Preoperative word-finding difficulties in children with posterior fossa tumours: a European cross-sectional study.

PURPOSE: Posterior fossa tumour surgery in children entails a high risk for severe speech and language impairments, but few studies have investigated the effect of the tumour on language prior to surgery. The current crosslinguistic study addresses this gap. We investigated the prevalence of preoperative word-finding difficulties, examined associations with medical and demographic characteristics, and analysed lexical errors. METHODS: We included 148 children aged 5-17 years with a posterior fossa tumour. Word-finding ability was assessed by means of a picture-naming test, Wordrace, and difficulties in accuracy and speed were identified by cut-off values. A norm-based subanalysis evaluated performance in a Swedish subsample. We compared the demographic and medical characteristics of children with slow, inaccurate, or combined slow and inaccurate word finding to the characteristics of children without word-finding difficulties and conducted a lexical error analysis. RESULTS: Thirty-seven percent (n = 55) presented with slow word finding, 24% (n = 35) with inaccurate word finding, and 16% (n = 23) with both slow and inaccurate word finding. Children with posterior fossa tumours were twice as slow as children in the norming sample. Right-hemisphere and brainstem location posed a higher risk for preoperative word-finding difficulties, relative to left-hemisphere location, and difficulties were more prevalent in boys than in girls. The most frequent errors were lack of response and semantically related sideordinated words. CONCLUSION: Word-finding difficulties are frequent in children with posterior fossa tumours, especially in boys and in children with right-hemisphere and brainstem tumours. Errors resemble those observed in typical development and children with word-finding difficulties.

Postoperative speech impairment and cranial nerve deficits after secondary surgery of posterior fossa tumours in childhood: a prospective European multicentre study.

PURPOSE: Brain tumours constitute 25% of childhood neoplasms, and half of them are in the posterior fossa. Surgery is a fundamental component of therapy, because gross total resection is associated with a higher progression-free survival. Patients with residual tumour, progression of residual tumour or disease recurrence commonly require secondary surgery. We prospectively investigated the risk of postoperative speech impairment (POSI) and cranial nerve dysfunction (CND) following primary and secondary resection for posterior cranial fossa tumours. METHODS: In the Nordic-European study of the cerebellar mutism syndrome, we prospectively included children undergoing posterior fossa tumour resection or open biopsy in one of the 26 participating European centres. Neurological status was assessed preoperatively, and surgical details were noted post-operatively. Patients were followed up 2 weeks, 2 months and 1 year postoperatively. Here, we analyse the risk of postoperative speech impairment (POSI), defined as either mutism or reduced speech, and cranial nerve dysfunction (CND) following secondary, as compared to primary, surgery. RESULTS: We analysed 426 children undergoing primary and 78 undergoing secondary surgery between 2014 and 2020. The incidence of POSI was significantly lower after secondary (12%) compared with primary (28%, p = 0.0084) surgery. In a multivariate analysis adjusting for tumour histology, the odds ratio for developing POSI after secondary surgery was 0.23, compared with primary surgery (95% confidence interval: 0.08-0.65, p = 0.006). The frequency of postoperative CND did not differ significantly after primary vs. secondary surgery (p = 0.21). CONCLUSION: Children have a lower risk of POSI after secondary than after primary surgery for posterior fossa tumours but remain at significant risk of both POSI and CND. The present findings should be taken in account when weighing risks and benefits of secondary surgery for posterior fossa tumours.

Ataxia and mobility in children following surgical resection of posterior fossa tumour: A longitudinal cohort study.

PURPOSE: To report the course of ataxia in children up to 2 years post-operatively, following surgical resection of a posterior fossa tumour (PFT). METHODS: Thirty-five children, (median age 9 years, range 4-15) having resection of PFT, were assessed using the Scale for the Assessment and Rating of Ataxia (SARA), Brief Ataxia Rating Scale (BARS) and the mobility domain of the Paediatric Evaluation of Disability Index (PEDI-m) at initial post-operative period (baseline), 3 months, 1 year and 2 years post-operatively. RESULTS: Baseline median scores of the SARA and BARS were 8.5 (range 0-35.5), and 7 (0-25) respectively. Ataxia improved at 3 months (median SARA and BARS reduction 3.5 and 4, respectively). Additional gradual improvements in SARA were recorded at 1 (median reduction 2) and 2 years post-operatively (median reduction 0.5). Median baseline PEDI-m was 54.75 (range 15.2-100) with improvement at 3 months (median increase 36.95) and small improvement at 1 year (median increase 2.5) and 2 years (median increase 5.8). Children with medulloblastoma and midline tumours (median baseline SARA 10 and 11, respectively) demonstrated more severe ataxia than children with low-grade gliomas and unilateral tumours (median baseline SARA 7.5 and 6.5, respectively). CONCLUSION: The largest improvement in ataxia scores and functional mobility scores is demonstrated within the first 3 months post-operatively, but ongoing gradual improvement is observed at 2 years. Children with medulloblastoma and midline tumour demonstrated higher ataxia scores long term.

The management of bifocal intracranial germinoma in children.

OBJECTIVES: Bifocal intracranial germinoma (BFG) is a tumour of the pineal and suprasellar regions, which is known to be highly radiosensitive. The definitive treatment and outcomes are not well defined, particularly in the paediatric population. We review our series of purely paediatric cases from a single institution and combine them with the limited reports in the literature to determine the results of different management strategies. METHODS: Four patients were treated at our institution with a median age of 15.3 years. A literature search identified a further 38 paediatric cases with a median age of 12.9 years. RESULTS: All four patients had normal serum and CSF tumour markers. One patient had a diagnosis made based on imaging findings of bifocal pineal and suprasellar lesions presenting with diabetes insipidus. Three others underwent biopsy. All had craniospinal radiotherapy, which has led to complete cure with no cases of progression at a mean follow-up of 3 years. The most common treatment modality in published cases is craniospinal irradiation. In the cases reviewed, limited radiation treatments (whole ventricle or focal) combined with chemotherapy regimens yield comparable outcomes where there is no spinal dissemination. Outcomes do not appear to be altered by biopsy in cases with negative tumour markers and characteristic imaging appearances. CONCLUSION: Patients who present with a classic appearance of germinoma, negative tumour markers and diabetes insipidus probably do not require a biopsy to confirm the diagnosis. No evidence of dissemination may obviate the need for craniospinal irradiation, but good quality long-term follow-up data are required to demonstrate the benefits of combined focal radiotherapy and chemotherapy regimes.

Cerebellopontine angle oligodendroglioma in a child: first case report.

The reported incidence of oligodendrogliomas in the paediatric population is less than 1 %. The posterior fossa is a rare location, with the vast majority arising in the cerebral hemispheres. We report the first paediatric case of a WHO grade II oligodendroglioma arising in the cerebellopontine angle (CPA). CPA oligodendrogliomas in children appear to behave aggressively and adjuvant therapy must be considered early; especially when complete resection cannot be achieved.

Prolonged survival associated with the use of intraoperative carmustine (Gliadel) in a paediatric patient with recurrent grade III astrocytoma.

A 15-year-old female presented with a middle cranial fossa anaplastic astrocytoma that was completely excised. She received local radiotherapy (54 Gy) and oral temozolomide. Five months after therapy, MRI showed local relapse. She underwent resection of the tumour with implantation of seven carmustine-impregnated wafers (Gliadel). She then received six cycles of procarbazine and lomustine therapy. Three years later, she is well and disease free. This case supports the further investigation of Gliadel in children and young people with relapsed high-grade glioma, particularly in the setting of a second complete resection.

Intra-operative 3-T MRI for paediatric brain tumours: challenges and perspectives.

MRI is the ideal modality for imaging intracranial tumours. Intraoperative MRI (ioMRI) makes it possible to obtain scans during a neurosurgical operation that can aid complete macroscopic tumour resection­a major prognostic factor in the majority of brain tumours in children. Intraoperative MRI can also help limit damage to normal brain tissue. It therefore has the potential to improve the survival of children with brain tumours and to minimise morbidity, including neurological deficits. The use of ioMRI is also likely to reduce the need for second look surgery, and may reduce the need for chemotherapy and radiotherapy. Highfield MRI systems provide better anatomical information and also enable effective utilisation of advanced MRI techniques such as perfusion imaging, diffusion tensor imaging, and magnetic resonance spectroscopy. However, high-field ioMRI facilities require substantial capital investment, and careful planning is required for optimal benefit. Safe ioMRI requires meticulous attention to detail and rigorous application of magnetic field safety precautions. Interpretation of ioMRI can be challenging and requires experience and understanding of artefacts that are common in the intra-operative setting.

Management strategies for recurrent ependymoma in the paediatric population.

INTRODUCTION: The management of recurrent ependymoma within the paediatric population remains a therapeutic challenge. The options available are varied and patients may have already received prior radio- or chemotherapy. As yet, no consensus exists regarding their optimal treatment. We review the literature and present our contemporary management strategies for this interesting group of patients. RESULTS AND DISCUSSION: Survival following recurrence is poor and those prognostic factors that predispose to recurrence include extent of surgical resection and the timing of administration of adjuvant therapy. The extent of resection at re-operation can confer a survival advantage, without a necessary increase in morbidity. Strategies aimed at improving surgical resection at first diagnosis include improving and centralising post-surgical radiological review, defining what are true residuals, and centralising surgical review of incompletely resected tumours. Re-irradiation can improve survival, and with the use of conformal radiation fields need not necessarily lead to neuropsychological damage. Cisplatin and etoposide remain the most effective chemotherapeutic agents to date and with an increase in the understanding of tumour biology this may improve further. Because of the complex nature of this group of patients, decisions regarding their management require the involvement of a paediatric neurosurgeon, paediatric neuro-oncologist and paediatric radiation oncologist.

Relapsed intracranial ependymoma in children in the UK: patterns of relapse, survival and therapeutic outcome.

Relapsed ependymoma in children poses difficult dilemmas in management. Clinico-pathological and treatment data of 108 children with relapsed ependymoma in the United Kingdom (UK) treated between 1985 and 2002 were reviewed to identify prognostic factors affecting survival. The primary site was the most common site of relapse (84%). Overall 25% had metastatic relapse. Surgery at relapse was attempted in only 55%. Radiotherapy was delivered at relapse in 66% infants and 50% of older children were re-irradiated. Overall 5-year survival was 24% and 27% for children less than 3 years of age at initial diagnosis and older children, respectively. Multivariate analysis showed that, for infants, surgery (p=0.01) and radiotherapy (p=0.001) at relapse were independent predictors of survival. For older children regardless of the previous radiotherapy, repeat irradiation was associated with better outcome (p=0.05). Relapse was associated with poor outcome in both age groups. A survival advantage conferred by both radiotherapy and surgery at relapse is independently significant.

Infantile myofibromatosis of the craniovertebral junction.

Infantile myofibromatosis (IM) is a rare pathological entity characterized by solitary or multiple nodular skin, soft tissues or bony lesions. Craniovertebral (CV) junction lesions are rare. We report the successful management of a solitary IM involving the posterior elements of the CV junction in a 6-month-old child.

Children's Cancer and Leukaemia Group (CCLG): guidelines for the management of intracranial meningioma in children and young people.

Primary tumours of the meninges occur extremely rarely in children and young people (less than five new cases annually in the UK) and have remained a poorly defined group compared with the common diagnosis of meningiomas in adults. Because of the rarity in children, paediatric meningiomas are often treated according to the adult practice. This may lead to inappropriate treatment considerations since paediatric meningiomas exhibit peculiarities that distinguish them from their adult counterpart. Striking the balance between late toxicities of adjuvant radiotherapy on the growing brain versus the risk of repeated recurrences necessitating surgical interventions in young patients is of importance and will require a clinical decision making process in the paediatric neuro-oncological/neurosurgical MDT tailored to each patient's age and clinical setting. The paediatric literature is based on small, single institution retrospective studies over extensive time periods during which the imaging facilities, pathological criteria and surgical advances have led to shifts in definitions of disease making comparison of results difficult to interpret. These guidelines have been developed following a comprehensive appraisal of the literature. Primary treatment is surgical resection. Careful pathological review and multidisciplinary discussions should be undertaken before considering postoperative treatment, such as radiotherapy for histologically anaplastic or clinically aggressive, relapsing meningioma.

The role of endoscopic third ventriculostomy in the management of hydrocephalus associated with cerebellopontine angle tumours.

BACKGROUND: Progressive hydrocephalus secondary to cerebellopontine angle tumours has been traditionally managed with ventriculo-peritoneal shunting. Endoscopic third ventriculostomy provides an alternative treatment option and the success rate in this patient group has not previously been reported. METHODS: We report a retrospective series of 11 patients with cerebello-pontine angle tumours who presented with symptomatic hydrocephalus, or developed hydrocephalus following radiosurgery, who underwent endoscopic third ventriculostomy. RESULTS: Seven patients (63.6%) remain shunt free. There were no complications following endoscopy in any patient. Where the ventriculostomy failed there was no additional morbidity. CONCLUSIONS: Endoscopic third ventriculostomy is a low morbidity procedure, which avoids the inherent problems of shunts, particularly infection and should be considered for patients with hydrocephalus and cerebello-pontine angle tumours.

Lumboperitoneal shunts: are the complications acceptable?

BACKGROUND: Lumboperitoneal shunts have long been used in the treatment of benign intracranial hypertension, postoperative pseudomeningocele, CSF leak and communicating hydrocephalus. Although they can provide a rapid and effective resolution of the symptoms there are major disadvantages associated with their use. METHOD: We retrospectively analyzed the records of 21 patients who underwent LP shunt insertion in our institution during the study period. FINDINGS: Eighteen patients (85.7%) underwent at least one shunt revision with a total of 63 revisions. Shunt related infections were observed in 7 cases and 7 patients developed Chiari malformation, 2 (9%) of these requiring further treatment. CONCLUSIONS: Despite the symptomatic control achieved with lumboperitoneal shunts we find the complication and revision rates unacceptably high. The technique should be re-evaluated and other treatment strategies considered.

Neurolisteriosis causing hydrocephalus, trapped fourth ventricle, hindbrain herniation and syringomyelia.

Central nervous system infection by Listeria monocytogenes is relatively uncommon, but is known to be highly morbid and fatal. We describe a case of listeria meningoencephalitis, wherein the acute episode was followed by a hitherto unreported conglomeration of severe and progressive neurological sequelae, in the form of supratentorial hydrocephalus, aqueduct block, trapped fourth ventricle, hindbrain herniation and syringomyelia. Pertinent literature is reviewed and the pathogenesis of the observed sequelae is explored.

Sudden death secondary to delayed closure of endoscopic third ventriculostomy.

An 11-year-old boy who underwent an initially successful endoscopic third ventriculostomy (ETV) died 4 months later secondary to acute hydrocephalus. This is the first report of sudden death caused by delayed closure of ETV. Guidelines to patients, parents and primary caregivers should include the possibility of ETV failure and encourage early neurosurgical consultation when symptoms of raised intracranial pressure occur.

Vestibular schwannomas in children.

OBJECTIVE: This article highlights the clinical presentation and management issues of unilateral vestibular schwannomas in children. We demonstrate how the presentation differs from neurofibromatosis type 2 (NF2) and from adult unilateral vestibular schwannomas. STUDY DESIGN: This article is composed of a series of three cases and a literature review. SETTING: The study was performed at a university hospital (tertiary referral center). PATIENTS: Three children, aged 9, 11, and 13 years, with histologically confirmed vestibular schwannomas were studied. All children under 16 years of age in the world literature with unilateral vestibular schwannomas were reviewed. INTERVENTION: Analysis of presentation and surgical management of these three children and those children reported in the literature. MAIN OUTCOME MEASURE: Pattern of presentation relative to children with NF2 and people with adult unilateral vestibular schwannomas. RESULTS: Two patients had multiple cranial nerve weakness and recurrence, and one patient had successful removal of the tumor with preservation of all functions of the cranial nerves, including the facial nerve. CONCLUSION: Vestibular schwannomas in children are very uncommon. It is likely that it is the first manifestation of NF2, but it may also be a variant of sporadic vestibular schwannomas. A presentation of three cases and a review of 36 other cases in the literature demonstrates how the presentation is different from adult sporadic vestibular schwannomas and NF2 because it lacks primary audiological symptoms. The study also provides evidence of non-NF2 vestibular schwannomas presenting in children and suggests that it is likely that these are a variant of unilateral sporadic vestibular schwannomas. The search for the features of NF2 in these cases remains mandatory.