Ulrich-wilhelm Thomale - Academia.edu (original) (raw)

Papers by Ulrich-wilhelm Thomale

Research paper thumbnail of Epilepsy Surgery in Children with Minimal Presurgical Video-EEG Monitoring

Abstracts of the 46th Annual Meeting of the Society for Neuropediatrics, 2021

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

Children, 2022

Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the... more 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 + ...

Research paper thumbnail of Severe Traumatic Brain Injury in children—paradigm of decompressive craniectomy compared to a historic cohort

Acta Neurochirurgica, 2022

Purpose Traumatic brain injury (TBI) is one of the leading causes of death and disability in chil... more 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...

Research paper thumbnail of The need for a consistent nomenclature for adjustable differential pressure and gravitational hydrocephalus valves. Results from a German Survey

Objective: The introduction of adjustable differential pressure (DP) and gravitational assisted (... more Objective: The introduction of adjustable differential pressure (DP) and gravitational assisted (GA) valves has advanced the treatment of hydrocephalus within the last years. Due to the mobility of these chronic patients, treatment may be performed by various neurosurgeons at different centers. Since[for full text, please go to the a.m. URL]

Research paper thumbnail of First experiences with gravitational valves (proGAV) in the pediatric population

Objective: CSF overdrainage is the source of complications after ventriculoperitoneal shunting wi... more Objective: CSF overdrainage is the source of complications after ventriculoperitoneal shunting with differential-pressure valves. Recently, an adjustable gravity-assisted valve (proGAV) had been developed to overcome overdrainage problems. Up to now, proGAV only had been used in adult hydrocephalus.[for full text, please go to the a.m. URL]

Research paper thumbnail of Tumor load rather than contrast enhancement is associated with the visual function of children and adolescents with optic pathway glioma – a retrospective Magnetic Resonance Imaging study

Journal of Neuro-Oncology, 2022

Introduction Optic pathway gliomas are often asymptomatic tumors occurring in children with neuro... more Introduction Optic pathway gliomas are often asymptomatic tumors occurring in children with neurofibromatosis type 1 (NF1 + OPG) or sporadically (spOPG). Treatment is usually prompted by visual loss and/or tumor progression on MRI. The aim of this study was to investigate the relationship between visual acuity (VA), tumor growth, and contrast enhancement to provide more distinct indications for the administration of gadolinium-based contrast agents. Methods Tumor load was retrospectively measured and enhancement semi-quantitatively scored on 298 MRIs of 35 patients (63% NF1 + OPG). Spearman rank correlation between tumor load and enhancement was calculated and a linear mixed model used to examine the influence of tumor load and enhancement on corresponding VA tests (LogMAR). Results The optic nerve width in NF1 + OPGs was strongly associated with VA (regression coefficient 0.75; confidence interval 0.61—0.88), but weakly with enhancement (0.06; −0.04—0.15). In spOPGs, tumor volume a...

Research paper thumbnail of Case Report: Hemispherotomy in the First Days of Life to Treat Drug-Resistant Lesional Epilepsy

Frontiers in Neurology, 2021

Background: Neonatal drug-resistant epilepsy is often caused by perinatal epileptogenic insults s... more Background: Neonatal drug-resistant epilepsy is often caused by perinatal epileptogenic insults such as stroke, ischemia, hemorrhage, and/or genetic defects. Rapid seizure control is particularly important for cognitive development. Since early surgical intervention and thus a short duration of epilepsy should lead to an optimal developmental outcome, we present our experience with hemispherotomy in an infant at the corrected age of 1 week.Methods: We report successful hemispherotomy for drug-resistant epilepsy in an infant with hemimegalencephaly at a corrected age of 1 week.Results: The infant was diagnosed with drug-resistant lesional epilepsy due to hemimegalencephaly affecting the left hemisphere. Given congruent electroclinical findings, we performed a left vertical parasagittal transventricular hemispherotomy after critical interdisciplinary discussion. No complications occurred during the surgery. Intraoperatively; 118 ml of red blood cells (30 ml/kg) and 80 ml of plasma wer...

Research paper thumbnail of Angulation towards coronal convexity measure and catheter length indication improves the quality of ventricular catheter placement – a smartphone-assisted guidance technique

World Neurosurgery, 2021

Accurate placement of a ventricular catheter is crucial to reduce the risk of shunt failure. In t... more Accurate placement of a ventricular catheter is crucial to reduce the risk of shunt failure. In the randomized, prospective, multicenter GAVCA trial, which evaluated the quality of ventricular catheter placement, the subgroup of patients with detailed length marked ventricular catheters (dVC) reflected a difference in the primary endpoint of optimal VC placement in contrast to the subgroup of patients with simplified length marked ventricular catheters (sVC). Objective of this analysis is to compare the dVC-group with the sVC-group as well as the smartphone-assisted guidance technique (GA) with the standard freehand technique (F) for catheter placement. We performed a further analysis of the GAVCA trial in two steps: 1st part) detailed length marked VCs (providing detailed distance from tip to base (3-13cm) in 0.5cm markings) compared to simplified length marked VCs with length indication at 5 cm and 10 cm from tip to base and 2nd part) comparing the smartphone-assisted guidance technique vs. freehand technique in the dVC-group. 1st part) Data of 137 patients (104 dVC patients vs. 33 sVC patients) was eligible for this analysis. Optimal VC placement was achieved in 72.1% of the dVC-group and 39.4% of the sVC-group (p=<0.001, odds ratio (OR) 3.9, 95% CI 1.7-9.3). 2nd part) The 104 dVC patients underwent a subgroup analysis concerning the catheter placement using different techniques for catheter placement (54 guidance technique (GA) vs. 50 freehand technique (F). Optimal catheter placement was achieved in 81.5% of the GA group and 62.0% of the F group (p=0.03, odds ratio 2.7, 95% CI 1.1-6.8). All patients who underwent guidance technique ventricular catheter placement (GA) were successful on primary puncture while for 8.7% of patients in the freehand group (F), multiple attempts were necessary (p=0.03). This analysis suggests the combination of a smartphone-assisted guidance technique (GA) and use of a detailed length marked ventricular catheter (dVC) to improve the rate of accurate ventricular catheter placement. Compared with freehand technique (F), patients' safety is increased by a reduction of unsuccessful ventricular catheter placement attempts.

Research paper thumbnail of The Influence of Perioperative Antibiotic Prophylaxis on Wound Infection and on the Colonization of Wound Drains in Patients After Correction of Craniosynostosis

Frontiers in Pediatrics, 2021

Objective: Evidence for the duration of perioperative antibiotic prophylaxis (PAP) after the corr... more Objective: Evidence for the duration of perioperative antibiotic prophylaxis (PAP) after the correction of craniosynostosis in children is scarce. We evaluated the necessary duration of PAP to ensure a minimal rate of postoperative wound infections.Methods: In this monocentric, retrospective, and prospective pilot study, two PAP protocols were compared. From August 2017 to May 2018, treatment group 1 (TG 1) was treated using the standard PAP protocol with at least three doses of antibiotics. Between May 2018 and March 2019, a shortened PAP with a single-shot administration was given to treatment group 2 (TG 2a and b). Endpoints of this study were wound infection rate, colonization rate of wound drains, and the course of treatment reflected by clinical and laboratory data.Results: A cohort of 187 children underwent craniosynostosis correction: 167 were treated according to protocols-−95 patients with at least three doses (TG 1) and 72 patients with a single-shot of cefuroxime (TG 2a)...

Research paper thumbnail of Pediatric thalamic incidentalomas: an international retrospective multicenter study

Journal of Neurosurgery: Pediatrics, 2021

OBJECTIVE Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain ... more OBJECTIVE Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain incidentalomas. Thalamic lesions have unique characteristics such as deep location, surgical complexity, and proximity to eloquent neuronal structures. Currently, the natural course of incidental thalamic lesions is unknown. Therefore, the authors present their experience in treating such lesions. METHODS A retrospective, international multicenter study was carried out in 8 tertiary pediatric centers from 5 countries. Patients were included if they had an incidental thalamic lesion suspected of being a tumor and were diagnosed before the age of 20 years. Treatment strategy, imaging characteristics, pathology, and the outcome of operated and unoperated cases were analyzed. RESULTS Overall, 58 children (23 females and 35 males) with a mean age of 10.8 ± 4.0 years were included. The two most common indications for imaging were nonspecific reasons (n = 19; e.g., research and developmental de...

Research paper thumbnail of Introduction to the annual issue on “Infant Hydrocephalus“

Child's Nervous System, 2021

Research paper thumbnail of Extended experience in parieto-occipital expansion surgery by meander technique—clinical and radiological evaluation

Child's Nervous System, 2021

Introduction Brachycephaly and anterior and posterior plagiocephaly appear as an isolated entity ... more Introduction Brachycephaly and anterior and posterior plagiocephaly appear as an isolated entity or manifest in syndromic conditions. In severe cases, possible treatment options currently comprise either cranioplasty or osteogenetic distraction. The aim of this paper is to retrospectively review the perioperative course of a series of children treated by posterior meander expansion technique at our institution with focus on the course of postoperative intracranial volume and eventual tonsillar descent evolution. Methods Forty-two children received a posterior cranial vault remodeling by means of a posterior meander technique during a 7-year period. Hospital records were reviewed, and pre- and postoperative MRIs were analyzed for intracranial volume, cephalic and asymmetry index, and tonsillar position over time. Results Median age at surgery was 11.5 months (range 17 days–10 years). Nineteen children had a symmetrical cranial deformity, twenty-three an asymmetrical synostosis. Half ...

Research paper thumbnail of Molecular characterisation of sporadic endolymphatic sac tumours and comparison to von Hippel–Lindau disease‐related tumours

Neuropathology and Applied Neurobiology, 2021

This is an open access article under the terms of the Creat ive Commo ns Attri butio n-NonCo mmer... more This is an open access article under the terms of the Creat ive Commo ns Attri butio n-NonCo mmerc ial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Research paper thumbnail of Surgical management of intracranial arachnoid cysts in pediatric patients: radiological and clinical outcome

Journal of Neurosurgery: Pediatrics, 2021

OBJECTIVE Congenital intracranial cysts account for a significant portion of intracranial lesions... more OBJECTIVE Congenital intracranial cysts account for a significant portion of intracranial lesions in the pediatric population. The efficiency of surgical treatment in a pediatric cohort with intracranial arachnoid cysts (ACs) at different locations regarding clinical symptoms and mass effect was evaluated. METHODS A retrospective study of all children who underwent surgical treatment of an intracranial AC during an 11-year period (2007–2018) was performed. Demographics, clinical symptoms, and radiological cyst size pre- and postoperatively, as well as the reoperation rate and possible treatment complications, were analyzed. RESULTS A total of 116 intracranial cysts at 8 different anatomical locations were surgically treated in 113 children (median age 5 years and 10 months) predominantly by endoscopic technique (84%). The complication rate was 3%, and the reoperation rate was 16%. Preoperative cyst enlargement was significantly more common in infants (p < 0.0001), as was the need...

Research paper thumbnail of Quantitative and qualitative comparison of morphometric outcomes after endoscopic and conventional correction of sagittal and metopic craniosynostosis versus control groups

Neurosurgical Focus, 2021

OBJECTIVE Surgical correction for sagittal and metopic craniosynostosis (SCS and MCS) aims to alt... more OBJECTIVE Surgical correction for sagittal and metopic craniosynostosis (SCS and MCS) aims to alter the abnormal cranial shape to resemble that of the normal population. The achieved correction can be assessed by morphometric parameters. The purpose of the presented study was to compare craniometric parameters of control groups to those same parameters after endoscopic and conventional (open) correction. METHODS The authors identified 4 groups of children undergoing surgical treatment for either SCS or MCS, with either endoscopic (SCS, n = 17; MCS, n = 16) or conventional (SCS, n = 29; MCS, n = 18) correction. In addition, normal control groups of nonaffected children who were 6 (n = 30) and 24 (n = 18) months old were evaluated. For all groups, several craniometric indices calculated from 3D photographs were compared for quantitative analysis. For qualitative comparison, averages of all 3D photographs were generated for all groups and superimposed to visualize relative changes. RES...

Research paper thumbnail of Seizures following surgery for supratentorial extratemporal low-grade tumors in children: a multicenter retrospective study

Journal of Neurosurgery: Pediatrics, 2020

OBJECTIVEResection of brain tumors may lead to new-onset seizures but may also reduce seizure rat... more OBJECTIVEResection of brain tumors may lead to new-onset seizures but may also reduce seizure rates in patients presenting with seizures. Seizures are seen at presentation in about 24% of patients with brain tumors. For lesional epilepsy in general, early resection is associated with improved seizure control. However, the literature is limited regarding the occurrence of new-onset postoperative seizures, or rates of seizure control in those presenting with seizures, following resections of extratemporal low-grade gliomas (LGGs) in children.METHODSData were collected retrospectively from 4 large tertiary centers for children (< 18 years of age) who underwent resection of a supratentorial extratemporal (STET) LGG. The patients were divided into 4 groups based on preoperative seizure history: no seizures, up to 2 seizures, more than 2 seizures, and uncontrolled or refractory epilepsy. The authors analyzed the postoperative occurrence of seizures and the need for antiepileptic drugs ...

Research paper thumbnail of Management of pineal region tumors in a pediatric case series

Neurosurgical Review, 2020

Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous h... more Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We evaluated our surgical experience concerning procurance of a histological diagnosis, management of hydrocephalus, and choice of antitumoral treatment. We analyzed the efficacy of neuroendoscopic biopsy and endoscopic third ventriculocisternostomy (ETV) in patients with pineal region tumors between 2006 and 2019 in a single-center retrospective cross-sectional study with regard to diagnostic yield, hydrocephalus treatment, as well as impact on further antitumoral management. Out of 28 identified patients, 23 patients presented with untreated hydrocephalus and 25 without histological diagnosis. One patient underwent open biopsy, and 24 received a neuroendoscopic biopsy with concomitant hydrocephalus treatment if necessary. Eighteen primary ETVs, 2 secondary ETVs, and 2 ventriculoperitoneal shunts (VPSs) were performed. Endosco...

Research paper thumbnail of Prognostic impact of distinct genetic entities in pediatric diffuse glioma WHO-grade II - report from the German/Swiss SIOP-LGG 2004 cohort

International Journal of Cancer, 2020

Reports on pediatric low-grade diffuse glioma WHO-grade II (DG2) suggest an impaired survival rat... more Reports on pediatric low-grade diffuse glioma WHO-grade II (DG2) suggest an impaired survival rate, but lack conclusive results for genetically defined DG2-entities. We analyzed the natural history, treatment and prognosis of DG2 and investigated which genetically defined sub-entities proved unfavorable for survival. Within the prospectively registered, population-based German/Swiss SIOP-LGG 2004 cohort 100 patients (age 0.8-17.8 years, 4% neurofibromatosis [NF1]) were diagnosed with a

Research paper thumbnail of Cranial and ventricular size following shunting or endoscopic third ventriculostomy (ETV) in infants with aqueductal stenosis: further insights from the International Infant Hydrocephalus Study (IIHS)

Child's Nervous System, 2020

Purpose The craniometrics of head circumference (HC) and ventricular size are part of the clinica... more Purpose The craniometrics of head circumference (HC) and ventricular size are part of the clinical assessment of infants with hydrocephalus and are often utilized in conjunction with other clinical and radiological parameters to determine the success of treatment. We aimed to assess the effect of endoscopic third ventriculostomy (ETV) and shunting on craniometric measurements during the follow-up of a cohort of infants with symptomatic triventricular hydrocephalus secondary to aqueductal stenosis. Methods We performed a post hoc analysis of data from the International Infant Hydrocephalus Study (IIHS)-a prospective, multicenter study of infants (< 24 months old) with hydrocephalus from aqueductal stenosis who were treated with either an ETV or shunt. During various stages of a 5-year follow-up period, the following craniometrics were measured: HC, HC centile, HC zscore, and frontal-occipital horn ratio (FOR). Data were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and sex. Results Of 158 enrolled patients, 115 underwent an ETV, while 43 received a shunt. Both procedures led to improvements in the mean HC centile position and z-score, a trend which continued until the 5-year assessment point. A similar trend was noted for FOR which was measured at 12 months and 3 years following initial treatment. Although the values were consistently higher for ETV compared with shunt, the differences in HC value, centile, and z-score were not significant. ETV was associated with a significantly higher FOR compared with shunting at 12 months (0.52 vs 0.44; p = 0.002) and 3 years (0.46 vs 0.38; p = 0.03) of follow-up.

Research paper thumbnail of Dynamic cerebellar herniation in Chiari patients during the cardiac cycle evaluated by dynamic magnetic resonance imaging

Neuroradiology, 2019

PurposeCerebellar herniation in Chiari patients can be dynamic, following the cerebrospinal fluid... more PurposeCerebellar herniation in Chiari patients can be dynamic, following the cerebrospinal fluid pulsatility during the cardiac cycle. We present a voxel intensity distribution method (VIDM) to automatically extract the pulsatility-dependent herniation in time-resolved MRI (CINE MRI) and compare it to the simple linear measurements. The degree of herniation is furthermore compared on CINE and static sequences, and the cerebellar movement is correlated to the presence of hydrocephalus and syringomyelia.MethodsThe cerebellar movement in 27 Chiari patients is analyzed with VIDM and the results were compared to linear measurements on an image viewer (visual inspection, VI) using a paired t test. Second, an ANOVA test is applied to compare the degree of herniation on static 3D MRI and CINE. Finally, the Pearson’s correlation coefficient is calculated for the correlation between cerebellar movement and the presence of hydrocephalus and syringomyelia.ResultsVIDM showed significant movement in 85% of our patients. Assuming that movement < 1 mm cannot be detected reliably on an image viewer, VI identified movement in 29.6% of the patients (p = 0.002). The herniation was greater on static sequences than on CINE in most cases, but this was not statistically significant. The cerebellar movement was not correlated with hydrocephalus or syringomyelia (Pearson’s coefficient < 0.3).ConclusionsVIDM is a sensitive method to detect tissue movement on CINE MRI and could be used for Chiari patients, but also for the evaluation of cyst membranes, ventriculostomies, etc. The cerebellar movement appears not to correlate with hydrocephalus and syringomyelia in Chiari patients.

Research paper thumbnail of Epilepsy Surgery in Children with Minimal Presurgical Video-EEG Monitoring

Abstracts of the 46th Annual Meeting of the Society for Neuropediatrics, 2021

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

Children, 2022

Optic pathway gliomas in children carry significant morbidity and therapeutic challenges. For the... more 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 + ...

Research paper thumbnail of Severe Traumatic Brain Injury in children—paradigm of decompressive craniectomy compared to a historic cohort

Acta Neurochirurgica, 2022

Purpose Traumatic brain injury (TBI) is one of the leading causes of death and disability in chil... more 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...

Research paper thumbnail of The need for a consistent nomenclature for adjustable differential pressure and gravitational hydrocephalus valves. Results from a German Survey

Objective: The introduction of adjustable differential pressure (DP) and gravitational assisted (... more Objective: The introduction of adjustable differential pressure (DP) and gravitational assisted (GA) valves has advanced the treatment of hydrocephalus within the last years. Due to the mobility of these chronic patients, treatment may be performed by various neurosurgeons at different centers. Since[for full text, please go to the a.m. URL]

Research paper thumbnail of First experiences with gravitational valves (proGAV) in the pediatric population

Objective: CSF overdrainage is the source of complications after ventriculoperitoneal shunting wi... more Objective: CSF overdrainage is the source of complications after ventriculoperitoneal shunting with differential-pressure valves. Recently, an adjustable gravity-assisted valve (proGAV) had been developed to overcome overdrainage problems. Up to now, proGAV only had been used in adult hydrocephalus.[for full text, please go to the a.m. URL]

Research paper thumbnail of Tumor load rather than contrast enhancement is associated with the visual function of children and adolescents with optic pathway glioma – a retrospective Magnetic Resonance Imaging study

Journal of Neuro-Oncology, 2022

Introduction Optic pathway gliomas are often asymptomatic tumors occurring in children with neuro... more Introduction Optic pathway gliomas are often asymptomatic tumors occurring in children with neurofibromatosis type 1 (NF1 + OPG) or sporadically (spOPG). Treatment is usually prompted by visual loss and/or tumor progression on MRI. The aim of this study was to investigate the relationship between visual acuity (VA), tumor growth, and contrast enhancement to provide more distinct indications for the administration of gadolinium-based contrast agents. Methods Tumor load was retrospectively measured and enhancement semi-quantitatively scored on 298 MRIs of 35 patients (63% NF1 + OPG). Spearman rank correlation between tumor load and enhancement was calculated and a linear mixed model used to examine the influence of tumor load and enhancement on corresponding VA tests (LogMAR). Results The optic nerve width in NF1 + OPGs was strongly associated with VA (regression coefficient 0.75; confidence interval 0.61—0.88), but weakly with enhancement (0.06; −0.04—0.15). In spOPGs, tumor volume a...

Research paper thumbnail of Case Report: Hemispherotomy in the First Days of Life to Treat Drug-Resistant Lesional Epilepsy

Frontiers in Neurology, 2021

Background: Neonatal drug-resistant epilepsy is often caused by perinatal epileptogenic insults s... more Background: Neonatal drug-resistant epilepsy is often caused by perinatal epileptogenic insults such as stroke, ischemia, hemorrhage, and/or genetic defects. Rapid seizure control is particularly important for cognitive development. Since early surgical intervention and thus a short duration of epilepsy should lead to an optimal developmental outcome, we present our experience with hemispherotomy in an infant at the corrected age of 1 week.Methods: We report successful hemispherotomy for drug-resistant epilepsy in an infant with hemimegalencephaly at a corrected age of 1 week.Results: The infant was diagnosed with drug-resistant lesional epilepsy due to hemimegalencephaly affecting the left hemisphere. Given congruent electroclinical findings, we performed a left vertical parasagittal transventricular hemispherotomy after critical interdisciplinary discussion. No complications occurred during the surgery. Intraoperatively; 118 ml of red blood cells (30 ml/kg) and 80 ml of plasma wer...

Research paper thumbnail of Angulation towards coronal convexity measure and catheter length indication improves the quality of ventricular catheter placement – a smartphone-assisted guidance technique

World Neurosurgery, 2021

Accurate placement of a ventricular catheter is crucial to reduce the risk of shunt failure. In t... more Accurate placement of a ventricular catheter is crucial to reduce the risk of shunt failure. In the randomized, prospective, multicenter GAVCA trial, which evaluated the quality of ventricular catheter placement, the subgroup of patients with detailed length marked ventricular catheters (dVC) reflected a difference in the primary endpoint of optimal VC placement in contrast to the subgroup of patients with simplified length marked ventricular catheters (sVC). Objective of this analysis is to compare the dVC-group with the sVC-group as well as the smartphone-assisted guidance technique (GA) with the standard freehand technique (F) for catheter placement. We performed a further analysis of the GAVCA trial in two steps: 1st part) detailed length marked VCs (providing detailed distance from tip to base (3-13cm) in 0.5cm markings) compared to simplified length marked VCs with length indication at 5 cm and 10 cm from tip to base and 2nd part) comparing the smartphone-assisted guidance technique vs. freehand technique in the dVC-group. 1st part) Data of 137 patients (104 dVC patients vs. 33 sVC patients) was eligible for this analysis. Optimal VC placement was achieved in 72.1% of the dVC-group and 39.4% of the sVC-group (p=<0.001, odds ratio (OR) 3.9, 95% CI 1.7-9.3). 2nd part) The 104 dVC patients underwent a subgroup analysis concerning the catheter placement using different techniques for catheter placement (54 guidance technique (GA) vs. 50 freehand technique (F). Optimal catheter placement was achieved in 81.5% of the GA group and 62.0% of the F group (p=0.03, odds ratio 2.7, 95% CI 1.1-6.8). All patients who underwent guidance technique ventricular catheter placement (GA) were successful on primary puncture while for 8.7% of patients in the freehand group (F), multiple attempts were necessary (p=0.03). This analysis suggests the combination of a smartphone-assisted guidance technique (GA) and use of a detailed length marked ventricular catheter (dVC) to improve the rate of accurate ventricular catheter placement. Compared with freehand technique (F), patients' safety is increased by a reduction of unsuccessful ventricular catheter placement attempts.

Research paper thumbnail of The Influence of Perioperative Antibiotic Prophylaxis on Wound Infection and on the Colonization of Wound Drains in Patients After Correction of Craniosynostosis

Frontiers in Pediatrics, 2021

Objective: Evidence for the duration of perioperative antibiotic prophylaxis (PAP) after the corr... more Objective: Evidence for the duration of perioperative antibiotic prophylaxis (PAP) after the correction of craniosynostosis in children is scarce. We evaluated the necessary duration of PAP to ensure a minimal rate of postoperative wound infections.Methods: In this monocentric, retrospective, and prospective pilot study, two PAP protocols were compared. From August 2017 to May 2018, treatment group 1 (TG 1) was treated using the standard PAP protocol with at least three doses of antibiotics. Between May 2018 and March 2019, a shortened PAP with a single-shot administration was given to treatment group 2 (TG 2a and b). Endpoints of this study were wound infection rate, colonization rate of wound drains, and the course of treatment reflected by clinical and laboratory data.Results: A cohort of 187 children underwent craniosynostosis correction: 167 were treated according to protocols-−95 patients with at least three doses (TG 1) and 72 patients with a single-shot of cefuroxime (TG 2a)...

Research paper thumbnail of Pediatric thalamic incidentalomas: an international retrospective multicenter study

Journal of Neurosurgery: Pediatrics, 2021

OBJECTIVE Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain ... more OBJECTIVE Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain incidentalomas. Thalamic lesions have unique characteristics such as deep location, surgical complexity, and proximity to eloquent neuronal structures. Currently, the natural course of incidental thalamic lesions is unknown. Therefore, the authors present their experience in treating such lesions. METHODS A retrospective, international multicenter study was carried out in 8 tertiary pediatric centers from 5 countries. Patients were included if they had an incidental thalamic lesion suspected of being a tumor and were diagnosed before the age of 20 years. Treatment strategy, imaging characteristics, pathology, and the outcome of operated and unoperated cases were analyzed. RESULTS Overall, 58 children (23 females and 35 males) with a mean age of 10.8 ± 4.0 years were included. The two most common indications for imaging were nonspecific reasons (n = 19; e.g., research and developmental de...

Research paper thumbnail of Introduction to the annual issue on “Infant Hydrocephalus“

Child's Nervous System, 2021

Research paper thumbnail of Extended experience in parieto-occipital expansion surgery by meander technique—clinical and radiological evaluation

Child's Nervous System, 2021

Introduction Brachycephaly and anterior and posterior plagiocephaly appear as an isolated entity ... more Introduction Brachycephaly and anterior and posterior plagiocephaly appear as an isolated entity or manifest in syndromic conditions. In severe cases, possible treatment options currently comprise either cranioplasty or osteogenetic distraction. The aim of this paper is to retrospectively review the perioperative course of a series of children treated by posterior meander expansion technique at our institution with focus on the course of postoperative intracranial volume and eventual tonsillar descent evolution. Methods Forty-two children received a posterior cranial vault remodeling by means of a posterior meander technique during a 7-year period. Hospital records were reviewed, and pre- and postoperative MRIs were analyzed for intracranial volume, cephalic and asymmetry index, and tonsillar position over time. Results Median age at surgery was 11.5 months (range 17 days–10 years). Nineteen children had a symmetrical cranial deformity, twenty-three an asymmetrical synostosis. Half ...

Research paper thumbnail of Molecular characterisation of sporadic endolymphatic sac tumours and comparison to von Hippel–Lindau disease‐related tumours

Neuropathology and Applied Neurobiology, 2021

This is an open access article under the terms of the Creat ive Commo ns Attri butio n-NonCo mmer... more This is an open access article under the terms of the Creat ive Commo ns Attri butio n-NonCo mmerc ial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Research paper thumbnail of Surgical management of intracranial arachnoid cysts in pediatric patients: radiological and clinical outcome

Journal of Neurosurgery: Pediatrics, 2021

OBJECTIVE Congenital intracranial cysts account for a significant portion of intracranial lesions... more OBJECTIVE Congenital intracranial cysts account for a significant portion of intracranial lesions in the pediatric population. The efficiency of surgical treatment in a pediatric cohort with intracranial arachnoid cysts (ACs) at different locations regarding clinical symptoms and mass effect was evaluated. METHODS A retrospective study of all children who underwent surgical treatment of an intracranial AC during an 11-year period (2007–2018) was performed. Demographics, clinical symptoms, and radiological cyst size pre- and postoperatively, as well as the reoperation rate and possible treatment complications, were analyzed. RESULTS A total of 116 intracranial cysts at 8 different anatomical locations were surgically treated in 113 children (median age 5 years and 10 months) predominantly by endoscopic technique (84%). The complication rate was 3%, and the reoperation rate was 16%. Preoperative cyst enlargement was significantly more common in infants (p < 0.0001), as was the need...

Research paper thumbnail of Quantitative and qualitative comparison of morphometric outcomes after endoscopic and conventional correction of sagittal and metopic craniosynostosis versus control groups

Neurosurgical Focus, 2021

OBJECTIVE Surgical correction for sagittal and metopic craniosynostosis (SCS and MCS) aims to alt... more OBJECTIVE Surgical correction for sagittal and metopic craniosynostosis (SCS and MCS) aims to alter the abnormal cranial shape to resemble that of the normal population. The achieved correction can be assessed by morphometric parameters. The purpose of the presented study was to compare craniometric parameters of control groups to those same parameters after endoscopic and conventional (open) correction. METHODS The authors identified 4 groups of children undergoing surgical treatment for either SCS or MCS, with either endoscopic (SCS, n = 17; MCS, n = 16) or conventional (SCS, n = 29; MCS, n = 18) correction. In addition, normal control groups of nonaffected children who were 6 (n = 30) and 24 (n = 18) months old were evaluated. For all groups, several craniometric indices calculated from 3D photographs were compared for quantitative analysis. For qualitative comparison, averages of all 3D photographs were generated for all groups and superimposed to visualize relative changes. RES...

Research paper thumbnail of Seizures following surgery for supratentorial extratemporal low-grade tumors in children: a multicenter retrospective study

Journal of Neurosurgery: Pediatrics, 2020

OBJECTIVEResection of brain tumors may lead to new-onset seizures but may also reduce seizure rat... more OBJECTIVEResection of brain tumors may lead to new-onset seizures but may also reduce seizure rates in patients presenting with seizures. Seizures are seen at presentation in about 24% of patients with brain tumors. For lesional epilepsy in general, early resection is associated with improved seizure control. However, the literature is limited regarding the occurrence of new-onset postoperative seizures, or rates of seizure control in those presenting with seizures, following resections of extratemporal low-grade gliomas (LGGs) in children.METHODSData were collected retrospectively from 4 large tertiary centers for children (< 18 years of age) who underwent resection of a supratentorial extratemporal (STET) LGG. The patients were divided into 4 groups based on preoperative seizure history: no seizures, up to 2 seizures, more than 2 seizures, and uncontrolled or refractory epilepsy. The authors analyzed the postoperative occurrence of seizures and the need for antiepileptic drugs ...

Research paper thumbnail of Management of pineal region tumors in a pediatric case series

Neurosurgical Review, 2020

Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous h... more Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We evaluated our surgical experience concerning procurance of a histological diagnosis, management of hydrocephalus, and choice of antitumoral treatment. We analyzed the efficacy of neuroendoscopic biopsy and endoscopic third ventriculocisternostomy (ETV) in patients with pineal region tumors between 2006 and 2019 in a single-center retrospective cross-sectional study with regard to diagnostic yield, hydrocephalus treatment, as well as impact on further antitumoral management. Out of 28 identified patients, 23 patients presented with untreated hydrocephalus and 25 without histological diagnosis. One patient underwent open biopsy, and 24 received a neuroendoscopic biopsy with concomitant hydrocephalus treatment if necessary. Eighteen primary ETVs, 2 secondary ETVs, and 2 ventriculoperitoneal shunts (VPSs) were performed. Endosco...

Research paper thumbnail of Prognostic impact of distinct genetic entities in pediatric diffuse glioma WHO-grade II - report from the German/Swiss SIOP-LGG 2004 cohort

International Journal of Cancer, 2020

Reports on pediatric low-grade diffuse glioma WHO-grade II (DG2) suggest an impaired survival rat... more Reports on pediatric low-grade diffuse glioma WHO-grade II (DG2) suggest an impaired survival rate, but lack conclusive results for genetically defined DG2-entities. We analyzed the natural history, treatment and prognosis of DG2 and investigated which genetically defined sub-entities proved unfavorable for survival. Within the prospectively registered, population-based German/Swiss SIOP-LGG 2004 cohort 100 patients (age 0.8-17.8 years, 4% neurofibromatosis [NF1]) were diagnosed with a

Research paper thumbnail of Cranial and ventricular size following shunting or endoscopic third ventriculostomy (ETV) in infants with aqueductal stenosis: further insights from the International Infant Hydrocephalus Study (IIHS)

Child's Nervous System, 2020

Purpose The craniometrics of head circumference (HC) and ventricular size are part of the clinica... more Purpose The craniometrics of head circumference (HC) and ventricular size are part of the clinical assessment of infants with hydrocephalus and are often utilized in conjunction with other clinical and radiological parameters to determine the success of treatment. We aimed to assess the effect of endoscopic third ventriculostomy (ETV) and shunting on craniometric measurements during the follow-up of a cohort of infants with symptomatic triventricular hydrocephalus secondary to aqueductal stenosis. Methods We performed a post hoc analysis of data from the International Infant Hydrocephalus Study (IIHS)-a prospective, multicenter study of infants (< 24 months old) with hydrocephalus from aqueductal stenosis who were treated with either an ETV or shunt. During various stages of a 5-year follow-up period, the following craniometrics were measured: HC, HC centile, HC zscore, and frontal-occipital horn ratio (FOR). Data were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and sex. Results Of 158 enrolled patients, 115 underwent an ETV, while 43 received a shunt. Both procedures led to improvements in the mean HC centile position and z-score, a trend which continued until the 5-year assessment point. A similar trend was noted for FOR which was measured at 12 months and 3 years following initial treatment. Although the values were consistently higher for ETV compared with shunt, the differences in HC value, centile, and z-score were not significant. ETV was associated with a significantly higher FOR compared with shunting at 12 months (0.52 vs 0.44; p = 0.002) and 3 years (0.46 vs 0.38; p = 0.03) of follow-up.

Research paper thumbnail of Dynamic cerebellar herniation in Chiari patients during the cardiac cycle evaluated by dynamic magnetic resonance imaging

Neuroradiology, 2019

PurposeCerebellar herniation in Chiari patients can be dynamic, following the cerebrospinal fluid... more PurposeCerebellar herniation in Chiari patients can be dynamic, following the cerebrospinal fluid pulsatility during the cardiac cycle. We present a voxel intensity distribution method (VIDM) to automatically extract the pulsatility-dependent herniation in time-resolved MRI (CINE MRI) and compare it to the simple linear measurements. The degree of herniation is furthermore compared on CINE and static sequences, and the cerebellar movement is correlated to the presence of hydrocephalus and syringomyelia.MethodsThe cerebellar movement in 27 Chiari patients is analyzed with VIDM and the results were compared to linear measurements on an image viewer (visual inspection, VI) using a paired t test. Second, an ANOVA test is applied to compare the degree of herniation on static 3D MRI and CINE. Finally, the Pearson’s correlation coefficient is calculated for the correlation between cerebellar movement and the presence of hydrocephalus and syringomyelia.ResultsVIDM showed significant movement in 85% of our patients. Assuming that movement < 1 mm cannot be detected reliably on an image viewer, VI identified movement in 29.6% of the patients (p = 0.002). The herniation was greater on static sequences than on CINE in most cases, but this was not statistically significant. The cerebellar movement was not correlated with hydrocephalus or syringomyelia (Pearson’s coefficient < 0.3).ConclusionsVIDM is a sensitive method to detect tissue movement on CINE MRI and could be used for Chiari patients, but also for the evaluation of cyst membranes, ventriculostomies, etc. The cerebellar movement appears not to correlate with hydrocephalus and syringomyelia in Chiari patients.