Torticollis with Atlantoaxial Rotatory Subluxation in Children: A Clinical Review.

A small proportion of children with a sudden onset torticollis ("wry neck") presents with an atlantoaxial rotatory subluxation, usually after mild trauma or recent head or neck infection. Torticollis is a clinical diagnosis and imaging is usually not indicated, though often performed in clinical practice. Atlantoaxial rotatory subluxation on imaging is often a physiological phenomenon in torticollis, and concomitant neurological symptoms are therefore rare. Treatment is primarily conservative, with analgesics, a rigid neck collar, and if needed benzodiazepines to counteract muscle spasms and anxiety. In case of treatment failure or chronic subluxation, cervical repositioning and fixation under general anesthesia may be considered. Surgical treatment is only indicated in a small percentage of patients with chronic refractory subluxation, concomitant cervical fractures, or congenital anomalies. Early diagnosis and treatment are important, since this is associated with a more successful conservative outcome than a prolonged approach.

Potentially Life-Threatening Interaction between Opioids and Intrathecal Baclofen in Individuals with a Childhood-Onset Neurological Disorder: A Case Series and Review of the Literature.

BACKGROUND: Spasticity and dystonia are movement impairments that can occur in childhood-onset neurological disorders. Severely affected individuals can be treated with intrathecal baclofen (ITB). Concomitant use of ITB and opioids has been associated with central nervous system (CNS) depression. This study aims to describe the clinical management of this interaction, based on a case series and review of literature. METHODS: Four individuals with childhood-onset CNS disorders (age 8-24) and CNS-depressant overdose symptoms after the concomitant use of ITB and opioids are described. The Drug Interaction Probability Scale (DIPS) was calculated to assess the cause-relationship (doubtful <2, possible 2-4, probable 5-8, and highly probable >8) of the potential drug-drug interaction. A literature review of similar previously reported cases and the possible pharmacological mechanisms of opioid-baclofen interaction is provided. RESULTS: After ITB and opioid co-administration, three out of four patients had decreased consciousness, and three developed respiratory depression. DIPS scores indicated a possible cause-relationship in one patient (DIPS: 4) and a probable cause-relationship in the others (DIPS: 6, 6, and 8). Discontinuation or adjusting ITB or opioid dosages resulted in clinical recovery. All patients recovered completely. In the literature, two articles describing nine unique cases were found. CONCLUSION: Although the opioid-ITB interaction is incompletely understood, concomitant use may enhance the risk of symptoms of CNS-depressant overdose, which are potentially life-threatening. If concomitant use is desirable, we strongly recommend to closely monitor these patients to detect interaction symptoms early. Awareness and monitoring of the potential opioid-ITB interaction is essential to reduce the risk of severe complications.

Robotic alignment system Cirq (Brainlab) for navigated brain tumor biopsies in children.

INTRODUCTION: The Cirq robotic alignment system (Brainlab, Munich, Germany) is a manually adjustable electronic arm with a robotic alignment module on its distal end, enabling the neurosurgeon to automatically and accurately align surgical instruments to a preoperatively planned trajectory. In this study, we share our first experiences and results using Cirq for intracranial tumor biopsy in children. METHODS: From May 2021 until October 2022, all consecutive patients that underwent a brain tumor biopsy using Cirq were included and compared to a historical cohort of patients biopsied with the non-robotic system Varioguide (Brainlab, Munich, Germany). Patient-related data, tumor-related data, and surgery-related data were collected. Registration accuracy was calculated for different patient-to-image registration methods. Pre- and postoperative images were fused, and entry error, target error, and angulation error were calculated. RESULTS: Thirty-seven patients, aged 1-19 years, were included (14 with Cirq and 23 with Varioguide). An integrated histopathological and molecular diagnosis was acquired in all cases. Patient-to-image registration was significantly more accurate when based on bone screw fiducials combined with intraoperative CT, as compared to surface matching or skin fiducials. The target error (Euclidian distance) was 5.3 mm for Cirq as compared to 8.3 mm for Varioguide, but this was not statistically significant. Entry error and angulation error were also not significantly different between both groups. CONCLUSION: Intracranial biopsy with the Cirq robotic system is feasible and safe, and its accuracy does not differ from the Varioguide system.

Surgical treatment options for spasticity in children and adolescents with hereditary spastic paraplegia.

PURPOSE: To provide an overview of outcome and complications of selective dorsal rhizotomy (SDR) and intrathecal baclofen pump implantation (ITB) for spasticity treatment in children with hereditary spastic paraplegia (HSP). METHODS: Retrospective study including children with HSP and SDR or ITB. Gross motor function measure (GMFM-66) scores and level of spasticity were assessed. RESULTS: Ten patients were included (most had mutations in ATL1 (n = 4) or SPAST (n = 3) genes). Four walked without and two with walking aids, four were non-walking children. Six patients underwent SDR, three patients ITB, and one both. Mean age at surgery was 8.9 ± 4.5 years with a mean follow-up of 3.4 ± 2.2 years. Five of the SDR patients were walking. Postoperatively spasticity in the legs was reduced in all patients. The change in GMFM-66 score was + 8.0 (0-19.7 min-max). The three ITB patients treated (SPAST (n = 2) and PNPLA6 (n = 1) gene mutation) were children with a progressive disease course. No complications of surgery occurred. CONCLUSIONS: SDR is a feasible treatment option in carefully selected children with HSP, especially in walking patients. The majority of patients benefit with respect to gross motor function, complication risk is low. ITB was used in children with severe and progressive disease.

Unique form of catheter malconnection following intrathecal baclofen surgery for spinal cord injury: a case report.

This case report concerns a patient suffering from traumatic spinal cord injury with severe spasticity treated with intrathecal baclofen therapy. After revision surgery for a confirmed catheter obstruction, progressive spasticity reappeared. Diagnostics demonstrated signs of catheter fracture or disconnection adjacent to the pump. During revision surgery, the silicone layer surrounding the sutureless pump connector was shown to be curled up, revealing the cause of dysfunction. As far as we know, this form of malconnection has not been reported before. Therefore, surgeons must be aware of this complication and additional inspection of the silicone connector prior to definite connection is advised.

Spinal cord anomalies in children with anorectal malformations: a retrospective cohort study.

PURPOSE: First, to assess the number of spinal cord anomalies (SCA), specifically tethered spinal cord (TSC) in patients with anorectal malformations (ARM), identified with spinal cord imaging (i.e. spinal cord US and/or MRI). Second, to report outcomes after TSC treatment. METHODS: A retrospective mono-center study was performed. All ARM patients born between January 2000 and December 2021 were included. Screening for SCA consisted of spinal cord US and/or MRI. Radiology reports were scored on presence of SCA. Data were presented with descriptive statistics. RESULTS: In total, 254 patients were eligible for inclusion, of whom 234 (92.1%) underwent spinal cord imaging. In total, 52 (22.2%) patients had a SCA, diagnosed with US (n = 20, 38.5%), MRI (n = 10, 19.2%), or both US and MRI (n = 22, 42.3%), of whom 12 (23.5%) with simple, 27 (52.7%) intermediate, and 12 (23.5%) complex ARM types. TSC was identified in 19 patients (8.1%), of whom 4 (21.1%) underwent uncomplicated neurosurgical intervention. CONCLUSIONS: SCA were present in 22% of ARM patients both in simple, as well as more complex ARM types. TSC was present in 19 patients with SCA, of whom 4 underwent uncomplicated neurosurgical intervention. Therefore, screening for SCA seems to be important for all ARM patients, regardless of ARM type. LEVEL OF EVIDENCE: Level III.

Uncommon presentations of a neurosurgical site infection: impaired wound healing with hypergranulation and crust formation.

Hypergranulation and crust formation after cranial neurosurgery is rare. We report three patients with an uncommon form of hypergranulation with extensive crust formation after cranial neurosurgery, associated with a St. Aureus infection of the scalp, and propose that this is a form of pyogenic dermatitis, as is commonly seen among domestic animals with a coat of fur. It can be treated conservatively. We propose a treatment algorithm.

The association between preoperative edema and postoperative cognitive functioning and health-related quality of life in WHO grade I meningioma patients.

BACKGROUND: Studies on the associations between preoperative cerebral edema, cognitive functioning, and health-related quality of life (HRQOL) in WHO grade I meningioma patients are virtually lacking. We studied the association between preoperative cerebral edema on postoperative cognitive functioning and HRQOL 6 months postoperatively in WHO grade I meningioma patients. METHODS: Twenty-one consecutive WHO grade I meningioma patients, who underwent surgery, were matched individually for age, gender, and educational level to healthy controls. Tumor and edema volume were assessed on preoperative T1- and T2-weighted MRI images, respectively. At least 5 months postoperatively, functional status, cognitive functioning, and HRQOL, using a cognitive test battery and the Short-Form Health Survey (SF-36), were determined. The correlation between preoperative tumor and cerebral edema volume with postoperative cognitive functioning and HRQOL was investigated using Kendall's tau coefficients. RESULTS: Compared to healthy controls, patients had lower verbal memory capacity (p = .012), whereas HRQOL was similar to matched healthy controls. In all cognitive domains, postoperative functioning was much lower in patients with preoperative cerebral edema than in those without. There were significant correlations between preoperative cerebral edema and tumor volume and postoperative cognitive functioning. Preoperative cerebral edema and/or tumor volume were not associated with HRQOL. CONCLUSIONS: Our results suggest that WHO grade I meningioma patients with larger volumes of preoperative cerebral edema are more at risk of experiencing limitations in longer-term cognitive functioning than patients with no or less edema preoperatively. This is an important knowledge for neurologists and neurosurgeons treating patients with a meningioma. More studies regarding the effect of peritumoral edema on cognitive functioning in meningioma patients are necessary.

Factors associated with success of conservative therapy in chronic subdural hematoma: a single-center retrospective analysis.

INTRODUCTION: Conservative therapy is a viable option for patients with chronic subdural hematoma (cSDH) who express no, or only mild symptoms. It is not clear which factors are associated with success of conservative therapy. This study aims to determine conservative therapy's success rate and to identify features possibly associated with success. METHODS: A monocenter retrospective cohort study, including cSDH patients treated conservatively (wait-and-watch) from 2012 to 2022, was performed. The primary outcome was success of conservative therapy, defined as 'no crossover to surgery' during the follow-up period. Secondary outcomes were (1) factors associated with success, analyzed with univariate and multivariable logistic regression analyses, (2) 30-day mortality (3) time to crossover and (4) reasons for crossover. RESULTS: We included 159 patients. Conservative therapy was successful in 96 (60%) patients. Hematoma volume (OR 0.79, 95% CI 0.69-0.92) and hypodense hematoma type (OR 3.57, 95% 1.38-9.23) were associated with success. Thirty-day mortality rate was 5% and the median duration between diagnosis and surgery was 19 days (IQR 8-39). Clinical deterioration was the most frequent reason for crossover (in 61/63 patients, 97%) and was accompanied by radiological hematoma progression in 42 patients (67%). CONCLUSION: In this selected group of patients, conservative therapy was successful in 60%. Smaller hematoma volume and hypodense hematoma type were associated with success. As time until crossover was approximately three weeks, deploying conservative therapy as primary treatment seems safe and could be rewarding as surgical complications can be avoided. Improvement in patient selection in future cohorts remains warranted.

Malignant glioma in L-2-Hydroxyglutaric Aciduria: thorough molecular characterization of a case and literature review.

L-2-hydroxyglutaric aciduria (L-2-HGA) is a rare neurometabolic disorder characterized by accumulation of L2-hydroxyglutarate (L-2-HG) due to mutations in the L2HGDH gene. L-2-HGA patients have a significantly increased lifetime risk of central nervous system (CNS) tumors. Here, we present a 16-year-old girl with L-2-HGA who developed a tumor in the right cerebral hemisphere, which was discovered after left-sided neurological deficits of the patient. Histologically, the tumor had a high-grade diffuse glioma phenotype. DNA sequencing revealed the inactivating homozygous germline L2HGDH mutation as well as inactivating mutations in TP53, BCOR and NF1. Genome-wide DNA-methylation analysis was unable to classify the tumor with high confidence. More detailed analysis revealed that this tumor clustered amongst IDH-wildtype gliomas by methylation profiling and did not show the glioma CpG island methylator phenotype (G-CIMP) in contrast to IDH-mutant diffuse gliomas with accumulated levels of D-2-HG, the stereoisomer of L-2-HD. These findings were against all our expectations given the inhibitory potential of 2-HG on DNA-demethylation enzymes. Our final integrated histomolecular diagnosis of the tumor was diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype. Due to rapid tumor progression the patient died nine months after initial diagnosis. In this manuscript, we provide extensive molecular characterization of the tumor as well as a literature review focusing on oncogenetic considerations of L-2-HGA-associated CNS tumors.

Surgical complications of intrathecal baclofen in children: A single centre, 20-year retrospective cohort study.

INTRODUCTION: Complications of intrathecal baclofen treatment (ITB) with an implanted pump can be severe and require surgery. Surgical implantation techniques and catheter materials for continuous ITB treatment have improved over the past years with the aim to reduce complications. OBJECTIVE: To assess: 1) the type and rate of complications of ITB that require surgical intervention, 2) which risk factors influence the occurrence of complications, and, specifically, 3) whether complication rate is influenced by type of catheter used. METHODS: A retrospective cohort study was conducted including all children (<18 years old) in one university medical center with pump implantation between 2001 and 2017. All complications requiring surgery were recorded. Risk factors for surgical intervention were determined using multiple logistic regression analysis. Catheter related complications between two types of catheters (silicone vs coated) were compared. RESULTS: In total, 88 complications of ITB treatment requiring surgery were found in 47 (36.2%) out of 130 children. These included catheter-related complications (55.7% of all complications), infections (21.6%), cerebrospinal fluid leakage (14.8%), and pump-related complications (7.9%). The silicone catheter type, used until 2012, was found to be a significant risk factor for complications (Odds Ratio 3.75; 95% CI: 1.30-10.83). Since the introduction of the coated catheter type, in 2012, the rate of catheter-related complications decreased, from 0.15 to 0.10 complications per pump year. CONCLUSION: The rate of surgical complications of intrathecal baclofen in children is high, and most frequently catheter-related. The number of complications decreased since the introduction of a new, coated, catheter in 2012. This study helps to inform children and their caregivers about the risk of possible complications of ITB, and to identify directions for future improvement of ITB care.

[Ventriculoperitoneal shunt dysfunction in children: various clinical presentations]. / Ventriculoperitoneale-shuntdisfunctie bij kinderen.

The clinical presentation of a ventriculoperitoneal shunt (VP-shunt) dysfunction depends on whether the cranial sutures are still unfused, and on the cause and severity of the VP-shunt obstruction. A suspicion of a VP-shunt dysfunction is always reason to consult with a neurosurgeon. A patient with a suspected VP-shunt dysfunction that presents with elevated intracranial pressure should be urgently assessed at the emergency department of a neurosurgical center. Conclusions about whether the ventricular system is enlarged should be based on comparison between the imaging made to demonstrate the VP-shunt dysfunction and a reference scan of the brain, made when the patient was in a good clinical condition. In a patient with small ventricles, but clinical indications of elevated intracranial pressure, a VP-shunt dysfunction can't be ruled out. In that case fundoscopy may be very valuable to rule out papilledema.

Prediction of Meningioma WHO Grade Using PET Findings: A Systematic Review and Meta-Analysis.

BACKGROUND AND PURPOSE: World Health Organization (WHO) grading of meningiomas reflects recurrence rate and prognosis. Positron emission tomography (PET) investigates metabolic activity, allowing for distinction between low- and high-grade tumors. As preoperative suspicion for malignant meningioma will influence surgical strategy in terms of timing, extent of resection, and risks taken to achieve a total resection, we systematically reviewed the literature on PET-imaging in meningiomas and relate these findings to histopathological analysis. METHODS: Searches in PubMed, EMBASE, and The Cochrane Library, from inception to September 2019, included studies of patients who had undergone surgery for a histologically verified intracranial meningioma, with a PET-scan prior to surgery and description of (semi)quantitative PET values for meningiomas from two different WHO groups. Studies comparing more than 1 patient per WHO group were included in the meta-analysis. RESULTS: Twenty-two studies (432 patients) were included. 18fluor-fluorodesoxyglucose (18F-FDG) PET was mostly described to differentiate benign from malignant meningiomas. Pooled data showed differences in mean (95% CI) Standardized Uptake Value (SUV) for WHO II/III compared to WHO I of 2.51 (1.36, 3.66), and in tumor-to-normal (T/N) ratio (T/N ratio) for WHO II/III versus WHO I of .42 (.12, .73). CONCLUSIONS: We found that SUV and T/N ratio in 18F-FDG PET may be useful to noninvasively differentiate benign from malignant meningiomas. T/N ratio seems to have a high specificity for the detection of high-grade meningiomas. Other PET tracers were studied too infrequently to draw definitive conclusions. Before treatment strategies can be adapted based on 18F-FDG PET, prospective studies in larger cohorts are warranted to validate the optimal T/N ratio cutoff point.

Meningioma surgery in younger and older adults: patient profile and surgical outcomes.

BACKGROUND: Due to increasing life expectancy, the number of older patients harboring a meningioma is expected to increase. We determined whether preoperative variables and postoperative clinical outcome differ between younger and older adults. METHODS: Medical records of meningioma patients were retrospectively analyzed. Preoperative variables were age, gender, neurological symptoms, Karnofsky Performance Status (KPS), American Society of Anesthesiologists Physical Status (ASA)-classification and tumor characteristics. Clinical outcome was assessed using complication rates, length of hospital stay and destination after discharge. After 6-12 and 12-18-month KPS, neurological symptoms and Glasgow Outcome Scale (GOS) scores were assessed for older (age ≥ 65 years) and younger adults (18-65 years) using Mann-Whitney U, T test, Pearson's Chi square or Fisher's exact. RESULTS: 89 patients were included (23 ≥ 65 years). Before surgery, older patients scored higher on ASA classification (p = 0.003) and lower on KPS (p = 0.017). There was no significant difference postoperatively in mortality, complications and duration of hospital stay. Less older patients were discharged directly to home compared to younger adults (52 vs 80%, respectively; p = 0.004). In surviving patients, less older subjects had a good recovery (GOS 4-5) at 6-12 months' follow-up compared to younger subjects (64 vs 93%, respectively; p = 0.035). At 12-18 months, there was no significant difference in good recovery between both age groups (82 vs 92%). CONCLUSION: In this cohort, outcome was worse for patients ≥ 65 years old in terms of discharge destination and good recovery at 6-12 months. At 12-18 months follow-up, older subjects performed not significantly different from younger ones. Careful patient selection seems essential to reach good results in meningioma surgery for patients ≥ 65 years old.

Agreement Between Extent of Meningioma Resection Based on Surgical Simpson Grade and Based on Postoperative Magnetic Resonance Imaging Findings.

BACKGROUND: The surgical Simpson grade, introduced in 1957, is the standard measure for meningioma resection and prediction of recurrences. We used an magnetic resonance (MR)-based grading system for the radiologic extent of resection, and assessed agreement of the extent of resection between the surgical Simpson grade and the MR-based scale. METHODS: Patients were prospectively included during a 2-year period. Immediately after surgery, the surgeon determined the Simpson grade. MR imaging was performed within 72 hours and at 3 months after surgery. Scans were assessed by a neuroradiologist, blinded to the surgeon's grading. Intraclass correlation coefficient (ICC) and absolute agreement were used to evaluate agreement between both scales. RESULTS: Thirty-five patients (41 tumors) were included. Absolute agreement was 76%, with an ICC of 0.613. At 3 months postoperatively, the ICC and absolute agreement were 0.682 and 78%. In 20% of cases, the extent of resection was less favorable on the early postoperative MR imaging than the surgeon's Simpson grade. CONCLUSIONS: Agreement for extent of meningioma resection between both scales was good in terms of the ICC. When the surgical Simpson grade is unclear, MR imaging at 3 months after surgery may be used as a baseline for further follow-up. In a substantial portion of cases, the extent of resection was less favorable on the early postoperative MR imaging than the surgeon's Simpson grade. The predictive value of the radiologic extent of resection for the risk of long-term recurrences is a subject for further research.

[A man with a skull trauma]. / Een man met een hoofdtrauma.

After suffering an accidental blow to the head during soccer, a 42-year old male presented with agitation, confused speech and variable dysphasia. CT-imaging revealed a depressed parietotemporal skull fracture with an associated epidural hematoma. The epidural hematoma was surgically evacuated. In this photo report CT- and clinical images of an impressive depressed skull fracture, with an associated linear fracture are demonstrated.