Analysis of bihemispheric language function in pediatric neurosurgical patients using repetitive navigated transcranial magnetic stimulation.

OBJECTIVE: Language dominance in the developing brain can vary widely across anatomical and pathological conditions as well as age groups. Repetitive navigated transcranial magnetic stimulation (rnTMS) has been applied to calculate the hemispheric dominance ratio (HDR) in adults. In this study, the authors aimed to assess the feasibility of using rnTMS to identify language lateralization in a pediatric neurosurgical cohort and to correlate the preoperative rnTMS findings with the postoperative language outcome. METHODS: A consecutive prospectively collected cohort of 19 children with language-associated lesions underwent bihemispheric rnTMS mapping prior to surgery (100 stimulation sites on each hemisphere). In addition to feasibility and adverse effects, the HDR (ratio of the left hemisphere to right hemisphere error rate) was calculated. The anatomical surgical site and postoperative language outcome at 3 months after surgery were assessed according to clinical documentation. RESULTS: Repetitive nTMS mapping was feasible in all 19 children (mean age 12.5 years, range 4-17 years; 16 left-sided lesions) without any relevant adverse events. Thirteen children (68%) showed left hemispheric dominance (HDR > 1.1), and 2 children (11%) showed right hemispheric dominance (HDR < 0.9). In 4 children (21%), the bihemispheric error rates were nearly the same (HDR ≥ 0.9 and ≤ 1.1). Sixteen children underwent surgery (14 tumor/lesion resections and 2 hemispherotomies) and 3 patients continued conservative therapy. After surgery, 4 patients (25%) showed an improvement in language function, 10 (63%) presented with stable language function, and 2 (12.5%) experienced deterioration in language function. Of the 6 patients with right hemispheric language involvement, 4 (80%) had glial tumors, 1 (20%) had focal cortical dysplasia, and 1 (20%) experienced hypoxic brain injury. Children with right hemispheric language involvement (HDR ≤ 1.1) did not show any language deterioration postoperatively. CONCLUSIONS: Bihemispheric rnTMS language mapping as a noninvasive mapping technique to assess lateralization of language function in the pediatric neurosurgical population is safe and feasible. Why relevant right hemispheric language function (HDR ≤ 1.1) was associated with postoperative unaltered language function needs to be validated in future studies. Bihemispheric rnTMS language mapping strengthens risk-benefit considerations prior to pediatric tumor/epilepsy surgery in language-associated areas.

The importance of routine genetic testing in pediatric epilepsy surgery.

Genetic variants in relevant genes coexisting with MRI lesions in children with drug-resistant epilepsy (DRE) can negatively influence epilepsy surgery outcomes. Still, presurgical evaluation does not include genetic diagnostics routinely. Here, we report our presurgical evaluation algorithm that includes routine genetic testing. We analyzed retrospectively the data of 68 children with DRE operated at a mean age of 7.8 years (IQR: 8.1 years) at our center. In 49 children, genetic test results were available. We identified 21 gene variants (ACMG III: n = 7, ACMG IV: n = 2, ACMG V: n = 12) in 19 patients (45.2%) in the genes TSC1, TSC2, MECP2, DEPDC5, HUWE1, GRIN1, ASH1I, TRIO, KIF5C, CDON, ANKD11, TGFBR2, ATN1, COL4A1, JAK2, KCNQ2, ATP1A2, and GLI3 by whole-exome sequencing as well as deletions and duplications by array CGH in six patients. While the results did not change the surgery indication, they supported counseling with respect to postoperative chance of seizure freedom and weaning of antiseizure medication (ASM). The presence of genetic findings leads to the postoperative retention of at least one ASM. In our cohort, the International League against Epilepsy (ILAE) seizure outcome did not differ between patients with and without abnormal genetic findings. However, in the 7/68 patients with an unsatisfactory ILAE seizure outcome IV or V 12 months postsurgery, 2 had an abnormal or suspicious genetic finding as a putative explanation for persisting seizures postsurgery, and 3 had received palliative surgery including one TSC patient. This study highlights the importance of genetic testing in children with DRE to address putative underlying germline variants as genetic epilepsy causes or predisposing factors that guide patient and/or parent counseling on a case-by-case with respect to their individual chance of postoperative seizure freedom and ASM weaning. PLAIN LANGUAGE SUMMARY: Genetic variants in children with drug-resistant epilepsy (DRE) can negatively influence epilepsy surgery outcomes. However, presurgical evaluation does not include genetic diagnostics routinely. This retrospective study analyzed the genetic testing results of the 68 pediatric patients who received epilepsy surgery in our center. We identified 21 gene variants by whole-exome sequencing as well as deletions and duplications by array CGH in 6 patients. These results highlight the importance of genetic testing in children with DRE to guide patient and/or parent counseling on a case-by-case with respect to their individual chance of postoperative seizure freedom and ASM weaning.

Navigated bedside implantation of external ventricular drains with mobile health guidance: technical note and case series.

PURPOSE: External ventricular drain (EVD) implantation is one of the fundamental procedures of emergency neurosurgery usually performed freehand at bedside or in the operating room using anatomical landmarks. However, this technique is frequently associated with malpositioning leading to complications or dysfunction. Here, we describe a novel navigated bedside EVD insertion technique, which is evaluated in a clinical case series with the aim of safety, accuracy, and efficiency in neurosurgical emergency settings. METHODS: From 2021 to 2022, a mobile health-assisted navigation instrument (Thomale Guide, Christoph Miethke, Potsdam, Germany) was used alongside a battery-powered single-use drill (Phasor Health, Houston, USA) for bedside EVD placement in representative neurosurgical pathologies in emergency situations requiring ventricular cerebrospinal fluid (CSF) relief and intracranial pressure (ICP) monitoring. RESULTS: In all 12 patients (8 female and 4 male), navigated bedside EVDs were placed around the foramen of Monro at the first ventriculostomy attempt. The most frequent indication was aneurysmal subarachnoid hemorrhage. Mean operating time was 25.8 ± 15.0 min. None of the EVDs had to be revised due to malpositioning or dysfunction. Two EVDs were converted into a ventriculoperitoneal shunt. Drainage volume was 41.3 ± 37.1 ml per day in mean. Mean length of stay of an EVD was 6.25 ± 2.8 days. Complications included one postoperative subdural hematoma and cerebrospinal fluid infection, respectively. CONCLUSION: Combining a mobile health-assisted navigation instrument with a battery-powered drill and an appropriate ventricular catheter may enable and enhance safety, accuracy, and efficiency in bedside EVD implantation in various pathologies of emergency neurosurgery without adding relevant efforts.

Tailored Concept for Accurate Neuroendoscopy: A Comparative Retrospective Single-Center Study on Image-Guided Neuroendoscopic Procedures.

OBJECTIVE: Precise planning and execution is key for neuroendoscopic interventions, which can be based on different available aiding technologies. The aim of this retrospective study is to report a case-based use of guided neuroendoscopy and to develop a stratification algorithm for the available technologies. METHODS: We reviewed consecutive neuroendoscopic cases performed at our center from 2016 to 2018. We distinguished between patients receiving a new burr hole (group A) and those with a preexisting burr hole (group B). Case-specific technical requirements for procedure planning and execution, complication rate, surgical outcome, and possible subsequent surgery were evaluated. From this experience, a stratification system was developed to tailor the available guiding technologies. RESULTS: A total of 309 neuroendoscopic interventions in 243 patients were included in the present study. The cases included hydrocephalic (81.6%) and nonhydrocephalic (18.4%) conditions. The interventions were supported by coordinate-based (group A, n = 49; group B, n = 67), guide-based (group A, n = 42; group B, n = 0), ultrasound-guided (group A, n = 50; group B, n = 7), or navigated augmented reality-guided (group A, n = 85; group B, n = 9) techniques. The overall complication rate was 4.5%. Stratified by the surgical indication, fontanel status, entry point localization, presence of a preexisting burr hole, ventricular size, and number of targets, an approach toward image-guided neuroendoscopy is suggested. CONCLUSIONS: Planning and technical guidance is essential in neuroendoscopic procedures. The stratified decision-making algorithm for different available technologies aims to achieve lower cost and time consumption, which was found to be safe and efficient. Further investigations are warranted to deliver solid data on procedure efficiency.

A European multicenter outcome study on the different perioperative airway management policies following midface surgery in syndromic craniosynostosis: a proposal for a Standard Operating Procedure.

BACKGROUND: Perioperative airway management following midface advancements in children with Apert and Crouzon/Pfeiffer syndrome can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications. METHODS: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc / le Fort III procedures. RESULTS: Ultimately, 275 patients (129 monobloc and 146 Le Fort III) were included; 62 received immediate extubation and 162 delayed extubation; 42 had long-term tracheostomies and nine perioperative short-term tracheostomies. Short-term tracheostomies were in most centers reserved for selected cases. Patients with delayed extubation remained intubated for three days (IQR 2 - 5). The rate of no or only oxygen support after extubation was comparable between patients with immediate and delayed extubation, 58/62 (94%) and 137/162 (85%) patients, respectively. However, patients with immediate extubation developed less postoperative pneumonia than those with delayed, 0/62 (0%) versus 24/161 (15%) (P = 0.001), respectively. Immediate extubation also appeared safe in moderate/severe OSA since 19/20 (95%) required either no or only oxygen support after extubation. The odds of developing intubation-related complications increased by 21% with every extra day of intubation. CONCLUSIONS: Immediate extubation following midface advancements was found to be a safe option, as it was not associated with respiratory insufficiency but did lead to fewer complications. Immediate extubation should be considered routine management in patients with no/mild OSA and should be the aim in moderate/severe OSA after careful assessment.

The Supraorbital Eyebrow Approach in Pediatric Neurosurgery: Perspectives and Challenges of Frontal Keyhole Surgery.

OBJECT: Supraorbital craniotomy via an eyebrow incision provides minimally invasive cosmetically favorable access to both orbital and intracranial pathologies. We describe the indication, surgical technique, and clinical course using this surgical approach in a cohort of patients from a single pediatric neurosurgery unit. METHODS: In a retrospective analysis, we identified all surgical cases between January 2013 and April 2022 who underwent the supraorbital craniotomy via an eyebrow incision. Craniotomy was performed using piezosurgery ultrasonic bone incision. An interdisciplinary team of an orbital surgeon and a neurosurgeon performed the orbital surgeries. Clinical and surgical characteristics, perioperative data, possible complications, or redo surgeries as well as ophthalmologic status were assessed. RESULTS: Clinical data of 37 interventions (cases) in 30 patients (age: 8 ± 6.5 years) were analyzed. The supraorbital craniotomy established access to the cranial, lateral, and central portions of the orbit (n = 11) and ipsilateral fronto-medial portions of the skull base (n = 26). Thirty cases suffered from tumor disease with heterogeneous histopathologic diagnoses, and in 13 cases, adjuvant therapy was required. The mean duration of surgery was 163 ± 95 min, and the mean time of hospital stay was 6.0 ± 2.8 days. In two cases (5.4%), the following complications were observed. One infection treated by puncture and antibiotics and one revision surgery was necessary due to loosening of osteosynthesis material. Postoperative visual function was stable compared to preoperative status after all interventions. After a mean follow-up time of 26 ± 25.9 months for oncologic cases the long term outcome was complete remission in 13, stable disease in 14, progressive disease in 1 and death in 2 patients. CONCLUSION: The supraorbital eyebrow approach is feasible and safe in pediatric neurosurgical cases as a minimally invasive and cosmetic favorable technique and should be considered for intraorbital as well as ipsilateral intracranial lesions adjacent to the skull base. Interdisciplinary cooperation enables a broader spectrum of surgical options in orbital and complex, fronto-basal, skull base pathologies.

Epilepsy surgery in early infancy: A retrospective, multicenter study.

Although epilepsy surgery is the only curative therapeutic approach for lesional drug-resistant epilepsy (DRE), there is reluctance to operate on infants due to a fear of complications. A recent meta-analysis showed that epilepsy surgery in the first 6 months of life can achieve seizure control in about two thirds of children. However, robust data on surgical complications and postoperative cognitive development are lacking. We performed a retrospective multicenter study of infants who underwent epilepsy surgery in the first 6 months of life. 15 infants underwent epilepsy surgery at a median age of 134 days (IQR: 58) at four centers. The most common cause was malformation of cortical development, and 13 patients underwent a hemispherotomy. Two thirds required intraoperative red blood transfusions. Severe intraoperative complications occurred in two patients including death in one infant due to cardiovascular insufficiency. At a median follow-up of 1.5 years (IQR: 1.8), 57% of patients were seizure-free. Three patients where reoperated at a later age, resulting in 79% seizure freedom. Anti-seizure medication could be reduced in two thirds, and all patients improved in their development. Our findings suggest that early epilepsy surgery can result in good seizure control and developmental improvement. However, given the perioperative risks, it should be performed only in specialized centers.

Neurosurgical morbidity in pediatric supratentorial midline low-grade glioma: Results from the German LGG studies.

Surgical resection is a mainstay of treatment for pediatric low-grade glioma (LGG) within all current therapy algorithms, yet associated morbidity is scarcely reported. As supratentorial midline (SML) interventions are particularly challenging, we investigated the frequency of neurosurgical complications/new impairments aiming to identify their risk factors. Records were retrospectively analyzed from 318 patients with SML-LGG from successive German multicenter LGG studies, undergoing surgery between May 1998 and June 2020. Exactly 537 operations (230 resections, 167 biopsies, 140 nontumor procedures) were performed in 318 patients (54% male, median age: 7.6 years at diagnosis, 9.5 years at operation, 11% NF1, 42.5% optic pathway glioma). Surgical mortality rate was 0.93%. Applying the Drake classification, postoperative surgical morbidity was observed following 254/537 (47.3%) and medical morbidity following 97/537 (18.1%) patients with a 40.1% 30-day persistence rate for newly developed neurological deficits (65/162). Neuroendocrine impairment affected 53/318 patients (16.7%), visual deterioration 34/318 (10.7%). Postsurgical morbidity was associated with patient age <3 years at operation, tumor volume ≥80 cm3 , presence of hydrocephalus, complete resection, surgery in centers with less than median reported tumor-related procedures and during the earlier study period between 1998 and 2006, while the neurosurgical approach, tumor location, NF1 status or previous nonsurgical treatment were not. Neurosurgery-associated morbidity was frequent in pediatric patients with SML-LGG undergoing surgery in the German LGG-studies. We identified patient- and institution-associated factors that may increase the risk for complications. We advocate that local multidisciplinary teams consider the planned extent of resection and surgical skills.

Biopsies of Caudal Brainstem Tumors in Pediatric Patients-A Single-Center Retrospective Case Series.

OBJECTIVE: The indication for performing biopsies in patients with diffuse lesions in the brain stem is controversial. The possible risks associated with the technically challenging interventions must be balanced against clarifying the diagnosis and the possible therapeutic options. We reviewed the feasibility, risk profile, and diagnostic yield of different biopsy techniques in a pediatric cohort. METHODS: We retrospectively included all patients aged <18 years who had undergone biopsy of the caudal brainstem region (pons, medulla oblongata) at our pediatric neurosurgical center from 2009 to 2022. RESULTS: We identified 27 children. Biopsies were performed using frameless stereotactic (Varioguide; n = 12), robotic-assisted (Autoguide; n = 4), endoscopic (n = 3), and open biopsy (n = 8) techniques. Intervention-related mortality was not observed. Three patients experienced a transient postoperative neurological deficit. No patient experienced intervention-related permanent morbidity. Biopsy yielded the histopathological diagnosis in all 27 cases. Molecular analysis was feasible for 97% of the cases. The most common diagnosis was H3K27M-mutated diffuse midline glioma (60%). Low-grade gliomas were identified in 14% of patients. Overall survival was 62.5% after 24 months of follow-up. CONCLUSIONS: Biopsies of the caudal brainstem in children were feasible and safe in the presented setup. The amount of tumor material acquired allowing for an integrated diagnosis and was obtained at reasonable risk. The selection of the surgical technique depends on the tumor location and growth pattern. We recommend the performance of brainstem tumor biopsies in children at specialized centers to better understand the biology and enable possible novel therapeutic options.

[Pediatric Intracranial Ependymoma - Recommendations for First-Line Treatment from the German HIT-MED study group]. / Kinder und Jugendliche mit intrakraniellem Ependymom ­ Empfehlungen der HIT-MED-Studiengruppe der GPOH zur Erstlinientherapie.

Biological subtypes of ependymoma (EPN) have been introduced by the recent WHO classification and appear to have great impact on the clinical course, but have not yet found their way into clinical risk stratification. Further, the overall unfavorable prognosis underlines the fact that current therapeutic strategies need further evaluation for improvement. To date, there is no international consensus regarding first-line treatment for children with intracranial EPN. Extent of resection is known to be the most important clinical risk factor, leading to the consensus that consequent evaluation for re-surgery of postoperative residual tumor needs to have highest priority. Furthermore, efficacy of local irradiation is unquestioned and recommended for patients aged>1 year. In contrast, efficacy of chemotherapy is still under discussion. The European trial SIOP Ependymoma II aims at evaluating efficacy of different chemotherapy elements, leading to the recommendation to include German patients. The BIOMECA study, as biological accompanying study, aims at identifying new prognostic parameters. These results might help to develop targeted therapies for unfavorable biological subtypes. For patient who are not qualified for inclusion into the interventional strata, the HIT-MED Guidance 5.2 provides specific recommendations. This article is meant as an overview of national guidelines regarding diagnostics and treatment as well as of treatment according to the SIOP Ependymoma II trial protocol.

How to define CSF overdrainage: a systematic literature review.

PURPOSE: Overdrainage (OD) is one of the most frequent complications related to drainage of the cerebrospinal fluid (CSF). It is mostly associated with valve-bearing shunt systems but should probably be considered as a risk factor in any type of CSF diversion procedure. There is extreme variation in the reported incidence of OD due to the lack of consensus on defining criteria and an unclear perception of the pathophysiology. Hence, OD is probably underreported and underestimated. The objective of this paper was to establish a definition of OD, based on a systematic review of the literature. METHODS: A systematic search was conducted in MEDLNE and EMBASE. Studies providing a definition or a description of diagnostic findings related to OD in ventriculoperitoneal shunt treated hydrocephalus were included. Non-English titles, abstracts and manuscripts were excluded. Extracted descriptions were graded into five groups (class I-V studies) based on how precise the terminology used to describe OD was. Class I studies were included for further analysis and characteristics of OD were extracted. The quality of included descriptions was assessed by a clinical expert panel. RESULTS: A total of 1309 studies were screened, 190 were graded into groups, and 22, which provided specific definitions or descriptions of OD, were graded as class I studies. We extracted 32 different characteristics consistent with OD (e.g., clinical symptoms, radiological signs, and syndromes). CONCLUSION: There was an overall agreement that CSF overdrainage following implantation of a ventriculoperitoneal shunt in a mixed pediatric and adult population is characterized as a persistent condition with clinically manifestations as postural dependent headache, nausea, and vomiting and/or radiological signs of slim ventricles and/or subdural collections.

Maneuver protocol for outpatient telemetric intracranial pressure monitoring in hydrocephalus patients.

INTRODUCTION: Telemetric intracranial pressure measurement (tICPM) offers new opportunities to acquire objective information in shunted and non-shunted patients. The sensor reservoir (SR) provides tICPM modality at a decent sampling rate as an integrated component of the CSF shunt system. The aim of this study is to perform tICPM during a defined protocol of maneuvers in an outpatient setting as feasibility study including either shunt-dependent patients or candidates for possible shunt therapy. METHODS: A total of 17 patients received a SR and were investigated within a protocol of maneuver measurements involving different body postures (90°, 10°, 0°, and - 10°), breathing patterns (hypo- and hyperventilation), and mild venous congestion (Valsalva, Jugular vein compression), while the latter two were performed in lying postures (10° and 0°). The cohort included 11 shunted and 6 non-shunted (stand-alone-SR) patients. All measurements were evaluated using an ICP-analysis software (ICPicture, Miethke, Germany) looking at ICP changes and amplitude (AMP) characteristics. RESULTS: The shunted patient group consisted of 11 patients (median age: 15.8 years; range: 4-35.2 years) with either a primary shunt (n=9) and 2 patients received a shunt after stand-alone-SR tICPM. Six patients were enrolled with a stand-alone SR (median age 11.9 years, range 3.6-17.7 years). In the stand-alone SR group, maneuver related ICP and AMP changes were more sensitive compared to shunted patients. Postural maneuvers caused significant ICP changes in all body positions in both groups. The highest ICP values were seen during Valsalva maneuver, provoked by the patients themselves. In the stand-alone group, significant higher ICP values during hyperventilation were observed compared to shunted individuals. In shunted patients, a significant correlation between ICP and AMP was observed only during hyperventilation maneuver, while this correlation was additionally seen in Valsalva and jugular vein compression in stand-alone patients. CONCLUSION: SR-related tICPM is helpful to objectify diagnostic evaluation in patients with CSF dynamic disturbances. The defined protocol did result in a wide range of ICP changes with promising potential for effective outpatient tICPM investigation. Since the correlation of ICP and AMP was observed during mild venous congestion maneuvers it appears to be specifically helpful for the evaluation of intracranial compliance. Further investigations of maneuver-related tICPM in a larger population, including variable pathologies, are needed to further establish the protocol in the clinical practice.

Importance of urodynamic evaluation of bladder function after secondary untethering in spina bifida patients: single center experience of 30 years.

INTRODUCTION: A TCS after primary closure of meningomyeloceles is a known complication of the spina bifida disease. Data on the outcome after SSCU surgery is heterogeneous and lacking standardization. Thus we aimed to find a reliable system for assessment of the bladder function before and after SSCU surgery and document postoperative outcome. METHODS: A retrospective study was performed on a cohort of patients with spina bifida diagnosis. In total, 130 patients underwent 182 SSCU surgeries, 56 of those met our inclusion criteria. A classification system, including two different methods, was used. The AC system used baseline pressure and detrusor over activity to define three levels of bladder dysfunction, the second method ranked the severity of bladder dysfunction by awarding points from 0 to 2 for bladder capacity, maximal detrusor pressure during autonomous contractions, leak point pressure and vesicoureteral reflux A high score is correlated with a severe bladder dysfunction. RESULTS: Gender distribution was equally (male: n = 29; 51.8%; female: n = 27; 48.2%). The median age at SSCU was 902 years (range 0.5-22.8 years). After SSCU, the stage improved in 11 patients (19.6%), worsened in 11 (19.6%) patients and remained the same in 34 patients (60.7%) after intervention (AC score). Non-worsening was observed in a total of 45 cases (80.4%) (p < 0.001). MHS score (n = 27, 48.2%) improved, remained unchanged (n = 12, 21.4%), 17 patients worsened (30.4%). Non-worsening in postoperative bladder functional outcome was demonstrated in 39 cases (69.6%) over all (p < 0.005). Regardless of whether bladder function is categorized by AC or MHS, postoperative outcome worsened significantly when SSCU was performed due to increasing deterioration in motor function alone (p < 0.05). Of the 24 cases with NOD as indication, 22 (91.7%) had an unchanged (n = 10; 41.7%) or improved (n = 12; 50.0%), meaning positive neuro-orthopedic outcome, only 2 (8.3%) deteriorated (p < 0.001). CONCLUSION: Our study presents reliable evaluation systems for bladder function in spina bifida patients. Since indications for SSCU surgery differ, it is important to know the possible effects on bladder function after this surgical procedure. Even a mild impairment of bladder function has a risk to deteriorate after SSCU surgery. Particularly interesting becomes this with regard to the fact that the prevalence of TCS might become more frequent with the rising numbers of prenatal closures of meningomyeloceles.

Underestimating isolated bilateral hygroma as non-accidental head injury with dramatic consequences: a case presentation.

OBJECTIVE: Abusive head injury (AHI) in infancy is associated with significantly worse outcomes compared to accidental traumatic brain injury. The decision-making of the diagnosis of AHI is challenging especially if the clinical signs are not presenting as a multifactorial pattern. METHOD: We present a case of isolated bilateral hygroma in which this differential diagnosis of AHI was evaluated but primarily not seen as such leading subsequently to extensive secondary AHI with fatal brain injury. RESULTS: The case of an 8-week-old infant with apparently isolated bilateral hygroma without any external signs of abuse and no retinal hemorrhages was interpreted in causative correlation to the perinatal complex course of delivery. At a second readmission of the case, severe brain injury with bilateral cortical hypoxia, subarachnoid and subdural hemorrhages, and skull and extremity fractures led to severe disability of the affected infant. CONCLUSION: Any early suspicion of AHI with at least one factor possibly being associated with abusive trauma should be discussed in multidisciplinary team conferences to find the best strategy to protect the child. Beside clinical factors, social factors within the family household may additionally be evaluated to determine stress-related risk for traumatic child abuse. In general, prevention programs will be essential in future perspective.

Long-term follow-up of surgical intervention pattern in pediatric low-grade gliomas: report from the German SIOP-LGG 2004 cohort.

OBJECTIVE: Neurosurgical treatment is an integral part of the treatment algorithms for pediatric low-grade glioma (LGG), yet patterns of surgical procedures are rarely challenged. The objective of this study was to evaluate surgical treatment patterns in pediatric LGG. METHODS: The German Societé Internationale d'Oncologie Pédiatrique (SIOP)-LGG 2004 cohort was analyzed to identify relevant patient and tumor characteristics associated with time to death, next surgery, number of resections, and radiological outcome. RESULTS: A total of 1271 patients underwent 1713 neurosurgical interventions (1 intervention in 947, 2 in 230, 3 in 70, and 4-6 in 24). The median age of the study population was 8.57 years at first surgery, and 46.1% were female. Neurofibromatosis type 1 (NF1) was found in 4.4%, and 5.4% had tumor dissemination. Three hundred fifty-four patients (27.9%) had chemotherapy and/or radiotherapy. The cumulative incidence of second surgery at 10 years was 26%, and was higher for infants, those with spinal and supratentorial midline (SML) tumors, and those with pilomyxoid astrocytomas. The hazard ratio for subsequent surgery was higher given dissemination and noncomplete initial resection, and lower for caudal brainstem and SML tumors. Among 1225 patients with fully documented surgical records and radiological outcome, 613 reached complete remission during the observation period, and 50 patients died. Patients with pilocytic astrocytoma had higher chances for a final complete remission, whereas patients with initial partial or subtotal tumor resection, dissemination, NF1, or primary tumor sites in the spinal cord and SML had lower chances. CONCLUSIONS: Neurosurgery is a key element of pediatric LGG treatment. In almost 50% of the patients, however, at least some tumor burden will remain during long-term follow-up. This study found that most of these patients reached a stable disease status without further surgeries. Multidisciplinary team decisions must balance the goal of complete resection, risk factors, repeated surgeries, and possible treatment alternatives in a wide range of heterogeneous entities. Procedural details and neurological outcome should be recorded to better assess their impact on long-term outcome.

Ictal EEG recording is not mandatory in all candidates for paediatric epilepsy surgery with clear MRI lesions and corresponding seizure semiology

OBJECTIVE: Epilepsy surgery can potentially cure drug-resistant epilepsy, but careful presurgical evaluation is vital to select patients who will profit from such an intervention. Many epilepsy surgery programs offer extensive presurgical evaluation including several days of video-EEG monitoring. Non-lesional epilepsy cases are rare among epilepsy surgery patients. We set up a lesion-orientated paediatric epilepsy surgery program for patients with clearly localized lesions with limited presurgical diagnostics, in particular, with a maximum of 48 hours of non-invasive EEG monitoring that did not necessarily include ictal EEGs. METHODS: We retrospectively evaluated the outcome of patients who were operated on within our epilepsy surgery program with respect to seizure freedom. RESULTS: Fifty-two children and adolescents with MRI lesions at a mean age of 8.27 ±4.83 years (range: 0.17-18.87) underwent a resective procedure. The most frequent surgery was a hemispherotomy. Overall seizure freedom was 81.8% after 12 months and 85.6% after a median observation period of 20.45 months. Seizure frequency was reduced >50% in all other patients. Preoperative recording of an ictal EEG on the side of surgery had no effect on postoperative seizure outcome (p= 0.697), nor did recording of epileptiform discharges on the ipsilateral (p= 0.538) and contralateral side (p= 0.147). SIGNIFICANCE: Our findings highlight the high success rate using a lesion-orientated epilepsy surgical approach with reduced presurgical video-EEG monitoring in the paediatric epilepsy population. Our data show that it is possible to reduce the complex pre-surgical work-up for epilepsy in children and adolescents by asking the basic question: "Is there any reason why the lesion should not be resected".

Surgical Management of Pre-Chiasmatic Intraorbital Optic Nerve Gliomas in Children after Loss of Visual Function-Resection from Bulbus to Chiasm.

Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the subgroup of pre-chiasmatic gliomas, intraorbital and intradural resection is a curative option after blindness. We present a two-center cohort using different surgical approaches. A retrospective analysis was performed, including 10 children. Mean age at surgery was 6.8 years. Interval between diagnosis and surgery was 1-74 (mean 24 ± 5.5, median 10) months. Indications for surgery were exophthalmos, pain, tumor progression, or a combination. Eight patients underwent an extradural trans-orbital-roof approach to resect the intra-orbital tumor, including the optic canal part plus intradural pre-chiasmatic resection. Gross total resection was achieved in 7/8, and none had a recurrence. One residual behind the bulbus showed progression, treated by chemotherapy. In two patients, a combined supra-orbital mini-craniotomy plus orbital frame osteotomy was used for intraorbital tumor resection + intradural pre-chiasmatic dissection. In these two patients, remnants of the optic nerve within the optic canal remained stable. No patient had a chiasmatic functional affection nor permanent oculomotor deficits. In selected patients, a surgical resection from bulb to chiasm ± removal of optic canal tumor was safe without long-term sequela and with an excellent cosmetic result. Surgery normalizes exophthalmos and provides an effective tumor control.

Severe Traumatic Brain Injury in children-paradigm of decompressive craniectomy compared to a historic cohort.

PURPOSE: Traumatic brain injury (TBI) is one of the leading causes of death and disability in children. Medical therapy remains limited, and decompressive craniectomy (DC) is an established rescue therapy in case of elevated intracranial pressure (ICP). Much discussion deals with clinical outcome after severe TBI treated with DC, while data on the pediatric population is rare. We report our experience of treating severe TBI in two different treatment setups at the same academic institution. METHODS: Forty-eight patients (≤ 16 years) were hospitalized with severe TBI (GCS ≤ 8 points) between 2008 and 2018 in a pediatric intensive care unit (ICU) at a specialized tertiary pediatric care center. Data on treatment, clinical status, and outcome was retrospectively analyzed. Outcome data included Glasgow Outcome Scale (GOS) at 3-, 12-, and 36-month follow-up. Data was compared to a historic cohort with 53 pediatric severe TBI patients treated at the same institution in a neurointensive care unit between 1996 and 2007. Ethical approval was granted (EA2/076/21). RESULTS: Between 2008 and 2018, 11 patients were treated with DC. Compared to the historic cohort, patients were younger and GCS was worse, while in-hospital mortality and clinical outcome remained similar. A trend towards more aggressive EVD placement and the internal paradigm change for treatment in a specialized pediatric ICU was observed. CONCLUSIONS: In children with severe TBI treated over two decades, clinical outcome was comparable and mostly favorable in two different treatment setups. Consequent therapy is warranted to maintain the positive potential for favorable outcome in children with severe TBI.