Sanami Kawada

Long-term results of surgically treated Moyamoya disease

Various surgical procedures have been tried for patients with Moyamoya disease. The most effective treatment, however, is still controversial. We retrospectively evaluated the long-term results of 71 patients (26 men and 45 women) with Moyamoya disease surgically treated in our institute. They consisted of 56 pediatric patients (younger than 15 years) and 15 adult patients. Symptoms in all patients were due to cerebral ischemia. We did 123 operations on 119 hemispheres: 18 superficial temporal artery--middle cerebral artery (STA-MCA) anastomoses, six STA-MCA anastomoses with indirect bypass (IB), 41 encephalo-duro-arterio-synangiosis (EDAS), 29 encephalo-duro-arterio-myo-synangiosis (EDAMS) and 29 ribbon EDAMS. Average follow-up periods for each procedure were: 7 years for STA-MCA anastomosis, 6.2 years for STA-MCA anastomosis with indirect bypass, 11 years for EDAS, 5.6 years for EDAMS and 2.6 years for ribbon EDAMS, respectively. The results of each procedure were satisfactory because the preoperative transient ischemic attacks disappeared. Analysis of follow-up angiograms shows excellent filling of the ACA and MCA territory in the patients undergoing ribbon EDAMS. However, long-term follow-up study shows that about 10% of the patients had severe difficulty in social or school life because of intellectual impairment.

Long-term follow-up study of patients with unilateral Moyamoya disease

Although a number of cases of unilateral Moyamoya disease have been reported, the natural history of this disease remains unclear. The clinical features of 17 patients initially diagnosed with unilateral Moyamoya disease at our hospital are reported. Age at onset was 3-45 years (mean, 13.5). Of these 12 cases had onset of symptoms in childhood and five had onset in adulthood. Seven were male and 10 were female. An ischemic attack was the initial episode in ten of the 12 pediatric cases, two of the five adult cases presented with intraventricular hemorrhage. Of the 12 pediatric patients six developed contralateral lesions between 4 and 34 months (mean, 20) after the diagnosis of a unilateral lesion. The remaining six pediatric patients and all adult patients did not develop lesions on the normal side. The mean age at onset for patients later developing contralateral lesions was 6.2 years. The pediatric cases remaining unilateral was 7.7 years. The normal hemisphere of three of the pediatric patients has remained unchanged on repeated follow-up angiograms for over 10 years. Young children tended to develop the vascular pathology bilaterally. However, there were some pediatric cases whose normal or atypical sides remained unchanged without development of bilateral lesions.

Spinal atypical teratoid/rhabdoid tumor in an infant.

Atypical teratoid/rhabdoid tumor of the central nervous system in infancy and childhood was established as an entity based on histological, immunohistochemical, and cytogenetic studies. We report the case of a 7-month-old girl who presented with progressive paraplegia and hypesthesia of her legs. Imaging studies revealed a spinal cord mass occupying the entire spinal canal below the T7 level. Through a T12-L3 laminectomy, the intramedullary tumor was partially debulked. Histologically, the tumor specimen had rhabdoid cells, and immunostaining showed vimentin and cytokeratin positivity. No abnormality of chromosome 22q was detected with the fluorescence in situ hybridization method.

Effect of external stenting and systemic hypertension on intimal hyperplasia in rat vein grafts.

OBJECTIVE Overdistention of vein grafts in arterial circulation and systemic hypertension are thought to be influential risk factors contributing to vein graft failures. This study tested the effects of external stenting in preventing systemic hypertension and overdistention of the rat vein graft in the long term. METHODS Jugular vein grafts were interposed into the carotid artery of normotensive (n = 39) and two-kidney, one-clip hypertensive (n = 30) rats. Jugular vein grafts wrapped with 1.5-mm-diameter polyester stents were used in normotensive (n = 26) and hypertensive (n = 25) rats. The vein grafts were harvested at 1, 2, 4, 8, 12, and 24 weeks after the grafting procedure. The neointimal area and wall thickness were measured by computerized planimetry, and Ki-67 immunohistochemistry was used to detect replicative smooth muscle cells in the graft wall. RESULTS In each group, intimal hyperplasia was apparent at 1 week and increased gradually to 24 weeks. The number of Ki-67-positive cells was most increased at 2 weeks after the grafting procedure and gradually decreased thereafter. The numbers of Ki-67-positive cells and the extent of intimal hyperplasia were not significantly different between normotensive and hypertensive rats. Both neointima formation and cell proliferation in the graft wall were significantly reduced by external stenting as compared with the results with unstented grafts. CONCLUSION Systemic hypertension by itself is not a risk factor for intimal hyperplasia and experimental vein graft failure in the long term. External stenting is effective against intimal hyperplasia, and it is possible to reduce the subsequent atherosclerotic change of the vein graft wall and improve the long-term patency of the vein graft with external stenting.

Embolization of cerebral arteriovenous malformations with cellulose acetate polymer: a clinical, radiological, and histological study.

OBJECTIVE A cellulose acetate polymer (CAP) solution was hypothesized to be useful for the embolization of arteriovenous malformations (AVMs). To investigate this possibility, we analyzed the clinical, radiological, and histological results of patients with AVMs embolized by using a CAP solution. METHODS We reviewed the cases of 11 patients with AVMs treated by embolization before surgical resection. We used two types of CAP solutions, CAP-M and CAP-L, which are mixtures of 250 mg of solid CAP and 1800 or 2250 mg of bismuth trioxide dissolved in 5.5 or 7.0 ml of dimethyl sulfoxide, respectively. After the embolization procedure, percent reduction of the nidus volume was measured, and thereafter, the clinical course and computed tomographic scans and/or magnetic resonance imaging scans were evaluated. All patients underwent surgical resection 1 to 51 days after embolization. Resected specimens were stained for light microscopic examination. RESULTS Thirty-nine feeding vessels were embolized. The reduction rate of the nidus volume ranged from 20% to nearly 100%. Transient and persistent ischemic deficits occurred in three patients and one patient, respectively, and there were no hemorrhagic complications. All AVMs but one were completely resected by surgery. The embolized AVMs were soft enough to be easily cut and retracted. The histological examinations disclosed no or mild inflammatory reactions within 2 weeks after embolization. The internal elastic lamina was preserved in every case. Recanalization through the cast of the CAP mass was not observed until 51 days after embolization. CONCLUSION CAP solution is a safe and useful embolic agent for AVMs.

Experimental Study of Intracisternal Administration of Tissue-Type Plasminogen Activator Followed by Cerebrospinal Fluid Drainage in the Ultra-Early Stage of Subarachnoid Haemorrhage

Summary¶ This experimental study evaluated the effect of intrathecal injection of tissue-type plasminogen activator followed by cisternal drainage in the ultra-early stage of aneurysmal subarachnoid haemorrhage to prevent vasospasm. Twenty Japanese white rabbits were divided into five groups. Either tPA (groups A, B, and E) or saline (groups C and D) was injected intrathecally 1 hour (groups A, B, C, and D) or 21 hours (group E) after the intrathecal injection of blood. Cerebrospinal fluid was drained 2, 4, and 6 hours after the intrathecal injection of blood (groups A, C, and E). On day 4, the angiographic caliber of the basilar artery in each group was as follows (mean±SD): A, 85.9±5.0%; B, 74.6±5.3%; C, 69.1±2.7%; D, 64.0±4.9%; E, 80.2±2.7% (compared with baseline). In the two groups in which CSF was drained (groups A and C), fibrinolysis with tPA significantly suppressed vasospasm. In the two groups treated with tPA (groups A and B), cisternal drainage significantly suppressed vasospasm. In the two groups treated with saline (groups C and D), however, cisternal drainage did not suppress vasospasm. Examination of the series of CSF samples (groups A and C) showed that fibrinolysis with tPA effectively cleared clots early. In the two groups treated with tPA and CSF drainage (groups A and E), early removal of subarachnoid clots reduced the degree of vasospasm. Early fibrinolysis with tPA and early removal of subarachnoid clots by drainage is effective for preventing vasospasm.

Progress in the management of patients with aneurysmal subarachnoid hemorrhage: A single hospital review for 20 years: Part I: Younger patients

BACKGROUND This study was carried out to clarify if there has been any improvement in the outcome of patients with aneurysmal subarachnoid hemorrhage during the past 20 years. Because elderly patients have apparently poorer prognoses than younger patients, patients older than 70 years were analyzed separately in the following article. METHODS Five hundred seventy-one patients with aneurysmal subarachnoid hemorrhage, under 70 years, who were consecutively admitted to Kagawa Prefectural Central Hospital from July 1972 to December 1992, were reviewed. These patients were divided into four groups according to the time of admission. The ultimate outcome was evaluated by means of Glasgow Outcome Scale 6 months after the ictus. RESULTS Changes in treatment protocol in this period included the induction of early surgery and the invention of a variety of modalities for the treatment of cerebral vasospasm. This resulted in a distinct increase in patients who actually underwent direct aneurysm clipping. Outcome has been significantly improved during this period, especially in patients with Hunt and Kosnik grade III (p<0.05, chi2). Patients in good clinical condition at follow-up (Glasgow Outcome Scale: good recovery) increased from 8.7% to 60.7%. Mortality decreased from 28.7% to 10.7%. CONCLUSIONS Current therapeutic modalities have significantly improved the outcome of patients with aneurysmal subarachnoid hemorrhage. Rebleeding before early surgery remains as a major cause of unfavorable outcome and further progress on this subject is mandatory.

Embolization of cerebral arteriovenous malformations with cellulose acetate polymer. Histological study of the resected specimens.

To test the usefulness of a cellulose acetate polymer(CAP) solution for brain arteriovenous malformations (AVMs), we analyzed the clinical and histological results of patients with AVMs embolized using CAP solution. We reviewed the cases of six patients with cerebral AVMs treated by embolization prior to surgical resection. We used two types of CAP solutions, CAP-M and CAP-L, which are mixtures of 250 mg of solid CAP and 1800 or 2250 mg of bismuth trioxide dissolved in 5.5 or 7.0 ml of dimethyl sulfoxide, respectively. All patients underwent surgical resection 1 to 37 days after the embolization procedure. Resected specimens were fixed in formalin and stained for light microscopic examination. Eighteen feeding vessels were embolized. The reduction rate of the nidus volume was between 20% and nearly 100%. Mild ischemic deficits occurred in one patients but there were no hemorrhagic complications related to the embolization procedures. All AVMs were completely resected by surgery. Direct inspection at surgery revealed that there was no apparent swelling or hematomas in the normal brain areas adjacent to the nidus, and the AVMs were soft enough to be easily retracted. The histological examinations disclosed no or mild inflammatory reactions within two weeks after embolization. The internal elastic lamina was preserved in every case. Recanalization of embolized vessels was not observed until 37 days after embolization. CAP solution is a safe and useful embolic agent for brain AVMs. Further study is needed to resolve the issue of recanalization.