Bilateral Carotid-cavernous Fistulas Research Articles

Rare Presentation of Bilateral Carotid Cavernous Fistula as Suprachoroidal Hemorrhage during Cataract Surgery.

To report a rare case of suprachoroidal haemorrhage (SCH) duringphacoemulsification in a patient with bilateral carotid-cavernous fistula (CCF). Case report. A 76-year-old female 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 an SCH. The corneal wound was opposed swiftly without an intraocular lens. Further evaluation after that revealed patient had a chronic headache for several years, and ocular examination showed bilateral esophoria. A computed tomography (CT) demonstrated features suggestive of bilateral CCF, which was confirmed with CT angiography later. Patients with CCF have elevated episcleral venous pressure and vortex venous pressure. Sudden decompression of the globe in these patients predisposes them to higher SCH risk, although this condition is generally rare in phacoemulsification.

Headache, vision changes and cranial nerve palsy after head trauma.

A 43-year-old female was brought to the hospital after a ground level fall and her Glasgow Coma Scale was 15. Non-contrast head computed tomography (CT) scan showed small bilateral subdural hematomas, left traumatic subarachnoid hemorrhage, left occipital bone and left longitudinal temporal bone fractures. She was managed conservatively. Interval repeat non-contrast head CT scans showed resolution of the hemorrhage. Two and a half months later she presented to the hospital with headaches, intermittent diplopia, blurry vision and a right lateral gaze palsy. Non-contrast head CT scan showed mild hyperattenuation in the cavernous sinus. Subsequently, a CT angiogram of the head was obtained that showed opacification of the bilateral cavernous sinus and distention of the orbital veins (Figure 1A,B). Evaluation with diagnostic cerebral angiogram showed direct bilateral carotid-cavernous fistulas (CCFs) (Figures 2 and 3). She underwent staged embolization of the CCFs. Direct CCF is the abnormal connection between the internal carotid artery and the cavernous sinus.1, 2 CCFs can present with the classic triad of chemosis, pulsatile proptosis and ocular bruit.3, 4 Other symptoms include orbital pain, abducens nerve palsy, deterioration of visual acuity and ophthalmoplegia.4, 5 Causes of CCF formation are traumatic, iatrogenic, connective tissue disorders and spontaneous.2, 6-9 Traumatic CCFs formation occurs in up to 1.25% of patients with traumatic brain injury or facial and skull base fractures.10, 11 Bilateral CCF formation is rare after trauma and symptoms develop in a delayed fashion.10-15 Treatment options include conservative management, open surgery, endovascular procedures and radiosurgery.16-18

Bilateral carotid-cavernous fistula after trauma presenting as hemorrhagic shock: Case report.

Bilateral carotid-cavernous fistula (CCF) is a rare complication associated with severe head injury and skull base fractures. Initial presentation with hemodynamically relevant epistaxis is unusual. We report a case of a 27-year-old male presenting with severe craniocerebral injury associated with massive epistaxis. To stabilize the patient hemodynamically, the bleeding was stopped by embolization of left internal carotid artery with coils, after checking that the Willis circle is well compensated. The left CCF was embolized later with flow diverter stent when it was safe to use platelet aggregation inhibitors. Reporting this case enlighten the management of bilateral CCF with hemorrhagic shock.

Gamma Knife radiosurgery for spontaneous carotid-cavernous fistulas: a preliminary report

Objective: Gamma Knife radiosurgery (GKRS) is used as an adjuvant treatment for carotid-cavernous fistulas (CCFs), and many previous reports have concluded that GKRS is a useful additional modality. We performed GKRS on CCFs without any endovascular treatment and present the preliminary results.Methods: Between December 2017 and December 2020, we performed GKRS in nine patients. Seven patients were female, and two patients were male (median age, 73 years; range, 46 to 86 years). Five fistulas were Barrow type B (one patient had bilateral CCF). Four fistulas were type C. One was type D. GKRS was performed with a marginal dose of 16 to 18 Gy (median, 17.5 Gy; 50% isodose line), and the mean targeted volume was 0.39 cm3 (range, 0.13 to 3.05 cm3). Results: All fistulas responded favorably to GKRS, with symptom improvement after 1 to 32 weeks (median, 3 weeks). The median follow-up period was 15.5 months (range, 12 to 43 months). Seven fistulas disappeared following treatment, which was confirmed by transfemoral angiography or orbit magnetic resonance imaging and brain magnetic resonance angiography (MRA). Three fistulas in the diameter of the superior ophthalmic vein decreased by more than 50%, as indicated by MRA. There were no cases of recurrence or adverse effects.Conclusion: Although further research requires a larger patient group and longer follow-up duration, GKRS can be considered a definitive treatment modality for any type of CCF, not only an adjuvant modality.

A rare case of nontraumatic bilateral carotid cavernous fistula Barrow type D – A case report

<br>Carotid cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the normal carotid arterial system. We are presenting a very rare case of spontaneous nontraumatic bilateral Barrow type D CCF. A 41-year-old patient presented to our tertiary eye care center with a history of long-standing bilateral congested eyes. On imaging, the patient was diagnosed with spontaneous bilateral Barrow type D CCF. The patient improved significantly by treatment with carotid decompression exercises. It is recommended to consider the diagnosis of bilateral CCFs as a differential diagnosis in patients with long-standing history of congested eyes nonresponsive to conventional topical treatment.<br>

Bilateral carotid cavernous fistula after trauma: a case report and literature review

BackgroundCarotid cavernous fistula is a rare complication that is typically associated with head trauma and skull base fractures. The traumatic bilateral carotid cavernous fistula are significantly rarer.Case presentationsWe report a case of a 61-year-old man presenting with unilateral exophthalmos, swollen eyelids, conjunctival congestion, and edema etiologically associated with severe trauma. Thereafter, the patient demonstrated symptoms of contralateral oculomotor nerve injury caused by skull base fracture, such as ptosis of eyelid, dilated pupils, and eye movement disorder, and was diagnosed with bilateral carotid cavernous fistula.ConclusionsThe patient recovered after undergoing endovascular embolization of bilateral cavernous sinus fistulas. The patient demonstrated the classic symptoms of an extremely rare condition known as bilateral carotid cavernous fistula, in only one eye. Reporting and analyzing this case will help us elucidate the underlying mechanisms of this disease.

POST TRAUMATIC CAROTID CAVERNOUS FISTULA PRESENTING AS PARATHYROID CRISIS

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: This is a case of two rare entities causing non-specific symptoms. Carotid-cavernous sinus fistula (CCF) arises from miscommunication of carotid arterial flow and the cavernous sinus. Post traumatic CCF presents bilaterally are uncommon, only 1-2% of patients. While already a rare diagnosis, our patient presented in an acute parathyroid crisis defined by symptomatology caused by markedly elevated calcium and parathyroid hormone, which may have masked our diagnosis of CCF. CASE PRESENTATION: A 57-year-old Colombian female presented with dizziness, anorexia, nausea, and vomiting. She also endorsed intermittent diplopia in the left eye. Physical exam revealed left sided ptosis. She reports a remote history of head trauma a month prior. Serum calcium on presentation was 13.6 mg/dL, ionized calcium of 6.8 mg/dL, and parathyroid hormone (PTH) was 219.8 pg/mL. Subsequently, a Technetium 99m sestamibi scan showed increased radioisotope along the right lower pole of the thyroid gland confirming primary hyperparathyroidism. She was resuscitated with normal saline fluid and started on Cinacalcet for hypercalcemia. Attempts to lower serum calcium can be achieved using bisphosphonates and may be effective within the first 24 hours. In our patient, Cinacalcet did not achieve sufficient calcium lowering thus, Zoledronic acid was used due to its potency with small doses. Nausea, vomiting, and positional dizziness resolved. Despite resolution of symptoms, she continued with ptosis; thus prompting further work up. Magnetic resonance angiography and venography revealed extensive time of flight enhancement throughout the bilateral cavernous sinuses. A cervical and cerebral angiography revealed bilateral carotid cavernous fistulas with ophthalmic vein arterialization. The next day, she underwent left cavernous sinus embolization with coiling. Parathyroidectomy was delayed due to recurrence of ptosis and need for a second embolization for persistent diplopia that resulted in resolution of symptoms. DISCUSSION: Each of these diagnoses are rare and both require timely evaluation and treatment. The patient's symptoms are classically seen in a parathyroid crisis, with nausea, vomiting, and anorexia being the most common symptoms in up to 48% of parathyroid crises. Like the aforementioned, CCF symptomatology also includes non-specific symptoms; however, may pose more of a challenge during diagnosis as symptoms wax and wane depending on the hemodynamic flow within the fistula. CONCLUSIONS: This case highlights the necessity of a thorough physical exam and further as a reminder that a clinician must be vigilant in ruling out alternative pathologies supported by the objective findings no matter how rare or unlikely. REFERENCE #1: Churojana A, Chawalaparit O, Chiewwit P, Suthipongchai S. Spontaneous occlusion of a bilateral post traumatic carotid cavernous fistula. Interv Neuroradiol. 2001;7(3):245-252. doi:10.1177/159101990100700311 REFERENCE #2: Singh DN, Gupta SK, Kumari N, et al. Primary hyperparathyroidism presenting as hypercalcemic crisis: Twenty-year experience. Indian J Endocrinol Metab. 2015;19(1):100-105. doi:10.4103/2230-8210.131763 REFERENCE #3: Lowell AJ, Bushman NM, Wang X, et al. Assessing the risk of hypercalcemic crisis in patients with primary hyperparathyroidism. J Surg Res. 2017;217:252-257. doi:10.1016/j.jss.2017.06.041 DISCLOSURES: No relevant relationships by Roman Bernstein, source=Web Response No relevant relationships by Ariol Labrada, source=Web Response No relevant relationships by Tram Nguyen, source=Web Response No relevant relationships by Elena Tellez, source=Web Response No relevant relationships by Yaswanraj Yuvaraj, source=Web Response

Clinical improvement in indirect carotid cavernous fistulas treated endovascularly: A patient based review

Patients with indirect carotid cavernous fistulas (CCF) were reviewed to emphasize the importance of diagnosing patients even with trivial findings and to raise awareness. Eighteen patients diagnosed as CCF were included. Neuro-ophthalmological findings before and after treatment, diagnostic investigations, treatment, clinical course was noted. Twelve patients were female (67%), 6 were males (33%) and the mean age at presentation was 54 years (range: 29–70 years). Conjunctival hyperemia was present in all patients. Seventeen (94%) patients had proptosis and diplopia, nine (50%) had orbital pain and/or headache, four (22%) had blurred vision, one (5.5%) had ptosis. On examination, 17 patients (94%) had restricted eye movements, four (22%) had low visual acuity and five patients had (28%) increased intraocular pressure (IOP). One patient had been diagnosed as myasthenia gravis and two as thyroid orbitopathy and had been on treatment accordingly before CCF was diagnosed. In two patients, bilateral findings were present despite unilateral CCF on angiography. Barrow Type B fistula was found in 7 (38%), Barrow Type D in 11 (62%) patients. In three bilateral CCF was detected. All were treated by endovascular intervention. Residual deficits at the sixth month control were, eye movement deficits in seven (39%), decrease in visual acuity in one (5.5%) and elevated IOP in one (5.5%) patient. Indirect CCF patients generally present with mild symptoms and the diagnosis may be overlooked. Mild progressive ophthalmoparesis with conjunctival hyperemia must be warning. Though rare bilateral CCF can be detected as well as unilateral CCF with bilateral findings.

Acute Onset Variable and Progressive Trochlear Nerve Palsy and Ophthalmoparesis Secondary to Bilateral Carotid Cavernous Fistula

ABSTRACT Purpose: To describe a patient who presented with right unilateral white-eyed posteriorly-draining carotid-cavernous fistulas (CCF) causing an isolated, acute-onset trochlear nerve palsy with atypical progression to a contralateral left red-eyed anteriorly-draining CCF associated with orbital congestion. Observation: A 74-year-old female presented with an acute onset incomitant right hypertropia consistent with the clinical diagnosis of a superior oblique palsy. Initial workup including MRI was normal. During a several-week course, she progressed to a comitant deviation. Six-weeks later she developed an episode of severe headache followed by worsening of her diplopia, reverse left hypertropia and left orbital congestive signs. CTA revealed mild opacification of the left cavernous sinus without a concomitantly present superior ophthalmic vein dilatation. A diagnostic angiogram was obtained which revealed a posterior-draining indirect CCF of the right internal carotid artery (ICA) and an anterior-draining indirect CCF of the left ICA. Transvenous Coil embolization resulted in immediate resolution of diplopia, orbital congestion, and headaches. Conclusion: Although it is atypical for a CCF – or other vascular pathology – to present with an isolated fourth nerve palsy, this case illustrates that vascular imaging should be considered in cases of acquired ocular motor paresis associated with headaches and cases with atypical progression despite normal initial cranial imaging.

Bilateral carotid-cavernous sinus fistula: Case reports and review of the literature

Carotid-cavernous fistula (CCF) is an abnormal vascular connection between the carotid artery and the cavernous sinus. There are various classifications based on haemodynamic, aetiology or anatomically. Haemodynamic classification is based on whether the fistula is high or low flow. Etiologically, it can be secondary to trauma or can develop spontaneously due to pre-existing aneurysm or medical conditions predisposing to arterial wall defects. Bilateral CCFs are very rare. We present two cases of bilateral CCF – one secondary to trauma and other occurred spontaneously. Both the patients presented with the signs of raised intraocular pressure – decreased vision, chemosis, proptosis and ophthalmoplegia. Magnetic resonance imaging and digital subtraction angiography confirmed a bilateral CCF. Both the patients underwent two settings of endovascular embolisation procedures, and complete embolisation of bilateral CCF was achieved. Available literature is unclear about the aetiology of bilateral CCF, technique of endovascular embolisation and the prognosis of bilateral CCF. In our both the cases, we were able to achieve complete embolisation in two settings.

A Rare Case Report of Bilateral Traumatic Carotid Cavernous Fistula

Objective : To increase the awareness for ophthalmologist in diagnosing rare case of bilateral traumatic carotid cavernous fistula that could lead to serious complications.&#x0D; Case description : A 44 years old woman came with chief complaint of squint and diplopia since one month after traffic accident. She had been underwent orbital bone fracture repair at previous hospital immediately. There was complaint of headache, blurred vision, double vision and pain on the eyes one month after trauma. There was no history of redness or proptosis. Examination revealed 6/6 for the right and 6/8.5 for the left eye corrected visual acuity, 30 degrees exotropia, normal intraocular pressures (IOP), restriction on eyes movement especially on the left eye, normal anterior and posterior segment, and no bruit. CT orbital revealed bilateral dilated and tortuous of the ophthalmica vein with suspicious of carotid cavernous fistula (CCF). Patient developed slowly progressive conditions such as proptosis non axial on the left eye, redness with corkscrew appearance on both eyes, restriction of the ocular movement and also founded bruit on both eyes within two months follow up. CT angiography revealed CCF bilateral type A. Patient underwent digital subtraction angiography (DSA) and balloon embolization four months after diagnosed.&#x0D; Conclusion : Although bilateral carotid cavernous fistula is a rare case, performing a suitable diagnostic examination would help to prevent devastating outcome. Prognosis depends on severity and time span from symptoms to treatment.

Bilateral carotid-cavernous fistula presented with unilateral symptomatology

Introduction. Carotid-cavernous fistula presents an abnormal communication between carotid arterial system and the venous cavernous sinus. Due to anatomical characteristics of the sinus, the clinical picture is first manifested as ophthalmic problem. These fistulas are either of spontaneous or traumatic origin with different hemodynamics. They are usually on one side. However, bilateral carotidcavernous fistulas are rare and deserve special interest. Case report. We presented a 76-years-old female patient, who complained on diplopia and right eye protrusion and esotropia. Ophthalmological findings aroused suspicion of a right-side spontaneous carotid-cavernous fistula. Further non-invasive and invasive investigations confirmed our initial diagnosis. Unexpectedly, digital subtraction angiography revealed another fistula, on her left side. Both fistulas were of low flow and did not need therapy. Conclusion. Carotid- cavernous fistula represent certain diagnostic and therapeutic challenge. Although they are not indicated in a clinical picture, sometimes bilateral fistulas can be found during appropriate diagnostic procedures.

Bilateral Carotid-Cavernous Fistulas: An Uncommon Cause of Pituitary Enlargement and Hypopituitarism.

Carotid-cavernous fistulas (CCFs) are rare, pathologic communications of the carotid artery and the venous plexus of the cavernous sinus. They can develop spontaneously in certain at risk individuals or following traumatic head injury. Typical clinical manifestations include headache, proptosis, orbital pain, and diplopia. We report a case of bilateral carotid-cavernous fistulas associated with these symptoms and also with pituitary enlargement and hypopituitarism, which improved following surgical intervention. Arterialization of the cavernous sinus and elevated portal pressure may interfere with normal venous drainage and the conveyance of inhibiting and releasing hormones from the hypothalamus, resulting in pituitary enlargement and hypopituitarism. This condition should be considered in the differential diagnosis of hypopituitarism associated with anterior pituitary enlargement.

Fístula carótido-cavernosa espontánea bilateral: reporte de caso y revisión de la literatura

Carotid cavernous fistulas are abnormal connections between the internal or external carotid artery or any branches and the cavernous sinus. They are characterised by proptosis, chemosis and orbital bruits. The carotid cavernous fistulas have a low incidence, especially if they are spontaneous and bilateral. They are associated with high morbidity, and require high clinical suspicion, early diagnosis, and specific treatment. They require emergency management when presented with signs of poor prognosis, such as rapidly progressive proptosis, intra-cerebral haemorrhage, or massive epistaxisThe case is presented of a 56 year-old patient with a history of bilateral glaucoma, high blood pressure (arterial hypertension), and chronic headache. She consulted due to changes in the pattern of headache, with signs of red flag due to the rapidly progressive severe pain, loss of consciousness and subsequent amnesia of the event. The cranial computed tomography scan showed asymmetry of the cavernous sinus and a hyperdense area to the right side. The magnetic resonance showed loss of cleavage in the segment of the cavernous portion of the right internal carotid artery, a finding suggestive of recent bleeding aneurysm. A cerebral angiography was performed, and a Barrow type D bilateral carotid cavernous fistula was diagnosed. Endovascular therapy was performed, with successful obliteration of both carotid cavernous fistulas.The literature review is presented along with evidence about the treatment of this disease.

Susceptibility-Weighted Imaging in Carotido-Cavernous Fistulas

This study determined the utility and accuracy of susceptibility-weighted MRI (SWI) for the detection of carotid cavernous fistulas. We retrospectively compared SWI images in nine patients (Group 1, case group) of DSA-proved carotid cavernous fistula (CCF) and 19 DSA negative cases for CCF as a control group (Group 2). Group 1 was again sub-grouped into direct and indirect types. Using uniform region-of-interest measurements, signal intensity within the superior ophthalmic vein (SOV) and the superior sagittal sinus (SSS) were measured on magnitude images of SWI. The SOV/SSS signal intensity ratio was calculated in each case and the mean values of the two groups were compared. Eleven SOV/SSS signal intensity ratios in Group 1 (7 unilateral and two bilateral CCF), 38 in Group 2 (both sides in 19 control subjects) were included. Median ± interquartile range of SOV/SSS ratios for Group 1, Group 2, indirect and direct type CCFs were 1.07 ± 0.43, 0.39 ± 0.23, 0.83 ±0.29, 1.4 ±0.38 respectively. Mann-Whitney test between Groups 1 and 2 was statistically significant with P<0.0001. All cases and controls were reliably distinguished with SOV/SSS signal intensity ratio of 0.64 as cut-off. Direct CCF cases had consistently higher ratios than indirect CCF. SWI was highly sensitive for detection and differentiation of both direct and indirect CCF. Only one case of corticovenous reflux was missed by SWI. SWI is useful for detection of CCF and to differentiate between direct and indirect CCF.

Recovery From Ophthalmoplegia and Proptosis After Repair of Bilateral Carotid-Cavernous Sinus Fistulas

A carotid-cavernous sinus fistula is an anomalous communication of dural branches from the intracerebral artery or external carotid artery to the cavernous sinus. Spontaneous carotidcavernous sinus fistulas have been associated with the presence of ruptured carotid aneurysms, atherosclerosis, diabetes mellitus, and genetic disorders such as Ehlers-Danlos syndrome or fibromuscular dysplasia. However, bilateral spontaneous carotidcavernous sinus fistulas are still considered uncommon.1,2 A 36-year-old man presented with 5 months of progressive headaches, diplopia, and progressive blurry vision. He had no history of head trauma, and both the headaches and the blurry vision were exacerbated by lying down in the supine position. Examination revealed significant proptosis, conjunctival injection, and abducens nerve palsies bilaterally (Figure, A and B). His visual field examination was within normal limits; however, his visual acuity was impaired, and tonometry revealed markedly elevated intraocular pressure (37 mm Hg in the left eye and 34 mm Hg in the right; reference range, 10-21 mm Hg). No retro-orbital bruit or upper motor neuron signs were noted. Magnetic resonance imaging of the brain showed an anterior displacement of the pituitary gland by a vascular structure, but no parenchymal changes were seen. An abnormal vascular structure was redemonstrated by magnetic resonance angiography, but the diagnosis of a dural fistula could not be confirmed. Conventional digital subtraction angiography revealed an indirect low-flow dural shunt between meningeal branches of both the internal carotid artery and the external carotid artery with the cavernous sinus bilaterally (Barrow type D; Figure, C). Endovascular embolization of the lesion (Figure, C and D) was performed using a combined anterior and posterior transvenous approach, which led to the immediate lowering of intraocular pressure and an improvement in visual acuity. At 1-month follow-up, the patient had a near complete resolution of his bilateral cranial nerve VI palsies (Figure, E and F; Video).

Spontaneous resolution of ophthalmologic symptoms following bilateral traumatic carotid cavernous fistulae

A 38-year-old woman developed bilateral carotid cavernous fistulae (CCF) following a motor vehicle collision. Her initial ophthalmologic findings included periorbital edema, palsies of the left oculomotor and abducens nerves, and residual dilated pupils. She subsequently developed significant optic disc edema and retinal vascular dilation bilaterally. Patients with similar injuries typically require neurosurgical or vascular intervention. In this case, the patient's signs resolved spontaneously by 21 months after onset, leaving no residual ocular deficits.

Bilateral indirect carotid cavernous fistula post trivial injury– A case report

Fifty-seven years old Malay lady, post menopausal with co-morbid of diabetes mellitus and hypertension presented with three months history of bilateral painful red eyes associated with double vision. Examination revealed both eyes proptosis, corkscrew vessels with present of bruit, secondary narrow angle with raised intraocular pressure, 6th cranial nerve palsy, and bilateral venous stasis retinopathy. CT angiogram showed bilateral dilated superior ophthalmic veins with cerebral angiogram findings of bilateral indirect carotid cavernous fistula involving small meningeal vessels. Indirect or dural cavernous sinus Fistula can easily be missed or misdiagnosed. Trivial injury especially in the predisposing patient can initiate the occurrence.

Teaching Neuro Images : Seeing double

A 64-year-old woman developed sudden onset of diplopia, headache, and bilateral abducens palsy. MRI revealed no intracranial mass, ischemia, or intraorbital pathology. Magnetic resonance angiography suggested bilateral carotid cavernous fistulas (CCFs, figure 1). Digital substraction angiogram revealed an intercavernous sinus dural arteriovenous fistula (DAVF), which was successfully embolized (figure 2). By 3-month follow-up, her …

Ophthalmopathy Induced by Bilateral Carotid Cavernous Fistula in a Patient with Graves' Disease

Received: 26 March 2011, Accepted: 12 July 2011 Corresponding author: Ji Hye Suk Department of Internal Medicine, Maryknoll Medical Center, 12 Daecheong-dong 4-ga, Jung-gu, Busan 600-730, Korea Tel: +82-51-461-2693, Fax: +82-51-465-7470, E-mail: Jihyesuk@gmail.com Ophthalmopathy Induced by Bilateral Carotid Cavernous Fistula in a Patient with Graves’ Disease Jong Kun Ha, Ji Hye Suk, A Ra Jo, Chan Woo Jung, Bong Jae Kim, Seong Oh Park, Sang Su Kim, Mi Kyung Kim Departments of Internal Medicine and Ophthalmology, Maryknoll Medical Center, Busan, Korea