Diagnosis and management of an intraorbital vascular fistula

A spontaneous intraorbital arteriovenous fistula is an extremely rare phenomenon. More commonly, arteriovenous fistulas occur spontaneously as carotid cavernous fistulas and dural cavernous fistulas or are secondary to trauma. Surgical approaches vary widely and each carries their own set of potential problems. The purpose of this single case report was to provide an example of a successful treatment of a rare, spontaneous intraorbital vascular fistula.     

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。     

Central retinal occlusion in the course of idiopathic carotid-cavernous fistula

PURPOSE: This study presents the case history of a 58-year-old woman with a diagnosed spontaneous carotid-cavernous fistula on the left side and mild hypertension. THE FIRST SIGNS OF THIS DISEASE WERE: Headache, double vision, proptosis, ptosis of the left upper lid, paresis of the left abducens nerve, conjunctival edema, dilatation and tortuosity of the vessels in conjunctiva and episclera. In the course of this disease a massive central retinal vein occlusion occurred in the left eye. The angiography demonstrated carotid-cavernous fistula on the left side with pathological blood flow. Embolization of the fistula was attempted, but it was not successful. During 6 months of follow up, the signs of central retinal vein occlusion and other manifestations disappeared. CONCLUSIONS: A spontaneous carotid-cavernous fistula should be considered as one of the contributing factors of the central retinal vein occlusion, particularly in menopausal women with concomitant arterial hypertension. The significant reduction of neurological and ophthalmological symptoms and signs may suggest, that arteriovenous shunt is closed.     

Spontaneous dural cavernous sinus fistula in a child

We describe the clinical course of a 9-year-old boy in whom a spontaneous dural cavernous sinus fistula developed. The patient initially presented with proptosis and conjunctival congestion believed to be caused by a cavernous sinus hemangioma and was prescribed oral prednisone. He subsequently developed pain, increased proptosis, and decreased vision. Emergent angiography revealed a dural cavernous sinus fistula, which was successfully embolized. This is the first case to our knowledge of a dural cavernous sinus fistula in a noninfant child that was treated successfully with embolization during angiography.     

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.     

Bilateral non-traumatic carotid cavernous sinus fistula with spontaneous closure

A case of bilateral spontaneous carotid cavernous sinus fistula is presented. The diagnostic value of Doppler ultrasound examination is discussed, as well as therapeutic measurements, such as manual compression of the carotid artery and embolization treatment. The frequent spontaneous closure of these fistulas is emphasized, and a conservative attitude is recommended.     

Eyelid Fistula: A Feature of Occult Sinus Disease

Purpose: To describe the clinical characteristics of patients presenting with eyelid fistula as a complication of occult sinus disease. Methods: A retrospective review of patients presenting, to the Orbital Clinic at Moorfields Eye Hospital, with eyelid fistula due to chronic sinus disease; the review including clinical features, management and outcome. Results: Eight patients presented with discharge from an upper lid or medial canthal fistula, of whom 6 had a history of variable lid swelling, 3 had ptosis and 2 had proptosis with restricted upgaze. All patients had ipsilateral frontal or ethmoid sinus opacification on CT, 5 had erosion of the bone and 2 had sinus mucocoeles. Management involved functional endoscopic sinus surgery to establish drainage of the affected sinuses and direct excision of the fistular tract. Conclusions: A discharging eyelid fistula may indicate occult sinus disease, which can remain undiagnosed for long periods. There is typically a history of recurrent upper lid erythema and swelling, with spontaneous drainage through a fistula sited in the supero-medial eyelid sulcus; local features include contracture and thickening of the skin and underlying tissues around the fistula.     

Spontaneous carotid-cavernous fistula and the Ehlers-Danlos syndromes

A 22-year-old woman with one of the Ehlers-Danlos syndromes (EDS) developed a spontaneous carotid-cavernous fistula (CCF), demonstrated by ophthalmic ultrasound and cerebral angiography. Therapy consisted of two balloon embolization procedures that significantly reduced blood flow through the fistula. The patient subsequently had a cerebral hemorrhage and expired. The cardinal features of EDS and its ophthalmologic findings are reviewed. Spontaneous CCF have been described in EDS patients, but case documentation is lacking in the ophthalmic literature. The rationale for treating a CCF and the various therapeutic approaches are discussed; the serious risks involved in any attempt to save vision in these patients are considered.     

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.     

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.     

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。     

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.     

Spontaneous carotico-cavernous fistulas

Conclusion It appears that the specific vascular loops are essential for the diagnosis of carotico-cavernous fistulas, so that the ophthalmologist has an important part to play; his opinion on the angiography can also carry weight. The intraocular pressure should also be checked and if glaucoma occurs it should be treated as indicated.In the case of spontaneous carotico-cavernous fistulas and very mild forms of traumatic fistula conservative therapy should be applied first because there is a possibility of spontaneous cure and avoidance of risky operations.     

Spontaneous choroidal detachment and ‘red-eyed shunt syndrome’: two clinical entities with the same cause?

The files of four cases of spontaneous choroidal detachment were reviewed in order to demonstrate a low pressure arteriovenous (A-V) fistula as the cause. The clinical course and symptoms were compared with those described in the red-eyed shunt syndrome. In one patient an A-V fistula was demonstrated by carotidography, while in another patient CT findings supported a similar aetiology. In two out of four cases the initial clinical diagnosis was a solid subretinal mass. In all cases echography demonstrated a choroidal detachment which spontaneously disappeared. A prolonged course, bilateral involvement with changing side recurrences are the characteristics of this disease.     

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.     

Ophthalmoscopic findings in spontaneous carotid cavernous fistula: An analysis of 20 patients

The pathophysiological influence of spontaneous carotid cavernous fistula (CCF) on retinal/orbital circulation is discussed. Of 20 patients, 1 case of papilloedema, 4 of choroidal detachment (CD), 2 of exudative retinal detachment (ERD) and 3 cases of central venous thrombosis (CVT) were seen, associations that on rarely reported in the literature. An elevation in the episcleral venous pressure was measured that was more than twice the normal, which explains the ophthalmoscopic findings.     

Dural arteriovenous fistula and spontaneous choroidal detachment: new cause of an old disease.

A case is presented in which bilateral spontaneous choroidal detachments appear to be the direce result of bilateral dural arteriovenous fistula of the cavernous sinus region. Rapid resolution of the clinical signs followed bilateral orbital decompression via the transfrontal approach. Similarities in clinical presentation of both entities are reviewed and a premise for their cause-and-effect relationship elaborated. A literature search for similar unrecognised cases is discussed. The paper suggests that this association may be more frequent than published reports would imply.     

Treatment of carotid cavernous fistulas

The diagnosis and treatment of carotid cavernous fistulas (CCF) is an interdisciplinary challenge for both ophthalmologists and interventional neuroradiologists. According to the clinical signs and symptoms the tentative diagnosis is made by the ophthalmologist. It is the task of the neuroradiologist to ascertain this diagnosis by intra-arterial angiography. If a fistula is suspected this invasive diagnostic procedure is indispensable, not only to establish the diagnosis but also to classify those types of fistula with an unfavourable spontaneous course possibly resulting in intracranial haemorrhage. The indication for therapy is based on the clinical symptoms and the angiographic findings. In a number of cases no therapy is required. Since a fistula may change over time, these patients have to be under close ophthalmological surveillance. In many patients a conservative therapeutic approach with manual compression of the carotid artery is sufficient as a fIrst step. Invasive treatment is performed via the endovascular approach in almost all cases. Direct CCF are predominantly treated transarterially with detachable balloons and/or coils. Recently, intracranial stents have been used increasingly. The embolisation of indirect CCF is most effective using the transvenous access with coils. There are several approaches to the cavernous sinus. The interventional occlusion of CCF is nowadays a very effective treatment associated with a comparatively high cure rate and low incidence of complications. By close cooperation between ophthalmologists and neuroradiologists the patients can be protected against visual loss, the development of a secondary glaucoma, and, most importantly, against intracranial haemorrhage.     

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.     

颈动脉一海绵窦瘘

目的：探讨31例TCCF及5例SCCF的临床特点与疗效。方法：回顾性地分析该病的诊断和治疗方法。结果：24例TCCF经血管内栓塞治疗后有22例。症状及体征消退，瘘口消失(治愈率91．7％)。好转2例(8．3％)，症状和体征减轻，瘘口基本闭塞。5例SCCF经保守治疗。效果良好。结论：DSA检查及血管内栓塞技术是较理想的诊断及治疗方法。