Clinical profile and management outcomes among patients with Carotid-Cavernous Fistula

Background@#A carotid-cavernous sinus fistula (CCF) is an abnormal communication between the internal carotid artery and/or the external carotid artery and the cavernous sinus. There is a paucity of information on the ophthalmic outcomes of endovascular treatment for CCF in the Philippines.@*Objectives@#This study aimed to describe the clinical experience of CCF in our institution in order to further our understanding of the disease. This study described the demographic profile, risk factors, and clinical features of patients with CCF, and compared the data of patients according to the type of CCF. The study discussed the changes in clinical features over time in CCF patients who chose not to undergo endovascular treatment. The study also discussed the clinical outcomes of CCF patients who underwent endovascular treatment and compared the clinical outcomes to CCF patients who did not undergo endovascular treatment.@*Methods@#A retrospective cohort study design was performed using a medical record review of patients clinically diagnosed with CCF from January 2011 to June 2019.@*Results@#One hundred twenty medical records of patients diagnosed with CCF were included. Based on angiographic findings, patients were grouped according to type of CCF, with 86 patients in Group 1 or Direct CCF, 23 patients in Group 2 or Indirect CCF, and 11 patients in Group 3 or Mixed type of CCF. The patients were also grouped according to treatment, with 109 patients in Group A, or patients who did not undergo endovascular treatment, and 11 patients in Group B, or patients who underwent endovascular treatment. There was a male predominance in CCF, most occurring in the age range of 26 to 35 years. Risk factors for CCF were trauma and hypertension. Clinical features included the presence of blurring of vision, proptosis, corkscrewing of conjunctival vessels, extraocular movement limitation, diplopia, audible bruit, elevated intraocular pressure, and pulsation. Findings on Computed Tomography scan included dilated superior ophthalmic vein, proptosis, and enlarged extraocular muscles. Direct CCF (Group 1) occurred mostly in males, with mean age of 39.1 years, and with trauma as the major risk factor. Indirect CCF (Group 2) occurred mostly in females, with mean age of 52.1 years, and with hypertension as the major risk factor. Regardless of the type of CCF, patients who did not undergo endovascular treatment can exhibit spontaneous improvement or worsening of clinical features. Patients who underwent endovascular treatment generally had favorable clinical outcomes, manifesting as either improvement or no worsening of features. There were minimal ophthalmic complications associated with treatment.@*Conclusion@#Endovascular treatment is safe and effective in the improvement of visual acuity, corkscrewing of conjunctival vessels, amount of proptosis, extraocular movement limitation, diplopia, and presence of audible bruit.

Outcomes of medical and surgical management in eyes with idiopathic elevated episcleral venous pressure

Purpose: Idiopathic elevated episcleral venous pressure (IEEVP) is a rare cause of secondary glaucoma and is a diagnosis of exclusion. The aim of this study was to describe the clinical presentation and analyze the outcomes of medical and surgical management in eyes diagnosed with idiopathic elevated episcleral venous pressure. Methods: A retrospective analysis of eyes diagnosed with IEEVP over a 5?year period between April 2012 and March 2016 was performed. The demographic details, medical history, and clinical course of the cases were obtained from the medical records. Data pertaining to the severity of glaucomatous damage, response to medical management, need for surgical intervention, and their outcomes were analyzed. Results: Fifteen eyes of 13 patients were included. Thirteen eyes (86.6%) had open angle configuration. Among the 13 eyes that had glaucoma, eight eyes (61.5%) had severe glaucoma, four eyes (30.7%) had moderate glaucoma, and one eye (7.6%) had mild glaucoma. The median follow?up was 210 days. Seven of the 15 eyes (46.6%) required a glaucoma filtration procedure, and three underwent prophylactic sclerotomies. 71.4% of these eyes had complete success. One out of the seven operated eyes required choroidal drainage post?operatively. Conclusion: IEEVP is an extremely rare condition and presents with raised intra?ocular pressure and tortuous episcleral vessels. The management of IEEVP is similar to that of primary open angle glaucoma. Uveal effusion is to be anticipated, and hence, combining trabeculectomy with prophylactic sclerotomies is advisable.

Delayed care in carotid-cavernous fistula due to the Covid-19 pandemic / Atraso no atendimento de casos de fístula carótido-cavernosa devido à pandemia de Covid-19

ABSTRACT Direct carotid-cavernous fistula is a high-flow communication between the internal carotid artery and the cavernous sinus that requires early transarterial embolization for its resolution. We report a case of a patient with a direct carotid-cavernous fistula who subsequently developed a central retinal vein thrombosis due to a delay in treatment related to the health collapse experienced in the first months of the Covid-19 pandemic in Spain.

RESUMO A fístula carótido-cavernosa direta é uma comunicação de alto fluxo entre a artéria carótida interna e o seio cavernoso que requer embolização trans-arterial precoce para sua resolução. É relatado aqui o caso de um paciente com fístula carótido-cavernosa direta que posteriormente desenvolveu uma trombose da veia central da retina devido a um atraso no tratamento relacionado ao colapso de saúde experimentado nos primeiros meses da pandemia de Covid-19 na Espanha.

A case of permanent vision loss due to a carotidcavernous fistula: importance of early diagnosis / Relato de caso de perda visual secundária a fístuda carótido-cavernosa: a importância de um diagnóstico adequado

ABSTRACT A 97-year-old female presented with spontaneous acute-onset palpebral hyperemia and edema of the right eye that had progressively worsen over the previous three days. These signs did not suggest possible carotid-cavernous fistula until a second examination 72 h later, during which the patient exhibited significant progression. Despite embolization, the patient exhibited sustained corneal edema, clots, and turbidity in the aqueous humor, which resulted in permanent visual loss. A greater level of clinical suspicion for possible carotid-cavernous fistula is warranted on initial presentation of palpebral hyperemia and edema to prevent possible irreversible vision loss.

RESUMO O objetivo deste relato é apresentar o caso de uma paciente de 97 anos com início agudo e espontâneo de hiperemia e edema palpebral. Estes sinais não levaram a uma suspeita diagnóstica de fístula carótido-cavernosa até um segundo momento, quando a paciente apresentou progressão importante do quadro clínico. Apesar da realização de tratamento efetivo com embolização da fístula, a paciente manteve alterações oculares como edema de córnea, coágulos e turvação no humor aquoso, e manteve perda visual definitiva.

Increased intraocular pressure in patients with Carotid-Cavernous Fistula seen at a Tertiary Eye Care Center

Objectives@#We described the demographic and clinical profiles of patients with carotid-cavernous fistula (CCF), determined the prevalence of increased intraocular pressure (IOP), and described the IOP outcomes after endovascular treatment.@*Methods@#This was a single-center, retrospective review of records of patients with clinical signs and radiologic evidence of CCF from January 2012 to December 2017. Outcome measures included the prevalence of increased IOP in those with CCF, mean and range of IOPs, average number of IOP-lowering medications needed, and percentage of eyes with normal, controlled, and uncontrolled IOP before and after endovascular intervention. @*Results@#Ninety-six (96) eyes of 92 patients with radiologic evidence of CCF on 4-vessel cerebral angiography were included. Fifty-nine (59) percent were between the ages of 20 to 39 years. Majority (65%) were males. Direct CCFs accounted for 70% of cases. Increased IOP was the third most common ocular sign with a prevalence of 78%, ranging from 10 to 56 mmHg (mean 20.3 ± 8.0). The average number of antiglaucoma medications for IOP control was 2. Eleven (11) underwent definitive management for CCF. Post-treatment, 33% of 13 eyes had normal, 27% controlled, and 40% uncontrolled IOPs.@*Conclusion@#There was a high prevalence of increased IOP in patients with CCF. Those who did not achieve IOP control should be referred for endovascular intervention to prevent serious complications, including secondary glaucoma.

Efficacy analysis of Matas test combined with endovascular intervention in 144 patients with traumatic internal carotid-cavernous fistula / 中华急诊医学杂志

Objective:To explore the efficacy of Matas test combined with endovascular intervention on patients with traumatic internal carotid-cavernous fistula.Methods:A retrospective case study was performed on 144 patients with traumatic internal carotid-cavernous fistula treated in our department from August 2012 to June 2018. The clinical symptoms, intraocular pressure and visual acuity data before and after surgery were counted, and the paired t test was used for statistical analysis. Logistic regression was performed to analyze factors affecting recurrence.Results:The main symptoms of traumatic internal carotid-cavernous fistula were proptosis, bulbar conjunctival hyperemia and cranial sound. Postoperative intraocular pressure was significantly lower than preoperative intraocular pressure [(11.13±2.97) mmHg vs. (22.37±6.64) mmHg] and postoperative visual acuity was significantly higher than preoperative visual acuity [(0.69±0.36) vs. (0.47±0.25)] and (both P<0.05). Postoperative intraocular pressure was an independent risk factor for recurrence within 3 months ( OR=0.357, 95% CI: 0.135-0.944, P=0.037), and the recurrence rate was 10.42%. Conclusions:Matas test combined with endovascular intervention in the treatment of traumatic internal carotid-cavernous fistula can effectively reduce intraocular pressure, improve visual acuity, and has a definite curative effect. It is one of the effective treatment methods for traumatic internal carotid-cavernous fistula.

Surgical Treatment of Carotid-cavernous Fistula Performed through the Brazilian Unified Health System

Introduction The carotid-cavernous fistula (CCF) is an abnormal communication between the arterial carotid system and the cavernous sinus. In most cases, spontaneous fistulas are due to the rupture of intracavernous carotid artery aneurisms. Traumatic fistulas occur in 0.2% of head injuries, and 75% of all CCFs are caused by automobile accidents or penetrating traumas. Objective To identify the data regarding the number of annual procedures, hospital expenses, length of hospital stay, and the number of deaths of patients admitted by the Brazilian Unified Health System (SUS, in the Portuguese acronym), in the period between 2007 and 2017, using the surgical code of the surgical treatment for CCF. Methods The present was an ecological study whose data were obtained by consulting the database provided by the Department of Computer Sciences of the Brazilian Unified Health System (Datasus, in Portuguese). Results A total of 85 surgical procedures were performed for the treatment of CCFs from January 2007 to October 2017 through the Unified Health System (SUS, in Portuguese), and there was a reduction of 71.42% in this period. The annual incidence of patients undergoing this surgical treatment during the period observed remained low, with 1 case per 13,135,714 in 2007, and 1 case per 51,925,000 in 2017. Conclusion Despite the low annual incidence of the surgical treatment of CCFs performed by the SUS in Brazil in the period of 2007­2017, based on the data obtained on the average length of stay and expenditures in hospital services, it is necessary that we develop an adequate health planning.

Transvenous Embolization of Dural Carotid Cavernous Fistula through the Supraorbital Vein

We describe a case of transvenous embolization through the dilated supraorbital vein to treat a dural carotid cavernous fistula. The approach through the common facial vein or direct access of the superior ophthalmic vein is a commonly used route to the superior ophthalmic vein when the approach via the inferior petrosal sinus is unavailable. In rare cases, the dilated supraorbital vein provides an alternative route and we discuss the technical details.

Síntomas neurológicos de fístula carotídeo carvenosa: reporte de caso / Neurological symptoms of cavernous carotid fistula: case report

RESUMEN Las fístulas carótido-cavernosas son comunicaciones anormales entre la arteria carótida interna o externa; o alguna de sus ramas (sistema arterial) y el seno cavernoso (sistema venoso); el espectro de presentación de esta entidad es muy variable, sin embargo existe un gran número de pacientes en los cuales los síntomas neurológicos conllevan a sospecha clínica. Los síntomas pueden variar desde cefalea de intensidad variable, síntomas de origen auditivo, tinitus, hipoacusia y vértigo hasta manifestaciones que son clínicamente evidenciables y signos derivados de congestión orbitaria como proptosis, quemosis, soplos orbitarios y pérdida de la visión, causas que motivan un diagnóstico y tratamiento oportuno. Se realiza reporte de un caso de un paciente de 60 años de edad que acude al servicio hospitalario de la Fundación Centro Colombiano y Enfermedades Neurológicas FIRE, con cefalea de 2 meses de evolución, consulta por diplopía binocular, dolor ocular bilateral, inyección conjuntival, secreción purulenta, epifora bilateral, vértigo con miositis orbitaria aguda, fístula carótido-cavernosa en ojo derecho y parálisis del sexto par craneal izquierdo. La panangiografía cerebral reporta fístula carótida-cavernosa indirecta tipo B de Barrow por aferencias del tronco meningohipofisiario derecho e izquierdo por lo que es llevado a terapia endovascular con obliteración exitosa de la fístula.

SUMMARY Carotid-cavernous fistulas are abnormal communications between the internal or external carotid artery or one of its branches (arterial system) and the cavernous sinus (venous system). The spectrum of presentation of this entity is very variable. However, patients may consult for headache and symptoms derived from orbital congestion such as proptosis, chemosis, orbital murmurs and loss of vision. They require timely diagnosis and treatment. We present the case of a 60 year old patient with a history of acute orbital myositis, carotid-cavernous fistula in the right eye, paralysis of the sixth left cranial nerve and a 2 month old headache who consults for binocular diplopia, bilateral ocular pain, conjunctival injection, purulent secretion outflow, bilateral epiphora, sensation of hallucination of movement with gait and photophobia. Cerebral panangiography reports remnant of Barrow's b-type indirect carotid-cavernous fistula by right and left meningohypophysis trunks. The pacient taken to endovascular therapy with successful fistula obliteration.

Complete Reversal of Diffusion Restriction after Treatment of Traumatic Carotid-Cavernous Fistula / 대한신경손상학회지

A 15-year-old man presented with stupor following a motorcycle traffic accident. The patient was diagnosed with a traumatic left carotid cavernous fistula (CCF) with pseudoaneurysm of the left internal carotid artery. Brain magnetic resonance imaging (MRI) showed transiently restricted diffusion in the left centrum semiovale white matter and lower temporo-occipital area extending to the splenium of the corpus callosum, with high signal intensity on diffusion-weighted imaging. On the 35th day of admission, the patient had complete neurological recovery and a follow-up brain MRI revealed complete resolution of the lesions in the left centrum semiovale and splenium of the corpus callosum. These clinical and radiological features are highly suggestive of complete reversal of diffusion restriction after successful embolization of traumatic CCF.

Direct, spontaneous carotid‑cavernous fistula with a contracted kidney: A rare association.

We report a case of a 20‑year‑old female having systemic hypertension who presented with right‑sided proptosis, chemosis, and diminished vision, preceded by an acute episode of unilateral throbbing headache. Imaging studies revealed a right‑sided direct, spontaneous carotid‑cavernous fistula (CCF), aneurysm of internal carotid artery, bleed in the parieto‑frontal lobe, and swelling of extraocular muscles. Abdominal ultrasound revealed a small contracted right kidney measuring 64 mm × 27 mm. A direct spontaneous CCF can occur spontaneously following rupture of intracranial aneurysm without any history of trauma or connective tissue disorder. Prompt diagnosis of intravascular malformations at initial presentation can prevent neurological complications and vision loss. A team approach including emergency physicians, neurosurgeons, and ophthalmologists is needed for the proper management of such patients.

Glaucoma secundário a fístula carótido-cavernosa / Secondary glaucoma following carotid cavernous fistula

RESUMO As fístulas carótido-cavernosas são uma causa rara, porém grave, de glaucoma secundário por aumento da pressão venosa episcleral. Apresenta-se um caso de uma mulher de 72 anos, negra, atendida no Hospital de Clínicas da Universidade Federal do Triângulo Mineiro (HC-UFTM) com dor, sensação de pulsação, proptose, engurgitamento episcleral e quemose em olho direito, com aumento da pressão intraocular (PIO) apesar do uso de mediação anti-glaucomatosa. Na arteriografia foi evidenciada fístula dural para o seio cavernoso à direita, com refluxo para as veias orbitárias ipsilaterais. Realizou-se tratamento com agente líquido de embolização e, após o tratamento cirúrgico, apresentou melhora completa da proptose e congestão dos vasos episclerais, porém manteve PIO aumentada e desenvolvimento de glaucoma de ângulo fechado pela presença de goniossinéquias em 270º, com controle satisfatório com medicação.

ABSTRACT Carotid-cavernous fistulas are a major cause of secondary glaucoma due to increased episcleral venous pressure. We present the case of a 72-year-old female patient, treated at the Hospital de Clínicas at the Universidade Federal do Triângulo Mineiro (HC-UFTM), with pain, proptosis, episcleral engorgement, chemosis and pulsation in the right eye, with increased intraocular pressure (IOP) on anti-glaucomatous medication.Arteriography showed dural fistula to the right cavernous sinus, with reflux to orbital veins.Treatment with liquid embolization agent was performed, following full recovery of proptosis and congestion of the episcleral vessels. However, the patient maintained increased IOP because of narrow-angle glaucoma development due to the presence of goniosynechiae in 270º, which was controlled satisfactorily with medication.

Carotid-Cavernous Fistula Due to Giant Aneurysm in a Postpartum Woman

A carotid-cavernous fistula (CCF) is an abnormal communication between the venous cavernous sinus and the carotid artery. The rupture of an intracavernous aneurysm is usually caused by trauma, but spontaneous rupture can also occur, with pregnancy being a contributing factor. We report a case of direct CCF due to rupture of a giant aneurysm in a postpartum woman.

The application value of MSCT in the diagnosis of carotid cavernous fistula / 实用放射学杂志

Objective To evaluate the application value of MSCT in the diagnosis of carotid cavernous fistula(CCF).Methods The data of 10 patients who had been confirmed with CCF by DSA were retrospectively analyzed to compare the imaging features of MSCT plain scan,CTA,post reconstruction imaging and other examination ways.Results 10 patients with CCF which were manifested by superior ophthalmic vein enlargement in CT plain scan,early appearance of superior ophthalmic vein and cavernous sinus enlargement in CTA were studied.The orificium fistulae can be displayed through the multi planar reconstruction (MPR)and the curved planar reconstruction (CPR).The focal region can be well displayed through the volume rendering (VR)and the maximum intensity projection (MIP). CT,MRI,DSA and color doppler ultrasonography have both advantages and limitations in the diagnosis of CCF.Conclusion MSCT can achieve characteristic imaging findings through scan,CTA technology and various post reconstruction methods,it has significant value in the early diagnosis and treatment of CCF.

Covered Stents for the Endovascular Treatment of a Direct Carotid Cavernous Fistula : Single Center Experiences with 10 Cases

OBJECTIVE: Covered stent has been recently reported as an effective alternative treatment for direct carotid cavernous fistulas (DCCFs). The purpose of this study is to describe our experiences with the treatment of DCCF with covered stents and to evaluate whether a covered stent has a potential to be used as the first choice in selected cases. METHODS: From February 2009 through July 2013, 10 patients underwent covered stent placement for a DCCF occlusion. Clinical and angiographic data were retrospectively reviewed. RESULTS: Covered stent placement was performed for five patients primarily as the first choice and in the other five as an alternative option. Access and deployment of a covered stent was successful in all patients (100%) and total occlusion of the fistula was achieved in nine (90%). Complete occlusion immediately after the procedure was obtained in five patients (50%). Endoleak persisted in five patients and the fistulae were found to be completely occluded by one month control angiography in four. The other patient underwent additional coil embolization by a transvenous approach. Balloon inflation-related arterial dissection during the procedure was noted in two cases; healing was noted at follow-up angiography. One patient suffered an asymptomatic internal carotid artery occlusion noted seven months post-treatment. CONCLUSION: Although endoleak is currently a common roadblock, our experience demonstrates that a covered stent has the potential to be used as the first choice in DCCF; this potential is likely to increase as experience with this device accumulates and the materials continue to improve.

Iatrogenic Carotid-Cavernous Fistula after Stent Assisted Coil Embolization of Posterior Communicating Artery Aneurysm

Stent assisted coiling (SAC) is a useful technique for the treatment of wide necked complex aneurysm. As the frequency of SAC increases, stent-related complications such as thromboembolism, aneurysm rupture, and vessel rupture have been reported. However, to the best of our knowledge, carotid-cavernous fistula (CCF) after SAC has never been reported. The authors experienced a case of direct CCF after a SAC procedure for treatment of a complex posterior communicating artery (PCoA) aneurysm regrowth, which was treated by clip ligation 12 years before. The patient was managed conservatively and angiograms performed three months after the procedure showed the complete obliteration of the left PcoA aneurysm and the spontaneous disappearance of CCF. Navigation of Solitaire stent lumen with microcatheter can cause unexpected arterial injury, especially when the proximal tip is placed in the curved portion. It seems to be desirable to place the proximal tip of Solitaire stent in the straight portion whenever possible to reduce the risk of inadvertent arterial injury which might be caused by future navigation of stent lumen.

Endovascular embolization with detachable balloon for traumatic carotid-cavernous fistulae：clinical experience in 188 cases / 介入放射学杂志

Objective To evaluate the clinical efficacy of endovascular embolization with detachable balloon, based on the characteristics of traumatic carotid-cavernous fistulae (TCCF), in treating TCCF. Methods The clinical data of 188 patients with TCCF, who had received endovascular embolization with detachable balloon via femoral artery access, were retrospectively analyzed. The risk factors for recurrence were statistically analyzed. Results Of the total 188 patients, complete cure after the first balloon embolization was obtained in 160, certain improvement of clinical symptoms was achieved in 22, and balloon embolization failed in 6, for whom other surgical options had to be carried out. Complications occurred in three patients. Recurrence was seen in 23 patients within the period from one day to 5 years after the treatment, and the recurrent lesion was successfully cured in all patients. Univariate analysis and chi square test or correction chi square test indicated that factors affecting postoperative recurrence were the use of multiple balloons for embolization and the presence of residual fistula after operation (P0.05). Multivariate logistic regression analysis revealed that the independent factors affecting recurrence included the number of used balloon≥2 (OR=7.80, 95%CI:2.28-26.73,P=0.001) and postoperative residual fistula that was observed immediately after the embolization (OR=10.46, 95%CI:2.99-36.50,P=0.000). Conclusion For the treatment of TCCF, transcatheter embolization with detachable balloon is minimally-invasive, safe and reliable with fewer complications, therefore, this technique should be regarded as the therapy of first choice. The use of multiple balloons and the presence of residual fistula observed immediately after the embolization procedure are the risk factors for recurrence. Other possible risk factors are still to be furtherstudied.

Cataract surgery in a case of carotid cavernous fistula.

A carotid‑cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. The ocular manifestations include conjunctival chemosis, proptosis, globe displacement, raised intraocular pressure and optic neuropathy. Although management of CCF in these patients is necessary, the ophthalmologist may also have to treat other ocular morbidities such as cataract. Cataract surgery in patients with CCF may be associated with many possible complications, including suprachoroidal hemorrhage. We describe cataract extraction surgery in 60‑year‑old female with bilateral spontaneous low‑flow CCF. She underwent phacoemulsification via a clear corneal route under topical anesthesia and had an uneventful postoperative phase and recovered successfully. Given the various possible ocular changes in CCF, one must proceed with an intraocular surgery with caution. In this communication, we wish to describe the surgical precautions and the possible pitfalls in cataract surgery in patients with CCF.

Spontaneous Carotid-Cavernous Fistula in the Type IV Ehlers-Danlos Syndrome

Ehlers-Danlos syndrome (EDS) is a rare inherited connective disease. Among several subgroups, type IV EDS is frequently associated with spontaneous catastrophic bleeding from a vascular fragility. We report on a case of carotid-cavernous fistula (CCF) in a patient with type IV EDS. A 46-year-old female presented with an ophthalmoplegia and chemosis in the right eye. Subsequently, seizure and cerebral infarction with micro-bleeds occurred. CCF was completely occluded with transvenous coil embolization without complications. Thereafter, the patient was completely recovered. Transvenous coil embolization can be a good treatment of choice for spontaneous CCF with type IV EDS. However, every caution should be kept during invasive procedure.

Endovascular treatment of traumatic carotid cavernous fistula with trapping technique.

Conventional endovascular treatment for carotid cavernous fistula (CCF) involves a direct delivery of either coils, detachable balloon or both to the fistula with end point of CCF resolution and carotid artery preservation. But in few cases with severe laceration of carotid artery, the feasible endovascular technique applicable is by blocking the filling of fistula from cerebral circulation. This method known as trapping technique which implicates carotid artery occlusion, was performed in our present case with good result.