Preoperative estimation of intracranial compliance in symptomatic children with Chiari malformation type 1: impact on outcome and risk of complications.

BACKGROUND: The role of reduced intracranial compliance (ICC) in the outcome after foramen magnum decompression (FMD) was demonstrated in adults with Chiari malformation Type 1 (CMI). However, similar observations from children treated for CMI are missing. METHODS: We reviewed pediatric cases of CMI referred to FMD between 2006 and 2022. Children with clinical and/or radiological signs suggesting reduced ICC (Group A) underwent overnight measurements of the pulsatile intracranial pressure (ICP): mean ICP wave amplitude (MWA) served as a surrogate marker of ICC. Children with more typical symptoms of CMI (Group B) underwent FMD without preoperative ICC estimation. This study presents the clinical, radiological, and outcome differences between these groups. RESULTS: Sixty-four children (mean age 11.1 ± 4.3 years) underwent FMD: In Group A (n = 30), the finding of reduced ICC as estimated from preoperative ICP measurement resulted in CSF diversion (ventriculoperitoneal shunt) before FMD in 11 children. Two patients required shunt due to complications after FMD (total shunt rate 43%). In Group B (n = 34) treated with FMD without preoperative ICC estimation, five children (15%) required shunting due to complications. In Group A, we found a significantly higher frequency of headache, nausea, fatigue, and dizziness. The outcome assessed by the modified Chicago Chiari Outcome Scale (mean follow-up 83 ± 57 months) was comparable between the groups, but the complication rate after FMD was significantly lower in Group A (7% vs. 32%; p = 0.011). The number of procedures (ICP measurement, FMD, shunt, re-do FMD, shunt revisions) was significantly higher in Group A (2.6 ± 0.9 vs. 1.5 ± 1.1 per patient; p < 0.001). CONCLUSION: In symptomatic children with CMI, the preoperative estimation of ICC from the overnight measurement of pulsatile ICP was more reliable for identifying those with reduced ICC than clinical and radiological assessment alone. When children with abnormally reduced ICC were identified and treated with CSF diversion before FMD, the complication rate was significantly reduced.

Ruptured Aneurysm of the Anterior Communicating Artery in a Newborn: A Case Report and Review of the Literature.

We report the case of a 3-week-old neonate who presented with massive subarachnoid and intraventricular hemorrhage from a ruptured aneurysm of the anterior communicating artery (ACommA). An attempt on endovascular treatment ended up with therapeutic closure of the parent artery. However, since further investigation revealed a disastrous supratentorial cerebral infarction as a result of the hemorrhage, active treatment was terminated and the neonate died a few days after the initial stroke. To the best of our knowledge and after reviewing available literature, this is one of only five cases of ACommA aneurysm in newborns reported to date. Bleeding from an ACommA aneurysm in a neonate thus represents an extreme clinical rarity. There are no available data comparing the efficacy and safety of microsurgical versus endovascular treatment in neonates and small infants, but the latter option may at least reduce the risk associated with open surgery and further blood loss in this age group.

[Moyamoya disease in children] / Moyamoya hos barn.

Moyamoya is a rare condition that affects the blood vessels of the brain in children and young adults. It can cause both ischaemic stroke and cerebral haemorrhage. Although established diagnostic criteria and examinations exist, limited knowledge of the condition often leads to a mistaken or delayed diagnosis. Treatment consists of antiplatelet drugs and surgical revascularisation. Prognosis after successful surgery is good, but the disease requires a dedicated medical team.

Whole-exome sequencing in syndromic craniosynostosis increases diagnostic yield and identifies candidate genes in osteogenic signaling pathways.

Craniosynostosis (CS) is a common congenital anomaly defined by premature fusion of one or more cranial sutures. Syndromic CS involves additional organ anomalies or neurocognitive deficits and accounts for 25%-30% of the cases. In a recent population-based study by our group, 84% of the syndromic CS cases had a genetically verified diagnosis after targeted analyses. A number of different genetic causes were detected, confirming that syndromic CS is highly heterogeneous. In this study, we performed whole-exome sequencing of 10 children and parents from the same cohort where previous genetic results were negative. We detected pathogenic, or likely pathogenic, variants in four additional genes (NFIA, EXTL3, POLR2A, and FOXP2) associated with rare conditions. In two of these (POLR2A and FOXP2), CS has not previously been reported. We further detected a rare predicted damaging variant in SH3BP4, which has not previously been related to human disease. All findings were clustered in genes involved in the pathways of osteogenesis and suture patency. We conclude that whole-exome sequencing expands the list of genes associated with syndromic CS, and provides new candidate genes in osteogenic signaling pathways.

Choroid Plexus Tumors in Children: Long-Term Follow-Up of Consecutive Single-Institutional Series of 59 Patients Treated over a Period of 8 Decades (1939-2020).

OBJECTIVE: To present long-term follow-up of a consecutive single-institutional series of patients treated for choroid plexus tumors over 8 decades. METHODS: From 1939 to 2020, 59 children were treated for choroid plexus tumors. Median age at diagnosis was 1.7 years. RESULTS: Gross total resection was achieved in 51 patients (86%). Ten patients (17%) underwent >1 resection. During the first 4 decades of the study (1939-1979), 14 patients with plexus papillomas were treated. Operative mortality was 50%, with 6 of the remaining 7 patients experiencing excellent survival with follow-up periods of 41-81 years. In the last 4 decades (1980-2020), 38 patients had low-grade tumors, and all were alive at the latest follow-up (range, 0.5-39 years). Observed 5-year survival in this subgroup was 100% (n = 30), as was observed 10-year survival (n = 26). One of 7 (14%) patients with atypical choroid plexus papilloma and 3 of 31 patients (10%) with choroid plexus papilloma underwent a second resection owing to recurrent tumor. At last follow-up, 47 patients (80%) were alive; 45 (96%) had a Barthel Index score of 100 and 2 had a Barthel Index score of 50. Today 25 patients are adults (20-59 years old); 17 work full-time, 4 work part-time, and 4 are unable to work. CONCLUSIONS: Low-grade choroid plexus tumors can be cured with gross total resection alone, with excellent long-term survival and functionality. The vast majority of survivors live independently as adults and work full-time. Recurrences are uncommon (8.7%), appear within the first few years after primary surgery, and can be treated with repeat resections.

Outcome after treatment of pediatric supratentorial ependymoma: long-term follow-up of a single consecutive institutional series of 26 patients.

BACKGROUND: Long-term outcome after surgical treatment of supratentorial ependymoma (STE) in children has not been extensively reported. FINDINGS: We identified 26 children who underwent primary tumor resection of STE between 1953 and 2011, with at least 8 years follow-up. Ten patients (38%) had anaplastic and 16 had low grade ependymoma. Four of 15 children (26%) treated in the years 1953-1976 survived more than 5 years, but the observed 10-year survival was only 7%. One patient lived for 37 years, and second surgery for a local recurrent lesion disclosed a glioblastoma, possibly secondary to radiotherapy. In contrast, the observed 5-year survival rate for 11 children treated in the years 1992-2011 was 8/11 (73%) and observed 10- and 25-year survival rates were 70% and 66%, respectively. Eight patients were alive and tumor-free with follow-up periods of 8-27 (median 18) years, all treated after 1992. Five of these long-term survivors were 23-39 years old with full-time (n = 3) or part-time (n = 2) work. The last three patients were still children (9-12 years old): one with good function and two with major neurological deficits. The majority of patients (n = 18) received adjuvant radiotherapy and eight children no adjuvant treatment. Repeated resections for residual or recurrent tumor were necessary in 11 patients (42%), mostly due to local disease with progressive clinical symptoms. Eight patients underwent only one repeat resection, whereas three patients had two or more repeat resections within 18 years after initial surgery. Four patients were tumor-free after repeated resections at the latest follow-up, 2-13 years after last surgery. CONCLUSION: Pediatric STE has a marked risk for local recurrence even after gross total resection and postoperative radiotherapy, but survival has increased following the introduction of modern treatment in recent years. Repeated surgery is an important part of treatment and may lead to persistent tumor control.

Outcome After Treatment of Spinal Ependymoma in Children and Adolescents: Long-Term Follow-up of a Single Consecutive Institutional Series of 33 Patients Treated Over Eight Decades.

BACKGROUND: Long-term outcomes for pediatric patients treated for spinal ependymoma are unknown. METHODS: We performed a retrospective analysis of outcome data from 33 children and young adults (0-22 years) who were operated on for a spinal ependymoma at our institution during the last 8 decades (1938-2019). RESULTS: Nineteen patients are alive, with follow-up period up to 60 years. Twelve of them are tumor-free, and 7 are alive with disease. Fourteen patients are dead, 9 of them due to recurrent and/or progressive disease 1-56 years (median: 11 years) after the initial surgery. Four of the deceased patients were treated before 1948, 3 of them with excellent long-term survival for 62-66 years after the initial surgery. Tumor recurrence was observed in half of the patients, both local at the site of the primary tumor resection as well as widespread intraspinal presentations. Recurrences were observed within months but also occurred after up to 20 years after initial treatment. After the implementation of magnetic resonance imaging in 1987, details of recurrent disease became more easily demonstrated. Repeated resections were performed when the symptomatic spinal disease was in progress (n = 11). Furthermore, 2 patients have intracranial tumor manifestations, 1 of them underwent resection of a suprasellar tumor in 1991. Four deceased patients experienced aggressive extraspinal progressive disease requiring multiple surgeries, including pulmonary metastasis in 1 of them. CONCLUSIONS: Pediatric spinal ependymomas can be treated with favorable results and functional outcome may be good even after more than half a century of follow-up. Nevertheless, unexpected and late recurrences may occur, and life-long follow-up is therefore recommended.

Outcomes in adulthood after neurosurgical treatment of brain tumors in the first 3 years of life: long-term follow-up of a single consecutive institutional series of 97 patients.

BACKGROUND: Long-term outcome for children who underwent surgery for brain tumors in the first 3 years of life is not well-known. METHODS: We performed a retrospective study on surgical morbidity, mortality rate, academic achievement, and work participation in children below 3 years of age who underwent primary tumor resection for a brain tumor in the period from 1973 to 1998. Gross motor function and activities of daily life were scored according to the Barthel Index. Long-term survivors were defined as with a survival from primary diagnosis of 20 years or more. FINDINGS: Ninety-seven consecutive children were included. No patient was lost to follow-up. Gross total resection was achieved in 67 children during the primary procedure, 25 had subtotal resections, and 5 had only partial resection. The 20-year survival figures for the 46 children with high-grade tumors was 33%, and the corresponding figures for 51 patients treated for low-grade tumors was 82%. Five of the 57 20-year-survivors died 21, 29, 30, 30, and 41 years, respectively, following primary surgery. Fifty of the 52 long-term survivors had a Barthel Index (BI) of 100, while the remaining two had a BI of 40. Twelve patients were long-term survivors after treatment for HG tumors (26%), while 40 of the 51 patients treated for LG tumors (78%) were alive. Thirty-two of the 52 long-term survivors were in full-time work and 29 of them after treatment for LG tumors. Another 10 were in part-time work, while the last 10 individuals had no working capacity. CONCLUSION: Survival is better for patients with low-grade tumors compared with those with high-grade tumors. The functional level of long-term survivors is affected by adjuvant therapy and radiotherapy in particular. Neurosurgical intervention in itself is safe and plausible for pediatric brain tumor patients below 3 years of age. However, there should be a focus on potential late affects, and survivors should be followed by knowledgeable clinical staff for the neoplastic disease as well as for potential side effects. In this consecutive series, a 33% 20-year survival for children treated for HG tumors and 82% for patients with LG tumors was observed. The patients with LG tumors who had been treated with surgical resection without any adjuvant therapy showed a good clinical outcome as adults, and two-thirds of them were in full-time work.

Social attainment in physically well-functioning long-term survivors of pediatric brain tumour; the role of executive dysfunction, fatigue, and psychological and emotional symptoms.

The purpose of this cross-sectional study was to investigate long-term social attainment in physically well-functioning adult survivors of pediatric brain tumour (PBT) and identify demographic, medical, and psychological factors related to poor social outcomes, with a special focus on the significance of executive dysfunction. One hundred and fourteen PBT survivors and a healthy control group provided personal data on social outcomes, i.e., education, work, and government benefits, and completed questionnaires on executive function (EF), psychological and emotional difficulties, and fatigue. A significantly higher number of survivors compared to healthy controls reported having received educational adjustments and substantial government benefits, and significantly more survivors than controls were currently not engaged in regular employment/training. PBT survivors and healthy controls did not differ on educational level or living situation. The factors most strongly associated with poor social outcomes were self-reported executive dysfunction, difficulties with adaptive functioning, and fatigue. The findings show that physically well-functioning PBT survivors are at risk of poorer social outcomes and financial dependence in adulthood compared to their healthy peers, and underline the importance of investigating EF in short- and long-term follow-ups. Future rehabilitation efforts should focus more on compensatory strategies for executive dysfunction and improving EF skills.

Benefits of clinical criteria and high-throughput sequencing for diagnosing children with syndromic craniosynostosis.

An accurate diagnosis of syndromic craniosynostosis (CS) is important for personalized treatment, surveillance, and genetic counselling. We describe detailed clinical criteria for syndromic CS and the distribution of genetic diagnoses within the cohort. The prospective registry of the Norwegian National Unit for Craniofacial Surgery was used to retrieve individuals with syndromic CS born between 1 January 2002 and 30 June 2019. All individuals were assessed by a clinical geneticist and classified using defined clinical criteria. A stepwise approach consisting of single-gene analysis, comparative genomic hybridization (aCGH), and exome-based high-throughput sequencing, first filtering for 72 genes associated with syndromic CS, followed by an extended trio-based panel of 1570 genes were offered to all syndromic CS cases. A total of 381 individuals were registered with CS, of whom 104 (27%) were clinically classified as syndromic CS. Using the single-gene analysis, aCGH, and custom-designed panel, a genetic diagnosis was confirmed in 73% of the individuals (n = 94). The diagnostic yield increased to 84% after adding the results from the extended trio-based panel. Common causes of syndromic CS were found in 53 individuals (56%), whereas 26 (28%) had other genetic syndromes, including 17 individuals with syndromes not commonly associated with CS. Only 15 individuals (16%) had negative genetic analyses. Using the defined combination of clinical criteria, we detected among the highest numbers of syndromic CS cases reported, confirmed by a high genetic diagnostic yield of 84%. The observed genetic heterogeneity encourages a broad genetic approach in diagnosing syndromic CS.

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.

Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival.

OBJECTIVE: Ependymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution. METHODS: The authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years. RESULTS: The authors identified 22 children (median age at the time of surgery 3 years, range 0-18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945-1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987-2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work. CONCLUSIONS: Pediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.

Persistent shunt dependency in children treated with CSF diversion for idiopathic intracranial hypertension (IIH).

Long-term shunt dependency rates in children treated for IIH with CSF diversion have not been established. We therefore present our experience with 4 children shunted for Idiopathic Intracranial Hypertension (IIH) during the years 1988-2000 with very long-time follow-up. Two out of these patients have experienced late or very late episodes of severe shunt failure during the second or third decade after initial shunt treatment. They were all boys and may not be representative for IIH patients as a whole. Two of them appear, however, to be permanently shunt dependent, indicating that long-term shunt dependency in children treated for IIH with CSF diversion may be more common than previously expected.

Adult outcome after neurosurgical treatment of brain tumours in the first year of life: long-term follow-up of a single consecutive institutional series of 34 patients.

BACKGROUND: Long-term results for adult patients who underwent surgery for paediatric brain tumours in the first year of life have not been reported. METHODS: We performed a retrospective study on surgical morbidity, mortality rate, academic achievement and/or work participation in patients who underwent primary tumour resection for a brain tumour as infants in the period from 1973 to 1998. Gross motor function and activities of daily life were scored according to the Barthel Index. FINDINGS: Thirty-four consecutive children were included. No patient was lost to follow-up. Twenty-two children (65%) underwent gross total resection (GTR), 10 had subtotal resections (STR) and 2 had only partial resection during primary surgery. Nine children were additionally surgically treated for hydrocephalus (HC), many of them with repeat operations for shunt malfunction during follow-up. Three children died within 30 days following GTR of highly vascular tumours. Seventeen (50%) of the infants had high-grade tumours with 1-month, 1-year and 20-year survival figures of 88, 30 and 30%. The corresponding figures for 17 patients treated for low-grade tumours were 94%, 88% and 88%, respectively. Seventeen patients (50%) are still alive as adult long-term survivors at median age of 29 years (range 20 to 43 years). Three died after 29, 30 and 41 years, respectively. At the latest follow-up, 16 out of 17 long-term survivors have a Barthel Index (BI) of 100, while the remaining one has a BI of 40. Two long-term survivors of a high-grade tumour treated 30 and 35 years ago are severely handicapped and have no working capacity. The 15 long-term survivors after treatment for a low-grade tumour are all in daily activity as students (4), in part-time work (3) or full-time work (8). CONCLUSION: A brain tumour occurring in the first year of life is a serious threat to the patient and the family, particularly in case of high-grade tumours. In our small cohort, the results from long-term survivors of high-grade tumour group are depressing and confirm the deleterious effect of radiotherapy given to infants decades ago. The infants with low-grade tumours who could be treated with surgical resection without any adjuvant therapy show a good clinical outcome as adults. For parents of these latter patients, the results are far better than feared in advance.

Neurosurgical treatment of pediatric pleomorphic xanthoastrocytomas: long-term follow-up of a single-institution, consecutive series of 12 patients.

OBJECTIVEThe authors conducted a study to delineate the long-term results of the surgical treatment of pediatric pleomorphic xanthoastrocytomas (PXAs).METHODSAll consecutive children and adolescents (0-20 years) who underwent primary tumor resection for a PXA during the years 1972-2015 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.RESULTSOf the 12 patients, 8 patients were in the 1st decade of life and 4 in the 2nd. The male/female ratio was 6:6. No patient was lost to follow-up. One patient presented with severe progressive tumor disease and died within 3 months after repeated resection. Another child died 3 days following a second surgical procedure involving gross-total resection (GTR) 8 years after the initial operation. The other 10 patients were alive at the latest follow-up when they reached the median age of 34 years (range 11-60 years). The median follow-up duration was 22 years (range 2-41 years). Barthel Index score was 100 in all 10 survivors. A total 18 tumor resections were performed. Five patients underwent a second tumor resection after MRI/CT confirmed recurrent tumor disease, from 6 months up to 17 years after the initial operation. Only one of our patients received adjuvant therapy: a 19-year-old male who underwent resection (GTR) for a right-sided temporal tumor in 1976. This particular tumor was originally classified as astrocytoma WHO grade IV, and postoperative radiotherapy (54 Gy) was given. The histology was reclassified to that of a PXA. Seven of 8 children whose primary tumor resection was performed more than 20 years ago are alive as of this writing-i.e., 88% observed 20-year survival. These are long-term survivors with good clinical function and all are in full- or part-time work.CONCLUSIONSPediatric patients with PXA can be treated with resection alone with rewarding results. Recurrences are not uncommon, but repeated surgery is well tolerated and should be considered in low-grade cases before adjuvant therapy is implemented. Follow-up including repeated MRI is important during the first postoperative years, since individual patients may have a more aggressive tumor course.

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.