Cerebrospinal fluid-lymphatic fistula in a child with generalized lymphatic anomaly treated with targeted blood patch - a rare case report and review of the literature.

Spontaneous intracranial hypotension may result in debilitating postural headaches and severe neurological symptoms due to secondary cerebellar sagging. The most common cause is the cerebrospinal fluid (CSF) leak within the spinal canal. Although previously reported in only a few cases, also paraspinal lymphatic malformations causing vertebral bone destruction may occasionally result in CSF leak to these pathological formations. Here, we present a case of a 9-year-old girl with generalized lymphatic anomaly (GLA) presenting with severe postural headache. Radiological imaging revealed a typical feature of cerebellar sagging. Myelography localized the CSF leakage into vertebral bodies of C7 and Th1, which both were partly involved in pathological paravertebral masses of known lymphatic anomaly, and from there along the right C8 nerve root sleeve into the anomaly. As the C8-nerve root could not be ligated due to the risk of significant neurological injury, we attempted image-guided targeted percutaneous epidural placement of a blood patch directly into the foramen at the affected level. The procedure resulted in obliteration of the fistula and regression of cerebellar sagging, with significant relief of symptoms. Although it is an extremely rare coincidence, patients with paraspinal lymphatic malformations may develop intraspinal CSF leak into these pathological formations. The present case report suggests that besides a direct surgical obliteration of the fistula and sacrificing the nerve root, a targeted percutaneous epidural blood patch may be a possible alternative in the case of a functionally important nerve root.

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.

Low versus High Intracranial Compliance in Adult Patients with Chiari Malformation Type 1-Comparison of Long-Term Outcome After Tailored Treatment.

BACKGROUND: In patients with Chiari malformation type 1 (CMI) presenting with low intracranial compliance (ICC), foramen magnum decompression (FMD) often fails and the complication rate may be higher. We therefore routinely perform a preoperative assessment of ICC from intracranial pressure measurement. Patients with low ICC are treated with ventriculoperitoneal shunt (VPS) before FMD. In this study, we assess the outcome of patients with low ICC, compared with patients with high ICC treated with FMD alone. METHODS: We reviewed the clinical and radiologic data of all consecutive patients with CMI treated between April 2008 and June 2021. ICC was assessed by overnight measurement of the pulsatile intracranial pressure: mean wave amplitude (MWA) above a previously defined threshold for abnormality was considered a surrogate marker for low ICC. The outcome was scored by Chicago Chiari Outcome Scale. RESULTS: Of 73 patients, 23 with low ICC (average MWA 6.8 ± 1.2 mm Hg) received VPS before FMD, whereas 50 with high ICC (average MWA 4.4 ± 1.0 mm Hg) received only FMD. After a mean follow-up of 78.7 ± 41.4 months, 96% of all patients subjectively improved. The mean Chicago Chiari Outcome Scale score was 13.1 ± 2.2. Enlargement of cerebrospinal fluid space in the foramen magnum was achieved in 95% and regression of syringomyelia in 74% of cases. We found no significant difference in outcome between the patients with low and high ICC. CONCLUSIONS: By identifying patients with CMI associated with low ICC and tailoring their treatment by VPS before FMD, we achieved favorable clinical and radiologic outcomes comparable to those with high ICC.

Long-term outcome of patients treated for craniopharyngioma: a single center experience.

PURPOSE: Treatment of craniopharyngiomas (CP) is challenging due to their proximity to critical neural structures, risk of serious complications and impaired quality of life after treatment. Recurrences may occur many years after surgical resection. However, long-term outcome data are still scarce. The purpose of this retrospective study was therefore to assess the long-term results after treatment of patients with CP. MATERIAL AND METHOD: Patients surgically treated for a histologically verified CP at Oslo University Hospital between 1992 and 2015 and with at least a 5-year follow-up were included. Patients' medical records and radiological studies were reviewed. RESULTS: Sixty-one patients (mean age 35.8 ± 22.2 years) were included; 18 patients (30%) were children <18 years of age. The incidence for the study period and the referral population was 1.1 cases/million/year, with trimodal peak incidence at 6, 32 and 59 years of age. The commonest presenting symptoms were visual disturbances (62%), headache (43%) and endocrine dysfunction (34%). The transcranial approach was utilized in 79% of patients. Gross total resection (GTR) was achieved in 59%. The surgical complication rate was 20%. Three patients (5%) received radiotherapy or radiosurgery after primary resection. The mean follow-up was 139 ± 76 months, with no patients lost to follow-up. Postoperatively, 59% of patients had panhypopituitarism and 56% diabetes insipidus. Eighteen patients (30%) developed tumour recurrence after a mean follow-up of 26 ± 25 months. The 10-year overall survival (OS) rate was 75%, whereas the disease-specific survival (DSS) rate was 84%, and recurrence-free survival (RFS) 61%. Subtotal resection (STR) (p = .01) and systemic comorbidity (p = .002) were associated with worse DSS. CONCLUSION: Surgical treatment of CP, even though combined with adjuvant radiotherapy in only selected cases, provides good long-time OS and DSS, and relatively good functional outcome in long-term survivors despite postoperative morbidity, particularly endocrine dysfunction. Systemic comorbidity and STR are individual negative prognostic factors.

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.

Left-handedness should not be overrated as a risk factor for postoperative speech impairment in children after posterior fossa tumour surgery: a prospective European multicentre study.

PURPOSE: Cerebellar mutism syndrome (CMS) is a severe neurological complication of posterior fossa tumour surgery in children, and postoperative speech impairment (POSI) is the main component. Left-handedness was previously suggested as a strong risk factor for POSI. The aim of this study was to investigate the relationship between handedness and the risk of POSI. METHODS: We prospectively included children (aged < 18 years) undergoing surgery for posterior fossa tumours in 26 European centres. Handedness was assessed pre-operatively and postoperative speech status was categorised as either POSI (mutism or reduced speech) or habitual speech, based on the postoperative clinical assessment. Logistic regression was used in the risk factor analysis of POSI as a dichotomous outcome. RESULTS: Of the 500 children included, 37 (7%) were excluded from the present analysis due to enrolment at a reoperation; another 213 (43%) due to missing data about surgery (n = 37) and/or handedness (n = 146) and/or postoperative speech status (n = 53). Out of the remaining 250 (50%) patients, 20 (8%) were left-handed and 230 (92%) were right-handed. POSI was observed equally frequently regardless of handedness (5/20 [25%] in left-handed, 61/230 [27%] in right-handed, OR: 1.08 [95% CI: 0.40-3.44], p = 0.882), also when adjusted for tumour histology, location and age. CONCLUSION: We found no difference in the risk of POSI associated with handedness. Our data do not support the hypothesis that handedness should be of clinical relevance in the risk assessment of CMS.

Molecular pathological insights reveal a high number of unfavorable risk patients among children treated for medulloblastoma and CNS-PNET in Oslo 2005-2017.

BACKGROUND: An unexplained regional difference in survival was observed in previous publications on outcome for children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor (CNS-PNET) in Norway. We aimed now to reevaluate and perform a retrospective molecular-based risk stratification of all embryonal brain tumors (excluding atypical teratoid rhabdoid tumors [ATRT]) in pediatric patients, who underwent surgery and treatment at Oslo University Hospital between 2005 and 2017. PROCEDURE: Specimens from all patients <20 years of age with initial diagnosis of medulloblastoma or CNS-PNET were reviewed. Molecular analyses comprised NanoString gene expression, molecular inversion probe profiling, Sanger sequencing, and 850K-methylation analysis. Whole chromosomal aberration signatures were assessed in standard-risk non-WNT/non-SHH medullobastomas for molecular risk stratification. RESULTS: We identified 53 non-ATRT embryonal tumors among which 33 were medulloblastomas. Molecular genetic parameters including whole chromosomal aberration signatures allowed classification of 17 medulloblastomas as molecular high risk. These patients had a significantly worse 5-year overall survival than the remaining 16 medulloblastoma patients (52.9% vs. 87.1% p = 0.036). Five patients in our cohort had tumors that are considered as new entities in the 2021 classification of tumors of the central nervous system. Five tumors were re-classified as nonembryonal tumors after review. CONCLUSION: Molecular-based risk stratification of standard-risk non-WNT/non-SHH medulloblastoma enabled superior identification of medulloblastomas with dismal prognosis. Our cohort demonstrated a significantly increased fraction of standard-risk non-WNT/non-SHH medulloblastoma with molecular high-risk profile compared to other studies, which might have contributed to previously reported unfavorable outcome data.

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.

Postoperative speech impairment and surgical approach to posterior fossa tumours in children: a prospective European multicentre cohort study.

BACKGROUND: Brain tumours are the most common solid tumours in childhood. Half of these tumours occur in the posterior fossa, where surgical removal is complicated by the risk of cerebellar mutism syndrome, of which postoperative speech impairment (POSI) is a cardinal symptom, in up to 25% of patients. The surgical approach to midline tumours, mostly undertaken by transvermian or telovelar routes, has been proposed to influence the risk of POSI. We aimed to investigate the risk of developing POSI, the time course of its resolution, and its association with surgical approach and other clinical factors. METHODS: In this observational prospective multicentre cohort study, we included children (aged <18 years) undergoing primary surgery for a posterior fossa tumour at 26 centres in nine European countries. Within 72 h of surgery, the operating neurosurgeon reported details on the tumour location, surgical approach used, duration of surgery, use of traction, and other predetermined factors, using a standardised surgical report form. At 2 weeks, 2 months, and 1 year after surgery, a follow-up questionnaire was filled out by a paediatrician or neurosurgeon, including neurological examination and assessment of speech. Speech was classified as mutism, reduced speech, or habitual speech. POSI was defined as either mutism or severely reduced speech. Ordinal logistic regression was used to analyse the risk of POSI. FINDINGS: Between Aug 11, 2014, and Aug 24, 2020, we recruited 500 children. 426 (85%) patients underwent primary tumour surgery and had data available for further analysis. 192 (45%) patients were female, 234 (55%) patients were male, 81 (19%) patients were aged 0-2 years, 129 (30%) were aged 3-6 years, and 216 (51%) were aged 7-17 years. 0f 376 with known postoperative speech status, 112 (30%) developed POSI, 53 (14%) developed mutism (median 1 day [IQR 0-2]; range 0-10 days), and 59 (16%) developed reduced speech after surgery (0 days [0-1]; 0-4 days). Mutually adjusted analyses indicated that the independent risk factors for development of POSI were younger age (linear spline, p=0·0087), tumour location (four levels, p=0·0010), and tumour histology (five levels, p=0·0030); surgical approach (six levels) was not a significant risk factor (p=0·091). Tumour location outside the fourth ventricle and brainstem had a lower risk of POSI (with fourth ventricle as reference, odds ratio (OR) for cerebellar vermis 0·34 [95% CI 0·14-0·77] and OR for cerebellar hemispheres 0·23 [0·07-0·70]). Compared with pilocytic or pilomyxoid astrocytoma, a higher risk of POSI was seen for medulloblastoma (OR 2·85 [1·47-5·60]) and atypical teratoid rhabdoid tumour (10·30 [2·10-54·45]). We did not find an increased risk of POSI for transvermian surgical approach compared with telovelar (0·89 [0·46-1·73]). Probability of speech improvement from mutism reached 50% around 16 days after mutism onset. INTERPRETATION: Our data suggest that a midline tumour location, younger age, and high-grade tumour histology all increase the risk of speech impairment after posterior fossa tumour surgery. We found no evidence to recommend a preference for telovelar over transvermian surgical approach in the management of posterior fossa tumours in children in relation to the risk of developing POSI. FUNDING: The Danish Childhood Cancer Foundation, the Swedish Childhood Cancer Foundation, the UK Brain Tumour Charity, the Danish Cancer Society, Det Kgl Kjøbenhavnske Skydeselskab og Danske Broderskab, the Danish Capitol Regions Research Fund, Dagmar Marshall Foundation, Rigshospitalet's Research Fund, and Brainstrust.

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.

The role of pulsatile and static intracranial pressure measurements in the management of children with craniosynostosis-an institutional experience from 49 patients.

BACKGROUND: Although measurement of intracranial pressure (ICP) has occasionally been utilized in children with craniosynostosis (CSS), data on parameters of pulsatile ICP in CSS are still lacking, and the role of pulsatile ICP measurements in the management of CSS is not well established. METHODS: From our department's database, we retrieved the data from children in whom the measurement of static and pulsatile ICP was a part of the diagnostic work-up in different clinical situations related to CSS. Both clinical and ICP data were retrospectively reviewed and analyzed. RESULTS: We identified 49 children with CSS, median age 4.4 years (range 0.2-18.9), in whom a total of 67 diagnostic ICP measurements were undertaken between 2002 and 2014. The CSS was syndromal in 23 cases. The rationale for ICP measurement was a question of indication for cranial vault expansion surgery (CVES) in 12 patients (Group 1), of its timing in 10 patients (Group 2), of suspected abnormally elevated ICP or hydrocephalus in 11 patients (Group 3), of indication for repeated CVES in 13 patients (Group 4), or shunt dysfunction in three patients (Group 5). The average mean ICP for the whole cohort was 15.1 ± 5.5 mmHg and mean wave amplitude (MWA) 5.3 ± 2.2 mmHg. There was no significant difference in ICP parameters when compared between Groups 1-5. Fundoscopy revealed papilledema in five out of 32 children (15.6%). There were significantly higher parameters of pulsatile ICP (MWA) in patients with papilledema, but no statistically significant difference in parameters of static ICP. CONCLUSIONS: In this cohort of pediatric patients with CSS presenting with various diagnostic challenges, we found the diagnostic measurement of static and pulsatile ICP useful in selecting the optimal treatment modality and timing of surgery. Papilledema was associated with elevated pulsatile ICP, a parameter that in previous studies has been shown to correlate with impaired intracranial compliance.

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.

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.

Chiari type 1-a malformation or a syndrome? A critical review.

OBJECTIVE: As the understanding of pathophysiology behind Chiari malformation still is limited, the treatment of Chiari malformation type 1 remains rather empirical. This may result in suboptimal treatment strategy and outcome in many cases. In this review, we critically address whether the condition known today as Chiari malformation type I should rather be denoted Chiari syndrome. METHODS: The current knowledge of Chiari malformation type 1 is summarized from the historical, etymological, genetic, clinical, and in particular pathophysiological perspectives. RESULTS: There are several lines of evidence that Chiari malformation type 1 represents a condition significantly different from types 2 to 4. Unlike the other types, the type 1 should rather be considered a syndrome, thus supporting the reasons to reappraise the traditional classification of Chiari malformations. CONCLUSION: We propose that Chiari malformation type 1 should rather be denoted Chiari syndrome, while the notation malformation is maintained for types 2-4.