Multimodal Approach for the Treatment of Complex Hypothalamic Hamartomas.

Hypothalamic hamartomas (HHs) are rare congenital lesions formed by heterotopic neuronal and glial cells attached to the mammillary bodies, tuber cinereum, and hypothalamus.They often present with an intractable epilepsy typically characterized by gelastic seizures but commonly associated with other types of refractory seizures. The clinical course is progressive in most of the cases, starting with gelastic seizures in infancy and deteriorating into complex seizure disorders that result in catastrophic epilepsy associated with cognitive decline and behavioral disturbances.Hamartomas are known to be intrinsically epileptogenic and the site of origin for the gelastic seizures. As antiepileptic drugs are typically ineffective in controlling HH-related epilepsy, different surgical options have been proposed as a treatment to achieve seizure control. Resection or complete disconnection of the hamartoma from the mammillothalamic tract has proved to achieve a long-lasting control of the epileptic syndrome.Usually, symptoms and their severity are typically related to the size, localization, and type of attachment. Precocious puberty appears mostly in the pedunculated type, while epileptic syndrome and behavioral decline are frequently related to the sessile type. For this reason, different classifications of HHs have been developed based on their size, extension, and type of attachment to the hypothalamus.The bigger and more complex hypothalamic hamartomas typically present with severe refractory epilepsy, behavioral disturbances, and progressive cognitive decline posing a formidable challenge for the control of these symptoms.We present here our experience with the multimodal treatment for complex hypothalamic hamartomas. After an in-depth review of the literature, we systematize our approach for the different types of hypothalamic hamartomas.

Extra-temporal pediatric low-grade gliomas and epilepsy.

Low-grade gliomas, especially glioneuronal tumors, are a common cause of epilepsy in children. Seizures associated with low-grade pediatric tumors are medically refractory and present a significant burden to patients. Often, morbidity and patients´ quality of life are determined rather by the control of seizures than the oncological process itself and the resolution of epilepsy represents an important part in the treatment of LGGs. The pathogenesis of tumor-related seizures in focal LGG tumors is multifactorial, and mechanisms differ probably among patients and tumor types. Pediatric low-grade tumors associated with epilepsy include a series of neoplasms that have a pure astrocytic or glioneuronal lineage. They are usually benign tumors with a neocortical localization typically in the temporal lobes, but also in other supratentorial locations. Gangliogliomas and dysembryoplastic neuroepithelial tumors (DNET) are the most common entities together with astrocytic gliomas (pilocytic astrocytomas and pleomorphic xanthoastrocytoma) and angiocentric gliomas, and dual pathology is found in up to 40% of glioneuronal tumors. The treatment of low-grade gliomas and associated epilepsy is based mainly on resection and the extent of surgery is the main predictor of postoperative seizure control in patients with a LGG. Long-term epilepsy-associated tumors (LEATs) tend to be well-circumscribed, and therefore, the chances for a complete resection and epilepsy control with a safe approach are very high. New treatments have emerged as alternatives to open microsurgical approaches, including laser thermal ablation or the use of BRAF inhibitors. Future advances in identifying seizure-related biomarkers and molecular tumor pathways will facilitate targeted treatment strategies that will have a deep impact both in oncologic and epilepsy outcomes.

Delayed hemorrhage after pediatric stereo-electroencephalography: delayed occurrence or delayed diagnosis?

BACKGROUND: Stereo-electroencephalography (SEEG) is a well-known invasive diagnostic method for drug-resistant epilepsy (DRE). Its rate of complications is relatively low, being the intracranial hemorrhage (ICH) the most relevant. Most centers perform immediate imaging studies after SEEG to rule out complications. However, delayed intracranial hemorrhages (DIH) can occur despite normal imaging studies in the immediate postoperative period. METHODS: We performed a retrospective review of DRE pediatric patients operated on SEEG between April 2016 and December 2020 in our institution. After implantation, an immediate postoperative CT was performed to check electrode placement and rule out acute complications. An additional MRI was performed 24 h after surgery. We collected all postoperative hemorrhages and considered them as major or minor according to Wellmer´s classification. RESULTS: Overall, 25 DRE patients were operated on SEEG with 316 electrodes implanted. Three ICHs were diagnosed on postoperative imaging. Two of them were asymptomatic requiring no treatment, while the other needed surgical evacuation after clinical worsening. The total risk of hemorrhage per procedure was 12%, but just one third of them were clinically relevant. Two hemorrhages were not visible on immediate postoperative CT, being incidentally diagnosed in the 24 h MRI. We recorded them as DIH and are reported in detail. CONCLUSION: Few reports of DIH after SEEG exist in the literature. It remains unclear whether these cases are late occurring hemorrhages or immediate postoperative hemorrhages undiagnosed on initial imaging. According to our findings, we recommend to perform additional late postoperative imaging to diagnose these cases and manage them accurately.

Life-threatening secondary hemophagocytic lymphohistiocytosis following vagal nerve stimulator infection in a child with CHD2 myoclonic encephalopathy: a case report.

Vagus nerve stimulation (VNS) is a surgical treatment available for patients affected by generalized refractory epilepsy. The authors report the case of a 15-year-old girl affected by CHD2-related myoclonic encephalopathy and BLM haploinsufficiency due to a deletion of 15q25.3q26.2 region, who suffered from secondary hemophagocytic lymphohistiocytosis (SHLH) after a VNS wound infection. SHLH has sporadically been described in epileptic patients. Based on indirect evidence that shows immune dysregulation in patients with CHD2 mutations and BLM mutations, we hypothesize that the genetic background of this patient may have played a critical role in the development of the syndrome.

Multinodular and vacuolating neuronal tumor associated with focal cortical dysplasia in a child with refractory epilepsy: a case report and brief review of literature.

Multinodular and vacuolating neuronal tumor (MVNT) is a rare newly identified benign lesion, firstly included in the World Health Organization classification of tumors of the central nervous system in 2016, whose neoplastic or dysplastic nature remains unclear but with a distinctive cytoarchitectural pattern and radiological features. It is usually discovered as late-onset refractory epilepsy, headache related, or an incidental lesion of adulthood. As it is typically a stable disease, many opt for follow-up, as long as it keeps remaining asymptomatic, leaving surgery for refractory epilepsy, neurological deficits, or intracranial hypertension symptoms. A subtotal or complete resection seems to control seizures and neurological manifestations. We herein present the case of a child with refractory epilepsy related to MVNT and focal cortical dysplasia, a dual pathology case in a less frequent age group and without the typical radiological imaging. We report its radiologic features, histologic description, and management, and we present a brief literature review on MVNT focusing on the pediatric cases reported. MVNT should now be another probable low-grade epilepsy-associated lesion (LEAT) in patients of all ages, with a benign and stable course as it constitutes a curable cause of focal epilepsy. As all the refractory cases, surgery should be indicated after a comprehensive evaluation of a multidisciplinary epilepsy surgery team.

Highly realistic simulation for robot-assisted hypothalamic hamartoma real-time MRI-guided laser interstitial thermal therapy (LITT).

PURPOSE: Real-time MRI-guided laser interstitial thermal therapy (LITT) is a challenging procedure due to its technical complexity, as well as the need for efficient multidisciplinary teamwork and transfer of an anesthetized patient between operating room (OR) and magnetic resonance (MR). A highly realistic simulation was developed to design the safest process before being applied to real patients. In this report, authors address the description of the methodology used for this simulation and its purposefulness. METHODS: The entire image planning, anesthetic, and surgical process were performed on a modified pediatric simulation mannequin with a brain made of medical grade silicone including a hypothalamic hamartoma. Preoperative CT and MR were acquired. Stereotactic insertion of the optical fiber was assisted by the Neuromate® stereotactic robot. Laser ablation was performed with the Medtronic Visualase® MRI-guided system in a 3T Phillips Ingenia® MR scanner. All the stages of the process, participants, and equipment were the same as planned for a real surgery. RESULTS: No critical errors were found in the process design that prevented the procedure from being performed with adequate safety. Specific proposals for team positioning and interaction in patient transfers and in MR room were validated. Some specific elements that could improve safety were identified. CONCLUSION: Highly realistic simulation has been an extremely useful tool for safely planning LITT, because professionals were able to take actions in the workflow based not on ideas but on lived experiences. It contributed definitively to build a well-coordinated surgical team that worked safely and more efficiently.

Frameless robot-assisted stereoelectroencephalography for refractory epilepsy in pediatric patients: accuracy, usefulness, and technical issues.

BACKGROUND: Stereoelectroencephalography (SEEG) is an effective technique to help to locate and to delimit the epileptogenic area and/or to define relationships with functional cortical areas. We intend to describe the surgical technique and verify the accuracy, safety, and effectiveness of robot-assisted SEEG in a newly created SEEG program in a pediatric center. We focus on the technical difficulties encountered at the early stages of this program. METHODS: We prospectively collected SEEG indication, intraoperative events, accuracy calculated by fusion of postoperative CT with preoperative planning, complications, and usefulness of SEEG in terms of answering preimplantation hypothesis. RESULTS: Fourteen patients between the ages of 5 and 18 years old (mean 10 years) with drug-resistant epilepsy were operated on between April 2016 and April 2018. One hundred sixty-four electrodes were implanted in total. The median entry point localization error (EPLE) was 1.57 mm (1-2.25 mm) and the median target point localization error (TPLE) was 1.77 mm (1.2-2.6 mm). We recorded seven intraoperative technical issues. Two patients suffered complications: meningitis without demonstrated germ in one patient and a right frontal hematoma in the other. In all cases, the SEEG was useful for the therapeutic decision-making. CONCLUSION: SEEG has been useful for decision-making in all our pediatric patients. The robotic arm is an accurate tool for the insertion of the deep electrodes. Nevertheless, it is an invasive technique not risk-free and many problems can appear at the beginning of a robotic arm-assisted SEEG program that must be taken into account beforehand.

Temporo-Parieto-Occipital Disconnection by Robot-Assisted Magnetic Resonance Imaging-Guided Laser Interstitial Thermal Therapy for Refractory Epilepsy in a Pediatric Patient: Proof-of-Principle Case Report and Surgical Nuances.

OBJECTIVE: Magnetic resonance imaging-guided laser interstitial thermal therapy (MRIgLITT) has been proven safe and effective for the treatment of focal epilepsy of different etiologies. It has also been used to disconnect brain tissue in more extensive or diffuse epilepsy, such as corpus callosotomy and hemispherotomy. METHODS: In this study, we report a case of temporo-parieto-occipital disconnection surgery performed using MRIgLITT assisted by a robotic arm for refractory epilepsy of the posterior quadrant. A highly realistic cadaver simulation was performed before the actual surgery. RESULTS: The patient was a 14-year-old boy whose seizures began at the age of 8. The epilepsy was a result of a left perinatal ischemic event that caused a porencephalic cyst, and despite receiving multiple antiepileptic drugs, the patient continued to experience daily seizures which led to the recommendation of surgery. CONCLUSIONS: A Wada test lateralized language in the right hemisphere. Motor and sensory function was confirmed in the left hemisphere through magnetic resonance imaging functional studies and NexStim. The left MRIgLITT temporo-parieto-occipital disconnection disconnection was achieved using 5 laser fibers. The patient followed an excellent postoperative course and was seizure-free, with no additional neurological deficits 24 months after the surgery.

A multidisciplinary approach to posterior quadrant disconnective epilepsy surgery in pediatric patients.

PURPOSE: Extensive lesions of the posterior quadrant are a relevant cause of pediatric drug-resistant epilepsy. Early surgery is the best treatment in these cases, but conventional multilobar resections carry a significant risk in pediatric patients. Despite temporo-parieto-occipital (TPO) disconnection being the preferable technique due to the preservation of motor function, studies reporting long-term longitudinal outcomes are still limited. This study aims to analyze seizures and developmental outcomes after TPO disconnection. METHODS: A prospective analysis was carried out on 12 children who underwent TPO disconnection. RESULTS: TPO disconnection was performed in 12 pediatric patients aged between 14 months and 18 years (median 6.29 years). The average age of seizure onset was 0.97 ± 1.22 years. Causes of TPO included perinatal ischemia in 4 patients and malformation of cortical development (MCD) in 8 patients. The presenting seizure types were focal motor impaired awareness seizures in 7 children and generalized in 5. The affected hemisphere was the right in 9 patients and the left in 3. In half of the patients, the temporal approach was performed through T1, and in the other 50%, it was performed through T2. After neuropsychological examination, 2 children improved, 7 remained stable, 2 patients presented stagnation and 1 declined. Regarding postoperative complications, nonresorptive hydrocephalus and an asymptomatic caudate nucleus infarct were observed. After a median follow-up of 2 years, 9 patients were in Engel's Class I seizure outcome. CONCLUSION: TPO disconnection is a safe and effective motor-sparing epilepsy surgery for children with refractory seizures located in the posterior quadrant that prevents further cognitive deterioration.

Completion of disconnective surgery for refractory epilepsy in pediatric patients using robot-assisted MRI-guided laser interstitial thermal therapy.

OBJECTIVE: Since 2007, the authors have performed 34 hemispherotomies and 17 posterior quadrant disconnections (temporoparietooccipital [TPO] disconnections) for refractory epilepsy at Sant Joan de Déu Barcelona Children's Hospital. Incomplete disconnection is the main cause of surgical failure in disconnective surgery, and reoperation is the treatment of choice. In this study, 6 patients previously treated with hemispherotomy required reoperation through open surgery. After the authors' initial experience with real-time MRI-guided laser interstitial thermal therapy (MRIgLITT) for hypothalamic hamartomas, they decided to use this technique instead of open surgery to complete disconnective surgeries. The objective was to report the feasibility, safety, and efficacy of MRIgLITT to complete hemispherotomies and TPO disconnections for refractory epilepsy in pediatric patients. METHODS: Eight procedures were performed on 6 patients with drug-resistant epilepsy. Patient ages ranged between 4 and 18 years (mean 10 ± 4.4 years). The patients had previously undergone hemispherotomy (4 patients) and TPO disconnection (2 patients) at the hospital. The Visualase system assisted by a Neuromate robotic arm was used. The ablation trajectory was planned along the residual connection. The demographic and epilepsy characteristics of the patients, precision of the robot, details of the laser ablation, complications, and results were prospectively collected. RESULTS: Four patients underwent hemispherotomy and 2 underwent TPO disconnection. Two patients, including 1 who underwent hemispherotomy and 1 who underwent TPO disconnection, received a second laser ablation because of persistent seizures and connections after the first treatment. The average precision of the system (target point localization error) was 1.7 ± 1.4 mm. The average power used was 6.58 ± 1.53 J. No complications were noted. Currently, 5 of the 6 patients are seizure free (Engel class I) after a mean follow-up of 20.2 ± 5.6 months. CONCLUSIONS: According to this preliminary experience, laser ablation is a safe method for complete disconnective surgeries and allowed epilepsy control in 5 of the 6 patients treated. A larger sample size and longer follow-up periods are necessary to better assess the efficacy of MRIgLITT to complete hemispherotomy and TPO disconnection, but the initial results are encouraging.

Presurgical evaluation of drug-resistant paediatric focal epilepsy with PISCOM compared to SISCOM and FDG-PET.

PURPOSE: Children with drug-resistant focal epilepsy have a compromised quality of life. Epilepsy surgery can control or significantly reduce the seizures. We assessed and compared the usefulness of PISCOM, a new nuclear imaging processing technique, with SISCOM and 18F-FDG PET (FDG-PET) in pre-surgical evaluation of paediatric drug-resistant focal epilepsy. METHODS: Twenty-two children with pharmcorefractory epilepsy, mainly extratemporal, who had undergone pre-surgical assessment including SISCOM and FDG-PET and with postsurgical favorable outcome (Engel class I or II) for at least two years, were included in this proof-of-concept study. All abnormalities observed in SISCOM, FDG-PET and PISCOM were compared with each other and with the known epileptogenic zone (EZ) based on surgical treatment, histopathologic and surgical outcome results. Global interobserver agreement, Cohen's Kappa coeficient and PABAK statistic were calculated for each technique. RESULTS: PISCOM concordance with the known EZ was significantly higher than SISCOM (p<0.05), and no statistically differences were found with FDG-PET. PISCOM showed successful identification in 19 of 22 cases (86%), successful concordant with FDG-PET in 17 (77%), and SISCOM in 11 (50%). If we consider PISCOM and FDG-PET results together, both techniques successfully localized the known EZ in all cases. The measures of agreement between two experts in nuclear medicine were higher in PISCOM than in SISCOM and FDG-PET. CONCLUSION: PISCOM could provide complementary presurgical information in drug-resistant paediatric focal epilepsy, particularly in cases in which FDG-PET is doubtful or negative, replacing SISCOM and sparing the use of interictal SPECT.

Robot-assisted, real-time, MRI-guided laser interstitial thermal therapy for pediatric patients with hypothalamic hamartoma: surgical technique, pitfalls, and initial results.

OBJECTIVE: Real-time, MRI-guided laser interstitial thermal therapy (MRgLITT) has been reported as a safe and effective technique for the treatment of epileptogenic foci in children and adults. After the recent approval of MRgLITT by the European Medicines Agency in April 2018, the authors began to use it for the treatment of hypothalamic hamartomas (HHs) in pediatric patients with the assistance of a robotic arm. In this study, the authors report their initial experience describing the surgical technique, accuracy of the robotic arm, safety, and efficacy. METHODS: The laser fiber was placed with the assistance of the stereotactic robotic arm. The accuracy of the robotic arm for this procedure was calculated by comparing the intraoperative MRI to the preoperative plan. Common demographic and seizure characteristics of the patients, laser ablation details, complications, and short-term seizure outcomes were prospectively collected. RESULTS: Sixteen procedures (11 first ablations and 5 reablations) were performed in 11 patients between 15 months and 17 years of age (mean age 6.4 years) with drug-resistant epilepsy related to HHs. The mean target point localization error was 1.69 mm. No laser fiber needed to be repositioned. The mean laser power used per procedure was 4.29 W. The trajectory of the laser fiber was accidentally ablated in 2 patients, provoking transient hemiparesis in one of these patients. One patient experienced postoperative somnolence and syndrome of inappropriate antidiuretic hormone secretion, and 2 patients had transient oculomotor (cranial nerve III) palsy. Fifty-four percent of the patients were seizure free after the first ablation (mean follow-up 22 months, range 15-33 months). All 5 patients who experienced an epilepsy relapse underwent a second treatment, and 4 remain seizure free at least 5 months after reablation. CONCLUSIONS: In the authors' experience, the robotic arm was sufficiently accurate for laser fiber insertion, even in very young patients. MRgLITT appears to be an effective treatment for selected cases of HH. MRgLITT for HH is a minimally invasive procedure with appealing safety features, as it allows delivery of energy precisely under real-time MRI control. Nonetheless, complications may occur, especially in voluminous HHs. The amount of delivered energy and the catheter cooling system must be closely monitored during the procedure. A larger sample size and longer follow-up duration are needed to judge the efficacy and safety of MRgLITT for HH more rigorously. This initial experience was very promising.

New stereoscopic virtual reality system application to cranial nerve microvascular decompression.

PURPOSE: Cranial nerve microvascular decompression is a habitual neurosurgical procedure. Authors describe a new application of the Dextroscope (Volume Interactions, Ltd.), a virtual reality environment, to plan and properly simulate this kind of procedures. METHODS: In three cases of hemifacial spasm refractory to drugs and botulinum toxin treatment, the authors used a virtual reality workstation (Dextroscope) to develop an interactive simulation of craniotomy, approach, and Gore-Tex implant optimal size and position in order to reach vascular decompression of facial nerve. RESULTS: Three-dimensional interactive environment allowed the authors to virtually carry out craniotomy, to visualize vascular and nerve relationship, and finally, to select and to simulate best Gore-Tex graft positioning in each case. During surgical procedures, facial nerve vascular compressions were exposed and Gore-Tex grafts were successfully placed as it was virtually planned. Patient outcomes were excellent, with an average improvement of two units (0-4 grades of severity scale). CONCLUSION: Virtual reality environment can help the neurosurgeon to plan and train vascular decompression procedures. Use of appropriate craniotomy, knowledge of vascular and nerve relationship, and selection of optimum paddy size and position are the main useful applications of the Dextroscope in these procedures.

Central nervous system malformation associated with methamphetamine abuse during pregnancy.

INTRODUCTION: Prenatal methamphetamine exposure is related to prematurity, fetal growth restriction, neurobehavioral effects and long-term motor and cognitive sequelae. PATIENT PRESENTATION: We report the case of a newborn from a Filipina with no prenatal care with a complex brain malformation. Methamphetamine was identified in maternal and neonatal urine and in maternal hair, raising our suspicion of methamphetamine as a cause of this malformation. DISCUSSION: Methamphetamine abuse is a growing problem worldwide. There are little data on its effect on the fetus. To our knowledge, no fetal brain abnormalities have been associated with its use. In our case, the lack of antenatal control does not allow us to date when this malformation appeared. CONCLUSION: The aim of our report is to generate awareness of the possible association between methamphetamine abuse during pregnancy and central nervous system malformations.

Tumor dissemination through surgical tracts in diffuse intrinsic pontine glioma.

OBJECTIVEDiffuse intrinsic pontine glioma (DIPG) is a highly aggressive and lethal brainstem tumor in children. In the 1980s, routine biopsy at presentation was abandoned since it was claimed "unnecessary" for diagnosis. In the last decade, however, several groups have reincorporated this procedure as standard of care or in the context of clinical trials. Expert neurosurgical teams report no mortality and acceptable morbidity, and no relevant complications have been previously described. The aim of this study was to review needle tract dissemination as a potential complication in DIPG.METHODSThe authors retrospectively analyzed the incidence of dissemination through surgical tracts in DIPG patients who underwent biopsy procedures at diagnosis in 3 dedicated centers. Clinical records and images as well as radiation dosimetry from diagnosis to relapse were reviewed.RESULTSFour patients (2 boys and 2 girls, age range 6-12 years) had surgical tract dissemination: in 3 cases in the needle tract and in 1 case in the Ommaya catheter tract. The median time from biopsy to identification of dissemination was 5 months (range 4-6 months). The median overall survival was 11 months (range 7-12 months). Disseminated lesions were in the marginal radiotherapy field (n = 2), out of the field (n = 1), and in the radiotherapy field (n = 1).CONCLUSIONSAlthough surgical tract dissemination in DIPG is a rare complication (associated with 2.4% of procedures in this study), it should be mentioned to patients and family when procedures involving a surgical tract are proposed. The inclusion of the needle tract in the radiotherapy field may have only limited benefit. Future studies are warranted to explore the benefit of larger radiotherapy fields in patients with DIPG.

[Clinical scale for the diagnosis of ventriculoperitoneal shunt malfunction in children in Emergency Department]. / Escala clínica para el diagnóstico de disfunción de válvula ventriculoperitoneal en niños en el Servicio de Urgencias.

INTRODUCTION: It is well known that some symptoms in children with ventriculoperitoneal shunt are associated with a higher risk of developing shunt malfunction. However none of those symptoms are sensitive or enough specific enough to diagnose the shunt malfunction. OBJECTIVE: To develop a diagnostic scale to identify children with an increased risk of shunt malfunction in the Emergency Department. MATERIAL AND METHODS: This is a prospective study including children aged one to eighteen years old admitted to the Emergency Department between April 2010 and March 2013 with symptoms of ventriculoperitoneal shunt malfunction. Logistic regression Analysis was used to determine whether or not the variables were considered independent risk factors for shunt malfunction. The results led to the development of a diagnostic scale. RESULTS: A scale was developed using 9 variables (erythema, swelling or discharge from the catheter trajectory, drowsiness, stiff neck, headache, afebrile, age>4 years, vomiting, recent neurological deficit, and time since last surgery ≤ 2 years) with a maximum score of 20 points. It was found that Scale scores ≥ 7 points were associated with an increased risk of shunt malfunction (OR 34.0, 95% CI 15.4-74.9; sensitivity 88.3%, specificity 81.1%, PPV 53.4%, NPV 96.7%). DISCUSSION: A diagnostic scale is designed for assessing the risk of shunt malfunction, selecting those patients with a higher risk. The use of this scale could help the management of these patients, reducing complementary tests, as well the usual radiation suffered by these children.

[Metastatic plexus papilloma in the paediatric age: a case report and review of the literature]. / Plexopapiloma metástasico en la edad pediátrica: caso clínico y revisión de la bibliografía.

INTRODUCTION: Tumours of the choroid plexus are uncommon, with a peak incidence in the early years of life. Clinically they usually produce intracranial hypertension. Histologically, they can be divided into papillomas and carcinomas. Metastasis rarely occurs in the case of papillomas. CASE REPORT: An 11-year-old female with an intracranial lesion in the 4th ventricle and a sacral intradural lesion. A sub-occipital craniotomy with total resection of the ventricular lesion was performed and this was followed later by partial exeresis of the spinal lesion. The histological study provides the diagnosis of typical choroid plexus papilloma in the intracranial lesion and choroid plexus papilloma metastasis in the spinal lesion. A review of the literature showed that choroid plexus papillomas with metastatic lesions are very rarely found at the paediatric age. There is no general agreement on the treatment of plexus papilloma metastasis at the paediatric age, expectant management being adopted in two cases and surgical treatment involving a laminectomy in the other. CONCLUSIONS: Choroid plexus papillomas are rare, benign lesions with a good prognosis. Metastasis seldom exists. Preferred treatment is total resection of the lesion. For the treatment of metastases, there is no evidence as to which is the best approach and different alternatives have been suggested.