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.

Republished: Primary intraventricular haemorrhage due to rupture of giant varix of the basal vein of Rosenthal in a patient with long-standing direct CCF: angiographic features and treatment considerations.

Direct carotid cavernous fistulae (CCF) are often detected early and treated promptly resulting in a paucity of literature regarding its long-term evolution. We present a case of high flow post-traumatic direct CCF that was neglected for over 6 years and presented with a rare manifestation of primary intraventricular haemorrhage. Occlusions of the primary venous outlets likely resulted in engorgement of the deep venous system. The segmental anatomy of the shunting basal vein is critical to the clinical presentation and may range from basal ganglia or brainstem oedema/infarctions to uniquely, as in our case, isolated intraventricular haemorrhage secondary to variceal rupture. Treatment in such chronic cases requires a consideration of cerebral hyperperfusion syndrome necessitating deconstructive techniques with subsequent anticoagulation to avoid accelerated thrombosis of the venous varices.

Relationship of clinical presentation and angiographic findings in patients with indirect cavernous carotid fistulae.

INTRODUCTION: Indirect cavernous carotid fistulae (ICCFs) can present with insidious, non-specific symptoms and prove difficult to diagnose. This study evaluates associations among ICCF symptoms and angiographic findings. METHODS: A retrospective analysis was performed of prospectively maintained records at four medical centers to identify patients with ICCFs evaluated with angiography. Patient demographics, symptoms, and angiographic findings were tabulated. Univariate and multivariate analyses were conducted to identify associations among these variables. RESULTS: Records sufficient for review existed for 267 patients evaluated with angiography. Patients were most commonly women, in the sixth or seventh decade of life, and had symptoms for months before a definitive diagnosis. The most common symptoms included proptosis, diplopia, cranial nerve palsy, and chemosis. Cortical venous reflux was most common in patients with chemosis, orbital pain, or bruit. Intracranial hemorrhage was associated with cortical reflux and bilateral inferior petrosal sinus occlusion. Patients with loss of symptoms demonstrated higher rates of inferior petrosal sinus occlusion and a trend towards rupture. CONCLUSION: A high index of suspicion is needed to promptly diagnose patients with ICCFs. High risk features are more common in the setting of chemosis, orbital pain, bruit, or spontaneous loss of symptoms. Patients with such symptoms warrant expedited angiographic evaluation.

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.

Indirect carotid cavernous fistula mimicking ocular myasthenia.

71-year-old woman with progressive left-sided, monocular diplopia and ptosis. Her symptoms mimicked ocular myasthenia, but she had an indirect carotid cavernous fistula (CCF). She was diagnosed with monocular myasthenia gravis (negative acetylcholinesterase antibody) after a positive ice test and started on Mestinon and underwent a thymectomy complicated by a brachial plexus injury. Months later, she developed left-sided proptosis and ocular bruit. She was urgently referred to neuro-interventional surgery and was diagnosed with an indirect high-flow left CCF, which was treated with Onyx liquid and platinum coil embolisation. Mestinon was discontinued. Her ophthalmic symptoms resolved. However, she was left with a residual left arm and hand hemiparesis and dysmetria secondary to a brachial plexus injury. Indirect CCF usually can present with subtle and progressive symptoms leading to delayed diagnosis or misdiagnosis. It is important for ophthalmologists to consider this differential in a patient with progressive ocular symptoms.

Combined surgical and endovascular approach to treat a carotid cavernous fistula with associated brainstem venous congestion.

Endovascular embolization is the standard approach for management of carotid cavernous fistulas (CCFs) due to the ease of access and reduced level of risk associated with the procedure compared with open surgery. We present here a case of a CCF that eventually led to the development of brainstem venous congestion from perimedullary venous drainage. This fistula was not amenable to endovascular embolization due to lack of either ophthalmic vein or petrosal sinus drainage. Therefore, a craniotomy with direct puncture of the cavernous sinus was performed, followed by coil embolization to completely treat this fistula. This case demonstrates an uncommon progression of venous drainage to Cognard grade V, rare development of symptomatic brainstem venous congestion and a unique method to combine an open surgical approach with endovascular embolization to treat CCFs.

Staged curative treatment of a complex direct carotid-cavernous fistula with a large arterial defect and an 'oversized' internal carotid artery.

This is a case of a high-flow, post-traumatic direct carotid-cavernous fistula with a widened arterial defect and a large-diameter internal carotid artery (ICA). The unique aspect of this case is the oversized ICA, >8mm in diameter, which is both a pathological and a therapeutic challenge, given the lack of available neuroendovascular devices for full vessel reconstruction. We present a planned two-stage embolisation paradigm for definitive treatment. Transarterial coil embolisation is performed as the first stage to disconnect the fistula and normalise flow in the ICA. A 3-month recovery period is then allowed for reduction in carotid diameter. Repair of the large vessel defect and pseudoaneurysm is performed as a second stage in a delayed fashion with a flow-diverting device. Follow-up angiography at 6 months demonstrates obliteration of the fistula and curative ICA reconstruction to a diameter <5mm.

Endovascular treatment of carotid-cavernous vascular lesions / Tratamento endovascular das lesões vasculares carótido-cavernosas

ABSTRACT Objective: to evaluate the endovascular treatment of vascular lesions of the cavernous segment of the internal carotidartery (ICA) performed at our institution. Methods: we conducted a descriptive, retrospective and prospective study of patients with aneurysms of the cavernous portion of the ICA or with direct carotid-cavernous fistulas (dCCF) undergoing endovascular treatment. Results: we included 26 patients with intracavernous aneurysms and ten with dCCF. All aneurysms were treated with ICA occlusion. Those with dCCF were treated with occlusion in seven cases and with selective fistula occlusion in the remaining three. There was improvement of pain and ocular proptosis in all patients with dCCF. In patients with intracavernous aneurysms, the incidence of retro-orbital pain fell from 84.6% to 30.8% after treatment. The endovascular treatment decreased the dysfunction of affected cranial nerves in both groups, especially the oculomotor one. Conclusion: the endovascular treatment significantly improved the symptoms in the patients studied, especially those related to pain and oculomotor nerve dysfunction.

RESUMO Objetivo: avaliar o tratamento endovascular de lesões vasculares da artéria carótida interna (ACI), segmento cavernoso, realizado na Santa Casa de São Paulo. Métodos: estudo descritivo, retrospectivo e prospectivo, de pacientes com aneurisma da porção cavernosa da ACI ou com fístulas carótido-cavernosas diretas (FCCd) submetidos a tratamento endovascular. Resultados: foram incluídos 26 pacientes com aneurismas intracavernosos e dez com FCCd. Todos os aneurismas foram tratados com oclusão da ACI. Os com FCCd foram tratados com oclusão, em sete casos, e com oclusão seletiva da fístula nos outros três. Houve melhora da dor e proptose ocular em todos os pacientes com FCCd. Nos pacientes com aneurisma intracavernoso, a incidência de dor retro-orbitária caiu de 84,6% para 30,8% após o tratamento. Após o tratamento endovascular houve uma melhora importante da disfunção de nervos cranianos afetados em ambos os grupos, sobretudo no nervo oculomotor. Conclusão: o tratamento endovascular trouxe melhora para os pacientes deste estudo, especialmente nos critérios dor e acometimento do nervo oculomotor.

The enlarged extraocular muscle: to relax, reflect or refer?

PurposeExtraocular muscle enlargement (EOME) is most commonly associated with thyroid eye disease, but there are other causes. We report our outcomes of investigating and managing non-thyroid-related EOME (NTR-EOME).MethodsRetrospective consecutive case series. Sixteen patients identified by clinical features and orbital imaging. Patient demographics, radiological features, and adjuvant tests including biopsy and final diagnosis were recorded.ResultsMean age at presentation 59.3 years (range 24-89 years). Mean follow-up 3.2 years (range 3 months to 5.5 years). Superior rectus (SR) was most commonly involved muscle (8/16 cases) followed by lateral rectus (4/16). Of the 16 cases, 14 were associated with underlying systemic neoplasia (5 lymphoma, 5 metastatic carcinoma, and 4 presumed paraneoplastic syndrome). All SR enlargement was associated with underlying neoplasia. All patients underwent orbital imaging followed by systemic imaging based on clinical index of suspicion (14/16 patients (13 full body CT (FBCT), 1 mammography)). Positive systemic radiological findings were detected in 12/14 cases. Of the remaining 2 patients, 1 underwent full body positron emission tomography-computed tomography (FBPET-CT), which detected thyroid carcinoma, and the second patient underwent FBCT for staging following orbital biopsy showing lymphoma. Four patients (25%) died within 3 years of follow-up due to disseminated systemic malignancy.ConclusionsAll cases of NTR-EOME should be viewed with a high level of clinical suspicion for systemic neoplasia, especially when the SR is involved. FBCT can help to identify a primary systemic cause. FBPET-CT is best reserved for cases negative on FBCT or for staging and monitoring systemic disease. NTR-EOME can be associated with significant mortality (25%), hence warrants prompt and thorough systemic investigation.

A case of delayed carotid cavernous fistula after facial gunshot injury presented as loss of vision with symptom resolution after endovascular closure procedure.

Carotid cavernous fistulas (CCFs) are abnormal connections between the carotid artery and the cavernous sinus (CS), and can occur as a result of blunt and penetrating head injuries. While occurrence is rare, diagnosis can be made in the emergency department. Described in the present report is the case of a 26-year-old man who presented with complaints of pain, redness, blurred and loss of vision in the right eye, and swelling of the upper face due to a gunshot injury he had sustained 35 days prior.

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.

TREATMENT OF RETINOPATHY AND MACULAR EDEMA SECONDARY TO A CAROTID-CAVERNOUS FISTULA.

PURPOSE: To present a case report on a patient with retinal complications from a carotid-cavernous fistula. METHODS: Observational case report. RESULTS: A 26-year-old patient sustained head trauma following a motorcycle accident. Examination and retinal imaging demonstrated a venous stasis retinopathy with cystoid macular edema. The edema resolved with aflibercept, but not with bevacizumab. CONCLUSION: The patient was diagnosed with venous stasis retinopathy secondary to carotid-cavernous fistula. Pathologic findings completely resolved with appropriate management.

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.

Transvenous embolization of a carotid cavernous fistula complicated by a hematoma at the tentorial edge.

Indirect (dural) carotid cavernous fistulae are generally treated by endovascular surgery primary transvenous embolization that is safe and effective. We describe here a case of a left indirect carotid cavernous fistula that presented with proptosis and eye redness. The patient underwent transvenous embolization of carotid cavernous sinus. The procedure was complicated by a haemorrhage from the cavernous sinus. The post procedural CT scan showed a haematoma at the tentorial edge. Precise diagnosis and prompt treatment could prevent severe complications.

Single intravitreal ranibizumab injection for optic disc neovascularisation due to possibly traumatic, direct carotid cavernous fistula.

We report a patient with optic disc neovasculariation due to possibly traumatic direct carotid cavernous fistula treated by a single dose of intravitreal ranibizumab prior to neurointervention. A 25-year-old man had a 10-month history of bilateral proptosis and left sixth nerve paralysis was evaluated. Conjunctival vessels were markedly dilated, especially in the left eye. Clinical examination and fundus fluorescein angiography revealed disc neovascularisation in the left eye with subtle peripheral retinal ischaemia. Magnetic resonance imaging suggested a high-flow carotid cavernous fistula on the left side and this was confirmed by catheter angiography. A single dose of intravitreal ranibizumab was injected prior to neuro-intervention. The disc neovascularisation regressed completely three days later. The left direct carotid cavernous fistula was later treated successfully with coil embolisation. Optic disc neovascularisation is a very rare feature of carotid cavernous fistula and intravitreal ranibizumab may be a useful therapeutic adjunct prior to neuro-interventional techniques to reduce neovascularisation-induced haemorrhage following the intervention.

Clinical presentation, aetiology and prognosis of orbital apex syndrome.

OBJECTIVES: To present the clinical features, determine the causes and evaluate the prognosis of orbital apex syndrome in patients of Songklanagarind Hospital. METHODS: A retrospective review was conducted of patients diagnosed with orbital apex syndrome between January 1, 2002 and December 31, 2008. RESULTS: In our series, the major cause of orbital apex syndrome was carotid-cavernous sinus fistula, for which 30 patients were diagnosed. After excluding those patients whose condition was caused by carotid-cavernous sinus fistula, 50 patients (58 eyes) were analyzed. The mean age of the patients was 47.60 ± 18.88 years (27 male patients and 23 female patients). The most common presenting symptom was blurred vision (86%). The median duration of the presenting symptom was 37.5 d. Of the 50 patients the most frequent aetiology of orbital apex syndrome was neoplasia (48%), of which lymphoma was the most common in this group. After treatment, the vision of 19.4% of the patients improved and proptosis improved by 68.4%. CONCLUSIONS: The most common presenting symptom of orbital apex syndrome was blurred vision. The most frequent aetiology of orbital apex syndrome of all patients was carotid-cavernous sinus fistula, while the next most frequent was neoplasm, of which lymphoma was the most common in this group. Patients had a good outlook, after treatment directed at the cause, with a modest improvement in vision and exophthalmos in more than half of patients.

Endovascular treatment of bilateral multiple carotid-cavernous fistulas in a patient with Ehlers-Danlos syndrome.

INTRODUCTION: In this article, we report the first patient with Ehlers-Danlos syndrome type IV (EDS IV) who developed multiple spontaneous contralateral direct carotid-cavernous fistulas (CCFs) after permanent occlusion of left internal carotid artery CCF 2 years before. We occluded the multiple fistulas and preserved the internal carotid artery (ICA) by using transarterial balloon-assisted embolization with coils and Onyx. CASE REPORT: A 39-year-old woman presented with severe sudden-onset periorbital pain, increasing swelling, and proptosis of her left eye. Digital subtraction angiography (DSA) demonstrated direct left CCF. This patient underwent successful occlusion of left ICA and CCF by using detachable balloons. Two years later, she complained of a pulsatile intracranial bruit and then swelling and proptosis of her right eye. INTERVENTION: The right carotid artery angiogram revealed direct high-flow CCF with two fistulas in the cavernous segment of right ICA. Taking a transarterial approach, we occluded the two fistulas with GDC coils and Onyx-18 by an assisted balloon. CONCLUSION: In the management of multiple CCFs in EDS IV patients, we are able to occlude the fistulas and leave the ICA intact by using transarterial balloon-assisted embolization. Intensive follow-up of treated arteries is mandatory, because EDS IV is widespread and can occur on contralateral vessels as well.