Treatment of 54 traumatic carotid-cavernous fistulas

A series of 54 traumatic carotid-cavernous fistulas has been treated with detachable balloon catheters. The balloon was introduced through one of three different approaches: the endarterial route; the venous route through the jugular vein, the inferior petrosal sinus, and the cavernous sinus; or surgical exposure of the cavernous sinus; with occlusion of the fistula by a detachable balloon directly positioned in the cavernous sinus. Full follow-up review demonstrated that the carotid blood flow was preserved in 59% of cases. The most frequent complication was a transient oculomotor nerve palsy, which occurred in 20% of cases. In three cases where both the fistula and the carotid artery were originally occluded by the balloon, the superior portion of the fistula was later found not to be completely occluded, and these patients had intracranial ligation of the supraclinoid portion of the carotid artery. Three patients had hemiparesis, transient in two cases and permanent in the other. The results show that the fistula was totally occluded in 53 cases; in the one exception the patient became asymptomatic but had a minimal angiographic leak.     

Spontaneous carotid-cavernous fistula in a patient with Ehlers-Danlos syndrome type IV--case report

A 30-year-old female complained of sudden onset of severe proptosis, chemosis, diplopia, and bruit. Right carotid angiography showed a high-flow direct carotid-cavernous fistula (CCF) draining into the engorged superior ophthalmic vein, inferior petrosal sinus, and pterygoid plexus. The patient experienced retroperitoneal bleeding from a ruptured right renal artery after undergoing cerebral angiography. We suspected Ehlers-Danlos syndrome (EDS) type IV, which was confirmed by showing cultured fibroblasts failed to secrete procollagen type III. Endovascular surgery cannot be considered the treatment method of choice in view of the fragility of the arteries and veins in patients with EDS type IV. We treated our patient with extracranial internal carotid artery ligation. Currently, there is no ideal treatment for CCF in patients with EDS type IV. Since CCF is rarely life-threatening, the investigative approach and course of treatment must consider the associated vascular fragility.     

Treatment of carotid-cavernous fistulas by cavernous sinus occlusion.

The author reports the occlusion of 33 carotid-cavernous fistulas in 31 patients using thrombogenic techniques. In one patient the carotid artery had been occluded previously, in one it was occluded deliberately, and with 31 fistulas it was preserved. There was no mortality and virtually no morbidity.     

Myocardial ischaemia associated with Ehlers-Danlos syndrome

A 38-yr-old man with an unusual type of Ehlers-Danlos syndrome presented for elective abdominal aortic aneurysm repair. During surgery he developed acute myocardial ischaemia, resulting in abandonment of the procedure. He was shown subsequently to have severe triple vessel coronary artery disease. Silent ischaemia associated with severe coronary artery disease, although rare, may be associated with the syndrome and is difficult to recognize as other cardiac abnormalities are frequently present.      

Spontaneous carotid-cavernous fistulae in Ehlers-Danlos syndrome Type IV. Case report

Spontaneous bilateral carotid-cavernous fistulae and cervical artery dissection is reported in a 20-year-old woman with Ehlers-Danlos syndrome Type IV. The clinical features of 16 previously published cases of spontaneous carotid-cavernous fistulae associated with Ehlers-Danlos syndrome Type IV are reviewed, for a total of 17 cases. The mean age of the 14 women and three men was 31.6 years. Only direct fistulae were encountered. Diagnostic neuroangiography carried morbidity and mortality rates of 36% and 12%, respectively; neuroradiological treatment resulted in death in one of six patients. The possible value of desmopressin in the management of these patients is discussed. In view of the risks of arterial puncture and surgery, the authors emphasize the importance of early recognition of Ehlers-Danlos syndrome.     

Irradiation of spontaneous carotid-cavernous fistulas

Two patients with spontaneous carotid-cavernous fistulas were successfully treated with cobalt 60 irradiation to the sellar region. Angiographically, one patient showed combined-type shunts comprising a dural internal carotid-cavernous fistula and a direct internal carotid-cavernous fistula; the other patient had a mixed dural external and internal carotid-cavernous fistula. The respective total radiation dose was 3,200 rads and 3,024 rads. The patients responded satisfactorily to the treatment, with disappearance of the fistulas on angiograms and patency of the internal and external carotid arteries.     

Long-term observations in cases with spontaneous carotid-cavernous fistulas

Summary Twenty-six cases with spontaneous carotid-cavernous fistulas were followed up for periods ranging between 4 months and 9 years 8 months. A complete regression of symptoms without reappearance for more than 6 months was noted in 19 cases, a marked improvement in 2 cases, and a moderate regression in 3 cases. In 2 cases, symptoms have continued for 9 years 8 months and for 1 year. The regression of symptoms was usually delayed in patients less than 60 years old, in cases in which the symptoms developed slowly, and in cases with multiple draining veins. According to our observations a regression of symptoms may occur after very slight changes of haemodynamics.Compression of the cervical carotid artery for a short time or a temporary occlusion of the carotid artery by a balloon catheter should be considered as the treatment of choice in the first instance in cases with spontaneous carotid-cavernous fistulas showing relatively low pressure and low flow shunt.     

Long-term observations in cases with spontaneous carotid-cavernous fistulas

Twenty cases with spontaneous carotid-cavernous fistulas were followed up for periods ranging between 9 months and 9 years 8 months. In five cases, a temporary reappearance of symptoms was noted within 6 months after their regression. A complete regression of symptoms without reappearance for more than 6 months (between 6 months and 6 years 10 months) was noted in 18 cases, and a marked improvement was noted in one case. The regression of symptoms was usually delayed in patients less than 60 years old, in cases in which the symptoms developed slowly, and cases with three draining veins. According to these observations, noninvasive treatment is basically recommended in cases with spontaneous carotid-cavernous fistulas.     

经动脉栓塞治疗颈内动脉海绵窦瘘的疗效

目的分析颈内动脉海绵窦瘘患者经动脉栓塞治疗的疗效。方法回顾性分析我院经动脉栓塞治疗的52例颈内动脉海绵窦瘘患者资料,分析其治疗的成功率、复发率及并发症,并随访其短期及长期疗效。结果 52例患者中,48例(48/52,92.31%)栓塞成功,4例栓塞失败后行外科手术治疗。48例栓塞成功患者中,41例(41/48,85.42%)成功闭塞瘘口且保持颈内动脉通畅。4例(4/52,7.69%)术后复发,分别经压颈、球囊、液态胶栓塞及闭塞颈动脉的方法治疗成功。44例无复发患者短期内所有患者眼部症状缓解,长期随访中无复发病例。结论经动脉栓塞治疗颈内动脉海绵窦瘘成功率高、复发率低,并发症少。可脱球囊栓塞为其首选方法,辅以弹簧圈、液态胶栓塞,其短期及长期疗效均可肯定。     

Treatment of spontaneous carotid-cavernous fistula in Ehlers-Danlos syndrome by transvenous occlusion with Guglielmi detachable coils. Case report and review of the literature

The authors report on a young woman with Ehlers-Danlos syndrome (EDS) Type IV in whom a spontaneous direct carotid-cavernous fistula (CCF) was treated by transvenous occlusion with regular and fiber-coated Guglielmi detachable coils. To the authors' knowledge, this is the first time this approach has been used in a patient with EDS. The different treatment options are discussed, and the literature on endovascular treatment of direct CCFs in EDS is reviewed.     

Direct intracavernous obliteration of high-flow carotid-cavernous fistulas

Four cases of high-flow carotid-cavernous sinus fistula (CCF), three of them posttraumatic and one spontaneous, have been treated by a direct surgical approach to the cavernous sinus. The CCF's were obliterated by the introduction into the cavernous sinus of muscle fragments and/or fibrin sealant. In the three cases with a preoperatively patent internal carotid artery (ICA), the CCF was occluded and the ICA flow preserved. One of these also had a posttraumatic false aneurysm that enclosed the two avulsed ends of a transected intracavernous ICA. This was treated by cervical ICA ligation following resolution of the CCF. A fourth patient, who had previously undergone an unsuccessful ICA trapping procedure elsewhere, also obtained a good result. The case histories and the surgical technique are presented. Direct intracavernous obliteration with muscle fragments and fibrin sealant fulfills the criteria for treatment of high-flow CCF's: occlusion of the arteriovenous fistula and preservation of the ICA circulation. While this surgical technique is a therapeutic option in some cases, it appears to have precise indications in others.     

Ehlers-Danlos syndrome associated with multiple spinal meningeal cysts--case report.

A 40-year-old female with Ehlers-Danlos syndrome was admitted because of a large pelvic mass. Radiological examination revealed multiple spinal meningeal cysts. The first operation through a laminectomy revealed that the cysts originated from dilated dural sleeves containing nerve roots. Packing of dilated sleeves was inadequate. Finally the cysts were oversewed through a laparotomy. The cysts were reduced, but the postoperative course was complicated by poor wound healing and diffuse muscle atrophy. Ehlers-Danlos syndrome associated with spinal cysts may be best treated by endoscopic surgery.     

Classification and treatment of spontaneous carotid-cavernous sinus fistulas

An anatomical-angiographic classification for carotid-cavernous sinus fistulas is introduced and a series of 14 patients with spontaneous carotid-cavernous sinus fistulas is reviewed to illustrate the usefulness of such a classification for patient evaluation and treatment. Fistulas are divided into four types: Type A are direct high-flow shunts between the internal carotid artery and the cavernous sinus; Type B are dural shunts between meningeal branches of the internal carotid artery and the cavernous sinus; Type C are dural shunts between meningeal branches of the external carotid artery and the cavernous sinus; and Type D are dural shunts between meningeal branches of both the internal and external carotid arteries and the cavernous sinus. The anatomy, clinical manifestations, angiographic evaluation, indications for therapy, and therapeutic options for spontaneous carotid-cavernous sinus fistulas are discussed.