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Masaru Yamada, Takao Kitahara, Akira Kurata, Kiyotaka Fujii, and Yoshio Miyasaka

Object. Intracranial vertebral artery (VA) dissection with subarachnoid hemorrhage is notorious for frequent rebleeding and a poor prognosis. Nevertheless, some patients survive with a good final outcome. The factors associated with the prognosis of this disease are not fully understood and appropriate treatment strategies continue to be debated. The authors retrospectively evaluated the clinical features of conservatively treated patients to elucidate the relationship between the clinical and angiographic characteristics of the disease and final outcomes.

Methods. This study includes 24 patients who were treated by conservative methods between 1990 and 2000. Conservative treatment was chosen because of delayed diagnosis, poor clinical condition, or anatomical features such as bilateral lesions and contralateral VA hypoplasia.

Of nine patients with an admission Hunt and Kosnik Grade I or II, eight had good outcomes (mean follow-up period 8 years and 4 months). All 15 patients with Grade III, IV, or V died and in 10 of these the cause of death was rebleeding. Among the 24 patients, 14 suffered a total of 35 rebleeding episodes; in 10 (71.4%) of these 14 patients rebleeding occurred within 6 hours and in 13 (93%) within 24 hours. Compared with the survivors, there was a female preponderance (0.022) among patients who died. These patients also had significantly shorter intervals between onset and hospital admission (p = 0.0067), a higher admission Hunt and Kosnik grade (p = 0.0001), a higher incidence of prehospitalization (p = 0.0296) and postadmission (p = 0.0029) rebleeding episodes, and a higher incidence of angiographically confirmed pearl-and-string structure of the lesion (p = 0.0049).

Conclusions. In our series of preselected patients, poor admission neurological grade, rebleeding episode(s), and lesions with a pearl-and-string structure were predictive of poor outcomes. Our findings indicate that patients with these characteristics may be candidates for aggressive attempts to prevent rebleeding during the acute stage. Patients without these characteristics may be good candidates for conservative treatment, especially those who survive the acute phase without rebleeding.

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Akira Kurata, Hidehiro Oka, Taketomo Ohmomo, Hitoshi Ozawa, Sachio Suzuki, Kiyotaka Fujii, Shinichi Kan, Yoshio Miyasaka, and Harue Arai

✓ This 44-year-old man with Ehlers—Danlos syndrome (EDS) Type IV presented with hemiparesis and the Gerstmann syndrome. Left carotid artery (CA) angiography revealed a dissecting aneurysm with severe stenosis located in the common CA; the lesion was successfully treated with a stent graft. The patient's clinical course after endovascular surgery was uneventful, without occurrence of megacolon. The literature for spontaneous CA dissection in EDS Type IV cases is reviewed and points for investigation and treatment are discussed.

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Yoshio Miyasaka, Kenzoh Yada, Takashi Ohwada, Takao Kitahara, Akira Kurata, and Katsumi Irikura

✓ The authors studied the venous drainage system and its impairment in relation to risk of hemorrhage in 108 cases of supratentorial arteriovenous malformation (AVM). The proportion of AVM's undergoing hemorrhage (hemorrhagic rate) was calculated in relation to: 1) the number of draining veins (one, two, or three or more); 2) the presence or absence of impairment in venous drainage (severe stenosis or occlusion in draining veins); and 3) the location of draining veins (deep venous drainage alone, superficial venous drainage alone, or a combination of the two). Statistical analysis demonstrated that AVM's with the following characteristics had a high risk of hemorrhage: 1) one draining vein (hemorrhagic rate 89% in 54 patients); 2) severely impaired venous drainage (hemorrhagic rate 94% in 18 patients); and 3) deep venous drainage alone (hemorrhagic rate 94% in 32 patients).

The present study suggests that the venous drainage system of AVM's is significantly associated with the risk of hemorrhage of these lesions. Therefore, careful preoperative angiographic evaluation of the venous drainage system is mandatory for decision making in the management of patients with AVM's.

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Akira Kurata, Yoshio Miyasaka, Takatomo Yoshida, Masatake Kunh, Kenzoh Yada, and Shinichi Kan

✓ A case is presented of tentorial dural arteriovenous malformation (AVM) associated with visual hallucinations and quadrant hemianopsia. Computerized tomography (CT) and magnetic resonance imaging showed an ischemic region, mainly affecting the white matter of the right occipital lobe, that was defined as an area of increased blood volume on dynamic CT scans and as a decrease in cerebral blood flow on N-isopropylp-123I-iodoamphetamine single-photon emission CT scans. Angiography demonstrated venous congestion, probably because the retrograde arterial inflow from the dural AVM into the corticomedullary vein was direct and not via the sinuses. The symptoms and radiological findings improved immediately after endovascular treatment. The origin of these symptoms was fully evaluated and confirmed to be a reversible ischemic change caused by disturbance of the volume of venous return over an extensive area.

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Chieki Wada, Akira Kurata, Ryuichi Hirose, Yoshiaki Tazaki, Shinichi Kan, Yoshihiro Ishihara, and Toru Kameya

✓ Ependymoblastoma is considered to be a primitive malignant glioma with ependymal differentiation. This rare tumor occurs in very early life and shows rapid growth and a diffuse infiltration through the leptomeningeal space. The tumor cells are highly immature, with numerous mitoses and multilayered ependymal rosettes. The ependymoblastoma described in this report was found in a 17-year-old girl. In spite of detailed clinical and postmortem examinations, no definite primary site was identified in the neuraxis. The lesion spread predominantly throughout the leptomeningeal space. Histological analysis strongly suggested that this tumor originated from a heterotopic glial nest in the subarachnoid space. The absence of immunohistochemical neural tissue markers, glial fibrillary acidic protein, S-100 protein, neuron-specific enolase, and neurofilaments ruled out neuronal or glial differentiation. The authors were unable to find any previous report of primary leptomeningeal ependymoblastoma.

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Yoshio Miyasaka, Kenzoh Yada, Takashi Ohwada, Takao Kitahara, Masataka Endoh, Motoyoshi Saito, Akira Kurata, and Hirotoshi Ohtaka

✓ Five cases of retrograde thrombosis of former feeding arteries after removal of an arteriovenous malformation (AVM) are reported. The clinical features of these patients were studied and compared to those of 71 patients without this complication. The following characteristics were found to correlate with retrograde thrombosis: 1) advancing age of the patient; 2) large AVM size; and 3) markedly dilated and elongated feeders. It is suggested that the slow flow in the former feeding arteries that was observed immediately after AVM removal and pathological changes in these vessels due to long-standing hemodynamic stresses contributed to the development of retrograde thrombosis. Neurological manifestations related to retrograde thrombosis were noted in three of the five cases. Although infrequent, this complication should be considered as a serious possibility following removal of an AVM.

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Sachio Suzuki, Akira Kurata, Taketomo Ohmomo, Takao Sagiuchi, Jun Niki, Masaru Yamada, Hidehiro Oka, Kiyotaka Fujii, and Shinichi Kan

✓Application of endovascular surgery for very small aneurysms is controversial because of technical difficulties and high complication rates. The aim in the present study was to assess treatment results in a series of such lesions at one institution.

Since 1997, endovascular surgery has been advocated for very small ruptured aneurysms (<3 mm in maximum diameter) that fulfill the criterion of a fundus/neck ratio greater than 1.5. Twenty-one patients were treated, for whom the World Federation of Neurosurgical Societies classification before treatment was Grade I in 10, Grade II in two, Grade III in two, Grade IV in five, and Grade V in two. The aneurysm location was the internal carotid artery in four, the anterior communicating artery in 11, the middle cerebral artery in one, and the vertebrobasilar system in five. In all patients, endovascular surgery was performed using Guglielmi detachable coils after induction of general anesthesia. Initially, the presumed volume of the lesions was calculated for each aneurysm. Thereafter, the appropriate coil length was decided according to the volume embolization ratio, as 30 to 40%. In all attempts to obliterate aneurysms a single coil was used.

All aneurysms were completely obliterated as confirmed by postembolization angiography, without procedure-related complications. During the follow-up period only one patient needed additional coil embolization for a growing aneurysm. Final outcomes were good recovery in 15 patients, moderate disability in five, and severe disability in one.

Appropriate selection of patients and coils, and use of sophisticated techniques allow a good outcome for patients with very small aneurysms.

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Akira Kurata, Sachio Suzuki, Kazuhisa Iwamoto, Kuniaki Nakahara, Madoka Inukai, June Niki, Kimitoshi Satou, Masaru Yamada, Kiyotaka Fujii, Shinichi Kan, and Toshiro Katsuta

Object

The transvenous approach via the inferior petrosal sinus (IPS) is commonly used as the most appropriate for carotid-cavernous fistula (CCF) or cavernous sinus sampling. However, sometimes the IPS is not accessible because of anatomical problems and/or complications, therefore an alternative route is needed. In this paper, the authors present and discuss the utility of a transvenous approach to the cavernous sinus via the inferior petrooccipital vein.

Methods

Four patients, 3 with dural CCFs and the other with Cushing disease, in whom endovascular surgical attempts failed using a conventional venous approach via the IPS, underwent a transvenous approach to the cavernous sinus via the inferior petrooccipital vein (IPOV). One dural CCF case had only cortical venous drainage, the second CCF also mainly drained into the cortical vein with slight inflow into the superior ophthalmic vein and inferior ophthalmic vein, and the third demonstrated drainage into the superior and inferior ophthalmic veins and IPOV.

Results

In all cases, the cavernous sinus could be accessed successfully via this route and without complications.

Conclusions

The transvenous approach to the cavernous sinus via the IPOV should be considered as an alternative in cases when use of the IPS is precluded by an anatomical problem and there are no other suitable venous approach routes.