Radiation-induced cavernoma in pediatric CNS tumors: a systematic review and treatment paradigm.

PURPOSE: This retrospective systematic literature review aimed to summarize available data regarding epidemiology, etiology, presentation, investigations, differentials, treatment, prevention, monitoring, complications, and prognosis for radiation-induced cavernous malformations (RICMs) in pediatric patients. METHODOLOGY: Review conducted per PRISMA guidelines. Google Scholar, PubMed, Trip Medical Database, and Cochrane Library searched utilizing a keyphrase, articles filtered per inclusion/exclusion criteria, duplicates excluded. Based on criteria, 25 articles identified, 7 further excluded from the systematic data but included in discussion (5 × insufficient data, 2 × other systematic reviews). RESULTS: Many studies did not contain all explored data. 2487 patients reviewed, 325 later found to have RICM (143 male, 92 female). Mean age at irradiation 7.6 years (range 1.5-19). Mean total radiation dose 56 Gy (12-112). Most common indications for radiation-medulloblastoma 133x, astrocytoma 23x, ependymoma 21x, germinoma 19x. Mean age at RICM diagnosis 18 years (3.6-57). Mean latency to RICM 9.9 years (0.25-41). Most common anatomic locations-temporal 36, frontal 36, parietal 13, basal ganglia 16, infratentorial 20. Clinical presentation-incidental 270, seizures 19, headache 11, focal neurological deficit 7, other 13. 264 patients observed, 34 undergone surgery. RICM bled in 28 patients. Mean follow-up 11.7 years (0.5-50.3). Prognostic reporting highly variable. CONCLUSIONS: From our data, pediatric RICMs appear to display slight male predominance, present about 10 years after initial irradiation in late teen years, and present incidentally in majority of cases. They are mostly operated on when they bleed, with incidental lesions mostly being observed over time. Further prospective detailed studies needed to draw stronger conclusions.

Outcomes of endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) in the treatment of paediatric hydrocephalus: Systematic review and meta-analysis.

PURPOSE: To evaluate the outcomes of endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) in the treatment of paediatric hydrocephalus. METHODS: We searched PubMed, MEDLINE, and Cochrane Central Register of Controlled Trials databases for articles published from 2000 to May 2023 (last search date May 6, 2023). Keywords searched included "endoscopic third ventriculostomy", "ventriculoperitoneal shunting", "paediatric population", and "outcomes". Using random-effects models, we compared success rates and complications of ETV and VPS. The primary outcome was ETV vs.VPS success rates, and the secondary outcome was post-treatment complications. Included studies reported on treatment success and complication rates. RESULTS: Out of 126 articles, 8 RCTs and 1 prospective study were included. Six studies reported primary outcome data (806 patients identified: 464 in ETV group, 342 in VPS group). Combined success rates were 81.8% (n = 283/346) for ETV and 86.7% (n = 182/210) for VPS (median follow-up 41 months). There was no difference in success rates between ETV and VPS groups (risk ratio 0.84, 95% confidence interval 0.80-0.90, I2 = 0%, p = 0.93). Combined complication rates were 4.6% (n = 16/346) in the ETV group and 27.1% (n = 57/210) in the VPS group. ETV had a lower rate of postoperative complications (risk ratio 0.76, 95% confidence interval 0.42-1.38, I2 = 53%, p = 0.04). CONCLUSIONS: Both ETV and VPS are viable surgical options for the management of paediatric hydrocephalus with similar success rates when used as first-line treatment. However, our study concluded that VPS results in a higher complication rate. REGISTRATION: This systematic review and meta-analysis was formally registered in the PROSPERO International database under the registration number CRD42023452907 on the 29th of August 2023.

Understanding the role of surgical intervention for patients with concomitant degenerative spinal disease without deformity and Parkinson's disease: a meta-analysis.

INTRODUCTION: Parkinson's disease is a common neurodegenerative disease. With an aging population, co-existent degenerative diseases of the spine in these patients will become more prevalent. RESEARCH QUESTION: This systematic review and meta-analysis aims to establish the incidence and risk of adverse outcomes following spinal surgery in patients with Parkinson's disease. Material and Methods A literature review was carried out in order to identify studies assessing the outcomes of adult patients with Parkinson's disease undergoing spinal surgery for degenerative conditions. Studies with less than ten subjects or those assessing surgery for spinal deformity or trauma were excluded. RESULTS: Of 74 studies identified, seven were included for meta-analysis. In the 689,578 participants, there was a significantly higher incidence, but not higher risk, of complications (0.54 95% CI [0.19-0.85] vs 0.07 95% CI [0.01-0.41]; p = 0.048) and revision surgery (0.6 95% CI [0.25-0.88] vs 0.1 95% CI [0.05-0.19]; p = 0.003) amongst patients with Parkinson's disease compared to controls. We also demonstrated a significantly lower incidence but not lower risk of clinical improvement (0.27 95% CI [0.16-0.41] vs 0.57 95% CI [0.36-0.76]; p = 0.02) after surgery in patients with Parkinson's disease. There was no difference in the incidence or risk of death following surgery. DISCUSSION AND CONCLUSION: Spinal surgery for concomitant degenerative diseases in patients with Parkinson's disease is associated with a lower incidence of clinical improvement and a higher incidence of complications. Medical management should be optimised before an individually tailored and well-considered surgical intervention is implemented.

A 5-year outcome of propranolol for the treatment of paediatric intracranial cavernoma: case report and a review of the literature.

We describe a case of a young male patient with KRIT1-driven familial cavernous malformation syndrome who developed multiple brain cavernomas, intracranial bleeding, and persistent seizures. Due to the relentless growth of cavernous malformations and recurrent intracranial bleeds, it was decided to enrol the patient in the "Propranolol for Intracranial Cavernoma" (PICC) pilot trial at our institution. Over the 5-year treatment period with 20 to 40-mg oral propranolol three times daily (TDS), we noted the near-complete arrest of the growth of cavernous malformations with no further evidence of intracranial bleeding or any further seizures. The observed outcome is consistent with the extremely limited published literature on the topic; thus, this case provides important evidence that supports the use of propranolol as a prophylactic treatment for paediatric intracranial cavernomas. We strongly encourage and recommend future prospective randomised controlled trials to definitively assess and characterize the therapeutic utility of propranolol in this patient population.

Treatment of primitive myxoid mesenchymal tumour of infancy: a management paradigm focusing on surgical nuances.

Primitive myxoid mesenchymal tumour of infancy (PMMTI) is a rare mesenchymal tumour that typically appears in those under 6 months of age and preferentially affects the deep soft tissues of the trunk and paravertebral spinal regions. PMMTI has only recently been described, and there is scarce literature reporting cases regarding the management paradigm of the tumour. We report the case of an 11-week-old male who presented with bilaterally reduced movement and brisk reflexes in his lower limbs, and irritability. Despite numerous radiological investigations, including MRI, PMMTI was only diagnosed upon biopsy and histopathology. Although PMMTI is known to be relatively unresponsive to chemotherapy, we observed a notable decrease in tumour size after a series of chemotherapy sessions. After two-staged surgical resection of the tumour, the patient is currently stable and under close follow-up. In this article, we aim to report on the patient's clinical presentation, investigations, diagnosis, and treatment, while also discussing the findings from a review of the literature pertaining to future approaches in managing PMMTI. Overall, this case highlights the importance of considering PMMTI in the differential diagnosis of deep soft tissue tumours in young infants and the potential for a combination of chemotherapy and surgical resection to be effective in treating this rare tumour.

The role of surgery in relieving calcified shunt site-related pain in patients with functioning VP shunt.

PURPOSE: Shunt calcification is a known late sequela of ventriculoperitoneal (VP) shunt insertion and is associated with shunt malfunction. However, in some patients, while shunt functionality is preserved despite calcification of the catheters, they experience nociceptive symptoms. In this paper, the authors present their surgical experience in managing patients with a functional VP shunt and experiencing pain secondary to shunt calcification. METHODS: We analysed outcomes of patients presenting with pain at the level of a calcified shunt who underwent surgical untethering of the calcified catheter from the soft tissues. This procedure was commenced by the senior author in 2015. Patients were collected prospectively from the databases of two institutions. Evidence of shunt calcification was confirmed on neuroimaging. RESULTS: Seven patients, two male and five female, were included. The mean age at untethering was 13.5 years. The mean time interval between primary shunt surgery and symptom onset was 12 years (range 6-16 years). The commonest site of tethering was the neck (50%) followed by abdomen and chest (both 25%). Six patients underwent untethering of the catheter from soft tissues. One patient had removal of a redundant segment of calcified shunt left in situ during a previous revision. All patients experienced pain relief following shunt untethering. CONCLUSION: Untethering of calcified VP shunt catheters from soft tissue can be considered an effective treatment of shunt site pain and offered to patients presenting with a functional VP shunt.

A rare presentation of a bilateral intracranial parameningeal embryonal rhabdomyosarcoma mimicking vestibular schwannoma in a two-year-old child: a case report.

Intracranial parameningeal rhabdomyosarcomas are rare, aggressive, rapidly progressive paediatric malignancies that carry a poor prognosis. The authors report a case of a 2-year-old boy who initially presented with a left facial palsy, ataxia and, shortly after, bloody otorrhoea. MRI imaging was initially suggestive of a vestibular schwannoma. However, there was rapid progression of symptoms and further MRI imaging showed very rapid increase in tumour size with mass effect and development of a similar tumour on the contralateral side. A histological diagnosis of bilateral parameningeal embryonal rhabdomyosarcoma was made. Despite treatment, progression led to hydrocephalus and diffuse leptomeningeal disease, from which the patient did not survive. Few intracranial parameningeal rhabdomyosarcomas have previously been reported and these report similar presenting symptoms and rapid disease progression. However, this is the first reported case of a bilateral intracranial parameningeal embryonal rhabdomyosarcoma which, on initial presentation and imaging, appeared to mimic a vestibular schwannoma.

Promoting padawans: a survey examining the state of mentorship in neurosurgical training in the United Kingdom.

BACKGROUND: Mentorship has long since been acknowledged as an integral part of Neurosurgical training. The authors sought to evaluate the state of mentorship in Neurosurgical training in the United Kingdom (UK). METHODS: A 28-point questionnaire was sent to all neurosurgical trainees in the UK and Ireland via the British Neurosurgical Trainee's Association (BNTA), comprising 180 trainees. RESULTS: There were 75 responses (180 trainees on the mailing list, 42% response rate). Despite all respondents reporting it to be at least somewhat important to have a mentor, 16% felt they had no mentors. The mean number of mentors was 2.91 with 72% of respondents having more than 1 mentor. In terms of the content of mentorship relationships, 63% were comfortable discussing career related topics with their mentor to a high or very high degree but only 29% felt comfortable discussing their general wellbeing. With regards to allocated educational supervisors, 43% thought this person to be a 'low' or 'very low' source of mentorship. The three most important traits of the ideal mentor as reported by respondents were: someone chosen by them (48%), working in the same hospital (44%) and having received formal mentorship training (36%). CONCLUSIONS: The current perception of mentorship in Neurosurgery from the surveyed trainees is mixed. A healthy majority of trainees benefit from mentorship of some kind, whilst a significant minority feel underserved. The surveyed trainees feel mentorship is slanted more towards clinical and professional aspects of development than it is towards personal ones. Suggestions for future insight would be an evaluation of senior registrar and consultant sentiments towards mentorship, whilst exploration into more flexible models for establishing mentoring relationships may help to address the heavy importance of trainee choice which is voiced by this survey's results.

The Management of Idiopathic and Refractory Syringomyelia.

Idiopathic syringomyelia (IS) and refractory syringomyelia (RS) are types of syringomyelia that often pose a management challenge and are associated with long-term clinical sequela. They are usually an epiphenomenon reflecting an underlying pathology where the treatment of the primary cause should be the aim for any surgical intervention. In the case of IS, the initial step is agreeing on the definition of the terms idiopathic and syringomyelia. After a rigorous exhaustive clinic-radiological workup, only IS patients with progressive neurology are treated, usually unblocking subarachnoid cerebrospinal fluid (CSF) pathway obstruction somewhere in the thoracic spinal canal and reserving shunting techniques to nonresponsive cases. Similar to IS, also RS is multifactorial, and its management varies based on the initial pathology, strongly supported by radiological and clinical features. We aim to address this topic focusing on the etiopathology, investigation paradigm, and surgical pathway, formulating algorithms of management with available evidence in literature. Surgical techniques are discussed in detail.

A 10-year retrospective observational study on the utility and prescription standards of dexamethasone in pediatric neuro-oncosurgery in a tertiary care center.

OBJECT: This study aimed to retrospectively assess dexamethasone utility in pediatric CNS tumor patients over a 10-year period, to better understand dosing variability, and highlight optimal practice. METHODS: All pediatric CNS tumor cases managed operatively for a 10-year period at a single center were reviewed. Information was gathered on demographics, dexamethasone doses, course durations, weaning regimes, PPI co-prescription, adverse events, and route of administration. Comparison within these groups was analyzed through use of statistical testing. RESULTS: One hundred twenty-seven patients received 193 dexamethasone courses. Median age was 7 years, with a median weight of 27.9 kg. Most common tumor type was astrocytoma (24.8%). Median daily dose was 8 mg, with twice-daily dosing most common. Median course duration was 8 days, ranging from 1 to 1103 days. Median weaning duration was 11.5 days. Daily dose was not correlated with patient weight and the median daily dose per kg was 0.2319 mg/kg. Incidence of adverse effects was 14.5% across all course lengths, with weight gain most common. The short-term course duration (<14 days) had the lowest adverse event incidence, with direct correlation between course length and adverse effect incidence. Dexamethasone dose per kg was not significantly different between patients with and without adverse effects. No relationship was noted between adverse effects incidence and administration route (intravenous compared to oral). 64.2% of patients received concurrent PPI with 35.8% receiving no PPI, with 1 gastrointestinal side effect noted in the PPI-receiving population. CONCLUSIONS: Large variation was seen in practice, with prescriptions appearing based on clinician preference and symptom severity rather than patient age or weight. Future guidelines should consider lower dose regimens than are currently presented with less frequent dosing as these may benefit quality of life. Weaning period can be relatively rapid for most patients, taking place in 2-3 days. PPI co-prescription does not seem to add significant benefit. We recommend using a standardized guideline of 0.2 mg/kg/day (max 8 mg/day) given OD or BD, with PPI cover where necessary. For acute presentations, we recommend limiting dexamethasone treatment to <14 days. These recommendations can be adjusted for individual cases to yield optimal results.

The benefits of perioperative music interventions for patients undergoing neurosurgery: a mixed-methods systematic review.

BACKGROUND: Several studies have demonstrated music intervention as a safe and inexpensive option for reducing anxiety and pain perioperatively. We performed a systematic review to evaluate its possible benefit in perioperative neurosurgical management. METHODS: The following databases were searched with no restrictions on publication date: PubMed, Embase, CINAHL, PsycINFO, CENTRAL and ClinicalTrials.gov. All studies that quantitatively or qualitatively assessed the effects of any music-based intervention administered within one week of cranial or spinal neurosurgery were eligible. Meta-analysis could not be performed, and quantitative findings were instead summarised narratively. We then synthesised qualitative observations through thematic analysis before conducting an integrative review. PROSPERO registration: CRD42019152626. RESULTS: Seven studies, of sample sizes ranging from 8 to 60, were included, with the timing, duration, frequency and type of music intervention varying considerably across studies. Quantitative analysis involving five studies showed a significant decrease in anxiety following music intervention in four studies (p < 0.05), and reduced pain perception in three studies (p < 0.05). Mixed evidence was obtained for physiological outcomes including heart rate and blood pressure. Risk of bias was moderate to high. Quantitative findings were generally supported by qualitative analysis which provided additional insight into the factors influencing music intervention's effectiveness. CONCLUSION: Despite the heterogeneity in study characteristics, this review, which is the first mixed-methods systematic review assessing the effects of perioperative music intervention, not only reveals a potential role for music intervention in neurosurgery, but also highlights the possible importance of considering qualitative evidence in future studies to better characterise its effectiveness.

Burnout amongst neurosurgical trainees in the UK and Ireland.

INTRODUCTION: Burnout is becoming an increasingly recognised phenomenon within the medical profession. This study aims to investigate the presence of burnout amongst neurosurgical trainees in the UK and Ireland as well as investigating potential exacerbating and protective factors. METHOD: An online survey was sent to all neurosurgical trainees in the UK and Ireland via the British Neurosurgical Trainees' Association (BNTA) mailing list. Responding participants anonymously completed the Copenhagen Burnout Inventory (CBI) and answered questions about known risk factors for burnout including workplace environment, workplace bullying, time spent on leisure activities and sleep and reported likelihood of leaving neurosurgery. We also collated data on responders' demographics. We compared CBI scores for participants with and without risk factors to determine correlation with CBI. RESULTS: There were 75 respondents (response rate 42%) from a range of ages and all training grades, 72% of whom were male. The median CBI score was 38.85 (IQR 17.76). Participants showed a higher degree of personal and workplace burnout (median CBIs of 47.02, IQR 25.00; and 49.14, IQR 19.64, respectively) compared with patient-related burnout (median CBI 18.67, IQR 25.00). Participants with the following self-reported risk factors were significantly more likely to have higher CBIs: workplace bullying (p = 0.01), getting on less well with colleagues (p < 0.05), working longer hours (p < 0.05) and insufficient sleep, exercise and leisure time (all p < 0.01). Those with higher CBI scores were more likely to consider leaving neurosurgical training (p = 0.01). CONCLUSION: We identified a high burnout incidence in a cohort representative of UK neurosurgical trainees, although our results may have been skewed somewhat by selection bias. We determined potential risk factors for burnout related to specific workplace stressors and time for non-work activities. In the future, changes to training curricula should address these issues, aiming to improve training, enhance patient care and reduce attrition rates.

Guideline for the management of long tunnelled external ventricular drains in chronic hydrocephalus.

This article reports on the journey of a child with an inoperable hypothalamic-origin pilocytic astrocytoma causing hydrocephalus, which was refractory to treatment with shunts, and required a new approach. With multidisciplinary support, excellent nursing care and parental education, the child's hydrocephalus was managed long term in the community with bilateral long-tunnelled external ventricular drains (LTEVDs). This article describes the patient's journey and highlights the treatment protocols that were created to achieve this feat. Despite the difficulties in initially setting up these protocols, they proved successful and thus the team managing the patient proposed that LTEVDs are a viable treatment option for children with hydrocephalus in the context of inoperable tumours to help maximise quality of life.

Custom-Made Three-Dimensional Models for Craniosynostosis.

The use of computer-aided design and computer-aided manufacturing in oral and maxillofacial surgery is an ever-growing field.The availability of 3D models, cutting guides, and customised surgical instruments gives surgeons the opportunity to modify and improve their surgical procedures.Here, we discuss the use of computer-aided design-computer-aided manufacturing to improve the management of a case of nonsyndromic metopic synostosis through the construction of: A cutting guide for the cranium, custom-made orbital protectors, a 3D model of the predicted postoperative meninges to allow off the table bone recontouring, and a template frontal bar to allow more specific recontouring of the frontal bar.

De novo distal middle cerebral artery aneurysm post-excision of intracerebral arteriovenous malformation in an 8-year old.

Cerebral arteriovenous malformations (AVMs) are frequently associated with concurrent aneurysms. These aneurysms are commonly haemodynamically related to the AVM and can be classified into extranidal or intranidal in reference to the AVM nidus. An aneurysm arising from an artery that does not angiographically contribute to the blood flow to the AVM is uncommon. A distal middle cerebral artery (dMCA) aneurysm itself is also a rare presentation, especially in paediatric population. We present a rare case of dMCA aneurysm that was noted after successful surgical management of a ruptured AVM in an 8-year-old child and its management. BACKGROUND: About 10-30% of patients with cerebral arteriovenous malformation (AVM) have an associated artery aneurysm. The majority of these aneurysms are flow-related to the malformation. These aneurysms can be classified into extranidal or intranidal in reference to the AVM nidus Rammos et al Am J Neuroradiol 37:1966-1971, [1]. An aneurysm arising from a different artery that does not angiographically contribute to the blood flow associated with the AVM is less common and would generally be regarded as unrelated to the AVM. Distal cerebral artery aneurysm itself is also a rare presentation, comprising of 1-7% of all middle cerebral artery aneurysm. In children, mycotic infection and dissection are the two most common causes for aneurysm in this location. Unlike in adults, berry aneurysms are uncommon in children. We describe a young patient who was found to have distal middle cerebral artery (dMCA) aneurysm in follow-up DSA (Digital Subtraction Angiogram) after the initial successful surgical treatment for a cerebral frontal AVM. In this particular case, endovascular repair is thought to be the best strategy to treat the aneurysm. However, there still remains a lack of consensus of the best management strategy (surgery or endovascular) in treating flow-related aneurysms in general. This is usually based on an individual case scenario and the treatment is tailored depending on various factors including the expertise of the treating team.

Comparison of Three Different Cranio-Cervical Decompression Procedures in Children with Chiari Malformation Type I: Does the Surgical Technique Matter?

BACKGROUND/AIMS: A broad spectrum of cranio-cervical decompression techniques (CCD) is employed for the treatment of Chiari malformation type I (CM1). The aim of this study was to compare the clinical and radiological outcome of 3 different CCD performed in a single paediatric centre. METHODS: A retrospective analysis of children treated between 2008 and 2014 was performed. Three different surgical techniques were offered to the patients: an extradural osseo-ligamentous bony decompression (BD), or a BD plus opening of the dura either without duroplasty (DOWD) or with watertight expansile duroplasty (DOPD). The primary clinical outcome was measured by utilizing the Chicago Chiari Outcome Scale (CCOS). RESULTS: Forty-six children underwent 51 CCD: 17 BD, 17 DOWD, and 17 DOPD. The median follow-up period was 46 months (16-98 months). Patients who underwent BD had a shorter length of hospital stay than those who underwent DPTC and DOWD. Clinical improvement, defined by the mean CCOS score, was comparable in patients receiving BD (n = 14.6) and DOPD (n = 14.5), but lower in patients with DOWD (n = 12). Post-operative complications were noted in 7 DOWD patients (41%) and 3 DOPD patients (17.6%). CONCLUSIONS: BD is a safe and effective procedure with comparable clinical and radiological outcomes to DOPD in children with CM1. The risk of post-operative complications and worse clinical outcome was noted to be higher when performing a DOWD.

Holoprosencephaly: antenatal and postnatal diagnosis and outcome.

OBJECTIVES: The objectives of this study are to ascertain the clinical outcome and overall survival of holoprosencephaly (HPE) patients diagnosed antenatally and postnatally, to determine the accuracy of antenatal diagnosis and to determine the role of neurosurgical intervention in HPE. DESIGN: This is a retrospective review over a 10-year period. PATIENTS: Sixty-three patients were included in the study, 45 were diagnosed by antenatal radiological imaging and 18 were diagnosed by postnatal radiological imaging. Patient data was drawn from Temple Street Children's University Hospital (the national paediatric neurosurgery centre), the National Maternity Hospital in Holle's Street, Dublin, and Our Lady of Sick Children Hospital, Dublin. METHODS: The study was carried out through a review of antenatal and postnatal radiological imaging and reports, clinical charts, GP letters from patient follow-up and telephone conversations with parents of HPE patients. RESULTS: Four patients in the antenatal diagnosis group had follow-up foetal MRI confirming HPE. Twelve in this group had radiological follow-up postnatally, and in five of these, HPE was confirmed. The remaining seven were identified as false positive. Alobar HPE constituted 55 % (21/38) of patients with 95 % mortality. Fifty-one percent had a normal karyotype. The overall survival in the antenatal diagnosis group was 13 %. In the postnatal group, 18 patients were identified, 67 % (12/18) lobar and 33 % (6/18) semilobar. Normal karyotype was found in 72 % (13/18), with an overall survival rate of 56 % (10/18). Neurosurgical intervention in both groups mainly consisted of CSF diversion in the form of ventriculoperitoneal (VP) or cystoperitoneal shunt (CP) (13/67). CONCLUSION: Foetal MRI should be routinely performed in suspected cases of HPE, and reliance on ultrasound alone in the antenatal period may not be sufficient. In our study, there is a high early mortality noted in severe cases of HPE, while milder forms of HPE in children tend to survive beyond infancy albeit with associated complications that required neurosurgical intervention and medical management for other associated systemic anomalies.