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Introducción: Las malformaciones arteriovenosas cerebrales (MAV) son un conjunto anormal de arterias y venas dilatadas del cerebro y caracterizadas por la pérdida de la organización vascular con una derivación arteriovenosa anormal. La probabilidad de ruptura de una MAV es baja, pudiendo causar déficit hasta en el 45% de los pacientes. La epilepsia puede asociarse con MAV siendo raro el estado epiléptico. La evolución a estado epiléptico refractario o estado epiléptico super refractario (EESR) es excepcional. Se presenta paciente con epilepsia asociada a MAV, con embolización y posterior complicación que evoluciona a EESR, donde se logró una resolución favorable con cirugía. Caso clínico: Paciente masculino de 70 años, con MAV temporal derecha no rota y epilepsia, libre de crisis desde el diagnóstico de ambas condiciones. Se realizó embolización logrando exclusión, pero con evolución a EESR. Tras estudio se realiza resección de MAV y resección parcial de zona de inicio de actividad epileptógena parieto-temporal. Además, se realizó una desconexión de región fronto-parietal central donde había evidencia de propagación de la actividad epiléptica. Paciente logra salir de estado crítico, con recuperación de conciencia y normalización de electroencefalogramas posquirúrgicos. A los 2 años evoluciona con epilepsia controlada (Engel IIa). Discusión y conclusiones: El abordaje quirúrgico es una opción en EESR y debe plantearse en un tiempo precoz de evolución (1-2 semanas). La cirugía se realizó para controlar EESR y fue una intervención exitosa. Esta estrategia puede lograr un cambio dramático en el pronóstico. La cirugía resectiva está indicada cuando hay evidencia etiológica en imágenes y focalidad eléctrica consistente en los estudios.
Introduction: Brain Arteriovenous Malformations (AVM) are an abnormal set of dilated arteries and veins within the brain and are characterized by loss of vascular organization with an abnormal arteriovenous shunt. The probability of AVM rupture is low, but it can cause deficits in up to 45% ofpatients. Epilepsy could be associated with AVM, and status epilepticus is rare. The evolution to refractory status epilepticus or super refractory status epilepticus (SRSE) is very rare. The objective is to present a patient with epilepsy associated with non-ruptured AVM, treated with embolization and a subsequent complication that evolves into SRSE, in which a favorable resolution was achieved after surgery Clinical case: 70-year-old male, with a diagnosis of non-ruptured right temporal AVM and epilepsy, who has been seizure free since the diagnosis, is reported. Embolization of the AVM was performed, achieving total exclusion, after the procedure presented seizures that evolved into SRSE. After investigation, an AVM resection and partial resection of the ictal onset zone on the right parieto-temporal region was performed, including a disconnection of the central fronto-parietal region, where there was evidence of propagation of ictal activity. Patient recovered from the critical condition after surgery, and also presented electrographic normalization. After 2 years, the epilepsy is well controlled (Engel IIa). Discussion and conclusions: The surgical approach is an option in SRSE, and it should be proposed early on, within a reasonable time of evolution (1-2 weeks). The active approach in this case, where the surgery was done for SRSE control, was a successful intervention. Especially when the features are consistent -there is etiological evidence in imaging and electrical focality in studies- there can be a dramatic change in the prognosis.
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Objective:To explore the application value of silent MR angiography (MRA) in imaging of brain arteriovenous malformation (BAVM) in children.Methods:A total of 20 children with BAVM confirmed by digital subtraction angiography (DSA) were retrospectively collected. All children were imaged by silent MRA and time-of-flight MRA (TOF MRA) in the same examination. The image quality of feeding artery, nidus and drainage vein of BAVM was evaluated using the four-point method. Wilcoxon rank-sum test was utilized to compare the image quality scores between silent MRA and TOF MRA. Weighted Kappa statistics used to evaluate the inter-modality agreement of silent MRA and TOF MRA with DSA in displaying of angioarchitecture characteristics and determination of Spetzler-Martin grading.Results:Among the 20 BAVMs, significant differences in image quality scores of the nidus (2.75±0.55 versus 2.20±0.70) and drainage vein (2.60±0.68 versus 2.20±0.77) were observed between silent MRA and TOF MRA ( Z=-3.05, P=0.002; Z=-2.13, P=0.033, respectively). The agreement between silent MRA and DSA was excellent in nidus size grading, deep venous drainage, associated aneurysm and SM grading (Kappa 0.91, 1.00, 0.83 and 0.93, respectively); The agreement between TOF MRA and DSA was fair to moderate (Kappa 0.46, 0.59, 0.35 and 0.47, respectively). Conclusions:Silent MRA showes better image quality compared to TOF MRA and improves the evaluation of angioarchitecture characteristics and Spetzler-Martin grading of BAVMs in children.
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Objective We evaluate if supplementary grading system can refine patient selection and accurately predict neurological outcome in BAVM. Methods We retrospectively study 221 BAVM patients who were treated micro?surgically by our hospital. The score of pre and post operation mRS and relative clinical, radiology data were collected. Two different logistic models (Spetzler-Martin, Supplementary Spetzler-Martin grading model) were constructed to com?pare the area under ROC. Results Some factors are significant different between worse outcome patients and good out?come patients:Non-hemorrhagic presentations prior surgery, AVM bigger than 3cm, diffuse shape of AVM and the elder patients. Predictive accuracy was higher for the supplementary model (ROC area, 0.91), than the Spetzler-Martin model (ROC area, 0.774). So the predictive accuracy of supplementary model was significantly better than that of the Spet?zler-Martin model (P=0.0362). Conclusions Supplementary Spetzler-Martin model can improve preoperative risk pre?diction and subgroup the patients more efficiently. When the score less than 5(including 5) in supplementary Spet?zler-Martin patients seem to have lower risk relative to surgery.
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Objective To evaluate the clinical application of multimodality treatment of brain arteriovenous malformation (BAVM) with Onyx embolization and microsurgery.Methods There were 48 patients with BAVM treated by combination of Onyx embolization and microsurgery.The clinical features,therapeutic process,complications and discharge status (by MRI,DSA and Glasgow outcome scale,GOS) were recorded in detail.The prognosis was estimated by modified Rankin scale (mRS) with 6-42 months' follow-up.Results The total resection rate was 97.9％.The therapy-related complications happened in 8 patients (16.7％).Among them,4 cases had dysfunction of central nervous system (8.3％),2 cases with intra-cerebral hemorrhage (4.2％),and 2 suffered from severe cerebral edema (4.2％).Good recovery was seen in 38 cases (79.2％) evaluated by GOS when discharged from hospital.The follow-up result mRS ＜ 3 was 97.9％.Conclusion Multimodality treatment of BAVM with Onyx embolization and microsurgery should be a good therapeutic strategy.
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Background: Central nervous system arteriovenous malformation (AVM) is a vascular malformation of the brain and involves entanglement of veins and arteries without an intervening capillary bed. Affecting predominantly young male patients, AVM presents with different clinical manifestations namely headache, seizures, neurological deficit and intracranial haemorrhage. The patients who present acutely with intracranial bleeding have a significant morbidity and mortality. The aim is to study the angioarchitecture of brain AVM (BAVM) and determine the risk factors for intracranial bleeding. Ultimately, the goal of the study is to look for the association between volume of haematoma and architecture of BAVM. Methods: A cross-sectional study of 58 patients was conducted at the Hospital Universiti Sains Malaysia. Data were collected over a period of seven years (2000 to 2007) to look for the association between the angioarchitecture of brain arteriovenous malformations (BAVM), haemodynamics and the natural history and risk of intracranial haemorrhage. Results: BAVM was predominantly found in young male patients in 65.5%. Small nidal size (P-value=0.004), deep location (P-value=0.003) and deep venous drainage (P-value=0.006) were found to be significant factors contributing to intracranial haemorrhage. All patients with coexisting intranidal or prenidal aneurysms presented with intracranial haematoma. Conclusion: The angioarchitecture of BAVM like nidal size, deep location and deep venous drainage can predict the risk of intracranial bleeding and can help in the management of high risk patients without any delay. Small sized and deep seated lesions have a diffuse type of intracranial bleed which eventually need more attention to the managing team as diffuse haematoma indicates more insult to brain.