Pulsatile Tinnitus Due to Stenosis of the Marginal Sinus: Diagnosis and Endovascular Treatment.

BACKGROUND AND PURPOSE: Venous pulsatile tinnitus is a disabling condition mainly caused by a stenosis of the lateral sinus. Here, we aimed to report a novel cause of venous pulsatile tinnitus, stenosis of the marginal sinus. MATERIALS AND METHODS: We retrospectively analyzed patients with isolated venous pulsatile tinnitus for which the suspected cause was a stenosis of the marginal sinus, treated or not, between January 2017 and December 2020. Patient charts and imaging were systematically reviewed. All patients underwent noncontrast temporal bone CT and MR imaging. RESULTS: Eight patients (7 women; median age, 36 years) were included. Six patients (75%) were overweight, and 1 patient had idiopathic intracranial hypertension. All patients presented with a typical venous pulsatile tinnitus. The stenosis of the marginal sinus was detected using oblique reconstructions on postcontrast 3D MR imaging. There was no other pathologic finding except ipsilateral stenosis of the lateral sinus in 3 patients. Four patients underwent endovascular therapy with placement of a stent in the marginal sinus, leading to complete resolution of the pulsatile tinnitus for all of them. No complication occurred. Of note, the symptoms of intracranial hypertension also regressed after stent placement in that patient. CONCLUSIONS: Marginal sinus stenosis is a novel cause of venous pulsatile tinnitus, which can be easily detected on MR imaging. Marginal sinus stent placement is safe and efficient. We hypothesized that the marginal sinus stenosis pathophysiology is similar to that of lateral sinus stenosis, which is a common and well-known cause of venous pulsatile tinnitus, explaining the similar clinical presentation and endovascular management.

Empty Sella Is a Sign of Symptomatic Lateral Sinus Stenosis and Not Intracranial Hypertension.

BACKGROUND AND PURPOSE: Empty sella has been reported in patients with idiopathic intracranial hypertension and is thought to be a sign of elevation of intracranial pressure. However, it can also be found in patients with lateral sinus stenosis presenting with isolated pulsatile tinnitus without signs of intracranial hypertension. We hypothesized that the volume of the sella turcica would be similar in both groups of patients undergoing stent placement for lateral sinus stenosis. MATERIALS AND METHODS: Consecutive patients with idiopathic intracranial hypertension or isolated venous pulsatile tinnitus and undergoing lateral sinus stent placement from January 2012 to December 2017 were included. The primary outcome was the estimated volume of the sella turcica based on preoperative CTA measurements. The ratio of the pituitary gland height/sellar height was calculated on preoperative MR imaging. Sellar volumes were compared among the 3 groups: pulsatile tinnitus, idiopathic intracranial hypertension, and a control group, matched by age and sex. RESULTS: Eighty-eight patients underwent lateral sinus stent placement. The median age was 37 years, and 94% were women. No difference in age, sex, or body mass index was found among the groups. Patients undergoing venous stent placement had significantly higher sellar volumes than the control group (P < 0.001). There was no difference in the sellar volumes (P = .63) or gland/sellar height ratios (P = .25) between the pulsatile tinnitus and idiopathic intracranial hypertension groups. CONCLUSIONS: Empty sella is found in 2 differing groups of patients undergoing lateral sinus stent placement, suggesting that it is a radiologic sign of symptomatic hemodynamic lateral sinus stenosis rather than elevated intracranial pressure.

The use of Onyx in different types of intracranial dural arteriovenous fistula.

BACKGROUND AND PURPOSE: Recently some series have been published about the use of Onyx for the treatment of DAFVs with satisfactory results. Our aim was to describe the treatment of different types of intracranial DAVFs with transcatheter injection of Onyx through an arterial approach. MATERIALS AND METHODS: At the Department of Interventional Neuroradiology, Hospital Lariboisière Paris, between January 2005 and January 2010, we treated 44 DAVFs in 42 patients. All patients were initially treated by arterial injection of Onyx. The average patient age was 56 years (range, 27-86 years), and there were 17 women and 25 men treated. RESULTS: A total of 58 arterial pedicles were catheterized, with the middle meningeal artery representing the most common site (n = 38). The average time of injection was 30 minutes (range, 15-60 minutes), and the average amount of Onyx was 2.5 mL (range, 0.6-6.5 mL). Of the 20 fistulas with direct venous drainage into a dural sinus (types I and II), we achieved the preservation of the sinus in 7 patients. Of the 44 fistulas embolized, 8 required a second embolization treatment and 1 fistula required a third treatment. In 9 cases, a complementary treatment was performed via transvenous embolization with coils and/or open surgery. Early complications were observed in 6 patients: Four had nerve injury (facial palsy, n = 2, and neuralgia, n = 2), and 2 had complications related to extension of venous thrombosis postembolization. All 6 patients had partial or complete resolution of these symptoms. CONCLUSIONS: The treatment of DAVFs by intracranial arterial injection of Onyx is safe, and, in most cases, results in the occlusion of the arterial venous shunt. In DAVFs with direct sinus drainage, sinus preservation was only possible in 7 of 20 patients (35%).

[Embolization of intracranial aneurysms: reimbursement and perspectives]. / Embolisations des anévrismes cérébraux: évolutions du financement et perspectives.

PURPOSE: To determine the costs related to the embolization of intracranial aneurysms compared to "rates per activity" (T2A) reimbursements. MATERIALS AND METHODS: The hospital admissions of patients with intracranial aneurysms treated with embolization and classified under diagnosis-related group (DRG) 01K02Z in 2007 were included. The costs related to the single-use devices, neurointerventional suite and hospital stay were calculated by analytical accounting. Revenues were calculated based on DRG-based medical information system (PMSI) and medical data using the diagnosis-related groups and reimbursements from 2007 (V10 of DRG) and 2009 (V11). RESULTS: Fifty-seven patients were included. The total cost was 932.278 euro and hospital revenues were 655.648 euro in 2007 and would have been 825.211 euro in 2009. The financial loss was on average 4.853 euro per admission in 2007 and 1.878 euro in 2009, and even more in two cases of ruptured aneurysm. CONCLUSION: In 2001, embolization of intracranial aneurysms, the treatment of choice for this pathology, results in a financial loss for the hospital, larger for ruptured aneurysms compared to non-ruptured aneurysms. The updated DRG, while improving the situation, remains insufficient.

A hereditary moyamoya syndrome with multisystemic manifestations.

OBJECTIVE: We report a detailed description of a family affected by a hereditary multisystem disorder associated with moyamoya syndrome. METHODS: In this family case report, we evaluated 9 members of the same family originating from Algeria. Investigations included neuroimaging, cardiologic and ophthalmologic evaluation, hormonal testing, hemoglobin electrophoresis, chromosomal karyotyping, muscle biopsy for morphology, immunohistochemistry and enzyme assays, mtDNA mutation screening, and haplotype analysis of 2 loci previously linked to moyamoya, on chromosomes 10 (ACTA2) and 17. RESULTS: Five males related through a maternal lineage were affected, suggesting an X-linked inheritance. Four of them had symptomatic moyamoya syndrome with an onset of acute neurologic manifestations between 4 and 32 years. Hypergonadotropic hypogonadism, azoospermia, short stature of postnatal onset (-2 to -4 SD in adulthood), premature graying of hair, and dysmorphism were present in all patients. The other features of the disease included early cataract in 4, dilated cardiomyopathy in 3, and partial growth hormone deficiency in 2 members. Muscle biopsy data did not reveal signs of a mitochondrial disorder. All conditions known to be associated with moyamoya syndrome such as Down syndrome, neurofibromatosis, and sickle cell disease were excluded. We also excluded linkage to the 2 loci previously reported to be involved in autosomal dominant syndromic and nonsyndromic moyamoya. Carrier females had normal phenotype and clinical history. CONCLUSIONS: These data strongly suggest that this family is affected by a hereditary moyamoya multisystem disorder with X-linked recessive pattern of inheritance.

Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach-Merritt syndrome.

Confusion in the nomenclature of vascular malformations has been a major obstacle to the understanding of these conditions, so that misdiagnosis and treatment inconsistencies are common. Coagulation abnormalities occurring in combination with venous malformations (VM) have been misdiagnosed as Kasabach-Merritt syndrome (KMS), despite marked differences in clinical features, pathology and treatment. A homogenous group of 24 patients with diffuse limb VM was entered into a retrospective chart review study. The VM affected an upper limb in 12 patients, a lower limb in 10 and both in two. Localized intravascular coagulation (LIC) was characterized by a decrease in fibrinogen (0.5-1 g/l), an increase in d-dimers (2-64 micro g/ml) and presence of soluble complex of fibrin (+ to +++). Platelet counts were normal or slightly decreased. Higher VM severity scores were associated with more severe LIC. A number of events such as sclerotherapy, surgery, bone fracture, prolonged immobilization and pregnancy or menstruation triggered conversion of the LIC to disseminated intravascular coagulation (DIC), with bleeding related to factor consumption and multiorgan failure related to disseminated microvascular thrombosis. Clinical symptoms associated with worsening of LIC were pain, thrombosis and bleeding at wound sites or during surgery. None of the patients had the large ecchymotic and inflammatory tumours seen in KMS. Graded permanent elastic compression with heparin therapy was the only effective treatment. In conclusion, VM-associated LIC is a distinctive lifelong coagulopathy that must be differentiated from KMS, which is characterized by platelet trapping within a vascular tumour of infancy. The treatment of the two conditions is very different.

A report of the clinical use of the detach-18 mechanical detachable platinum coil in 41 patients.

BACKGROUND AND PURPOSE: The purpose of this study was to determine the safety and reliability of the mechanical detachment system of a new platinum coil, Detach-18, when used as a vascular occlusive device for neurovascular disease. METHODS: Forty-one patients (nine male and 32 female patients; age range, 26-75 years; mean age, 54.4 years) underwent treatment at seven centers. Twenty-two patients had dural arteriovenous fistulae of the transverse sinus treated by a transvenous route. Fourteen patients underwent internal carotid artery occlusion in the treatment of aneurysms, meningioma, facial tumor, or carotid injury. One patient underwent occlusion of the basilar artery and one patient underwent occlusion of the vertebral artery for treatment of aneurysms. In two patients, coils were used as part of the treatment of their arteriovenous malformations. In all cases, the Detach-18 coils were delivered through a microcatheter with two distal markers. Two types of coils, a 0.015-inch-diameter "regular" coil and a 0.014-inch-diameter "soft" coil, were used. RESULTS: A total of 569 coils were used, 541 of which were detached. The number of coils in ranged from four to 53 (average number of coils, 14). The coils passed easily through the microcatheter. The detachment maneuver occurred within 10 to 25 s with 20 turns of the introducing wire. No premature coil detachment occurred. Complete occlusion of the vessel lumen was achieved in 35 cases. In three cases, 80% to 90% occlusion was achieved. In two cases, 70% to 80% occlusion was achieved. There were no device-related complications. CONCLUSION: The detachment system was safe, reliable, consistent, and fast. This is a useful system for coil embolization in neurovascular applications.

Carotid stenting for radiation-induced stenoses: A report of 7 cases.

BACKGROUND AND PURPOSE: Radiation-induced stenoses of the carotid artery are associated with fibrosis of the arterial layers and tissue planes that renders their surgical treatment difficult. We present our clinical experience in carotid angioplasty stenting (CAS) of patients harboring such stenoses. METHODS: Seven patients underwent transfemoral CAS of 10 radiation-induced stenoses located on either the common or the internal carotid artery. Six patients presented neurological symptoms. Four patients had undergone previous radical neck dissection, and 3 had permanent tracheostomies. Stenoses were primarily covered with a self-expandable stent before carotid dilation. RESULTS: All interventions were successful, with residual stenoses <20%. No permanent complication occurred. The mean follow-up was 8 months. Patients were symptom free at the last clinical examination, and Doppler control showed no evidence of restenosis. CONCLUSIONS: Carotid stenting appears very attractive for such "hostile neck" patients and seems a safe and efficient treatment for radiation-induced stenoses.

Linac radiosurgery for cerebral arteriovenous malformations: results in 169 patients.

PURPOSE: To present the SALT group results using Linac radiosurgery (RS) for AVM in 169 evaluable patients treated from January 1990 thru December 1993. METHODS AND MATERIALS: Median age was 33 years (range 6-68 years). Irradiation was the only treatment in 55% patients. Other treatment modalities had been used prior to RS in 45%: one or more embolizations in 36%, surgery in 6%, and embolization and surgery in 3% patients. Nidus were supratentorial in 94% patients, infratentorial in 6% patients. Circular 15 MV x-ray minibeams (6-20 mm) were delivered in coronal arcs by a GE-CGR Saturne 43 Linac. Patient set-up included a Betti arm-chair, a Talairach frame. Prescribed peripheral dose was 25 Gy on the 60%-70% isodose (max dose 100%). Arteriographic results were reassessed in December 1997 at 48 to 96 months follow-up. RESULTS: The overall obliteration rate (OR) was 64% (108/169). AVM volumes ranged from 280 to 19,920 mm(3), median 2460 mm(3). OR was 70% for AVM

Major complications of percutaneous embolization of skull-base tumors.

The technique of direct intratumoral injection of permanent liquid polymerizing agent was initially described in 1994 and has evolved significantly with experience. We report complications that occurred in two patients during injection of Histoacryl and offer suggestions to prevent such complications in the future. In one patient, the glue settled in the right middle cerebral artery; in the second, the glue entered the left ophthalmic artery through a collateral branch. Although the fundamental injection technique has not changed, we suggest additional precautions and modifications to make this procedure a safer and more valuable element in the overall management of patients with difficult skull-base tumors.

Combined management of intracranial aneurysms by surgical and endovascular treatment. Modalities and results from a series of 395 cases.

The selective occlusion of saccular intracranial aneurysms may be achieved by two techniques: microsurgical clipping and endovascular coiling. Each of them have particular indications which need to be defined. We report on a series in which both techniques were applied. From September 1992 to June 1996, 395 consecutive patients with small or large aneurysm were treated either by surgery (N = 102) or by endovascular coiling (N = 293). Coiling was chosen each time the shape of the aneurysm seemed to be appropriate for this treatment: narrow neck and ratio neck diameter by sac diameter less than one third. Satisfactory results with complete or subtotal obliteration and no recanalization on the following controls at 1, 6, 12 and 36 months, were obtained in 92% before retreatment and in 98.8% after retreatment. Unsatisfactory results were observed after surgery in 7 cases and in 25 cases after embolization. After retreatment, it remains 3 post-surgical and 2 post-endovascular cases. In the overall series, good and excellent clinical outcome was noted in 90% for small aneurysms and in 86.5% for large ones; mortality was of 4.8%. In a series in which were applied both types of treatment, surgery in 25% and endovascular technique in 75%, good results in terms of aneurysm occlusion and clinical results were achieved. These results are as good as the best series in which surgery was the only choice. Therefore with appropriate selection, endovascular treatment is a good alternative for treatment of the majority of saccular aneurysms.

Dural arteriovenous fistulas as a cause of intracranial hypertension due to impairment of cranial venous outflow.

OBJECTIVES: A retrospective study was carried out on 13 patients with intracranial dural arteriovenous fistulas (DAVFs) who presented with isolated or associated signs of intracranial hypertension. METHODS: Nine patients presented with symptoms of intracranial hypertension at the time of diagnosis. Ocular fundoscopy available in 12 patients showed bilateral papilloedema in eight and optic disk atrophy in four. Clinical evolution was particularly noticeable in five patients because of chronic (two patients) or acute (after lumbar shunting or puncture: three patients, one death) tonsillar herniation. RESULTS: Two patients had a type I fistula (drainage into a sinus, with a normal antegrade flow direction). The remaining 11 had type II fistulas (drainage into a sinus, with abnormal retrograde venous drainage into sinuses or cortical veins). Stenosis or thrombosis of the sinus(es) distal to the fistula was present in five patients. The cerebral venous drainage was abnormal in all patients. CONCLUSION: Type II (and some type I) DAVFs may present as isolated intracranial hypertension mimicking benign intracranial hypertension. Normal cerebral angiography should be added as a fifth criterion of benign intracranial hypertension. The cerebral venous drainage pattern must be carefully studied by contralateral carotid and vertebral artery injections to correctly evaluate the impairment of the cerebral venous outflow. Lumbar CSF diversion (puncture or shunting) may induce acute tonsillar herniation and should be avoided absolutely. DAVF may induce intracranial hypertension, which has a poor long term prognosis and may lead to an important loss of visual acuity and chronic tonsillar herniation. Consequently, patients with intracranial hypertension must be treated, even aggressively, to obliterate the fistula or at least to reduce the arterial flow and to restore a normal cerebral venous drainage. The endovascular treatment may associate arterial or transvenous embolisation and/or surgery. Patients in whom the fistula is not obliterated after an endovascular therapeutic procedure, need continuous clinical and angiographical follow up.

Therapeutic approach to arteriovenous malformations of the tongue.

Arteriovenous malformations (AVM) are developmental errors with a high complication rate because they are hemodynamically active. The aim of our study is to evaluate management of the rare subgroup of tongue AVM. From 1982 to 1994, 25 patients with AVM of the tongue presented to our department. All patients were discussed in our multidisciplinary staff consultation and were treated by embolization, surgery, or were followed-up clinically with no intervention. Thirteen patients were asymptomatic in a hemodynamically quiescent phase and did not require any kind of intervention. The 12 others underwent embolization procedures following which 6 became stable, 2 had complementary surgery, 1 was lost to follow-up, 1 had two minor bleeding episodes without the need for hospitalization, and 2 were improved. Arteriovenous malformations of the tongue must be followed clinically and treated only if they become active. Permanent embolization with a polymerizing liquid administered through supra-selective catheterization or by direct puncture of the malformation is recommended.

Management of intracranial aneurysms by surgical and endovascular treatment--modalities and results from a series of 395 cases.

The selective occlusion of saccular intracranial aneurysms may be achieved by two techniques: microsurgical clipping and endovascular coiling. Each of them have particular indications which need to be defined. From September 1992 to June 1996, 395 consecutive patients with small or large aneurysms were treated either by surgery (n = 102) or by endovascular coiling (n = 293). Coiling was chosen each time the shape of the aneurysm seemed to be appropriate for this treatment: narrow neck and ratio neck diameter by sac diameter less than one third. Satisfying results with complete or subtotal obliteration and no recanalization on the following controls at 1, 6, 12, and 36 months were obtained in 92% before retreatment and in 98.8% after retreatment. Unsatisfying results were observed after surgery in seven cases and in 25 cases after embolization. After retreatment, it remains three post-surgical and two post-endovascular cases. Good and excellent clinical outcome was noted in 90% for small aneurysms and in 86.5% for large ones. Mortality is of 4.8% in the overall series. In a series in which were applied both types of treatment, surgery in 25% and endovascular techniques in 75%, good results in terms of efficiency and clinical results were achieved. These results are as good as the best series in which surgery was the only choice. Therefore with appropriate selection, endovascular treatment is a good alternative for treatment of the majority of saccular aneurysms.

[Cerebromeningeal hemorrhages in Rendu-Osler disease. 2 cases treated by embolization]. / Hémorragies cérébroméningées de la maladie de Rendu-Osler. Deux cas traités par embolisation.

BACKGROUND: Treatment for cerebral vascular malformations in patients with hereditary hemorrhagic telangiectasia, also called Osler-Weber Rendu disease, has been greatly improved with advances in interventional neuroradiology. CASE REPORTS: Two patients with Osler-Weber Rendu disease experienced intracranial hemorrhage. In the first case, the disease was diagnosed at the age of 60 years in a man with recurrent epistaxis and numerous angiomas on the face and trunk as well as in the buccal cavity. The first neurological signs appeared seven years later and brain MRI revealed a 2-3 cm temporo-occipital arteriovenous malformation. Six months later sudden onset headache following exertion led to a CT-scan which showed tetraventricular hemorrhage. The second case was a 63-year-old man referred to neurosurgery for cerebromeningeal hemorrhage. Clinical examination and history taking found several small skin angiomas and a family and personal history of epistaxis. CT-scan revealed a dural fistula in the posterior fossa. Good clinical outcome was obtained in both cases by endovascular embolisation. DISCUSSION: Intracranial hemorrhage is observed in 2 to 3% of patients with Osler-Weber Rendu disease. Telangiectasia is not a pathognomonic sign and non-specific vascular malformations may be observed, particularly arteriovenous malformations or arterial aneurysms. Most can be reached by endovascular catheterism. Most of these usually unique malformations can be successfully treated by first-line embolisation.

[Persistent trigeminal artery and its variant. Contribution of MRI and MR-angiography]. / La persistance de l'artere trigéminée et sa variante. Apport de l'IRN et de l'angio-RM.

We describe the magnetic resonance imaging and magnetic resonance angiography findings in 5 cases of persistent trigeminal artery, and in two cases of variant trigeminal artery. Six patients underwent complete four-vessel angiography, performed to depict other pathology. Magnetic resonance imaging and magnetic resonance angiography allowed in each case to visualize this artery and to precise its latero or intrasellar situation. The variant is not defined on conventional magnetic resonance imaging, but may be individualized on magnetic resonance angiography. In each case, identification of these persistent artery is crucial if surgery of sellar or gasserian region is planned.

Treatment of brain arteriovenous malformations by embolization and radiosurgery.

Embolization was used to reduce the size of brain arteriovenous malformations (AVMs) prior to radiosurgical treatment in 125 patients who were poor surgical candidates or had refused surgery. Of these patients, 81% had suffered hemorrhage, and 22.4% had undergone treatment at another institution. According to the Spetzler-Martin scale, the AVMs were Grade II in 9.6%, Grade III in 31.2%, Grade IV in 30.4%, and Grades V to VI in 28.8% of the cases. Most embolizations were performed using cyanoacrylate delivered by flow-guided microcatheters. Radiosurgery was performed using a linear accelerator in 62 patients treated by the authors, and 34 patients were treated at other institutions using various methods. Embolization produced total occlusion in 11.2% of AVMs and reduced 76% of AVMs enough to allow radiosurgery. Radiosurgery produced total occlusion in 65% of the partially embolized AVMs (79% when the residual nidus was < 2 cm in diameter). Embolizations resulted in a mortality rate of 1.6% and a morbidity rate of 12.8%. No complications were associated with radiosurgery. The hemorrhage rate for partially embolized AVMs was 3% per year. No patient with a completely occluded AVM experienced rehemorrhage. Angiographic follow-up review of AVMs embolized with cyanoacrylate demonstrated a 11.8% revascularization rate, occurring within 1 year. It is concluded that after partial embolization with cyanoacrylate, the risk of hemorrhage from the residual nidus is comparable to the natural history of AVMs and that the residual nidus can be irradiated with results almost as good as for a native AVM of the same size.

[Treatment of 315 intracranial aneurysms using electrically controlled detachable coils]. / Traitement par spires (coils) à détachement contrôlé électrique de 315 anévrysmes intracrâniens.

The purpose of this study was to evaluate the results of 315 intracranial saccular aneurysms in 290 patients selectively treated with GDC in a single Center. One hundred and fifty eight patients were treated at the acute phase of the sub-arachnoid hemorrhage (SHA), sixty patients were treated more than three weeks after the SHA, seventy two patients were treated for unruptured aneurysms. We found at three months post-embolization good clinical results in 88,6%, a moderate disability in 3,4%, a severe disability in 2,7%, a 5,2% mortality. The complete aneurysm occlusion rate was 80,6% on the immediate control angiogram. Smaller the aneurysms, better were the anatomical results. The GDC treatment appears as an alternative treatment of surgery for the ruptured aneurysms at the acute-phase of SHA.