Three cases of carotid cavernous sinus fistulas where the main ocular manifestation was restricted ocular motility]

BACKGROUND: Some cases of carotid cavernous sinus fistulas (CCFs) have few ocular congestive signs and symptoms. These cases need to be accurately diagnosed as having CCFs. CASES: Three patients with CCFs without ocular congestive signs or symptoms developed unilateral abducens restriction and visited our clinic. FINDINGS: Color Doppler imaging revealed retrograde flow of the superior ophthalmic veins bilaterally in the three cases. The diagnose of CCF was confirmed by cerebral angiography. CONCLUSION: It is sometimes difficult to diagnose CCF, when a patient without ocular congestive signs or symptoms shows disturbed ocular motility as the only ocular manifestation. In such cases, color Doppler imaging may show the presence of CCF.     

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.     

The dural shunt syndrome. Pathophysiology and clinical course

Dural shunts are vascular communications in which blood flows through small meningeal branches of the carotid arteries to enter the venous circulation near the cavernous sinus. The features of 10 patients with dural shunts are described. Most of these patients were postmenopausal women with dilated episcleral vessels, unilateral elevation of intraocular pressure, and exophthalmos. Pain or discomfort was common and ptosis or limited eye movement was usually present. These findings were characteristically more mild than those associated with carotid-cavernous sinus fistulas. CT scans usually showed enlargement of the extraocular muscles and the superior ophthalmic vein. Arteriography visualized partial or complete occlusion of intracranial venous drainage posterior to the cavernous sinus in all patients. Vascular dynamics of dural shunts are reviewed, and it is suggested that many of these communications may be congenital and that the onset of clinical abnormalities may be associated with the occurrence of intracranial venous thrombosis. Most patients eventually had resolution of their clinical abnormalities without treatment, and in some cases this improvement occurred soon after carotid arteriography.     

Supraclinoid carotid artery fistula mimicking a carotid-cavernous fistula

Pulsatile exophthalmos syndrome (PES) is characterized by conjunctival injection, orbital bruit, chemosis, exophthalmos, and palpebral edema. It is usually associated with glaucoma, decreased visual acuity, and extrinsic ophthalmoplegia, and mostly secondary to carotid-cavernous fistulas (CCFs), an abnormal communication between the intracavernous segment of the carotid artery and the venous channels in cavernous sinus. We present a case of an abnormal communication between the supraclinoid segment of the carotid artery and the coronary sinus that drains into the contralateral ophthalmic vein simulating a CCF. It was succesfully treated by coil embolization using a percutaneous approach. Other reported rare causes of PES and treatment modalities by embolization are reviewed.     

Carotid-cavernous and orbital arteriovenous fistulas: ocular features,diagnostic and hemodynamic considerations in relation to visual impairment and morbidity

The author investigated 101 cases with direct dural carotid-cavernous and orbital arteriovenous fistulas (CCF). The characteristic clinical findings, such as specific epibulbar arterialized loops, are described and the differential diagnosis of the striking diagnostic triad (exophthalmos, the above-mentioned loops and glaucoma) is discussed, together with the exclusion criteria for other causes of red eyes, episcleral measurements and blood flow. The results of various diagnostic procedures, such as ultrasonography, Doppler hematotachography and color Doppler of the orbit and carotid systems, magnetic resonance imaging and angiography, and of conservative treatment and embolization processes are dealt with successively.The classification of different types of carotid-cavernous fistulas is presented, together with the clinical signs in relation to morbidity and mortality during or after conservative or intervention therapies.The importance of patient follow-up, in the clinic as well as with Doppler methods, is emphasized in order to differentiate a progressive or diminished clinical condition caused by spontaneous thrombosis in the healing process or more arteriovenous flow. A ‘decision tree’ for use in daily practice is provided. In this study, of the 101 cases in which the localization was diagnosed by angiography, 42 were direct (30 traumatic, 12 spontaneous), 31 were dural (3 traumatic, 28 spontaneous) and 10 were orbital CCFs. In 18 other cases, usually dural or orbital shunts, angiography was not performed. For the management of 42 direct fistulas, conservative treatment was used in 12 cases (7 with success; 58%) and balloon embolization was performed in 18 cases (17 with success; 94.5%); the other cases were treated by direct or indirect surgery. Of the 48 (spontaneous and traumatic) dural fistulas, 39 were treated conservatively (32 recovered or were much improved: 82%, of the total cases, 67%). All seven cases in which embolization was performed were cured and/or much improved. In two cases, one fistula was conservatively treated while one was embolized at another location, both with success. Of the 10 orbital arteriovenous shunts showing signs of dural fistulas, the features disappeared in 8 cases, although after a much longer follow-up period than for the typical dural carotid-cavernous sinus fistulas; in one patient, direct surgery was performed successfully and in one patient the original, non-progressive, orbital features could still be observed.     

Carotid-cavernous and orbital arteriovenous fistulas: ocular features,diagnostic and hemodynamic considerations in relation to visual impairment and morbidity

The author investigated 101 cases with direct dural carotid-cavernous and orbital arteriovenous fistulas (CCF). The characteristic clinical findings, such as specific epibulbar arterialized loops, are described and the differential diagnosis of the striking diagnostic triad (exophthalmos, the above-mentioned loops and glaucoma) is discussed, together with the exclusion criteria for other causes of red eyes, episcleral measurements and blood flow. The results of various diagnostic procedures, such as ultrasonography, Doppler hematotachography and color Doppler of the orbit and carotid systems, magnetic resonance imaging and angiography, and of conservative treatment and embolization processes are dealt with successively. The classification of different types of carotid-cavernous fistulas is presented,(1-3) together with the clinical signs in relation to morbidity and mortality during or after conservative or intervention therapies. The importance of patient follow-up, in the clinic as well as with Doppler methods, is emphasized in order to differentiate a progressive or diminished clinical condition caused by spontaneous thrombosis in the healing process or more arteriovenous flow. A 'decision tree' for use in daily practice is provided. In this study, of the 101 cases in which the localization was diagnosed by angiography, 42 were direct (30 traumatic, 12 spontaneous), 31 were dural (3 traumatic, 28 spontaneous) and 10 were orbital CCFs. In 18 other cases, usually dural or orbital shunts, angiography was not performed. For the management of 42 direct fistulas, conservative treatment was used in 12 cases (7 with success; 58%) and balloon embolization was performed in 18 cases (17 with success; 94.5%); the other cases were treated by direct or indirect surgery. Of the 48 (spontaneous and traumatic) dural fistulas, 39 were treated conservatively (32 recovered or were much improved: 82%, of the total cases, 67%). All seven cases in which embolization was performed were cured and/or much improved. In two cases, one fistula was conservatively treated while one was embolized at another location, both with success. Of the 10 orbital arteriovenous shunts showing signs of dural fistulas, the features disappeared in 8 cases, although after a much longer follow-up period than for the typical dural carotid-cavernous sinus fistulas; in one patient, direct surgery was performed successfully and in one patient the original, non-progressive, orbital features could still be observed.     

Ophthalmic Findings in Dural Arteriovenous Shunts

Five cases of dural carotid arteriovenous shunts in the region of the cavernous sinus are reported. Discussion of the specific carotid angiographic features stresses the pathophysiologic characteristics of these shunts in relation to the ophthalmic findings. In all cases, the meningohypophyseal artery was involved as the sole or predominant source of the afferent arterial supply, and the degree of proptosis, epibulbar congestion and secondary glaucoma correlated with retrograde filling of the superior ophthalmic vein from the cavernous sinus. The ophthalmologist's early recognition of this entity as a “spontaneous” pathologic occurrence is important, since he is intimately involved in its diagnosis and long-term management.     

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。     

颈动脉海绵窦瘘介入治疗观察

目的探讨颈动脉海绵窦瘘介入治疗效果。方法对8眼(8例)颈动脉海绵窦瘘采用介入治疗并随访分析。结果7例经颈内动脉置入成功。1例经颈内动脉失败后，经眼上静脉置入成功。术后症状逐渐减轻，1～6月随访观察症状体征消失，瘘口消失，血管造影证实颈内动脉通畅，海绵窦不显影。结论颈动脉海绵窦瘘介入治疗安全性高，创伤轻，恢复快，并发症少。是一种较为理想的治疗方法。     

Ophthalmologic outcome of transvenous embolization of spontaneous carotid-cavernous fistulas: a preliminary report

We give a preliminary report of theophthalmologic outcome offour patients with transvenousembolization studied for their spontaneouscarotid-cavernous fistulas. One ofthem is a direct type while threeof them are indirect dural shunts. Inindirect dural shunts, traditionaltransarterial embolization rarelyachieves a complete clinical cure in ashort period of time. All caseshad an ophthalmologic disturbancejustified for endovascularintervention. We performed catheterization,and subsequently embolization withGuglielmi Detachable coils, tothe cavernous sinus via the femoralvein and inferior petrosal sinus/superiorophthalmic vein. All fourpatients achieved clinical andangiographic improvement with afollow-up period range from twoto sixteen months except for onepatient who had residual bilateralsixth nerve palsy. The transvenousapproach offers an effective andsafe alternative for the management ofspontaneous carotid-cavernous fistula.     

Cavernous sinus dural arteriovenous malformations: patterns of venous drainage are related to clinical signs and symptoms

OBJECTIVE: To provide evidence that venous congestion and drainage patterns are responsible for the manifestations of cavernous sinus area dural arteriovenous malformations (CSdAVMs). DESIGN: Retrospective observational case series. PARTICIPANTS: Records of 85 patients with complete clinical and angiographic evaluations of CSdAVMs were evaluated for the clinical features of the disorder. A neuroradiologist analyzed patterns of venous drainage to and from the cavernous sinus without knowledge of the clinical features. Four venous drainage patterns (reversal of flow from the CSdAVMs into the anterior cavernous sinus, ophthalmic vein thrombosis, drainage into the inferior petrosal sinus or drainage into the superior petrosal sinus) were statistically tested for their predictive value of signs and symptoms using logistic regression. MAIN OUTCOME MEASURES: The power of prediction of orbital congestion, elevated IOP, extraocular muscle dysfunction, optic neuropathy, venous-stasis retinopathy, choroidal effusion, anterior chamber shallowing, bruits, cranial nerve paresis, and central nervous system dysfunction from four patterns of venous drainage. RESULTS: Reversal of drainage into the anterior cavernous sinus and ophthalmic veins was highly predictive (P = 0) of orbital congestion, which was seen in 77 (91%) patients. In contrast, eight (9%) patients without orbital congestion had shunts that did not drain into the anterior cavernous sinus and ophthalmic veins. Cavernous sinus dural arteriovenous malformation drainage into the anterior cavernous sinus and ophthalmic veins also predicted elevated IOP (P = 0.0023) and optic neuropathy (P = 0.047). Ophthalmic vein thrombosis significantly predicted cases with choroidal effusion (P = 0.002) and anterior chamber shallowing (P = 0.01). Third nerve paresis could be predicted from flow toward the inferior petrosal sinuses (P = 0.017). Central nervous system symptoms or dysfunction, occurring in 7 (8%) patients, was predicted by venous drainage into the superior petrosal sinus (P = 0.0008). CONCLUSIONS: The clinical features found in patients with CSdVAMs are related to the abnormal venous drainage and can be predicted by these venous drainage patterns. Venous congestion and hypertension seem to cause the clinical dysfunction in this disorder.     

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.     

Arterio-venous fistula of cavernous sinus. A comparison of iatrogenic direct fistula with low flow dura shunt syndrome

PURPOSE: Comparison of symptoms of the low-flow dura shunt syndrome as a small arterial anomaly in the cavernous sinus with the direct, traumatic originated internal carotid artery fistula. MATERIAL AND METHODS: The clinical case report for two patients. The first case with a direct cavernous sinus fistula was diagnosed using angiography and the patient was treated with a detachable balloon catheter through the inferior petrousal sinus. The second case after the diagnosis was followed up with doppler sonography. RESULTS: The first case developed the direct cavernous sinus fistula after second thrombarteriotomy of the right internal carotid stenosis. The diagnosis, by typical clinical symptoms, was confirmed through the cavernous sinus angiogram with enlarged superior and inferior ophthalmic veins. The drainage was accomplished through the inferior petrousal sinus and the intercavernous sinus with accompanying signs of cortical drainage. All clinical symptoms, except for the abducens nerve palsy and the incomplete oculomotor nerve palsy, were reduced after neurosurgical occlusion of the fistula. The second case with the low-flow dura shunt syndrome was symptomatic by hypertension crisis and some spontaneous reduction was noticed. CONCLUSIONS: The iatrogenic direct cavernous sinus fistula is seldom but a very dangerous vital complication of the internal carotid arterial stenosis surgery and must be immediately closed through the endovascular embolisation therapy. The low-flow dura shunt syndrome may be in 50% occluded spontaneously.     

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.     

栓塞治疗颈动脉海绵窦瘘的良好效果(英文)

目的:报告外伤后颈动脉海绵窦瘘患者应用螺圈栓塞的成功经验。方法:连续病例。结果:两名颈动脉海绵窦瘘(CCF)患者成功接受了螺圈栓塞治疗。他们分别在机动车事故后2wk和4mo出现CCF的临床症状。一名患者视力良好但眼内压(IOP)升高,另一名则视力低下伴有临界眼内压。CCF的诊断均得到脑血管造影证实,采用螺圈栓塞治疗后两名患者视力恢复正常,在未用任何抗青光眼药物前提下眼压降至正常范围。CCF的症状诸如眼球突出,眼肌麻痹,结膜充血水肿,眼眶杂音及眼底改变也随之消失。结论:颈动脉海绵窦瘘宜早诊断和治疗以避免发生视力丧失严重并发症的发生,血管内栓塞是当代治疗选择。     

自发性颈动脉海绵窦瘘眼部表现的临床分析

目的:探讨自发性颈动脉海绵窦瘘眼部表现特点及诊断要点,提高对该病的认识,减少临床漏诊、误诊。方法:回顾分析14例就诊于我院的自发性颈动脉海绵窦瘘患者的眼部症状及体征、影像学检查、治疗方法等。结果:所选14例病例中最常见的眼部表现为眼球突出(100%)、结膜及浅层巩膜充血扩张(93%)。行CT或MRI检查的12例病例中,眼上静脉增粗者9例,海绵窦增宽或高信号者6例。4例行栓塞治疗者眼部症状和体征得到明显改善。结论:自发性颈动脉海绵窦瘘的眼部临床谱广泛多样,特征性眼部表现结合影像学检查可有效提高诊断。栓塞治疗对改善眼部表现效果明显。     

Embolization of carotid cavernous fistulas via the superior ophthalmic vein

Background: Carotid cavernous fistulae are treatable by various means, one of which is embolization via the superior ophthalmic vein (SOV). The use of this technique over the past 12 years at the Royal Melbourne Hospital, Victoria, Australia, is reviewed.
Methods: Five patients with carotid cavernous fistulas were treated with embolization of the fistula via the surgically isolated SOV.
Results: Each patient had successful endovascular closure of the fistula using embolization via the SOV approach.
Conclusions: Treatment of certain types of carotid cavernous fistulas that cannot be closed by the conventional endovascular (arterial and venous) approaches can be successfully closed by using a transvenous approach through the SOV     

Dural carotid cavernous fistula: definitive endovascular management and long-term follow-up

PURPOSE: To describe the endovascular treatment and clinical outcome in patients with indirect carotid cavernous fistulas (CCFs) over a 15-year period. To our knowledge, this is the largest series in the medical literature. DESIGN: Interventional case series. METHODS: A retrospective evaluation of 135 consecutive patients who underwent examination and treatment for indirect CCF was performed. Patients received independent evaluations by ophthalmologists, neurologists, or neuro-ophthalmologists before, during, and after endovascular treatment. Patients initially received noninvasive imaging followed by cerebral arteriography for definitive diagnosis and stratification by angiographic risk factors. Endovascular treatment was performed in 133 (98%) patients and clinical follow-up was achieved in 135 (100%) patients on an average of 56 +/- 4.3 months (range: 2 months-14 years). Angiographic follow-up was performed in 72 (54%) patients with ongoing symptoms or a history of fistula with high-risk angiographic features. Arteriographic cure with long-term clinical outcome is summarized by modified Rankin scale (mRS) and Barthel index (BI). RESULTS: At a mean follow-up of 56 months, 121 (90%) patients were clinically cured. At latest clinical follow-up, 131 (97%) patients showed good recovery (mRS, 1-2; BI 90-100), one (1%) had moderate disability (mRS, 3; BI, 50-60), and three (2%) (mRS, 4; BI, 40-50) were severely disabled. Procedure-related permanent morbidity was 2.3%. There was no operative mortality. CONCLUSIONS: With the observed favorable outcomes and low rate of procedural morbidity in this patient population with long-term angiographic and clinical follow-up, endovascular therapy should be the primary treatment for patients with indirect (dural) fistulas of the cavernous sinus.     

栓塞治疗颈动脉海绵窦瘘的良好效果

目的：报告外伤后颈动脉海绵窦瘘患者应用螺圈栓塞的成功经验。 方法：连续病例。 结果：两名颈动脉海绵窦瘘（CCF）患者成功接受了螺圈栓塞治疗。他们分别在机动车事故后2wk和4mo出现CCF的临床症状。一名患者视力良好但眼内压（10P）升高，另一名则视力低下伴有临界眼内压。CCF的诊断均得到脑血管造影证实，采用螺圈栓塞治疗后两名患者视力恢复正常，在未用任何抗青光眼药物前提下眼压降至正常范围。CCF的症状诸如眼球突出，眼肌麻痹，结膜充血水肿，眼眶杂音及眼底改变也随之消失。 结论：颈动脉海绵窦瘘宜早诊断和治疗以避免发生视力丧失严重并发症的发生，血管内栓塞是当代治疗选择。