Primary endovascular treatment for acute ischemic stroke in teenage patients: a short case series.

PURPOSE: To analyze the safety and efficacy of primary endovascular treatment (EVT) for acute ischemic stroke (AIS) in patients younger than 18 years of age. METHODS: Review of 4 patients < 18 years of age with AIS, prospectively enrolled in an electronic database registry for acute ischemic stroke patients who underwent thrombectomy at tertiary centers, from January 2011 to February 2017. Clinical and imaging data were analyzed. RESULTS: All patients were female. Patients 1 to 4 were 14, 13, 16, and 13 years old, respectively. Patients 1 and 3 had left middle cerebral artery occlusion, patient 2 basilar occlusion, and patient 4 right tandem occlusion. Mean NIHSS score was 13 (7-19) on arrival and 4 (0-5) at 24 h. Patient 2 had Osler-Weber-Rendu disease and patient 4 a previously surgically repaired complete atrioventricular canal. All patients presented with clinical-radiological mismatch. CT/CTA was used in patients 1 and 4 and MRI/MRA in patients 2 and 3. Stent retriever was used in 3 patients (patients 1, 3, and 4) and direct aspiration first-pass technique in 1 (patient 2). All 4 procedures resulted in successful recanalization and 3-month functional independence. CONCLUSION: Primary EVT is reported in patients 13 to 16 years of age with AIS due to large vessel occlusion and clinical-radiological mismatch. Procedures were safe and effective with prompt recanalization and good clinical outcome.

Intra-arterial chemotherapy for retinoblastoma. Challenges of a prospective study.

PURPOSE: To report the efficacy and complications of intra-ophthalmic artery melphalan (IAM) for treatment of patients with advanced intra-ocular retinoblastoma. METHODS: Patients with newly diagnosed, unilateral, group D retinoblastoma were included in a phase II protocol. Children with relapsed-refractory disease after systemic chemoreduction were later treated under the same guidelines.Melphalan (3­5 mg/procedure) was injected through a 1.2 F microcatheter placed into the ophthalmic artery every 21 days. RESULTS: Eleven patients (12 eyes, eight as primary treatment) received 33 IAM procedures. The phase II protocol closed prematurely because of low accrual. The IAM technique was overall safe and could be performed successfully in 31 of 33(94%) attempts. After the second administration of IAM, very good partial response was achieved in all treated eyes. With a median follow-up time of 29.5 months (range 6­57), ocular salvage was achieved in 7 of 12 (58%) eyes. No systemic adverse events were observed. Two patients developed diffuse arteriolar sclerosis, hyperpigmentation of the retinal pigment epithelium and partial retinal atrophy after the second IAM. Both eyes were preserved with no tumour activity, good motility and perception of light, 56 and 30 months after the last IAM treatment. Multinucleated macrophages with intracytoplasmic foreign material were found in the choroid and the retina in 2 of 5 enucleated eyes. CONCLUSION: Our study reports the activity and reproducibility of IAM in advanced retinoblastoma but also underlines the challenges of performing prospective studies on this treatment modality. Toxicity was limited to only ocular vascular events.

Endovascular treatment of a pial arteriovenous fistula of a posteroinferior cerebellar artery with a double origin.

Pial arteriovenous fistulas (pAVF) of the posterior fossa are rare and may present with symptoms secondary to mass effect, venous hypertension or hemorrhage, among others. A case is presented of a previously healthy 2-year-old boy with neurological deficit arising from pAVF of the posteroinferior cerebellar artery. The pAVF was successfully treated with endovascular occlusion.

Feasibility of estimating regional mechanical properties of cerebral aneurysms in vivo.

PURPOSE: In this article, the authors studied the feasibility of estimating regional mechanical properties in cerebral aneurysms, integrating information extracted from imaging and physiological data with generic computational models of the arterial wall behavior. METHODS: A data assimilation framework was developed to incorporate patient-specific geometries into a given biomechanical model, whereas wall motion estimates were obtained from applying registration techniques to a pair of simulated MR images and guided the mechanical parameter estimation. A simple incompressible linear and isotropic Hookean model coupled with computational fluid-dynamics was employed as a first approximation for computational purposes. Additionally, an automatic clustering technique was developed to reduce the number of parameters to assimilate at the optimization stage and it considerably accelerated the convergence of the simulations. Several in silico experiments were designed to assess the influence of aneurysm geometrical characteristics and the accuracy of wall motion estimates on the mechanical property estimates. Hence, the proposed methodology was applied to six real cerebral aneurysms and tested against a varying number of regions with different elasticity, different mesh discretization, imaging resolution, and registration configurations. RESULTS: Several in silico experiments were conducted to investigate the feasibility of the proposed workflow, results found suggesting that the estimation of the mechanical properties was mainly influenced by the image spatial resolution and the chosen registration configuration. According to the in silico experiments, the minimal spatial resolution needed to extract wall pulsation measurements with enough accuracy to guide the proposed data assimilation framework was of 0.1 mm. CONCLUSIONS: Current routine imaging modalities do not have such a high spatial resolution and therefore the proposed data assimilation framework cannot currently be used on in vivo data to reliably estimate regional properties in cerebral aneurysms. Besides, it was observed that the incorporation of fluid-structure interaction in a biomechanical model with linear and isotropic material properties did not have a substantial influence in the final results.

Genome-wide association study of intracranial aneurysm identifies three new risk loci.

Saccular intracranial aneurysms are balloon-like dilations of the intracranial arterial wall; their hemorrhage commonly results in severe neurologic impairment and death. We report a second genome-wide association study with discovery and replication cohorts from Europe and Japan comprising 5,891 cases and 14,181 controls with approximately 832,000 genotyped and imputed SNPs across discovery cohorts. We identified three new loci showing strong evidence for association with intracranial aneurysms in the combined dataset, including intervals near RBBP8 on 18q11.2 (odds ratio (OR) = 1.22, P = 1.1 x 10(-12)), STARD13-KL on 13q13.1 (OR = 1.20, P = 2.5 x 10(-9)) and a gene-rich region on 10q24.32 (OR = 1.29, P = 1.2 x 10(-9)). We also confirmed prior associations near SOX17 (8q11.23-q12.1; OR = 1.28, P = 1.3 x 10(-12)) and CDKN2A-CDKN2B (9p21.3; OR = 1.31, P = 1.5 x 10(-22)). It is noteworthy that several putative risk genes play a role in cell-cycle progression, potentially affecting the proliferation and senescence of progenitor-cell populations that are responsible for vascular formation and repair.

Transient encephalopathy from angiographic contrast: a rare complication in neurointerventional procedures.

Neurotoxicity from contrast media used in angiography is a rare complication from these procedures. The infrequency with which it is encountered makes it a diagnostic challenge. We present the case of a 51-year-old male who, 30 min after successful angiography for treatment of a right carotid-ophthalmic fusiform aneurysm with a stent, developed psychomotor agitation, disorientation, and progressive left faciobrachial hemiparesis (4/5). An emergency nonenhanced CT showed marked cortical enhancement and edema in the right cerebral hemisphere. Cortical enhancement is thought to be secondary to contrast extravasation due to disruption of the blood-brain barrier. Angiography was performed immediately, without any pathologic findings. After this procedure there was an increase in the left faciobrachial hemiparesis (3/5), right gaze deviation, Gerstmann syndrome, and left anosognosia and left homonymous hemianopsia. Endovenous dexamethasone and mannitol were initiated. Twenty-four hours later an MRI showed no signs of acute infarct, just gyriform signal increase in the right cerebral hemisphere on FLAIR and a decrease in the edema observed before. The patient had progressive improvement of his neurological deficit. A control MRI done 5 days later was normal. The patient recovered completely and was discharged. This rare entity should be kept in mind but diagnosed only when all other causes have been ruled out, because more important and frequent causes, such as acute infarct, must be excluded promptly.

Use of angioembolization as an effective technique for the management of pediatric solid tumors.

PURPOSE: In oncology practice, angioembolization has been reported for tumor reduction before surgery, treatment of life-threatening conditions, and for palliative care. Nevertheless, the overall experience with angioembolization for the treatment of tumors is limited. We report our experience in 7 nonvascular solid pediatric tumors. MATERIALS AND METHODS: A retrospective review was carried out of medical records from pediatric patients (0-18 years) with solid nonvascular tumors who underwent angioembolization in the last 5 years at our institution. RESULTS: Seven patients underwent embolization: 2 neuroblastomas, 1 metastatic paraganglioma, 1 hepatoblastoma, 1 myofibroblastic tumor, 1 osteosarcoma, and 1 undifferentiated sarcoma. The reason for angioembolization was preparation for surgery (3), treatment of a life-threatening event (1), or palliative care (3). Each case is presented and discussed. The outcome was subsequent complete surgical resection in 3 cases, tumor vanished in 1 case, symptom control was achieved in 1, and the other 2 patients improved their survival and quality of life, however, died of disease progression. CONCLUSIONS: Tumor angioembolization may enter the treatment algorithm for selected patients who have to face difficult or unwarranted surgical procedures or have diseases where conventional therapies have failed.

Treatment of disseminated paraganglioma with gemcitabine and docetaxel.

A male with metastatic paraganglioma received different chemotherapy regimens and 14 arterial embolizations with no or short-lasting clinical benefit. He was started on gemcitabine and docetaxel and, after two cycles, remission of all clinical signs occurred over 2 months. A complete biochemical response was achieved and angiographic signs of portal hypertension disappeared. He received 18 cycles of therapy and no limiting side effects were observed. More than 2 years after gemcitabine and docetaxel treatment, the patient remains symptom free. Gemcitabine and docetaxel could be a potential therapeutic strategy for this patient.

Moyamoya en la población infantil no asiática. Ampliación de casuística / Moyamoya disease in non Asian population

La enfermedad o síndrome de moyamoya es una rara afección que provoca una oclusión de las carótidas intracraneales y determina una red vascular colateral anormal en la base del cráneo y que, por su aspecto arteriográfico, hace ya más de 50 años un grupo de neurocirujanos japoneses denominaron moyamoya, y de la cual persisten muchas interrogantes sin resolver. Se realizó un estudio descriptivo y longitudinal de 19 niños con síndrome o enfermedad de moyamoya. La edad promedio de inicio fue de seis años. Del total de niños incluidos, doce debutaron en forma aguda con infarto cerebral; dos pacientes (con antecedentes de epilepsia y retraso) lo hicieron, uno con infarto y otro con hemorragia cerebral, otros dos con epilepsia y retraso, uno con trastorno del lenguaje, siendo en los dos restantes un hallazgo casual. Entretanto doce pacientes se consideraron idiopáticos (portadores de enfermedad de moyamoya) y siete asociados a enfermedades sistémicas (síndrome de moyamoya). El diagnóstico inicial se confirmó por angiorresonancia en diez casos y por arteriografía convencional en nueve. El seguimiento promedio fue de cinco años, once evidenciaron progresión clínica y ocho estabilidad, con un seguimiento promedio de siete y dos años respectivamente. Todos mostraron progresión angiográfica. En siete niños se realizaron diez cirugías de revascularización sin complicaciones y con buenos resultados clínicos y angiográficos. Dos niños fallecieron (uno por hemorragia cerebral y otro por un infarto expansivo) y catorce presentaron secuelas leves a moderadas. Resulta importante identificar los niños que se pueden beneficiar con la cirugía de revascularización, una de las pocas opciones terapéuticas para evitar la progresión y complicaciones de esta grave enfermedad.

Moyamoya's disease or syndrome is a rare illness wich causes symptoms of cerebral ischemia due to intracranial arteries' stenosis, with secondary abnormal vasculature networks at the base of the brain. Since the first description of moyamoya disease done by a group of Japanese neurosurgeons more than 50 years ago, this rare illness is still considered an intriguing disease. The clinical features, treatment, imaging findings and outcomes of a series of not Asian children with Moyamoya disease are described in this study. A total of 19 six year old patients were analyzed. The initial presentation in 12 patients was infarctions, 2 with epilepsy and mental retardation, another 2 who had personal history of epilepsy and mental retardation developed symptoms of ischemic stroke (one of them), and hemorrhagic stroke the other one; 1experienced language problems, while in the last two the diagnosis was casual. On the other hand, 12 patientes were considered idiopathic cases (moyamoya's disease) and 7 were related to systemic illnes (moyamoya's syndrome). Initial diagnosis was achieved by magnetic resonance angiography in 10 cases, and conventional angiography in 9. All patients were followed up for a mean period of 5 years. While 11 patients experienced clinical worsening of symptoms, 6 asymptomatic. However, all of them showed angiographic progression. Seven patients underwent bypass surgery with no further complications, 2 passed away and 14 remained with moderate handicaps. A number of surgical procedures have been developed for revascularization, improving the outcome of some of these patients by preventing ischemic and hemorrhagic stroke. Due to the fact that moyamoya disease is not a silent disorder and its progress cause complications, surgical revascularization should always be considered in the management of these patients.

Percutaneous treatment of lumbar intervertebral disk hernias with radiopaque gelified ethanol: a preliminary study.

STUDY DESIGN: Prospective clinical trial. OBJECTIVE: Demonstrate the safety and efficacy of gelified ethanol in the percutaneous treatment of lumbar disk hernias. SUMMARY OF BACKGROUND DATA: After the commercial withdrawal of Chymopapain, the need for new substances to treat intervertebral disk hernias was evident. Good results were obtained with pure ethanol, but this substance was difficult to handle. We decided to use a similar substance mixed with ethylcellulose to increase its viscosity and enhanced with radiopaque material. METHODS: Two hundred seventy-six consecutive patients sent to be treated of a lumbar intervertebral disk hernia percutaneously were included in this preliminary study and treated with radiopaque gelified ethanol (RGE) and intra-articular steroids. Three groups were set, group A for patients to be treated only with RGE and groups B and C for difficult cases presenting a narrow canal, foraminal hernia, or hiperalgic sleepless hernia, treated with RGE plus another intradiscal technique, automatized percutaneous diskectomy for group B and radiofrequency nucleoplasty for group C. RESULTS: Very good or good results were obtained in 202 (91.4%) of the 221 patients in group A. Of the 44 patients in group B, 37 patients (84%) presented very good or good results and in 9 (82%) of the 11 patients of group C, we obtained similar results. There was no allergic complication in any of our patients. Short-term follow-up with magnetic resonance showed little or no changes in the intervertebral disk but there was discordance with clinical signs. Long-term follow-up magnetic resonance showed a dramatic reduction in hernia volume. CONCLUSIONS: This preliminary study shows the efficacy and inocuity of this new substance that could take over the Chymopapain therapeutic field.