Diagnosis by Doppler color echography of dural carotid-cavernous fistula of ophthalmological manifestation]

The case reported here, concerns a spontaneous low-flow fistula between the external carotid arterial network and the cavernous sinus, with ophthalmological symptoms (exophthalmos, red eye) in an old woman with cardiac failure. The shunt was diagnosed by color-Doppler-imaging, which showed a flow reversal with a systolic component in the superior and inferior enlarged ophthalmic veins. This finding led the authors to extend the arterial filling sequence since the shunt was not detectable on standard arterial views. Embolization was performed during angiography which remains necessary to localize the shunt and to treat the fistula. The clinical symptoms progressively returned to normal and the correction of the hemodynamic disturbances could be followed by color-Doppler imaging, a non-invasive technique which can be easily repeated.     

Clinical spectrum of spontaneous carotid-cavernous fistula

A carotid-cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. A CCF is divided into two categories, direct and indirect. Direct fistulas usually account for 70 to 90% of all CCF. Spontaneous, low-flow fistulas are usually associated with atherosclerosis, hypertension and collagen vascular disease or may develop in females during peripartum period. The elderly age group, especially women are at increased risk. We report three cases of spontaneous CCF presenting with ocular manifestations and hypertension, without any collagen vascular disease. One case was a direct variety and the other two were of indirect variety.     

Doppler sonography in the diagnosis of dural carotidcavernous fistula

Intracranial dural fistulas in the region of the cavernous sinus usually occur in middle-aged or elderly women in whom physical findings of ocular pulsation, orbital bruit, and ocular injection are subtle or absent. We examined a 19-year-old man with hemophilia who had a red, mildly proptosed left eye. Typical features of a carotidcavernous sinus fistula were absent except for the presence of dilated, tortuous episcleral vessels. Directional Doppler ultrasound evaluation detected retrograde blood flow in the left frontal vein and thus confirmed the presence of an arteriovenous shunt. After a selective internal carotid angiogram demonstrated a shunt from a dural branch of the internal carotid artery, the patient's symptoms remitted spontaneously. Directional Doppler ultrasonography is a noninvasive test that can detect the presence of a low flow, retrobulbar arteriovenous shunt in the region of the cavernous sinus.     

Carotid artery fistula after cataract surgery

To determine carotid cavernous fistula associated with choroidal detachment after cataract surgery. A 77-year-old woman underwent cataract surgery in both eyes. Postoperatively, proptosis and dilation of episcleral vessels in her left eye occurred and gradually increased. One month later, choroidal detachment developed in her left eye. Computed tomography showed an enlarged superior ophthalmic vein. Selected cerebral angiography showed fistulas between the megingeal branches of both the internal and external carotid arteries and the cavernous sinus. After the neurosurgical treatment, these symptoms disappeared. The development of carotid cavernous fistula after cataract surgery, as demonstrated in our patient, may be uncommon. [Ophthalmic Surg Lasers 1998;30:160-162.] Carotid cavernous fistula (CCF) is an abnormal communication between the internal carotid artery and the cavernous sinus. Ocular manifestation of the fistula includes proptosis, pulsation of the globe, orbital bruit, episcleral vein dilation, and chemosis. CCF is divided into spontaneous or traumatic by cause and direct or dural by angiographic findings. To our knowledge, CCF development after cataract surgery may be uncommon. We recently examined a patient with such a condition.     

Dural arteriovenous fistula of the sigmoid sinus with a clinical expression on the opposite eye: A case report

We report the case of an 87-year-old patient who developed a dural arteriovenous fistula of the sigmoid sinus with progressive exophthalmia in the opposite eye. The patient had a unilateral decrease in visual acuity; the vessels of the conjunctiva were dilated, and a progressive central retinal vein occlusion occurred in the left eye. A dural carotid cavernous fistula of the right sigmoid sinus was diagnosed with an angiography exam and was successfully treated with embolization of the arteriovenous shunt using Onyx(?). A central partial facial nerve paralysis occurred after embolization and spontaneously resolved in 6 months. Six months after the treatment, visual acuity improved to 7/10 and the exophthalmia and central retinal vein occlusion signs regressed. Dural arteriovenous fistulae are abnormal shunts between a collateral vessel of the carotid artery and the cavernous sinus; the unilateral pulsating exophthalmia is the most frequent associated symptom. Cerebral MRI is useful to analyze indirect signs of the arteriovenous shunt showing an abnormal dilatation of the veins, but angiography of the carotid artery is essential to confirm the diagnosis. Furthermore, it allows treatment of the fistula at the same time, the main complication being the embolization of normal vessels close to the abnormal shunt. The clinical expression of the dural arteriovenous fistula on the opposite eye is infrequent and is related to the presence of complex septae within the cavernous sinus, resulting in a bilateral drainage of the cerebral venous system. The early diagnosis and management of the disease is necessary to improve the visual prognosis.     

28例颈动脉海绵窦瘘影像诊断分析

目的探讨超声、CT、MRI和数字减影血管造影(DSA)等各种影像学检查在颈动脉海绵窦瘘(CCF)诊断中的价值。方法收集我院经DSA证实诊断的CCF共28例,所有患者行眼科标准化A／B超声、DSA检查,部分患者行彩色多普勒超声、CT及MRI检查中的一项或多项。结果超声检查可发现眼上静脉增粗与脉搏同周期的搏动等,并能检测血流频谱。CT和MRI检查可发现海绵窦扩大及异常表现。DSA检查显示CCF分为颈内动脉海绵窦段直接供血和颈动脉的供应硬膜的动脉供血,供血动脉包括：颈内动脉海绵窦区细小脑膜分支;颈外动脉来源的脑膜中动脉、副脑膜动脉、咽升动脉、圆孔动脉等;瘘血向眼静脉、岩下窦、海绵问窦、侧裂静脉及皮层引流等。结论超声、CT、MRI等影像学检查对CCF的诊断各有所长,联合应用多可做出正确的临床诊断;DSA是诊断CCF的“金标准”,可明确瘘的大小、瘘血来源及引流方向。（中华眼科杂志,2004,40：674-678)     

Ophthalmoplegia in carotid cavernous sinus fistula.

The aetiology of ophthalmoplegia in 15 patients with carotid-cavernous sinus fistula is discussed, and the clinical findings are correlated with angiographic and orbital CT appearances. After closure of the fistula the majority of patients with generalised ophthalmoplegia recovered full ocular movements rapidly, while patients with an isolated abduction weakness required much longer to return to normal. Orbital CT studies showed enlarged extraocular muscles in the patients with generalised ophthalmoplegia but muscles of normal size in those with abduction failure alone. After closure of the fistula repeat CT studies of patients with enlarged extraocular muscles showed a diminution in muscle size. We suggest that generalised ophthalmoplegia in carotid cavernous sinus fistula is due to hypoxic, congested extraocular muscles. Isolated abduction weakness is due to a sixth nerve palsy, which probably occurs either in the cavernous sinus or more posteriorly near the inferior petrosal sinus. A combination of these 2 mechanisms may be found in some patients.     

Transvenous embolization of a dural carotid-cavernous sinus fistula via the inferior ophthalmic vein

A 76-year-old woman presented with an acute onset of right periocular pain, diplopia, ocular injection, progressive proptosis, and periocular swelling. She had an unremarkable past medical history, and the erythrocyte sedimentation rate and complete blood count were normal. A carotid-cavernous sinus fistula was suspected, and an MRI demonstrated enlargement of the superior ophthalmic vein posterior to the globe and enlargement of the inferior ophthalmic vein throughout its entire course. Cerebral arteriography demonstrated a dural cavernous sinus fistula. The inferior ophthalmic vein was accessed via the inferonasal orbital space and was catheterized for delivery of multiple platinum coils to the cavernous sinus fistula. Follow-up venograms demonstrated occlusion of the fistula. At 2-month follow-up, there was a residual sixth nerve palsy and resolution of symptoms, including proptosis and periocular swelling.     

The results of serial dynamic enhanced computed tomography in patients with carotid-cavernous sinus fistulas

PURPOSE: To assess serial dynamic enhanced computed tomography (serial DE-CT) as a diagnostic tool for carotid-cavernous sinus fistula (CCF). METHODS: Serial DE-CT was performed in seven patients (ages 31-74) with CCF. Contrast material was injected intravenously at a dose of 60 mL with an injection speed of 4 mL per second. Serial axial images of the cavernous sinus were undertaken every 3 seconds using a helical computed tomography system. This relatively low-risk technique provides direct evidence of the arteriovenous shunt in the cavernous sinus. RESULTS: In early imaging after the injection, enhancement of the cavernous sinus on the side of the CCF was noted at the arterial phase in all patients, whereas early enhancement of the cavernous sinus was not observed on the contralateral uninvolved side. CONCLUSIONS: These findings suggest the usefulness of serial DE-CT as a diagnostic tool for the initial diagnosis of both high- and low-flow CCFs.     

The association of carotid cavernous fistula with Graves’ ophthalmopathy

Graves’ ophthalmopathy (GO) is one of the frequent manifestations of the disorder which is an inflammatory process due to fibroblast infiltration, fibroblast proliferation and accumulation of glycosaminoglycans. Eye irritation, dryness, excessive tearing, visual blurring, diplopia, pain, visual loss, retroorbital discomfort are the symptoms and they can mimic carotid cavernous fistulas. Carotid cavernous fistulas are abnormal communications between the carotid arterial system and the cavernous sinus. The clinical manifestations of GO can mimic the signs of carotid cavernous fistulas. Carotid cavernous fistulas should be considered in the differential diagnosis of the GO patients especially who are not responding to the standard treatment and when there is a unilateral or asymmetric eye involvement. Here we report the second case report with concurrent occurrence of GO and carotid cavernous fistula in the literature.     

Orbital arteriovenous fistula with symptoms contralateral to the arterial supply. A case report

Carotid-cavernous sinus fistulas presenting signs and symptoms contralateral to the arterial supply of the fistulas are not uncommon. We describe a thoroughly documented case of an orbital arteriovenous fistula with symptoms exclusively contralateral to the arterial source, a rarer entity. A carotid angiography performed on a patient who presented a red chemotic and proptotic left eye showed a shunt between the meningeal branches of the right internal carotid artery and a left orbital vein.     

Stereotactic gamma surgery combined with endovascular surgery for treatment of a spontaneous carotid cavernous sinus fistula

A Barrow type D carotid cavernous sinus fistula (CCF) with varices proved unamenable to endovascular surgery. Follow-up angiography showed a persistent CCF fed by a recurrent meningeal branching of the ophthalmic artery division with drainage into the ophthalmic vein, resulting in a secondary glaucoma and central retinal thrombosis. Stereotactic gamma surgery was successfully performed, and aggravation of the visual symptoms was prevented. The CCF disappeared and intraocular pressure normalized one month after the radiosurgery. Stereotactic radiosurgery is thus an appropriate treatment for cavernous sinus dural arteriovenous fistulas resistant to endovascular treatment, especially with small, slow flow target residual fistulas.     

Unilateral Direct Carotid Cavernous Fistula Causing Bilateral Ocular Manifestation

Unilateral carotid cavernous fistula presents with ipsilateral ocular findings. Bilateral presentation is only seen in bilateral fistulas, usually associated with indirect (dural) carotid cavernous fistulas. Direct carotid cavernous fistulas are an abnormal communication between the internal carotid artery and the cavernous sinus. They typically begin with a traumatic disruption in the artery wall into the cavernous sinus, presenting with a classic triad of unilateral pulsatile exophthalmos, cranial bruit and episcleral venous engorgement. We report the case of a 38-year-old male with traumatic right carotid cavernous sinus fistula and bilateral ocular presentation successfully treated by interventional neuroradiology.Key Words: Exophthalmos, Papilledema, Carotid artery injury, Carotid cavernous sinus fistula, Endovascular procedure, Therapeutic embolization     

Surgical transvenous embolization of spontaneous carotid cavernous sinus fistulas in two patients

BACKGROUND: Arteriovenous communications in which blood flows from meningeal branches of the internal and external carotid arteries into the venous circulation around and in the cavernous sinus are termed spontaneous (dural) carotid sinus cavernous fistulas. Due to their mostly low shunt volume they are rarely life threatening, but without treatment they may cause severe ocular complications like episcleral secondary glaucoma, central vein occlusion or exudative retinal detachment. Traditional therapy is the transarterial approach by an interventional neuroradiologist. If such an approach is not possible or unsuccessful a transvenous route has to be considered. PATIENTS AND METHODS: Two patients underwent anterior orbitotomy via sub brow incision or infraciliary incision with cannulation of the superior ophthalmic vein or the inferior ophthalmic vein and embolization of the cavernous sinus with platinum coils. RESULTS: Successful closure was achieved on angiography and normalisation of clinical symptoms after a short period of progressive venous congestion. CONCLUSIONS: For arteriovenous fistulas that cannot be embolized arterially the surgical transvenous orbital route may work as a method of second choice. When performed by an interdisciplinary team (orbital surgeon, interventionell neuroradiologist) it is a technically straightforward, effective and promising approach.     

首诊于眼科的颈动脉海绵窦瘘7例回顾性分析

目的 探讨颈动脉海绵窦瘘眼部表现特点及诊断要点。方法 回顾分析5年中7例首诊于我科的外伤性颈动脉海绵窦瘘者的眼部症状及体征、影像学检查、治疗方法等。所有病例中最常见的眼部表现为搏动性眼球突出、球结膜高度充血水肿、眼压升高等。CT或MRI检查所有病例均显示眼上静脉扩张和海绵窦增宽,数字减影血管造影检查明确诊断。结果 7例行血管内介入栓塞治疗均一次成功,眼部症状和体征得到明显改善。结论 在临床工作中,对于可疑病例,应考虑颈动脉海绵窦瘘的可能,血管造影是该病诊断的金标准,血管内介入栓塞治疗对颈动脉海绵窦瘘是有效的。     

颅底动脉瘤的眼部改变

目的：探讨颅底动脉瘤的早期眼部改变，避免误诊。方法：回顾性对30例颅底动脉瘤的眼部改变进行分析。结果：临床表现有4组：①单纯颅神经受压症状6例。②自发性颈动脉海绵窦瘘5例。③轻度蛛网膜下腔出血伴有颅神经受压症状6例。④蛛网膜下腔出血为主者4例。总计颅神经受压症状中，动眼神经麻痹16只眼(53％)，外展神经麻痹11只眼(37％)，三叉神经麻痹11只眼(37％)，视神经受损导致的视力减退9只眼(30％)。视神经萎缩5只眼(17％)，视乳头水肿2只眼(7％)。结论：中年以上原因不明的明显头痛伴有动眼神经等颅神经障碍者，自发性颈动脉海绵窦瘘者应警惕到颅底动脉瘤的可能。     

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.     

Blindness associated with enlarging mycotic aneurysm after cavernous sinus thrombosis

PURPOSE: We report a patient with an enlarging internal carotid mycotic aneurysm secondary to septic cavernous sinus thrombosis presenting with acute visual loss. DESIGN: Single observational case report. METHODS: Retrospective review of the medical record and review of the literature. RESULTS: A 19-year-old man with residual left sixth nerve palsy and decreased vision in his left eye caused by left cavernous sinus thrombosis secondary to pansinusitis was seen 2 weeks after discharge with acute decreased visual acuity in the right eye. A workup revealed an enlarging left carotid/ophthalmic aneurysm that compressed the optic chiasm and right optic nerve. The patient was taken to the interventional angiography suite, where his left internal carotid artery was occluded endovascularly. The patient's vision improved on discharge. CONCLUSIONS: Visual loss caused by a mycotic carotid aneurysm is an infrequent sequelae after cavernous sinus thrombosis and is not well described in the literature. To our knowledge, this is the first reported case of acute visual loss associated with a mycotic ophthalmic aneurysm. The result of treatment was good in this case, with the patient's visual acuity returning to pretreatment status.     

Dural arteriovenous malformation

The carotid-cavernous fistula is a well-known clinical entity in which the internal carotid artery ruptures within the cavernous sinus. There is a lesser known but similar clinical entity known as the dural arteriovenous malformation which represents rupture of one of the smaller branches of the internal carotid artery within the cavernous sinus. This paper represents the clinical presentation of one such case of dural arteriovenous malformation and subsequent discussion.     

Secondary glaucoma following carotid cavernous fistula resolved after fistula's embolization: case report

Spontaneous carotid cavernous fistulas are a rare entity occurring more frequently in middle aged women. The authors report a case of a spontaneous carotid cavernous fistula in an elderly woman resulting in glaucoma of hard management. After surgical embolization of the fistula, the symptoms regressed and the intraocular pressure moved back to regular levels.