Implantation of Depth Electrodes in Children Using VarioGuide® Frameless Navigation System: Technical Note.

BACKGROUND: Electrode placement in epilepsy surgery seeks to locate the sites of ictal onset and early propagation. An invasive diagnostic procedure, stereoelectroencephalography (SEEG) is usually implemented with frame-based methods that can be especially problematic in young children. OBJECTIVE: To evaluate the feasibility and accuracy of a new technique for frameless SEEG in children using the VarioGuide® system (Brainlab AG, München, Germany). METHODS: A frameless stereotactic navigation system was used to implant depth electrodes with percutaneous drilling and bolt insertion in pediatric patients with medically refractory epilepsy. Data on general demographic information of electrode implantation, duration, number, and complications were retrospectively collected. To determine the placement accuracy of the VarioGuide® frameless system, the mean Euclidean distances were calculated by comparing the preoperatively planned trajectories with the final electrode position observed on postoperative computed tomography scans. RESULTS: From May 2011 to December 2015, 15 patients (8 males, 7 females; mean age: 8 yr, range: 3-16 yr) underwent SEEG depth electrode implantation of a total of 111 electrodes. The mean error measured by the Euclidean distance from the center of the entry point to the intended entry point was 3.64 ± 1.78 mm (range: 0.58-7.59 mm) and the tip of the electrode to the intended target was 2.96 ± 1.49 mm (range: 0.58-7.82 mm). There were no significant complications. CONCLUSION: Depth electrodes can be placed safely and accurately in children using the VarioGuide® frameless stereotactic navigation system.

Pediatric cerebellar giant cavernous malformation: case report and review of literature.

BACKGROUND AND IMPORTANCE: Giant cavernous malformations (GCM) are low flow, angiographically occult vascular lesions, with a diameter >4 cm. Cerebellar GCMs are extremely rare, with only seven cases reported based on English literature. These lesions are most commonly seen in the pediatric age group, which is known to have an increased risk of hemorrhage, being surgery clearly recommended. CLINICAL PRESENTATION: An 18-month-old girl presented with a 6-month history of cervical torticollis and upper extremities clumsiness. An MRI revealed a 57 × 46 × 42 mm multi-cystic, left cerebellar hemisphere mass, showing areas of hemorrhages and cysts with various stages of thrombus. There was no enhancement with contrast. Cerebral angiography ruled out an arteriovenous malformation. She underwent a left paramedian occipital craniotomy, and macroscopic gross total resection was accomplished. Histopathologic examination was consistent with a cavernous malformation. After surgery, the patient had no new neurological deficit and an uneventful postoperative recovery. Follow-up MRI confirmed total removal of the lesion. CONCLUSION: Cerebellar GCMs in children are symptomatic lesions, which prompt immediate surgical treatment. These are rare lesions, which can radiologically and clinically mimic a tumor with bleed, having to be considered in the differential diagnosis of neoplastic lesions. Cerebellar GCMs might be suspected in the presence of large hemorrhagic intra-axial mass with "bubbles of blood," multi-cystic appearance, surrounded by hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect. Careful radiological study provides a preoperative diagnosis, but its confirmation requires histopathological examination. Complete surgical removal should be attempted when possible.

Neuroendoscopic Intraventricular Biopsy in Children with Small Ventricles Using Frameless VarioGuide System.

Endoscopic biopsy for intraventricular tumors in pediatric patients with small ventricles is a challenging procedure because of the risk of morbidity during the intraventricular approach. We describe the use of the VarioGuide system for intraventricular endoscopic biopsy in 9 consecutive pediatric patients with intraventricular lesions and small ventricular size. All patients had lesions in the anterior part of the third ventricle with a median frontal and occipital horn ratio of 0.33. Patients presented with growth failure (n = 4), visual disturbances (n = 4), and seizures (n = 1). The VarioGuide system consists of an ergonomic arm with 3 joints for gross adjustment. The 3 rotational joints on the distal side of the system are adjusted according to the angles of the planned trajectory. The endoscope is adjusted to the distal side of the VarioGuide and inserted through the ring, previously set for the diameter of the endoscope and for the planned trajectory. The accuracy of the trajectory and correct ventricular cannulation are confirmed under endoscopic guidance. The biopsy is carried out according to the standard technique. In all cases, the biopsy sample provided the definitive diagnosis. Diagnoses included germinomas in 4 patients, hamartoma in 1 patient, hypothalamic astrocytoma in 2 patients, and craniopharyngioma in 2 patients. The use of the VarioGuide system for intraventricular endoscopic biopsy is highly recommended for pediatric patients with small ventricle size. This technique may help minimize the risk of unnecessary brain damage during the entrance to small ventricles.

Tratamiento de los tumores de plexos coroideos en el Hospital Infantil Niño Jesús de Madrid: nuestra experiencia en las últimas tres décadas / Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades

Objetivo: Realizar una revisión de los pacientes en edad infantil con tumores de plexos coroideos (TPC) intervenidos en el Hospital Infantil Niño Jesús de Madrid desde enero de 1981 hasta septiembre de 2014. Material y método: Los casos registrados fueron analizados en base a la epidemiología, el grado tumoral, las características clínicas, la localización, las características de la diseminación, la actitud terapéutica, el pronóstico y las complicaciones. Resultados: Se registraron 17 pacientes con TPC en edad infantil. Los casos se distribuyeron de modo que 9 casos fueron diagnosticados como papiloma de plexos coroideos (PPC) (52,9%), 2 casos como PPC atípicos (11,7%) y 6 casos como carcinoma de plexos coroideos (CPC) (35,2%). La edad al diagnóstico fue menor de 2 años en 14 de los 17 pacientes (82,3%) y la incidencia fue mayor en los varones (82,3% de los casos). Se realizó resección completa en 16 pacientes (94,1%). Recibieron tratamiento complementario 6 pacientes (todos diagnosticados de CPC) (35,2%). Dos de los 17 pacientes fallecieron (11,7%), mostrando una densidad de incidencia de 0,01 muertes/año. Conclusiones : Nuestra serie de casos es concordante con series previas publicadas en la literatura científica en cuanto a epidemiología, grado tumoral, presentación clínica, características radiológicas y actitud terapéutica. El patrón oro en el tratamiento de los TPC es la resección quirúrgica completa. La quimioterapia y radioterapia deben reservarse para el tratamiento complementario de CPC y recidiva o resto tumoral de PPC atípico

Objective: To review childhood patients with choroid plexus tumors (CPT) who underwent surgery at Hospital Infantil Niño Jesús of Madrid since January 1981 to September 2014. Material and methods: Registered charts were analyzed based on the epidemiology, tumor grade, clinical profile, location, dissemination characteristics, therapy, prognosis and complications. Results: Seventeen childhood patients were recorded with CPT. Cases were distributed so that 9 cases were choroid plexus-papilloma (CPP) (52.9%), 2 cases atypical CPP (11.7%) and 6 cases choroid plexus-carcinoma (CPC) (35.2%). Age at diagnosis was less than 2 years in 14 of the 17 patients (82.3%) and the incidence was higher in males (82.3% of the cases). Gross total resection was performed in 16 patients (94.1%). Adjuvant treatment was used in 6 patients (all this cases with CPC) (35.2%). Two of the 17 patients died (11.7%), showing an incidence density of 0.01 deaths/year. Conclusions: Our case series is consistent with previous published in scientific literature regarding epidemiology, tumor grade, clinical presentation, radiological features and therapeutic approach. Gross total resection is considered the therapeutic gold standard for choroid plexus tumors. Chemotherapy and radiotherapy should be used as adjuvant treatment in CPC and recurrent or remaining atypical CPP

[Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades]. / Tratamiento de los tumores de plexos coroideos en el Hospital Infantil Niño Jesús de Madrid: nuestra experiencia en las últimas tres décadas.

OBJECTIVE: To review childhood patients with choroid plexus tumors (CPT) who underwent surgery at Hospital Infantil Niño Jesús of Madrid since January 1981 to September 2014. MATERIAL AND METHODS: Registered charts were analyzed based on the epidemiology, tumor grade, clinical profile, location, dissemination characteristics, therapy, prognosis and complications. RESULTS: Seventeen childhood patients were recorded with CPT. Cases were distributed so that 9 cases were choroid plexus-papilloma (CPP) (52.9%), 2 cases atypical CPP (11.7%) and 6 cases choroid plexus-carcinoma (CPC) (35.2%). Age at diagnosis was less than 2 years in 14 of the 17 patients (82.3%) and the incidence was higher in males (82.3% of the cases). Gross total resection was performed in 16 patients (94.1%). Adjuvant treatment was used in 6 patients (all this cases with CPC) (35.2%). Two of the 17 patients died (11.7%), showing an incidence density of 0.01 deaths/year. CONCLUSIONS: Our case series is consistent with previous published in scientific literature regarding epidemiology, tumor grade, clinical presentation, radiological features and therapeutic approach. Gross total resection is considered the therapeutic gold standard for choroid plexus tumors. Chemotherapy and radiotherapy should be used as adjuvant treatment in CPC and recurrent or remaining atypical CPP.

Estenosis de canal a nivel del atlas en un niño con síndrome de Down. Presentación de un caso y revisión de la literatura / No disponible

INTRODUCCIÓN: La aparición de anomalías congénitas a nivel del atlas es frecuente en pacientes con alteraciones neurales, como en el síndrome de Down. La presencia de estenosis clínica por alteración en el arco posterior de C1 sin que existiera una subluxación atlantoaxial previa no ha sido descrita en la literatura hasta el momento. CASO CLÍNICO: Presentamos un caso excepcional de mielopatía por compresión a nivel del atlas en un niño de 5 años con síndrome de Down provocado por una anomalía del desarrollo del arco posterior de C1. Se realizó una laminectomía posterior a ese nivel con mejoría de la sintomatología previa. CONCLUSIONES: Se debe prestar especial atención a los niños con síndromes asociados a alteraciones de la condrogénesis, como es el caso de los que presentan síndrome de Down, para poder beneficiarse de un tratamiento precoz, ya que en la mayoría de las ocasiones son diagnosticados cuando los síntomas son muy severos

INTRODUCTION: The appearance of congenital anomalies at the level of atlas is frequent inpatients with neural alterations, as well as in the Down syndrome. The presence of clinicalstenosis for alteration in the posterior arch of C1 without a previous atlantoaxial subluxationhasn’t been described in the literature thus far. CASE REPORT: We report an exceptional case of myelopathy due to compression at the levelof the atlas in a 5-year-old boy with Down syndrome provoked by a developmental anomaly of the posterior arch of C1. A posterior laminectomy was achieved at that level withimprovement of the previous symptoms. CONCLUSIONS: We have to pay special attention in children with syndromes associated withchondrogenesis alterations, as in the case of those with Down syndrome, to benefit fromearly treatment, since in most of the time they are diagnosed when symptoms are verysevere

[Spinal stenosis at the level of atlas in a boy with Down syndrome. A case report and literature review]. / Estenosis de canal a nivel del atlas en un niño con síndrome de Down. Presentación de un caso y revisión de la literatura.

INTRODUCTION: The appearance of congenital anomalies at the level of atlas is frequent in patients with neural alterations, as well as in the Down syndrome. The presence of clinical stenosis for alteration in the posterior arch of C1 without a previous atlantoaxial subluxation hasn't been described in the literature thus far. CASE REPORT: We report an exceptional case of myelopathy due to compression at the level of the atlas in a 5-year-old boy with Down syndrome provoked by a developmental anomaly of the posterior arch of C1. A posterior laminectomy was achieved at that level with improvement of the previous symptoms. CONCLUSIONS: We have to pay special attention in children with syndromes associated with chondrogenesis alterations, as in the case of those with Down syndrome, to benefit from early treatment, since in most of the time they are diagnosed when symptoms are very severe.

Prognosis of cerebellar astrocytomas in children.

OBJECTIVE: Our main objective is to review a large series of cerebellar astrocytomas in children and evaluate the outcome of the patients depending on astrocytoma class. The effect of astrocytoma characteristics on the children's prognosis was determined by grouping a series of cerebellar astrocytomas by their location, radiological aspect, size, and histology and determining whether this was related with outcome. MATERIALS AND METHODS: Two hundred and three children with cerebellar astrocytomas were retrospectively reviewed, and their tumors were classified by location, macroscopic radiological appearance, size, and histology. We have distinguished between patients operated before and after 1974 because of the introduction of new diagnostic (computed tomography, magnetic resonance) and more sophisticated treatment techniques after this year (microsurgery, laser, cavitron, etc). RESULTS: Our patients' results were classified according to the Lapras scale/classification as normal, with some neurological deficit but able to lead a normal life, and those with severe post surgical deficits. Recurrences and mortality were also noted. Normal or good results were obtained in 111 patients, some neurological deficit in 55, and severe deficits in nine. There were six recurrences and 22 deaths because of the disease. CONCLUSIONS: Two main factors affected prognosis. One was whether the tumor was completely resected or not; this was the treatment in most cases in this series. The second factor was the location, size, and macroscopic appearance of the tumor. The best prognosis was associated with being located in one hemisphere, being cystic, being cystic with a posterior nodule, and/or being small.