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.

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.

Microvascular and neural alterations in carotid cavernous fistulas: An optical coherence tomography angiography study.

BACKGROUND: Current modalities for diagnosing carotid cavernous fistula (CCF) are inaccurate in analysing retinal microcirculations and nerve fibre changes. Retinal microvascular and neural alterations occur in CCF patients and can be quantitatively measured using optical coherence tomography angiography (OCTA). We measured the neurovascular changes in the eyes of CCF patients and used OCTA as a supplementary method. METHODS: This cross-sectional study studied 54 eyes of 27 unilateral CCF subjects and 54 eyes of 27 healthy age- and sex-matched controls. OCTA parameters in the macula and optic nerve head (ONH) were analysed using a one-way analysis of variance with further Bonferroni corrections. Parameters with statistical significance were included in a multivariable binary logistic regression analysis and receiver operating characteristic (ROC) curves were generated. RESULTS: There was significantly less deep-vessel density (DVD) and ONH-associated capillary density in both eyes of CCF patients than in controls, while the differences between the affected and contralateral eyes were insignificant. The retinal nerve fibre layer and ganglion cell complex thickness were lower in the affected eyes than in the contralateral or controlled eyes. ROC curves identified DVD and ONH-associated capillary density as significant parameters in both eyes of CCF patients. CONCLUSION: The retinal microvascular circulation was affected in both eyes of unilateral CCF patients. Microvascular alterations occurred before retinal neural damage. This quantitative study suggests a supplementary measurement for diagnosing CCF and detecting early neurovascular impairments.

Diagnostic clues of IOP pulsation on applanation tonometry in carotid-cavernous fistula patients.

BACKGROUND: Carotid-cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system and exhibits typical symptoms of red eye, diplopia, blurred vision, headache, and murmur. However, the symptoms for CCF may vary and can lead to misdiagnosis. IOP pulsations provide a hint leading to suspicion of CCF. We report three cases related to CCF differential diagnosis: two cases of CCF patients and one case of conjunctivitis with corkscrew conjunctival vessels. CASE PRESENTATION: The case 1 patient, with a typical unilateral CCF, exhibited significant IOP pulsation in Goldmann tonometry measurements in the affected eye. The case 2 patient did not show typical symptoms of CCF except asymmetric upper eyelid swelling (right > left). In clinical evaluation, IOP elevation in the right eye and IOP pulsation in both eyes were noted. Based on radiology, the patient was diagnosed with bilateral CCF. The case 3 patient was referred to our institution for differential diagnosis of CCF. The patient had corkscrew conjunctival vessels in both eyes, which had appeared after he had been revived through CPR (cardiopulmonary resuscitation) 25 years prior. IOP pulsation was not observed in Goldmann tonometry. Radiology test result for arterio-venous fistula was negative in the case 3 patient. CONCLUSION: For diagnosis of CCF, IOP pulsation by Goldmann applanation tonometry exhibits a good correlation with the disease in our cases and provides useful diagnostic clues.

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.

Carotid-cavernous fistula(CCF) presenting as paroxysmal painful ophthalmoplegia.

BACKGROUND: Painful ophthalmoplegia can be caused by various etiologies, and broad differential diagnosis is needed. Carotid-cavernous fistula (CCF) is a rare cause of painful ophthalmoplegia, and early diagnosis is quite difficult. CASE PRESENTATION: Here, we present a case of paroxysmal painful ophthalmoplegia caused by CCF. The episodic symptoms were nonstereotypical and lasted minutes to hours. Magnetic resonance imaging (MRI) and computed tomography angiography (CTA) results were normal, which confounded efforts to determine a diagnosis. Subsequently, digital subtraction angiography (DSA) revealed a posterior-draining CCF. The CCF was treated at an early stage without residual symptoms. CONCLUSIONS: We propose that symptoms could be relapsing or remitting during an early stage of CCF and that posterior-draining CCF is prone to misdiagnosis due to atypical manifestations. Normal CTA results cannot exclude carotid-cavernous fistula, and DSA should be performed once CCF is suspected.

Carotid-cavernous fistula after endovascular intervention for chronic carotid artery total occlusion.

BACKGROUND AND PURPOSE: In addition to head trauma and cranial surgery, endovascular intervention for chronic carotid artery occlusion (CAO) may also result in carotid-cavernous fistula (CCF). The management and prognosis of iatrogenic CCF during CAO recanalization have never been well described and discussed in the literature. MATERIALS AND METHODS: We conducted a retrospective analysis for CAO recanalization attempts in National Taiwan University Hospital and affiliated hospitals. Incidence and presentation, demographic and angiographic variables, and clinical follow-up of the development of iatrogenic CCF were carefully reviewed. RESULTS: A total of 138 consecutive de novo CAO endovascular recanalization attempts were reviewed. The technical success rate was 61.6% (85/138). Complication rate, including death, stroke, and intracranial or sub-arachnoid hemorrhage (ICH or SAH) was 4.3% (6/138). CCF developed in 11 patients (8.0%), and none resulted in death, stroke, or ICH/SAH within 30 days. Female gender and distal carotid artery reconstitution at communicating or ophthalmic segments were associated with development of CCF. Imaging follow-ups were performed in eight patients and none showed persistent CCF. CONCLUSION: CCF may develop during chronic CAO endovascular recanalization attempts. It is usually self-limited and can be managed conservatively.

Post-traumatic right carotid-cavernous fistula resulting in symptoms in the contralateral eye: a case report and literature review.

BACKGROUND: To report a case of a carotid-cavernous fistula (CCF) that occurred after a motor vehicle accident and review the uniqueness of this case and the main confusing points for the diagnosis of such cases. CASE PRESENTATION: A 22-year-old man complained of left eyelid swelling, eye redness, visual decrease and occasional headache after motor vehicle accident 4 months prior during which he experienced a head injury. He was initially thought to have glaucoma, but he was finally diagnosed with a right CCF based on magnetic resonance imaging (MRI) and digital subtraction angiography (DSA). Timely embolization surgery resulted in obvious relief of the ocular symptoms and an improved prognosis. CONCLUSION: This is the first reported case of a post-traumatic unilateral CCF with contralateral symptoms in direct CCF, it is very infrequent and deserves our attention. We should maintain high suspicion of CCF and confirm the diagnosis by DSA when managing such patients to prevent serious consequences. Early diagnosis and treatment can improve the prognosis of patients.

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.

[Treatment of a Traumatic Direct Carotid Cavernous Fistula with an Intradural Internal Carotid Artery Pseudoaneurysm Using a Low-profile Visualized Intraluminal Support(LVIS)Stent:A Case Report].

Traumatic carotid cavernous fistula(CCF)is known to present a direct connection between the cavernous segment of the internal carotid artery(ICA)and the cavernous sinus(CS). In rare cases, the fistula is formed between the intradural internal carotid artery(ICA)and the cavernous sinus(CS)via a pseudoaneurysm(pAN), requiring appropriate management and aggressive surgical treatment. We describe a 58-year-old man who sustained a severe head injury diagnosed as traumatic CCF treated with an intradural pAN procedure and transarterial coil embolization combined with a Low-profile Visualized Intraluminal Support(LVIS)stent. While slow arteriovenous shunt flow persisted at the end of the surgery, the fistula was completely occluded on the digital subtraction angiography obtained 2 weeks after the procedure. It was suspected that the flow-diversion effect of the LVIS stent might have caused the curable progression of the fistula occlusion.

Efficacy of Orbital Color Doppler Imaging and Neuroimaging in the Diagnosis of Carotid Cavernous Fistulas.

PURPOSE: To evaluate the diagnostic sensitivity and specificity of orbital color Doppler imaging (CDI) and conventional neuroimaging (CT/MRI) compared with cerebral angiography in patients with carotid-cavernous fistulas (CCFs). METHODS: The study design was a retrospective patient chart and imaging review. The authors reviewed 655 charts of all patients who underwent CDI and neuroimaging (CT/MRI) between 2006 and 2015 at one institution. Sixty patients had a presumptive diagnosis of CCF without thrombosis. Thirty-seven patients with 43 events met the inclusion criteria of the study. The diagnostic sensitivity of the 3 noninvasive imaging modalities (CDI, CT, MRI) for CCF was compared with the gold standard 6-vessel cerebral angiography. Significance testing was performed using the 2-tailed Fisher test. RESULTS: Color Doppler imaging had high sensitivity (96.8%) but low specificity (41.7%) for the diagnosis of CCFs with anterior orbital findings. A negative CDI had more diagnostic value than a positive CDI. While an arterial wave form in the superior ophthalmic vein was the most common finding of CCF on CDI, enlargement of the superior ophthalmic vein was the only statistically significant finding. Posterior cortical venous drainage was noted in about 10% of the patients with indirect (low-flow) fistulas, who presented with unilateral orbital signs and symptoms, a finding not previously reported in the literature. CONCLUSION: Color Doppler imaging is a useful noninvasive, radiation-free modality for diagnosis of CCF with anterior drainage, with higher sensitivity than CT or MRI, but equivalent specificity. A significant limitation of CDI is the lack of usefulness in diagnosing fistulas with posterior cortical venous drainage, which carry a risk of intracerebral hemorrhage and stroke. In this series, 10% of unilateral CCFs with anterior orbital signs and symptoms showed angiographic evidence of posterior cortical venous drainage.

Patient with a spontaneously evolving carotid cavernous fistula in the emergency department.

Background - Approximately 2% of patients admitted to the emergency department present with headache, which is often associated with vomiting, ocular pain, and earache. In rare cases, the presence of an abnormal communication between a cavernous sinus and the carotid arterial system that creates a carotid cavernous fistula is the main cause of these symptoms. Case presentation - A 32-year-old woman presented at the emergency department with unilateral headache associated with earache on the same side, and pulsating tinnitus. On examination, we observed unusual appearance of our patient (small stature, unusually visible skin, lobeless ears). In the first 5 hours of our observation no neurological symptoms had been present, but after a severe vomiting, exophthalmos, subconjunctival suffusion and moderate ptosis developed. First, regarding the initial general symptoms, otorhinolaryngologist assessed the patient, and did not find any abnormality. Further, we ordered computed tomography and consulted a neurologist. Despite of the negative results we continued the observation because her symptoms did not improve. After appearance of neurological symp-toms, carotid cavernous fistula was suspected. Magnetic resonance imaging and ophthalmologist consultation verified the diagnosis. For therapy, she was transferred to interventional neuroradiology. Because of the unusual appearance and carotic cavernous fistula, we ordered genetic examination. This indicated the presence of Ehlers-Danlos syndrome type IV in the background. The first major manifestation of the syndrome was observed at our department. Conclusions - Carotid cavernous fistula is an uncommon diagnosis in the emergency department; however, the early recognition of symptoms and early treatment can prevent further consequences of this potentially severe condition.