Transient fourth nerve palsy preceding cervical myelopathy due to carotid-cavernous fistula.

Carotid-cavernous fistulas (CCFs) are rare intracranial vascular malformations. Among the various classifications available, the most recently proposed highlights the strong correlation between venous drainage pattern and clinical presentation. We present the case of a woman in her 70s with a history of transient palsy of the fourth cranial nerve who presented with subacute cervical myelopathy, which was caused by a CCF with venous drainage into the peribulbar and perimedullary plexus.Given this atypical presentation of CCF and the diagnostic challenges it poses, we conducted a comprehensive PubMed search looking for CCFs presenting with cervical myelopathy and our results confirmed their rarity and allowed us to identify clinical elements that may help clinicians diagnose and manage this potentially treatable condition.

Clinical Presentation of Carotid-Cavernous Fistula and Outcomes of Endovascular Balloon Embolization

Objectives: To describe the clinical presentation of carotico-cavernous fistula (CCF) and outcomes of endovascular balloon embolization in a tertiary care center in a developing country. Materials and Methods: This retrospective interventional case series included 18 patients who underwent endovascular balloon embolization from 2019 to 2022 at Lahore General Hospital in Lahore, Pakistan. The analyzed data consisted of age, gender, cause and type of CCF, clinical presentation, diagnostic technique used, intervention, and the results of two-month follow-up. Patients with incomplete records and coil embolization were excluded. Digital subtraction angiography was done in all cases followed by endo-arterial balloon embolization. Procedures were carried out under general anesthesia via femoral artery approach. A single balloon was sufficient to close the fistula in all cases. Results: There were 18 patients who met the inclusion criteria. Sixteen patients had direct CCF, and the mean age of the patients was 27.2±12.6 years. The commonest cause of CCF was trauma, and the mean time of presentation after trauma was 7.89±7.19 months. The male-to-female ratio was 8:1. Preoperative visual acuity was worse than 6/60 in 8 patients, between 6/60 and 6/18 in 7 patients, and better than 6/18 in 3 patients. The mean intraocular pressure was 16.06±3.37 mmHg preoperatively and 14.83±3.49 mmHg postoperatively (p=0.005). Endovascular embolization was successful in 15 patients (83.3%). One patient developed epidural hematoma as a complication of the procedure, which was drained later. There was no mortality related with the procedure. Conclusion: Balloon embolization via the femoral artery is an efficient technique in direct as well as indirect CCF. It is safe and simple with very good results if performed in a timely manner.

Inferior Ophthalmic Vein-Dominant Dural Cavernous Fistula Embolization via Combined Orbitotomy and Direct Puncture of Inferior Ophthalmic Vein: A Case Report and Literature Review.

Dural carotid-cavernous fistulas (DCF) typically drain into the superior ophthalmic vein. Predominant involvement of the inferior ophthalmic vein (IOV) is rare, with only 4 documented cases in the literature. Here, the authors describe a case of a 51-year-old man who presented with acute left-sided proptosis, dysmotility, and vision loss and was found to have an IOV-dominant type D dural carotid-cavernous fistulas. The fistula could not be embolized by transfemoral endovascular access or orbitotomy alone and was ultimately managed with combined orbitotomy and direct IOV puncture. All previous reports of IOV-dominant dural carotid-cavernous fistulas in the literature were similarly inaccessible via the transfemoral approach. This case highlights the challenges of IOV cutdown and proposes an alternative management strategy. When IOV cutdown is precluded by the fragile, collapsed, or deep nature of the vessel, conversion to percutaneous IOV puncture may offer a safe and effective approach and mitigate the risks of direct puncture alone.

High-flow carotid cavernous fistula and 'white eye': A rare and easily misdiagnosed presentation - A case report and literature review.

The three classic symptoms of carotid cavernous fistula (CCF) are pulsating exophthalmos, bruit and conjunctival chemosis. Here, we present a clinical case of isolated abducens nerve palsy due to a high-flow CCF in an 84-year-old woman, without the typical congestive orbito-ocular features. It was a diagnostic challenge because, for patients older than 50 years with cardiovascular risk factors, ischaemic mononeuropathy is the most frequent aetiology. This case illustrates the least common type of CCF that can be easily misdiagnosed. Physicians should consider fistula as a possible diagnosis in a patient with isolated abducens nerve palsy even without the classic triad.

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.

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.

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.

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.

RARE PRESENTATION OF BILATERAL CAROTID-CAVERNOUS FISTULA AS SUPRACHOROIDAL HEMORRHAGE DURING CATARACT SURGERY.

OBJECTIVE: To report a rare case of suprachoroidal hemorrhage during phacoemulsification in a patient with bilateral carotid-cavernous fistula. METHOD: Case report. RESULT: A 76-year-old woman with underlying hypertension presented left eye poor vision due to an underlying dense cataract. Her initial preoperative assessment was uneventful, and she underwent phacoemulsification. During epinucleus removal, there was sudden, unexpected anterior chamber shallowing, resulting in posterior capsule rupture. While the surgeon extended the wound to facilitate epinucleus removal, there was a further decrease of red reflex, followed by hardening of the globe, indicating a suprachoroidal hemorrhage. The corneal wound was opposed swiftly without an intraocular lens. Further evaluation after that revealed the patient had a chronic headache for several years, and ocular examination showed bilateral esophoria. A computed tomography demonstrated features suggestive of bilateral carotid-cavernous fistula, which was confirmed with computed tomography angiography later. CONCLUSION: Patients with carotid-cavernous fistula have elevated episcleral venous pressure and vortex venous pressure. Sudden decompression of the globe in these patients predisposes them to higher suprachoroidal hemorrhage risk, although this condition is generally rare in phacoemulsification.

Hemicranial postural headache as a first symptom of a spontaneous carotid cavernous fistula: A case report.

RATIONALE: Spontaneous carotid cavernous fistula (CCF) is rare, and the expression of headache caused by it can be variable. PATIENT CONCERNS: A case of a man hospitalized for high-intensity hemicranial headache which was aggravated by lying down and relieved when standing or sitting. The pain was of a pulsating character, localized on the right, behind the eye, followed by nausea and vomiting. He gradually appeared with ophthalmoplegia, decreased visual acuity and epistaxis. DIAGNOSIS: Digital subtraction angiogram (DSA) showed a pseudoaneurysm arising from the internal carotid artery (ICA) that projected anteriorly and medially into the sphenoid sinus with occluded fistula. INTERVENTIONS: The pseudoaneurysm was successfully treated with covered stent. OUTCOMES: The patient was then followed up clinically at the outpatient and seen in the outpatient clinic with no further episodes of nasal bleeding or new neurologic deficit. The vision loss and ophthalmoparesis were unchanged. LESSONS: Hemicranial postural headache may be the first and characteristic sign of spontaneous CCF.