovascular embolization that resulted in complete occlusion of the fistula and reflux of embolysate into one of its connecting veins. CONCLUSIONS: The morphology of the venous drainage can lead to atypical hemorrhagic presentation, whereas dilatation of one of the tributary veins with cortical venous reflux should warn the interventionist the path the embolysate may follow. We provide our experience with this unique presentation and its treatment.

Concomitant occurrence of clinoid and cavernous segment aneurysms complicated with carotid cavernous fistula: A case report.

RATIONALE: Dual aneurysms arising from the internal cerotic artery (ICA) is a very rare occurrence. Clinoid segment aneurysms (CSAs) are often seen at the carotid dural rings while cavernous carotid aneurysms (CCAs) are often a direct communication between the ICA and the cavernous sinus (CS). We present a case of complex concomitant occurrence of a CSA and a CCA complicated with delay aneurysmal rupture (DAR) resulting in carotid cavernous fistula (CCF) after our initial treatment of the patient with pipeline embolization devices (PLEDs) PATIENT CONCERNS:: We present a 64-year old female who we admitted at our institution due to one-year history of double vision. Neurological examinations were unremarkable. DIAGNOSIS: Magnetic resonance imaging (MRI) and computer tomography (CT)-scan revealed dual aneurysms on the ICA. Digital subtracting angiogram (DSA) confirmed a small CSA and a large CCA on the right ICA. INTERVENTIONS: We treated both aneurysms with PLED and subsequently observed DAR of CCA as a complication. OUTCOMES: We successfully occluded the fistula with ONYX (ev3, Irvine, CA) via the trans-venous approach. LESIONS: PLED was the best endovascular treatment option though DAR was inevitable. Although the trans-arterial approach may be the gold standard for the managing of CCF, the complex nature of our case made us opt for trans-venous approach. The trans-venous route is very appropriate for fistulas with complex parent arteries.

Intraocular Pressure in the Eyes of Patients With Carotid-Cavernous Fistulas: Profile, Intereye Asymmetry, and Treatment Outcomes.

PRECIS: Secondary ocular hypertension (OHT) is common in carotid-cavernous fistulas (CCFs). Management of elevated intraocular pressure (IOP) is possible with a multidisciplinary approach. The ipsilateral normal eyes may have higher IOP than the contralateral eyes. PURPOSE: To study the IOP profile of the eyes of patients with a CCF, treatment outcomes for elevated IOP, and intereye IOP asymmetry in the eyes with normal IOP. METHODS: This was a retrospective case series. A total of 64 eyes of 60 patients with digital subtraction angiography-proven CCF diagnosed from the year 2000 to 2016 were included. The demographics, clinical features, management, and outcomes were recorded. The primary outcome included understanding of the cause of elevated IOP. The secondary outcomes included comparison of the IOP between contralateral eyes and ipsilateral normal eyes (IOP <21 mm Hg) and management outcomes for elevated IOP. RESULTS: The mean age of the patients was 45.6±18.2 years. In the study population, 70% of the patients were males. Indirect CCF was present in 55% of the eyes. It was found that 64.06% (n=41) of the eyes had elevated IOP, glaucoma, or were glaucoma suspects. Among all the eyes, 40.62% (n=26) of the eyes had secondary OHT due to elevated episcleral venous pressure, whereas 7.81% (n=5) of the eyes had secondary open-angle glaucoma. The mean IOP was higher in the ipsilateral eyes than in the other eyes (22.95±7.1vs. 15.11±2.99 mm Hg; P<0.001). The mean IOP in the ipsilateral normal eyes was higher than that in the contralateral eyes, with a mean difference of 2.92±2.29 mm Hg (confidence interval of the mean difference: 1.90-3.94 mm Hg; P<0.0001). IOP reduction (<21 mm Hg) was achieved in 70.7% of the patients following CCF management with intermittent carotid massage, endovascular treatment, IOP-lowering medications, or a combination among these. CONCLUSIONS: Secondary OHT due to elevated episcleral venous pressure was more common than secondary open-angle glaucoma. Ipsilateral normal eyes had higher IOP than contralateral eyes. IOP-lowering agents and management of CCF resulted in IOP control in most patients.

Carotid Cavernous Fistulas and Dural Arteriovenous Fistulas of the Cavernous Sinus: Validation of a New Classification According to Venous Drainage.

BACKGROUND: Multiple carotid cavernous fistula (CCF) classifications have been proposed. However, they lacked predictive factors for the clinical presentation, natural history, and hemorrhagic risk. Our aim was to externally validate a new classification according to venous drainage (i.e., the Thomas classification [TC]) to assess its relationship with symptoms, endovascular treatment, and outcomes. METHODS: We performed a multicenter retrospective review of CCFs at 2 major academic institutions. The CCFs were classified using the Barrow classification (BC) and TC systems. RESULTS: The data from 94 patients with a diagnosis of CCF were collected during a study period 23 years, 4 months. Of these 94 patients, 89 had undergone CCF treatment and 5 had experienced spontaneous occlusion. Complete occlusion was achieved in 89.9% of the treated patients. Complications occurred in 5.3% of the patients, including permanent deficits in 2.1%. TC type 4 was associated with cortical symptoms compared with type 2 (P = 0.003) and type 3 (P < 0.001). The BC was not able to detect significant differences among the symptom types. Significant differences were found using the TC for the transarterial-only, transvenous anterior-only, and transvenous posterior-only approaches (P < 0.001, P = 0.03, and P = 0.001, respectively). The transvenous posterior and transvenous anterior approach were significantly associated with type 2 and 3 TC, respectively. Excluding direct CCFs, the BC was not related to the treatment approach. No significant differences in the outcomes were found. However, a trend toward a lower occlusion rate for TC type 4 compared with type 3 was observed. CONCLUSION: The TC provided useful information regarding the fistula anatomy and venous hemodynamics, which correlated with the clinical symptoms and treatment strategy.

Post-traumatic carotid-cavernous fistula with bilateral proptosis simulating cavernous sinus thrombosis.

A 48-year-old woman presented with bilateral non-pulsatile proptosis and ophthalmoplegia after 3 days following blunt orbital trauma. It was associated with fever, malaise and loss of vision in right eye. She was provisionally diagnosed with cavernous sinus thrombosis and was treated with intravenous antibiotics with no improvement. A subtle bruit was present on examination, and digital subtraction angiography revealed a right direct (type A) carotid-cavernous fistula (CCF). The patient underwent right coil embolisation of direct CCF. On follow-up at 4 months, her proptosis resolved completely and extraocular movements improved.

Endovascular treatment of carotid-cavernous sinus fistulas: ophthalmic and visual outcomes.

Purpose: The main objective was to report the epidemiology, clinical manifestations, angiography features, treatment modality and post-treatment outcomes in patients diagnosed with carotid cavernous fistulas (CCF). Methods: A retrospective review of the medical imaging database in conjunction with medical records from 2004 to 2017 at the Royal Brisbane and Women's Hospital (RBWH) was conducted.We identified 39 patients with CCF (16 direct, 23 indirect). A total of 37 diagnoses were confirmed by direct catheter angiography. The remaining two cases were diagnosed using magnetic resonance imaging/magnetic resonance angiography. Results: Coils were deployed in 100% of direct and 83% of treated indirect fistulas that were treated. Other embolic agents were deployed alone or in combination with coils. Successful angiographic closure was achieved in 93% of direct and 92% of indirect fistulas. Multiple treatments were required in 33% of direct and 16% of indirect fistulas. Visual acuity improved in patients with direct fistulae(p = 0.02) and was preserved in those with indirect fistulae. Post-treatment diplopia persisted in six patients with direct fistulas and three patients with indirect fistulas. Four patients with indirect fistulas experienced persistent ocular hypertension post-treatment compared to two patients with direct fistulas. Conclusions: Endovascular coils are the most commonly deployed treatment for CCF. Both indirect and direct fistulas achieved high rates of closure; however, indirect fistulas were less likely to require multiple treatments. Good post-procedural vision was achieved for both groups.

A red eye induced by a spontaneous carotid cavernous fistula.

A 73-year-old woman initially presented to our ophthalmology department with complaint of a red eye with gradual proptosis and mild restricted movement of the right eye who was misdiagnosed with conjunctivitis during 2 months. One week later she returned to the emergency department with a painful, swollen right eye and a high intraocular pressure of 41 mm Hg and limitation of her extraocular muscles in nearly all fields of gaze. As her IOP was high, she was diagnosed as having secondary glaucoma and was prescribed timolol 0.5% (twice a day) and Latanoprost eye drop at night in the right eye. During follow-up, her IOP remained in high level despite maximum medications. Computed tomography scan of the orbit showed an enlarged superior ophthalmic vein (SOV). In view of her persistent elevated IOP despite maximum medications and dilated conjunctival veins, she was advised digital subtraction angiography (DSA) and imaging revealed a carotid cavernous fistula (CCF). The purpose of this article is to describe ophthalmological complaints of CCFs to help ophthalmologist in understanding the clinical features of spontaneous CCF and avoid misdiagnosis.

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.

Transvenous Coil Embolization for the Treatment of Carotid Cavernous Fistula after Pipeline Placement: A Case Report.

The Pipeline embolization device (PED), a type of flow diverter, has become an appealing alternative treatment option for large or giant and wide-necked intracranial aneurysms. Carotid cavernous fistula (CCF) resulting from delayed aneurysmal rupture is a rare complication of PED placement with unknown pathophysiology. Here, we describe a case of CCF resulting from aneurysmal rupture following PED placement, and present the details of treatment by transvenous coil embolization. An 81-year-old woman was referred to our hospital for treatment of an 18.0 × 10.3 mm intracranial aneurysm located in the cavernous segment of the left internal carotid artery, noted during an examination of her diplopia. Endovascular treatment was conducted by positioning a single PED (4.75 × 25 mm) across the neck of the aneurysm. The postoperative course was uneventful, and the patient was discharged 8 days post procedure. On post-procedure day 10, her left oculomotor nerve palsy had worsened and she had developed left abducens nerve palsy, left exophthalmos, and left chemosis. Angiography demonstrated left direct CCF because of rupture of the aneurysm that had been treated with PED. Transvenous coil embolization was performed on post-procedure day 11 to treat the CCF, and complete resolution of the CCF and significant thrombus formation within the aneurysm sac were confirmed 11 days after the second procedure. Our angiographic results suggest that the aneurysmal rupture was caused by aneurysmal volume expansion associated with PED-induced thrombosis. Transvenous coil embolization for the treatment of CCF following PED placement constitutes a new challenge.

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.

An anatomical study of the pathophysiology of carotid cavernous sinus fistula associated with Le Fort III osteotomy.

UNLABELLED: Carotid-cavernous sinus fistula (CCF) is a rare complication occurring after the Le Fort osteotomy. We aimed to elucidate the cause of CCF in the Le Fort osteotomy. METHODS: Eleven fresh cadavers were used. After craniotomy, a Le Fort III bipartition osteotomy was then performed. On the left side, both the lateral wall of the maxilla and the pterygomaxillary junction (PMJ) were divided, in addition to the conventional osteotomy line. On the right side, those parts were kept intact. After the osteotomy, a tensiometer was fixed to the skull base. The sensor was linked with the wall of the carotid artery in the cavernous portion. A down-fracture was then performed initially from the left, followed by the right. Tensile force data of both sides were recorded. RESULTS: In all cases, a higher tensile load was observed on the right side compared to the left side. In right side, two skull base fractures complexed with high pterygoid process fractures, and nine pure high fractures of the pterygoid process were identified. CONCLUSIONS: During down-fracture, the wall of the carotid artery experiences significant tensile load. The tensile load on the arterial wall may collapse the fine branches of the carotid artery in the cavernous portion.

4D flow preliminary investigation of a direct carotid cavernous fistula due to a ruptured intracavernous aneurysm.

Inadequate information is available about the cerebral blood flow and surgical strategies of a direct aneurysmal carotid cavernous fistula (daCCF). We report a quantitative analysis of flow velocity and volume using preoperative time-resolved phase-contrast MRI (four-dimensional (4D) flow MRI) in a daCCF. This is the first report of 4D flow findings with a daCCF. A 55-year-old woman developed a sudden headache and bruit of the right orbit, and MRI suggested the presence of a daCCF. Quantitative analysis using preoperative 4D flow MRI revealed the flow volume of the right internal carotid artery. The daCCF was successfully treated by high-flow bypass using a radial artery graft and internal carotid artery trapping. Postoperative angiography showed a complete obliteration of the daCCF. Studies to collect data from additional cases are required so that 4D flow findings can be further used in the management of daCCFs.

Intrafistula pressure measurement in traumatic carotid cavernous fistulas--key to increasing safety and effectiveness of endovascular coiling.

BACKGROUND: Very few diseases demostrate the tremendous variation and capricious results common with the clinical ensemble of carotid cavernous fistulas (CCFs). The treatment modality for CCFs has changed from surgical to endovascular therapy. Many options exist in endovascular therapy ranging from balloons to coils and liquid embolization agents like Onyx. This study was undertaken to assess the role of recording intra-fistula pressure (IFP) during endovascular coiling of CCFs in order to help us better understand the angiodynamics of the fistula and to make coiling safer and effective. METHODS: IFP measurement was done in 15 cases of traumatic CCF undergoing endovascular coiling. Patients were prospectively analyzed by pre- and post- procedural clinical profile, degree of recovery and time until fistula occlusion. Univariate analysis was used to find the correlation between the reduction in IFP, the degree of fistula obliteration and time until occlusion. RESULTS: Of the 15 patients who underwent endovascular coiling 13 had total occlusion of the fistula, 1 patient had subtotal occlusion and 1 patient had no occlusion of the fistula. There was 100 % internal carotid artery (ICA) patency. Univariate analysis showed a strong correlation between the degree of reduction in IFP and time required for fistula occlusion (p < 0.001). Patients with a significant drop in IFP were also preceived as having a higher chance of fistula occlusion after waiting 30 min. CONCLUSION: To our knowledge this is the largest series in published literature focused on using only detachable coils as the first line embolizing agent in treatment of traumatic CCFs. We conclude that IFP monitoring will play an important role in making treatment of CCFs safer and more effective. The aim of coiling the fistula is not to tightly pack the fistula as during treatment of aneurysms; rather it is to reduce flow across the fistula leading to thrombosis thus reducing the requirement of coils.

Clúster-tic como comienzo clínico de una fístula dural carótido cavernosa / Cluster-tic syndrome as the first clinical manifestation of a dural carotid-cavernous fistula

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Balloon test occlusion of the internal carotid artery with stump pressure ratio and venous phase delay technique.

Balloon test occlusion (BTO) is crucial before sacrificing parent arteries. We proposed a simple paradigm combining clinical tolerance with venous phase technique and stump pressure ratio as a criterion for sufficient collateral flow. Internal carotid artery (ICA) occlusion was considered safe for asymptomatic patients who exhibited less than 2 seconds of venous phase delay or had a stump pressure ratio greater than 60%. A total of 37 BTO procedures were performed on 31 patients. Twenty-three patients were monitored clinically and 3 were symptomatic. Venous phase comparison was performed on 27 patients, and 5 failed the test. The stump pressure was measured in all patients, and 7 patients failed the test. In summary, 7 patients failed the BTO, of which 6 received high-flow bypass and 1 of these 6 were symptomatic and exhibited stump pressure ratios less than 60% in the second BTO procedure. Occlusion of the ICA was performed on 29 patients. Only 1 patient developed delayed vasospasm and brain infarction. Adequate collateral flow may be indicated by a stump ratio of 60% or greater with or without a high-flow bypass. Combined with clinical assessment and venous phase technique, ICA occlusion may be a safe option that does not result in delayed ischemic complications.

Lymphoblastic leukaemia presenting as a carotid-cavernous fistula.

A carotid-cavernous fistula is a life-threatening condition characterised by an abnormal communication between the carotid arterial vessels and the cavernous venous system. Although these fistulae can arise spontaneously, they mainly occur after trauma, especially road traffic accidents, falls and penetrating cranial or orbital injuries. The mainstay of treatment involves endovascular embolization, but in those patients where this is not possible or where embolization fails, direct surgical intervention and ligation of the artery may be necessary. Here we describe an interesting case of a suspected carotid-cavernous fistula which turned out to be cavernous sinus syndrome secondary to lymphoma.

Spontaneous carotid cavernous fistula complicating pregnancy.

Carotid cavernous fistula is a well-documented but rare condition in pregnancy, about which there are a limited number of reports in the literature. We report such a case in a 41-year-old woman presenting with right-sided headache, proptosis, and diplopia at 37 weeks of gestation. She was subsequently diagnosed to have carotid cavernous fistula based on angiography. Embolisation was performed in the postpartum period. Carotid cavernous fistula has the potential of serious morbidity including visual loss and intracranial haemorrhage. It can be treated effectively by endovascular embolisation, which confers a good prognosis. Although headache is a common complaint during pregnancy, obstetrician should be aware of this condition if the clinical presentation is suspicious.

[Unusual presentation of a carotid-cavernous fistula in Ehlers-Danlos type IV].

Carotid-cavernous fistulae may present clinically in different ways, depending on the diameter and flow of the artery involved. In a direct fistula between the internal carotid artery and the cavernous sinus (high - flow fistula) the clinical presentation is usualLy dramatic and the signs and symptoms are severe. Indirect fistulae, in contrast, result from the connection of branches of the internal carotid, external carotid, or both to the cavernous sinus (low - flow fistula) and often present in a moderate and/or indolent manner. Patients with Ehlers-Danlos type IV suffer from vascular wall fragility resulting in a high frequency of aneurysms, dissections and fistulae. We describe a patient with Ehlers-Danlos vascular type IV who presented with mild signs and symptoms, suggesting the existence of a low-flow fistula. After two weeks, the patient's condition rapidly deteriorated. Endovascular catheterization demonstrated direct carotid cavernous fistula which was successfully treated. This case demonstrates the importance of early consultation with an endo-vascular surgeon in every patient with Ehlers-Danlos suspected of harboring a carotid cavernous fistula.