Functional hemispheric disconnection procedures for chronic epilepsy: history, indications, techniques, complications and current practice in Europe. A consensus statement on behalf of the EANS functional neurosurgery section.

Introduction: The surgical procedure for severe, drug-resistant, unilateral hemispheric epilepsy is challenging. Over the last decades the surgical landscape for hemispheric disconnection procedures changed from anatomical hemispherectomy to functional hemispherotomy with a reduction of complications and stable good seizure outcome. Here, a task force of European epilepsy surgeons prepared, on behalf of the EANS Section for Functional Neurosurgery, a consensus statement on different aspects of the hemispheric disconnection procedure. Research question: To determine history, indication, timing, techniques, complications and current practice in Europe for hemispheric disconnection procedures in drug-resistant epilepsy. Material and methods: Relevant literature on the topic was collected by a literature search based on the PRISMA 2020 guidelines. Results: A comprehensive overview on the historical development of hemispheric disconnection procedures for epilepsy is presented, while discussing indications, timing, surgical techniques and complications. Current practice for this procedure in European epilepsy surgery centers is provided. At present, our knowledge of long-term seizure outcomes primarily stems from open surgical disconnection procedures. Although minimal invasive surgical techniques in epilepsy are rapidly developing and reported in case reports or small case series, long-term seizure outcome remain uncertain and needs to be reported. Discussion and conclusion: This is the first paper presenting a European consensus statement regarding history, indications, techniques and complications of hemispheric disconnection procedures for different causes of chronic, drug-resistant epilepsy. Furthermore, it serves as the pioneering document to report a comprehensive overview of the current surgical practices regarding this type of surgery employed in renowned epilepsy surgery centers across Europe.

Norwegian population-based study of effectiveness of vagus nerve stimulation in patients with developmental and epileptic encephalopathies.

OBJECTIVE: Evaluate the long-term efficacy of vagus nerve stimulation (VNS) in patients with developmental and epileptic encephalopathies (DEE) compared with epilepsy patients without intellectual disability (ID). METHODS: Long-term outcomes from a Norwegian VNS quality registry are reported in 105 patients with DEEs (Lennox-Gastaut syndrome [LGS] n = 62; Dravet n = 16; Rett n = 9; other syndromes n = 18) were compared with 212 epilepsy patients without ID, with median follow-up of 88 and 72 months, respectively. Total seizure reduction was evaluated at 6, 12, 24, 36, and 60 months. Effect on different seizure types was evaluated at baseline and last observation carried forward (LOCF). RESULTS: Median monthly seizure frequency at LOCF was reduced by 42.2% (p < 0.001) in patients with DEE and by 55.8% (p < 0.001) in patients without ID. In DEE patients, ≥50% seizure reduction at 6 and 24 months were 17.1% and 37.1%, respectively, and 33.5% and 48.6% for patients without ID. Seizure reduction ≥75% at 60 months occurred in 14.3% of DEE patients and 23.1% of patients without ID. Highest median reduction was for atonic seizures, most notably 64.6% for LGS patients. A better effect was seen at 2 years among DEE patients with unchanged medication compared with those with changed medication (54.5% vs. 35.6% responders, p = 0.078). More DEE patients were reported to have greater improvement in ictal or postictal severity (43.8% vs. 28.3%, p = 0.006) and alertness (62.9% vs. 31.6%, p < 0.001) than patients without ID. For both groups, use of the magnet reduced seizure severity. Hoarseness was the most common adverse effect in both groups. In addition, DEE patients were frequently reported to have sleep disturbance, general discomfort, or abdominal problems. SIGNIFICANCE: Our data indicate that VNS is very effective for atonic seizures. Patients without ID had best overall seizure reduction, however, patients with DEE had higher retention rates probably due to other positive effects. PLAIN LANGUAGE SUMMARY: DEE refers to a group of patients with severe epilepsy and intellectual disability. Many of these patients have restricted lifestyles with frequent seizures. VNS is a treatment option for patients who do not respond well to medicines, either because of insufficient effect or serious adverse effects. Our study shows that VNS is well tolerated in this patient group and leads to a reduction in all seizure types, most notably for seizures leading to fall. Many patients experience other positive effects like shorter and milder seizures, as well as improvement in alertness.

Temporal lobe epilepsy. / Temporallappsepilepsi.

The temporal lobes are the part of the brain most likely to give rise to epileptic seizures. Seizures originating in the temporal lobes vary greatly in character; some may be so unusual that they are not even recognised as epileptic. For patients who have been diagnosed with hippocampal sclerosis and whose seizures cannot be controlled with drugs, epilepsy surgery may be a good treatment option. In this brief clinical review, we summarise the key features of epilepsy and highlight the importance of accurate and early diagnosis for achieving good clinical outcomes.

ANT-DBS in epilepsy shows no effect on selected neuropsychiatric tests.

OBJECTIVES: Deep brain stimulation of the anterior thalamic nucleus (ANT-DBS) is an established option in treatment-resistant epilepsy and obtained FDA approval in 2018. Increased psychiatric comorbidity is well known in epilepsy. The main objective of this study was to investigate possible neuropsychiatric treatment-related changes in patients receiving ANT-DBS. MATERIALS AND METHODS: Bilateral ANT electrodes were implanted in 18 adult patients with refractory epilepsy in a randomized, double-blinded study. Immediately after implantation, patients were randomized to stimulation ON (n = 8) or OFF (n = 10) for the first 6 months (blinded phase). During the next six months (open phase), both groups received active stimulation. Neuropsychiatric assessment was conducted before implantation (T1), at the end of the blinded period (T2), and 1 year after implantation (T3). RESULTS: Comparing preoperative status (T1) and 12 months (T3), postoperative outcome in all patients did not show significant differences between the two groups for any of the applied tests. Groupwise comparisons across the two first time points (the blinded period, representing the randomized controlled trial) showed no significant differences between the two groups in any of the neuropsychiatric parameters studied. Comparing test results after 6 months of stimulation in both groups (sum of ON group T1 to T2 and OFF group T2 to T3) did not show significant changes for any of the psychiatric assessments. CONCLUSIONS: Our results indicate that ANT-DBS has limited effect concerning psychiatric issues. Subjective side effects were, however, reported in individual patients.

Cognitive change after DBS in refractory epilepsy: A randomized-controlled trial.

OBJECTIVES: Deep brain stimulation of the anterior thalamic nucleus (ANT-DBS) reduces seizure frequency in patients with refractory epilepsy. There are, however, few studies on treatment-related changes in cognitive functions. The main objective of this study was to investigate cognitive changes in patients receiving ANT-DBS. We also explored whether possible effects were related to stimulation duration and whether change in seizure frequency was associated with cognitive changes. MATERIALS AND METHODS: Bilateral ANT electrodes were implanted in 18 patients with refractory epilepsy, aged 18-52 years. Immediately after implantation, patients were randomized to stimulation ON (n = 8) or OFF (n = 10) for the first 6 months (blinded phase). During the following 6-month open phase, both groups received stimulation. Neuropsychological assessments were conducted before implantation (T1), at the end of the blinded period (T2), and 1 year after implantation (T3). RESULTS: Groupwise comparisons across the three time points revealed changes in performance in two of 22 cognitive test scores: motor speed and sustained attention. We found no significant group differences in cognitive change from T1 to T2. Patients reported fewer symptoms of executive dysfunction after 12 months of stimulation. Patients showing significant improvement in seizure frequency had better performance in a measure of verbal learning. CONCLUSION: Our results indicate that ANT-DBS has very limited effects on cognitive functioning, as measured by formal tests after 6- or 12-month stimulation. ANT-DBS may have a positive influence on executive function. Our findings provide limited support for an association between change in seizure frequency and cognitive functioning.

Long-term outcome of posterior fossa medulloblastoma in patients surviving more than 20 years following primary treatment in childhood.

The aim of the study was to analyze the long-term outcome (>20 years) after treatment of posterior fossa medulloblastoma (MB) in childhood. We analyzed data from patients treated for posterior fossa MB between 1974 (introduction of the first international treatment protocol in Norway) and 1987 (when use of radiotherapy was abandoned in children under 4 years of age). Out of 47 children, 24 survived >20 years. At the time of analysis, 16 patients (median age 41 years, range 32-52) were alive (median follow-up 34 years, range 30-42), while 8 patients died 22-41 years (median 31 years) after primary treatment: one late death (after 22 years) was due to tumor recurrence whilst other 7 deaths (after 23 to 41 years) were related to the detrimental effects of the treatment (secondary tumors, stroke, severe epilepsy and depression). Observed 20- and 30-year survival rates were 51% and 44%, respectively. Despite successful treatment of MB in childhood and satisfactory tumor control during the first 20 years following primary treatment, our data indicates that even long-term survivors may die from tumor recurrence. However, the main factors causing late mortality and morbidity in long-term survivors seem to be the complications related to radiotherapy given in childhood.

Direct visual targeting versus preset coordinates for ANT-DBS in epilepsy.

OBJECTIVES: Deep brain stimulation (DBS) of the anterior thalamic nucleus (ANT) may be used against refractory focal epilepsy, but only two randomized double-blinded trials have been performed. The Oslo study was discontinued prematurely since reduction in seizure frequency was less than expected. The aim of the present study was to review the targeting used in the Oslo study and to identify the actual positions of the contacts used for stimulation. MATERIAL AND METHODS: BrainLab MRI data were available from 12 Oslo study patients. Based on MRI the coordinates of the center of the ANT were identified. The coordinates were considered as the visually identified preferred target and were compared with the target originally used for ANT electrode implantation and with the actual electrode positions estimated from post-operative CT scans. RESULTS: We found considerable differences between the visually identified preferred target, the originally planned target, and the actual electrode position. The total distance between the active electrode position and the visually identified preferred target was on average 3.3 mm on the right and 2.9 mm on the left side. CONCLUSION: Indirect targeting based on preset coordinates may contribute to explain the modest effect of ANT-DBS on seizure frequency seen in the Oslo study. Observed differences between the center of the ANT and the actual electrode position may at least in part be explained by variations in position and size of the ANT. Direct identification of the target using better MRI imaging protocols is recommended for future ANT-DBS surgery.

Clinical, Radiological, and Demographic Details of Benign External Hydrocephalus: A Population-Based Study.

BACKGROUND: Benign external hydrocephalus has an incidence of about 0.4 per 1000 live births. It affects infants and is characterized by an increasing head circumference and typical neuroimaging findings. Previously published studies on benign external hydrocephalus often contain groups of few and selected patients. METHODS: This is a follow-up of a recently published article reporting the incidence of benign external hydrocephalus. This retrospective and population-based study covers two large health regions in Norway, over a 10-year period (1994 to 2003). Infants with increasing head circumference, combined with typical radiological findings of enlarged subarachnoid spaces, were included. Information about head circumference development, neuroimaging findings, and birth delivery methods, as well as demographic details, was retrieved from the hospital medical records. RESULTS: A total of 176 children with benign external hydrocephalus were included, 86.4% being boys. At birth, the head circumference was close to normal. Mean age for when the head circumference reached abnormal values, i.e., crossing two percentiles or reaching the 97.5 percentile, was 3.4 months; none was older than seven months. Around four of five children had dilated lateral ventricles in addition to enlarged subarachnoid spaces. The neuroimaging findings tended to normalize after age 12 months. About half of the patients ended up with head circumferences at or above the 97.5 percentile. CONCLUSIONS: Most infants with benign external hydrocephalus are born with a normal head circumference that increases too fast and reaches abnormally high values before age six months. This age and gender distribution is very similar to that described for infant subdural hemorrhage.

Anterior thalamic deep brain stimulation in refractory epilepsy: A randomized, double-blinded study.

OBJECTIVES: The safety and effect on seizure frequency of anterior thalamic nucleus deep brain stimulation were studied in this prospective, randomized, double-blinded study. Patients were followed for 12 months. The first 6 months were blinded with regard to active stimulation or not. After 6 months, all patients received active stimulation. MATERIAL AND METHODS: Bilateral ANT electrodes were implanted into 18 patients suffering from focal, pharmacoresistant epilepsy. Antiepileptic treatment was kept unchanged from three months prior to operation. The Liverpool seizure severity scale (LSSS) was used to measure the burden of epilepsy. RESULTS: There was no significant difference between the 2 groups at the end of the blinded period at 6 months. However, when considering all patients and comparing 6 months of stimulation with baseline, there was a significant, 22% reduction in the frequency of all seizures (P = 0.009). Four patients had ≥50% reduction in total seizure frequency and 5 patients ≥50% reduction in focal seizures after 6 months of stimulation. No increased effect over time was shown. LSSS at 6 months compared to baseline showed no significant difference between the 2 groups, but a small, significant reduction in LSSS was found when all patients had received stimulation for 6 months. CONCLUSIONS: Our study supports results from earlier studies concerning DBS as a safe treatment option, with effects even in patients with severe, refractory epilepsy. However, our results are not as encouraging as those reported from many other, mainly unblinded, and open studies.

Quality of life and physician-reported developmental, cognitive, and social problems in children with benign external hydrocephalus-long-term follow-up.

INTRODUCTION: Benign external hydrocephalus (BEH) is characterized by too rapidly increasing head circumference in infants, combined with typical neuroimaging findings. Psychomotor developmental delay is typically seen during the first few years of life; after that, the children's development assumedly normalizes. However, little is known about the long-term effects of BEH. METHODS: In this retrospective population-based study, children diagnosed with BEH during the years 1994-2003 in Southern Norway were asked to participate. Included patients (age 8-18 years old) and their parents answered the PedsQL questionnaire. The patient's family physicians contributed by giving information from medical records, with special emphasis on developmental, cognitive, and social function. RESULTS: One hundred seventy-six children were identified with BEH. One hundred three patients and 86 parents completed the PedsQL questionnaire. Supplemental medical information for 142 of the patients was received, mainly from their family physicians. Children and adolescents with BEH score themselves better than the normative mean on health-related quality of life, while the parents score their BEH children within the normative mean, except for the school functioning subgroup, where they score significantly lower. Various developmental, physical, and social problems are reported, like mental retardation, speech problems, epilepsy, motor impairment, psychiatric disorders, and cognitive difficulties. Among these patients, there is a discrepancy in some areas between the child-reported and parent-reported quality of life. CONCLUSIONS: Children and adolescents who were diagnosed with BEH during infancy generally do well. However, for some patients, there appear to be various developmental, social, and cognitive problems, and they seem to struggle more in school than their healthy peers.

Neurosurgical treatment of gangliogliomas in children and adolescents: long-term follow-up of a single-institution series of 32 patients.

OBJECT: The object of this study was to delineate long-term results of the surgical treatment of pediatric tumors classified as ganglioglioma or gangliocytoma. METHODS: A cohort of consecutive patients 19 years or younger who had undergone primary resection of CNS tumors during the years 1980-2016 at a single institution were reviewed in this retrospective study of surgical morbidity, mortality, and academic achievement and/or work participation. Gross motor function and activities of daily living were scored using the Barthel Index (BI). RESULTS: Patient records for 32 consecutive children and adolescents who had undergone resection for a ganglioglioma were included in this study. Of the 32 patients, 13 were in the first decade at the first surgery, whereas 19 were in the second decade. The male/female ratio was 1.0 (16/16). No patient was lost to follow-up. The tumor was localized to the supratentorial compartment in 26 patients, to the posterior fossa in 5 patients, and to the spinal cord in 1 patient. Only two of the tumors were classified as anaplastic. Of the 30 low-grade tumors, 2 were classified as gangliocytomas, 6 were desmoplastic infantile gangliogliomas, and 22 were ordinary gangliogliomas. The aim of primary surgery was gross-total resection (GTR) and was achieved in 23 patients (71.9%). Altogether, 43 tumor resections were performed. Eight patients underwent a second resection from 1 to 10 years after primary surgery and three of these also had a third resection from 2 to 24 years after initial surgery. The reason for further resection was clinical (seizure control failure/recurrence of epilepsy or progressive neurological deficit) and/or residual tumor progression/recurrence. There was no operative mortality in this series and all 32 patients are alive with follow-up periods from 0.5 to 36 years (median 14 years). Observed 14-year survival is thus 100%. One out of two children with primary anaplastic tumor received local radiotherapy (proton) postoperatively. The other 31 patients did not have any kind of non-surgical adjuvant therapy. Twenty-one out of 26 children with supratentorial tumor had epilepsy as one of their presenting symptoms. Nineteen of these became seizure-free after initial surgery (18 of them after GTR), but 3 patients experienced recurrence of seizures within some years. Functional outcome in terms of ADL, schooling, and work participation was gratifying in most patients. Five patients have persistent hydrocephalus (HC), treated with ventriculoperitoneal (VP) shunts. CONCLUSION: Low-grade gangliogliomas (GGs) can be surgically treated with good long-term results including seizure and tumor control as well as school and working participation.

Epidemiology of Benign External Hydrocephalus in Norway-A Population-Based Study.

BACKGROUND: Benign external hydrocephalus is defined as a rapidly increasing head circumference (occipitofrontal circumference) with characteristic radiological findings of increased subarachnoid cerebrospinal fluid spaces on neuroimaging. The incidence of benign external hydrocephalus has not been previously reported, and there is no available information on the ratio of benign external hydrocephalus in the population of hydrocephalic children. METHODS: This study is retrospective and population-based study, geographically covering two health regions in the southern half of Norway with a total mean population of 3.34 million in the ten-year study period, constituting approximately 75% of the Norwegian population. Children with a head circumference crossing two percentiles, or greater than the 97.5th percentile, and with typical imaging findings of enlarged frontal subarachnoid spaces with or without enlarged ventricles were included. Children were excluded if they had a history of head trauma, intracranial hemorrhage, central nervous system infection, other known causes of hydrocephalus, or were born preterm defined as birth before 37 weeks of gestation. RESULTS: A total of 176 children fitting the criteria were identified, giving an incidence of 0.4 per 1000 live births. One hundred fifty-two (86.4%) of the patients were male, and mean age at referral was 7.3 months. Increasing head circumference was the main reason for referral in 158 (89.8%) patients and the only finding in 60 (34.1%) patients. Thirty-seven (21%) children had normal ventricles on imaging; the remainder had increased ventricular size. The incidence of pediatric hydrocephalus in Norway is reported to be 0.75 per 1000 live births, thus benign external hydrocephalus accounts for approximately 50% of hydrocephalic conditions in this population. CONCLUSIONS: The incidence of benign external hydrocephalus was found to be 0.4 per 1000 live births in this population.

Use of advanced therapies for Parkinson's disease in Norway. / Bruk av avansert behandling ved Parkinsons sykdom i Norge.

BACKGROUND: Many patients with Parkinson's disease with severe motor fluctuations benefit from advanced therapies ­ either deep brain stimulation or continuous infusion therapy with levodopa-carbidopa intestinal gel or apomorphine. In Norway, deep brain stimulation is provided as a shared national or multi-regional service. The treatment is currently available at Oslo University Hospital and St. Olavs Hospital; prior to 2012 it was also available at Haukeland University Hospital. Infusion therapy has no similar geographical restrictions. We therefore wished to examine geographical differences in the use of the two most common forms of advanced therapy for Parkinson's disease. MATERIAL AND METHOD: The county of residence of all patients receiving deep brain stimulation or infusion therapy with levodopa-carbidopa intestinal gel in the period 2009 ­ 2013 was recorded using data from hospital episode statistics and the Norwegian Prescription Database, respectively. RESULTS: A total of 262 patients with Parkinson's disease began advanced therapy, 146 with deep brain stimulation and 116 with levodopa-carbidopa infusion. Four counties differed significantly from the others in their use of the two methods. Møre og Romsdal, Nordland and Sør-Trøndelag treated a significantly greater proportion of patients with deep brain stimulation, while Rogaland treated a significantly greater proportion with levodopa-carbidopa infusion therapy. INTERPRETATION: Advanced therapies for Parkinson's disease are offered throughout Norway, but there are significant geographical differences in the type of therapy initiated. One possible explanation is that patients in different counties receive different information about the therapeutic options available.

Seizure outcomes of temporal lobe epilepsy surgery in patients with normal MRI and without specific histopathology.

BACKGROUND: Seizure outcome following surgery in pharmacoresistant temporal lobe epilepsy patients with normal magnetic resonance imaging and normal or non-specific histopathology is not sufficiently presented in the literature. METHODS: In a retrospective design, we reviewed data of 263 patients who had undergone temporal lobe epilepsy surgery and identified 26 (9.9%) who met the inclusion criteria. Seizure outcomes were determined at 2-year follow-up. Potential predictors of Engel class I (satisfactory outcome) were identified by logistic regression analyses. RESULTS: Engel class I outcome was achieved in 61.5% of patients, 50% being completely seizure free (Engel class IA outcome). The strongest predictors of satisfactory outcome were typical ictal seizure semiology (p = 0.048) and localised ictal discharges on scalp EEG (p = 0.036). CONCLUSION: Surgery might be an effective treatment choice for the majority of these patients, although outcomes are less favourable than in patients with magnetic resonance imaging-defined lesional temporal lobe epilepsy. Typical ictal seizure semiology and localised ictal discharges on scalp EEG were predictors of Engel class I outcome.

Neurocognitive and psychosocial function in children with benign external hydrocephalus (BEH)-a long-term follow-up study.

INTRODUCTION: Long-term impact of benign external hydrocephalus (BEH) on cognition is largely unknown, and indication for neurosurgical CSF diversion procedure is debated. This study reports neuropsychological and psychosocial function in operated and non-operated BEH children. METHODS: Eighty-six children (76 males) between 8 and 18 years (mean 13.9) diagnosed with BEH before 12 months were included, of whom 30.2 % were operated. Participants completed neuropsychological tests and questionnaires covering quality of life (PedsQL) and executive function (BRIEF). RESULTS: Both operated and non-operated BEH children performed significantly below normative means on several neuropsychological tests. The children scored themselves higher than the norm average on PedsQL; however, the parents reported life quality comparable to other children. Operated children performed poorer compared with non-operated children on tests of psychomotor speed, attention span, executive function, motor speed and coordination, and on the BRIEF subscale Monitoring. Operated children, but not their parents, reported more problems on PedsQL subscale School than non-operated children. DISCUSSION: Children with BEH display long-term subtle neurocognitive difficulties. Non-operated children performed significantly better on some neuropsychological measures and reported less psychosocial problems. This difference may be caused by a selection bias: neurosurgical intervention was more likely in children with clinically more pronounced symptoms.

Seizure outcomes in relation to the extent of resection of the perifocal fluorodeoxyglucose and flumazenil PET abnormalities in anteromedial temporal lobectomy.

BACKGROUND: The area of predominant perifocal [(18)F]fluorodeoxyglucose ((18)F-FDG) hypometabolism and reduced [(11)C]flumazenil ((11)C-FMZ) -binding on PET scans is currently considered to contain the epileptogenic zone and corresponds anatomically to the area localizing epileptogenicity in patients with temporal lobe epilepsy (TLE). The question is whether the volume of the perifocal pre-operative PET abnormalities, the extent of their resection, and the volume of the non-resected abnormalities affects the post-operative seizure outcome. METHODS: The sample group consisted of 32 patients with mesial temporal sclerosis who underwent anteromedial temporal lobe resection for refractory TLE. All patients had pathologic perifocal findings on both of the PET modalities as well as on the whole-brain MRI. The volumetric data of the PET and MRI abnormalities within the resected temporal lobe were estimated by automated quantitative voxel-based analysis. The obtained volumetric data were investigated in relation to the outcome subgroups of patients (Engel classification) determined at the 2-year post-operative follow-up. RESULTS: The mean volume of the pre-operative perifocal (18)F-FDG- and (11)C-FMZ PET abnormalities in the volumes of interest (VOI) of the epileptogenic temporal lobe, the mean resected volume of these PET abnormalities, the mean volume of the non-resected PET abnormalities, and the mean MRI-derived resected volume were not significantly related to the outcome subgroups and had a low prediction for individual freedom from seizures. CONCLUSIONS: The extent of pre-surgical perifocal PET abnormalities, the extent of their resection, and the extent of non-resected abnormalities were not useful predictors of individual freedom from seizures in patients with TLE.

Posterior fossa ependymoblastoma diagnosed in the second month of life: uneventful 12 years survival after gross total resection followed by chemotherapy.

We report on an infant who underwent gross total resection (GTR) of a posterior fossa ependymoblastoma in the second month of life followed by chemotherapy with uneventful long-term survival for 12 years. Postoperative radiotherapy has been considered obligate to have a chance for prolonged survival, but is inadvisable in infants. To our knowledge, this is the first reported long-term survival in an infant treated for ependymoblastoma.

Neurosurgical treatment of brain tumors in the first 6 months of life: long-term follow-up of a single consecutive institutional series of 30 patients.

OBJECT: The aim of this study is to delineate the long-term results for patients going through surgery for pediatric brain tumors in the first 6 months of life. METHODS: Thirty consecutive children (1-182 days old) who underwent primary resection for a brain tumor during the years 1973-2012 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily life were scored according to the Barthel index. RESULTS: Of the 30 patients, 11 children had surgery in the first 3 months of life (1 to 88 days) and 19 were aged 3 to 6 months (94-182 days) at the time of surgery. The male/female ratio was 1.0 (15/15). No patients were lost to follow-up. Two patients died in the postoperative period (30 days). Another eight patients died during the follow-up. Twenty patients are alive, with follow-up times from 2 to 38 years, median 13 years. Among the 28 children who survived the primary resection, eight underwent repeat surgery from 6 months to 5 years after the first operation. Two children were operated three times, and one of these also a fourth time. Gross total resection (GTR) was achieved in 20 of the primary resections, subtotal resection (STR) in 6, and in the last 4, only a biopsy or a partial resection was performed. Nine children received adjuvant chemotherapy and three of these also radiotherapy (in the years 1979-1987). Among the 20 survivors, the Barthel index is normal (100) in 18 patients, 40 in one, and 20 in the last one. Eight tumors were located to the posterior fossa, and 22 were supratentorial. Eighteen tumors were histologically low-grade (WHO grade I-II), most of these were plexus papillomas (7) or astrocytomas (7), and 12 were high-grade (WHO grade III-IV); PNET/medulloblastomas (6), ependymoma (2), glioblastoma (2), teratoma, and plexus carcinoma. CONCLUSION: Infants with brain tumors may clearly benefit from surgical resection with favorable results even for prolonged periods of time. Ten children died, two of them with prolonged survival for 9 and 29 years. Among the 20 survivors, a stable very long-term result appears obtainable in 18 also when it comes to quality of life. Four of the survivors have been treated for highly malignant tumors with a follow-up of 5, 11, 14, and 26 years. One of our infant patients treated for GBM in 1982, lived for 29 years, however, with a progressive decline in the quality of life probably due to postoperative whole-brain radiation.

Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients.

OBJECT: The authors delineate the long-term results of surgical treatment for pediatric low-grade midbrain glioma. METHODS: A series of 15 consecutive patients (age range 0-15 years) who underwent primary tumor resection for a low-grade midbrain glioma during the years 1989-2010 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index. RESULTS: Of the 15 patients, 10 were in their 1st decade (age 0-9 years) and 5 were in their 2nd decade of life (age 10-15 years) at the time of surgery. The male/female ratio was 0.50 (5:10). No patients were lost to follow-up. One patient died in the postoperative period (32 days posttreatment). Another 2 patients died during follow-up. One patient succumbed to acute bleeding in the resection cavity 8 months after surgery, and the other died of shunt failure 21 years after initial treatment. Twelve patients are alive at the time of this writing, with follow-up periods from 3 to 24 years (median 8 years). Among the 12 survivors, the Barthel Index scores were normal (100) in 11 patients and 80 in 1 patient. A total of 25 tumor resections were performed. In 1 patient, further resection was performed 5 days after initial resection due to MRI-confirmed residual tumor. Another 5 patients underwent repeat tumor resection after MRI-confirmed progressive tumor disease and clinical deterioration ranging from 3 months to 4 years after the initial operation. Three of these 5 patients also underwent a third resection, and 1 of the 3 underwent a fourth operation. Six children received adjuvant therapy: local radiotherapy in 2 patients, chemotherapy in 3 patients, and both in 1 patient. Twelve (80%) of the 15 patients needed treatment for persistent hydrocephalus. CONCLUSIONS: Selected cases of low-grade midbrain gliomas may clearly benefit from resection with favorable results, even for prolonged periods. Three patients in the present series died, one of whom had a prolonged survival period of 21 years. Among the 12 survivors, stable long-term results appeared obtainable in at least 9. One patient died of acute hemorrhage 8 months after initial resection; otherwise, rapid tumor progression and death were not observed. Forty percent of the patients received adjuvant treatment, with local radiotherapy, chemotherapy, or both.