Revascularizacion cerebral en el moyamoya del adulto / Cerebral revascularization in adult moyamoya

Introducción: el Moyamoya (MM) es una enfermedad crónica esteno-oclusiva de los vasos arteriales intracraneanos asociado al desarrollo secundario de arteriolas dilatadas en la base del cerebro. La historia natural de la enfermedad predispone a infartos y/o hemorragias cerebrales. El objetivo del presente trabajo es mostrar nuestra experiencia en el manejo quirúrgico del MM del adulto. Material y método: se realizó una revisión retrospectiva de 11 pacientes adultos con diagnóstico de MM tratados entre mayo 2014 y mayo 2020. Se analizaron las historias clínicas, estudios diagnósticos, protocolos y videos operatorios. En el preoperatorio se utilizó la escala angiográfica de Suzuki y para la valoración clínica pre y postoperatoria la escala de Rankin modificada. Resultados: se operaron 11 pacientes (8 femeninos), 9 son portadores de EMM y 2 con SMM secundario a NF1 y enfermedad de Hashimoto. Se realizaron 16 procedimientos de revascularización en 11 pacientes (en 5 pacientes la revascularización cerebral fue bilateral), 12 fueron combinados (bpTS más EDMS) y en 4 sólo sinangiosis tipo EDAMS.El aumento del flujo sanguíneo cerebral se constató en todos los casos mediante ADC luego de los 6 meses de la cirugía. La permeabilidad del bpTS se comprobó en 10 de 12 bpTS (83.3 %) y la revascularización de la sinangiosis fue muy buena en todos los pacientes. El 82 % de los pacientes mejoraron un punto o más en la escala de Rankin y un paciente presentó un infarto frontal en TAC postoperatoria sin repercusión clínica. Conclusiones: la evolución natural de la enfermedad conduce al deterioro neurológico por ACV isquémico y/o hemorrágico. En nuestra experiencia, la mejor opción terapéutica en el adulto es la revascularización cerebral combinada porque disminuye la incidencia de ACV con resultados favorables y escasa morbilidad

Introduction: Moyamoya is a chronic steno-occlusive disease of the intracranial vessels associated to the secondary development of dilated arterioles at the base of the brain. The natural history of the disease predisposes to cerebral infarcts and/or hemorrhages. The objective of this paper is to show our experience and surgical management of adult moyamoya. Materials and Methods: retrospective review of 11 adult patients with moyamoya treated from May 2014 to May 2020 was performed. Clinical charts, neuroimaging studies, operative records and surgical videos were analyzed. The Suzuki scale was used as a preoperative score, and the modified Rankin scale was used for pre and postoperative clinical assessment. Results: Eleven patients (8 females) were operated at our Institution: 9 are MMD and 2 MMS secondary to NF1 and Hashimoto disease. We performed 16 cerebral revascularizations in 11 patients (in 5 patients thecerebral revascularization was bilateral), 12 were combined (STA-MCA bypass plus EDMS) and in only 4 EDAMS synangiosis was done.The increase in cerebral blood flow was verified in all cases by follow-up DSA at 6 months. The STA-MCA bypass permeability was verified in 10 of 12 cases (83.3%) and the revascularization by synangiosis was demonstrated in all patients. Clinical follow-up showed that 82 % of the patients improved one point or more on the modified Rankin scale and one patient presented a frontal infarction on postoperative CT without clinical worsening. Conclusions: The natural course of the disease leads to neurological deterioration due to ischemic and hemorrhagic stroke. According to our experience, the best therapeutic option in adults is combined cerebral revascularization because it decreases the incidence of stroke with good results and low morbidity

Postoperative Transient Neurologic Dysfunction: A Proposal for Pathophysiology

BACKGROUND AND PURPOSE: Sudden neurological deterioration which cannot be explained by structural change, ischemia or seizure is often observed among neurosurgical patients. We aimed to provide new insight into the pathophysiology of postoperative transient neurologic dysfunction. METHODS: We describe prolonged but fully reversible focal neurologic dysfunction of unknown origin based on the initial evaluation in 8 patients who had received encephalo-duro-arterio-synangiosis for moyamoya disease. We performed brain imaging, including diffusion weighted imaging and perfusion magnetic resonance imaging or single photon emission computed tomography, and electroencephalography (EEG) during the episodes and after resolution of the symptoms. RESULTS: The symptoms consisted of dysarthria, hemiparesis, or hemiparesthesia of limbs contralateral to the operated side. These symptoms developed between 12 hours and 8 days after surgery and lasted between 12 hours and 17 days. Structural imaging did not show any significant interval change compared with the immediate postoperative images. Perfusion imaging showed increased cerebral blood flow in the symptomatic hemisphere. EEG revealed low amplitude arrhythmic slowing in the corresponding hemisphere. Follow-up imaging and EEG after recovery did not show any abnormalities. CONCLUSIONS: Transient neurologic dysfunction can occur during the postoperative period of brain surgery. Although this may last more than usual transient ischemic attack or seizure, it eventually resolves regardless of treatment. Based on our observation, we propose that this is the manifestation of the transient cortical depression triggered by mechanical stimulation, analogous to migraine aura associated with cortical spreading depression.

Clinical features and surgical efficacy of moyamoya syndrome in children / 中国脑血管病杂志

Objective Toinvestigatetheclinicalfeaturesandsurgicalprognosisofmoyamoya syndromeinchildren.Methods Theclinicaldataof12childrenwithmoyamoyasyndromeadmittedto the 307th Hospital of People′s Liberation Army from December 2002 to October 2013 were analyzed retrospectively. Eleven of them underwent encephalo-duro-arterio-synangiosis (EDAS). A total of 550 children with moyamoya disease in the same period were used as a control group. The clinical characteristics and surgical efficacy of the children with moyamoya syndrome were summarized and concluded by comparing the clinical data of the two groups,including sex,age of onset,initial symptom,progress symptoms, Suzukiinstallments,imagingfeatures,andsurgicalefficacy.Results Themaleandfemaleratioof the children with moyamoya syndrome was 1∶2. Their mean age of onset was 12 ± 5 years old. There were significant differences in the initial symptom (cerebral infarction and cerebral hemorrhage )and disease progress between the children with moyamoya syndrome group and the control group (5/12 vs. 14. 5%[80/550], 3/12 vs. 61. 8%[340/550],and 5/12 vs. 8.7%[48/550],respectively;all P<0. 05). Within the follow-up period,of the 11 children underwent EDAS,7 cases had no further attack,and 4 cases were improved significantly. There was significant difference in the modified Rankin scale (mRS)between the beforeandaftersurgery(0[0,1]vs.2[1,2];P<0.05).Conclusions Theclinicalfeaturesofthe children with moyamoya syndrome have some differences with those with moyamoya disease. Timely and effective EDAS treatment may effectively prevent disease progression and improve the prognosis of patients.

Fibromuscular Dysplasia with Cerebral Infarction in a Child: Case Report

An 11-year-old child presented with left hemiparesis and a seizure caused by fibromuscular dysplasia of the right internal carotid artery with cerebral infarction is presented. On conventional angiography, the authors found the classical `beaded' lesions. These pathognomic changes were most important to diagnosis. On brain single-photon emission computed tomography, we found the impaired vascular reserve of right basal ganglia, frontal and temporal area. We tried an antiplatelet agent and an encephaloduroarterio-synangiosis on the right hemisphere.

The Role of Basic Fibroblast Growth Factor in Moyamoya Disease

OBJECTIVES AND OMPORTANCE: The present study investigated the levels of basic fibroblast growth factor(bFGF) in the CSF of patients with moyamoya disease and its clinical significance. METHODS: The levels of bFGF in CSF, taken from 26 hemispheres of 14 moyamoya patients and 20 patients without vascular anomaly(control group), were measured by an enzyme-linked immunosorbent assay. We analyzed the correlation between the level of bFGF and the clinical factors such as age, onset pattern, development of neovascularization, and cerebral circulation. RESULTS: The CSF of moyamoya patients contained a high concentration of bFGF to a significant extent. The bFGF level was apparently elevated in the patients in whom neovascularization from indirect revascularization, such as modified encephalo-duro-arterio-synangiosis(EDAS) was well developed. A linear correlation between the values of bFGF and clinical progression was noted. CONCLUSIONS: The elevation of bFGF in moyamoya disease seems to be specific. Clinically, the bFGF level may be considered a useful indicator to predict the efficacy of indirect revascularization.

Two Operative Cases of Moya Moya Disease in Children

The treatment of moya moya disease, a chronic occlusive cerebrovascular disease of unknown etiology, isn't settled ut various operative methods to maximize cerebral revascularization have been reported. Two cases in children treated surgically are presented, one with cerebroarteriosynangiosis and the other with encephalo-duro-arterio-synangiosis(EDAS). The methods of cerebral revascularization are discussed in detail.

Treatment of Cerebral Rete Mirabile in Children by Encephalo-duro-arterio-synangiosis: Report of Two Operative Cases

Cerebral rete mirabile is a unusual form of chronic cerebrovascular occlusive disease characterized usually by bilateral stenosis of distal internal carotid arteries and their vicinity, by a hazy network of collateral circulation at the base of brain called moyamoya vessels and clinically by recurring hemispheric ischemic attack in children. We have reported here 2 cases of cerebral rete mirabile in children and performed newly developed operative procedure which we think is an ideal surgical method for treatment of this disease in children and is compared with other surgical treatment.