Martin U Schuhmann

University of Tuebingen | EKU Tübingen · Department of Neurosurgery

10044 Background: 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...

Chronic pleural cerebrospinal fluid (CSF) effusion is a rare complication after ventriculoperitoneal (VP) shunt insertion and only 18 cases in children and adults have been described so far without catheter dislocation to the intrathoracic cavity. We report on a 4-year-old girl with a complex history of underlying neurogenetic disorder, a hypoxic i...

Background Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive CNS tumors of infancy and early childhood. Hallmark is the surprisingly simple genome with inactivating mutations or deletions in the SMARCB1 gene as the oncogenic driver. Nevertheless, AT/RTs are infiltrated by immune cells and even clonally expanded T cells. However, it is...

Aims An ependymoma shows divergent morphological and molecular features depending on their location. The paired box 6 ( PAX6 ) transcription factor is a putative tumour suppressor and drives cancer cells towards a stem cell-like state. A transcriptome study reported high PAX6 expression in ependymal tumours, but data on protein expression are lacki...

A bstract Atypical teratoid/rhabdoid tumors (AT/RT) are highly aggressive CNS-tumors of infancy and early childhood. Hallmark is the surprisingly simple genome with inactivating mutations or deletions in the SMARCB1 gene as the oncogenic driver. Nevertheless, AT/RTs are infiltrated by immune cells and even clonally expanded T cells. However, it is...

Background: Frame-based stereotactic procedures are still the gold standard in neurosurgery. However, there is an increasing interest in robot-assisted technologies. Introducing these increasingly complex tools in the clinical setting raises the question about the time efficiency of the system and the essential learning curve of the surgeon. Meth...

Background: In the treatment of childhood hydrocephalus, 3D volumetry seems to have many advantages over classical planar index measurements for dedicated monitoring of changes in cerebrospinal fluid and brain volume. Nevertheless, this method requires extensive technical effort and access to the complete three-dimensional data set. Against this b...

Background: Neurofibroma/schwannoma hybrid nerve sheath tumors (N/S HNSTs) are neoplasms associated with larger nerves that occur sporadically and in the context of schwannomatosis or neurofibromatosis type 2 or 1. Clinical management of N/S HNST is challenging, especially for large tumors, and established systemic treatments are lacking. Methods...

Tumors of the pineal region comprise several different entities with distinct clinical and histopathological features. Whereas some entities predominantly affect adults, pineoblastoma (PB) constitutes a highly aggressive malignancy of childhood with a poor outcome. PBs mainly arise sporadically, but may also occur in the context of cancer predispos...

Purpose It is assumed that the width of the optic nerve sheath diameter (ONSD) is dependent on intracranial pressure (ICP) and pulsatility and thus constitutes a non-invasively accessible “window” for qualitative assessment of ICP. Data on the correlation to invasively measured ICP in children are scarce and have often been obtained from sedated pa...

Purpose Secondary craniostenosis is a relevant problem pediatric neurosurgeons are confronted with and poses challenges regarding reliable diagnosis of raised ICP, especially in case of absent or questionable papilledema. How to identify children with elevated ICP is still controversial and diagnostics vary. We report on our experience with compute...

Background Hydrocephalus shunt malfunction can—also in children—occur insidiously without clear symptoms of raised intracranial pressure (ICP) or changes in ventricular size, imposing a diagnostic challenge. Computerized shunt infusion studies enable quantitative shunt function assessment. We report on feasibility and results of this technique in c...

Decision-making in pediatric neurosurgery routinely involves assessing intracranial pressure (ICP). The level of injury and severity of disease play an important role in determining which method of ICP measurement is preferred. Invasive and non-invasive techniques have described indications, advantages and shortcomings. This collection of papers fr...

Purpose Previous studies correlating ultrasound (US)-based optic nerve sheath diameter (ONSD) and intracranial pressure (ICP) in children were performed under general anesthesia. To apply ONSD in daily clinical routine, it is necessary to investigate patients awake. It is furthermore essential for ICP-assessment with ONSD to know if ONSD-ICP correl...

Background In childhood hydrocephalus, both the amount of cerebrospinal fluid and the brain volume are relevant for the prognosis of the development and for therapy monitoring. Since classical planar measurements of ventricular size are subject to strong limitations, imprecise and neglect brain volume, 3D volumetry is most desirable. We used and ev...

Aims: DNA methylation-based central nervous system (CNS) tumour classification has identified numerous molecularly-distinct tumour types, and clinically relevant subgroups among known CNS tumour entities that were previously thought to represent homogeneous diseases. Our study aimed at characterising a novel, molecularly-defined variant of glioneu...

Introduction In a retrospective magnetic resonance imaging (MRI)-based study, we showed that changes of the third ventricle diameter (TVD) are a reliable mirror of changes of the entire ventricular system. The third ventricle is easily accessible in more than 90% of children and adults using ultrasound (US) via the transtemporal bone-window; thus i...

Background: Intracardial migration of a ventriculoperitoneal (VP) shunt is a rare complication and it has been described in only single case reports. Herein, we report the successful interdisciplinary management of an intracardial migration of a VP shunt and provide a review of the literature. Case description: A 38-year-old-patient with shunt-d...

Hearing-preserving partial resection of neurofibromatosis type 2 (NF2) associated vestibular schwannomas (VS) is a preferred treatment strategy, particularly for children and adolescents. However, the residual tumors do grow and lead at some point to continued hearing deterioration. An adjuvant bevacizumab treatment may provide an option for slowin...

Unilateral sensorineural hearing loss is a common symptom of vestibular schwannomas in adolescent patients with neurofibromatosis type 2 or sporadic vestibular schwannomas and is often the initial clinical feature. While rare cases of sensorineural impairment presenting as vision or hearing loss due to metastatic medulloblastoma are known, hearing...

Hearing preservation is a major goal in the treatment of neurofibromatosis type 2 (NF2) associated vestibular schwannoma (VS), particularly in children and adolescents. In this study, we retrospectively reviewed hearing and volumetry data sets of 39 operated tumors (ears) in 23 patients under the age of 25 and in a follow-up period of 21 to 167 mon...

Objective: The authors' aim was to evaluate the tumor volume and growth rate of neurofibromatosis type 2 (NF2)-associated vestibular schwannomas (VSs) and the clinical factors or type of mutations before and after surgery in children and adults younger than 25 years at the time of diagnosis. Methods: A total of 579 volumetric measurements were p...

Abstract Acetylsalicylic acid has been linked to a lower risk for different cancer types, presumably through its inhibitory effect on cyclooxygenase 2. This has also been investigated in vestibular schwannomas with promising results suggesting an antiproliferative effect and recently the intake has been recommended for vestibular schwannomas as a c...

Background: Medulloblastoma is the most common malignant brain tumor in childhood and adolescence. Although some patients present with distinct genetic alterations, such as mutated TP53 or MYC amplification, pediatric medulloblastoma is a tumor entity with minimal mutational load and low immunogenicity. Methods: We identified tumor-specific muta...

As an essential pillar of today’s cancer treatment, radiation therapy has led to improved survival rates of patients with childhood malignancies including leukemia and central nervous system (CNS) tumors. However, long-term complications such as radiation-induced malignancies occur in a subset of patients following radiation therapy, especially obs...

Purpose Sporadic mononeuropathies without trauma or compression are challenging to diagnose. Nerve ultrasound has recently proven its usefulness in the diagnosis of traumatic neuropathies, tumors and polyneuropathies. However, its role in mononeuropathies currently remains unclear. We describe ultrasonography follow-up results in 12 patients with s...

Objectives: The transition from single-ventricle lesions with surgically placed systemic-to-pulmonary artery shunt to the circulation following a bidirectional cavopulmonary connection results in higher pressure in the superior vena cava when compared with the preceding circulation. The aim of this study was to evaluate the impact of this transiti...

Background: Craniopharyngioma is a tumor of low histological malignancy resulting from an anomaly of embryonic development. Affected children and adolescents are being studied with respect to their quality of life, progression-free survival, and overall survival in the framework of the ongoing KRANIOPHARYNGEOM 2007 project. Methods: This prospec...

Purpose: Regular measurement of ventricular size is important in children with hydrocephalus. After closure of the fontanelle this is currently addressed by repetitive cranial MRI or CT imaging, coming along with risks of anaesthesia or radiation. As the third ventricle is accessible via the temporal bone window using ultrasound, determination of...

Low grade gliomas (LGGs) constitute the largest, yet clinically and (molecular-) histologically heterogeneous group of pediatric brain tumors of WHO grades I and II occurring throughout all pediatric age groups and at all central nervous system (CNS) sites. The tumors are characterized by a slow growth rate and may show periods of growth arrest. Ar...

Purpose: Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive paediatric brain tumour with fatal outcome. The Individualised Therapy For Relapsed Malignancies In Childhood (INFORM) registry study offers comprehensive molecular profiling of high-risk tumours to identify target alterations for potential precision therapy. We analysed molec...

Tumors arising in the pineal region comprise a spectrum of different entities with distinct clinical and histopathological characteristics. Pineocytoma (PC), pineal parenchymal tumors of intermediate differentiation (PPTID) and papillary tumors of the pineal region (PTPR) mainly occur in adult patients and are low to moderately aggressive neoplasms...

Abstract: Purpose: Diffuse midline gliomas, H3 K27M-mutant were introduced as a new grade IV entity in WHO classification of tumors 2016. These tumors occur often in pediatric patients and show an adverse prognosis with a median survival less than a year. Most of the studies on these tumors, previously known as pediatric diffuse intrinsic pontine...

Purpose To demonstrate the clinical use of FDG-PET/MRI for monitoring enlargement and metabolism of plexiform neurofibromas (PNF) in patients with neurofibromatosis type 1 (NF1), in whom the development of a malignant peripheral nerve sheath tumor (MPNST) is often a life limiting event. Methods NF1 patients who underwent a simultaneous FDG-PET/MRI...

Introduction External hydrocephalus (eHC) is commonly defined as a subtype of infant “hydrocephalus” consisting of macrocepahly associated with enlarged subarachnoid space and no or mild ventriculomegaly. This status is thought to be related to impaired CSF absorption because of arachnoid villi immaturity. However, other factors like the venous sys...

Purpose: Pilocytic astrocytoma is the most common childhood brain tumor, characterized by constitutive MAPK activation. MAPK signaling induces oncogene-induced senescence (OIS), which may cause unpredictable growth behavior of pilocytic astrocytomas. The senescence-associated secretory phenotype (SASP) has been shown to regulate OIS, but its role i...

Optimizing oxygen delivery to the brain is one of the main goals in children with congenital heart defects after surgery. It has been shown that cerebral oxygen saturation (cSO2) is depressed within the first day after neonatal cardiopulmonary bypass surgery. However, peri-operative cerebral oxygen metabolism has not yet been assessed in previous s...

Purpose Presurgical functional MRI (fMRI) and diffusion MRI tractography (dMRI tractography) are widely employed to delineate eloquent brain regions and their connections prior to brain tumor resection in adults. However, such studies are harder to perform in children, resulting in suboptimal neurosurgical care in pediatric brain tumor surgery as c...

Table S1. Summary of some general recommendations re: data processing for diffusion MR tractography, functional MRI, or both, in a clinical context in children.

Background and Purpose: Neurofibromatosis Type 2 (NF2) is an autosomal-dominant tumor-prone disorder characterized by the manifestations of central nervous system lesions. However, the first clinical signs of disease are often non-tumorous. Cerebrovascular insults are known in NF2, however, not yet described as first symptom in young NF2 patients....

Objective: The aim of this study is to present the clinical experience of two neurosurgical centers with the use of a 2-micron continuous wave laser (2μ-cwL) system as standard tool in neuroendoscopic procedures and to discuss the safety and efficacy of this system. Methods: 469 patients underwent neuroendoscopic procedures using 2μ-cwL between...

The application of both functional MRI and diffusion MR tractography prior to a neurosurgical operation is well established in adults, but less so in children, for several reasons. For this review, we have identified several aspects (task design, subject preparation, actual scanning session, data processing, interpretation of results, and decision‐...

Diffuse leptomeningeal glioneuronal tumors (DLGNT) represent rare CNS neoplasms which have been included in the 2016 update of the WHO classification. The wide spectrum of histopathological and radiological features can make this enigmatic tumor entity difficult to diagnose. In recent years, large-scale genomic and epigenomic analyses have afforded...

Paediatric CNS tumours are the most common cause of childhood cancer-related morbidity and mortality, and improvements in their diagnosis and treatment are needed. New genetic and epigenetic information about paediatric CNS tumours is transforming the field dramatically. For most paediatric CNS tumour entities, subgroups with distinct biological ch...

Background: The classical dura opening for midline posterior fossa tumors in and around the 4th ventricle and the craniocervical junction is a Y-shaped incision. Several potential problems are associated with this technique. We used a technical variant, the U-shaped durotomy, previously described by Rhoton, offering several advantages. We report o...

Atypical teratoid/rhabdoid tumors (AT/RTs) are commonly regarded as immunologically cold tumors, as they rank among malignancies with the lowest mutational load. However, low mutational burden is not necessarily correlated with poor immunogenicity, as tumor-exclusive peptides bound in the tumor cell’s MHC represent potential targets for immune resp...

Diffuse leptomeningeal glioneuronal tumors (DLGNT) represent a rare CNS neoplasm included in the latest WHO classification update from 2016. The wide spectrum of histological and radiological features can make this enigmatic tumor entity difficult to diagnose. In recent years, large-scale genomic and epigenomic analyses have provided insight into k...

The main challenge in the clinical management of pilocytic astrocytoma (PA) is its unpredictable growth behavior. PA cells are driven into oncogene-induced senescence (OIS) by aberrant MAPK activation. In other senescence models OIS was shown to be regulated and maintained by the senescence-associated secretory phenotype (SASP). In this study, the...

Conclusion: A symptomatic FLH of the median nerve is rare in children below the age of 5 years but has to be kept in mind as differential diagnosis in case of wrist and/or palm swelling, macrodactyly, and pain in hand or forearm. MRI is diagnostic, with very characteristic features, which can also be identified in high-resolution nerve ultrasound....

Bei den Phakomatosen handelt es sich um eine Gruppe von Erkrankungen, bei denen kutane und ZNS-Manifestationen gemeinsam vorkommen. Sie werden deswegen oft auch als neurokutane Syndrome bezeichnet. Von kinderneurochirurgischer Relevanz sind die Tumormanifestationen im Rahmen der Neurofibromatose Typ 1 und Typ 2 sowie bei der tuberösen Sklerose. Die...

Periphere Neurotomien zur Behandlung von Spastizität wurden in Deutschland zu Beginn des 20. Jahrhunderts erstmals eingesetzt, ähnlich wie die dorsale Rhizotomie (damals als noch unselektives Verfahren). Durch die mikrochirurgische Dissektion des Nerven in einzelne Faszikel und die Anwendung von elektrophysiologischer Stimulation ist das Verfahren...

Der kindliche Hydrozephalus ist pathophysiologisch vom Erwachsenenhydrozephalus nicht nur in Bezug auf seine Ätiologie, sondern auch in Bezug auf die wirksamen pathophysiologischen Mechanismen so unterschiedlich, dass er grundsätzlich getrennt betrachtet werden sollte. Eine alles vereinende schlüssige pathophysiologische Hypothese ist ebenso fehlen...

Das intensivmedizinische Management von Kindern mit schwerem Schädel-Hirn-Trauma ist nur in enger Kooperation und Abstimmung von Kinderneurochirurgie und pädiatrischer Intensivmedizin möglich. Aufgabe der Kinderneurochirurgie darf dabei nicht nur die Anlage von Hirndrucksonden, Entfernung von raumfordernden Hämatomen und ggf. Dekompressionskraniekt...

Introduction: Chronic overdrainage affects shunted patients producing a variety of symptoms that may be misdiagnosed. The best known symptoms are so-called shunt-related headaches. There is mounting evidence that changes in cerebrospinal venous system dynamics are a key factor to the pathophysiology of chronic overdrainage syndrome. Clinical pres...

Purpose: Ventriculo-peritoneal (VP) shunts are effective for treatment of hydrocephalus in all age groups; however, they are associated with complications, a common one being ventricular catheter (VC) obstruction. VC position is likely to influence VC survival; however, most VCs are positioned freehand without guidance. This paper describes the ac...

Background: Many radiological signs are known for the diagnosis of idiopathic normal pressure hydrocephalus (iNPH). However, there is little information about these signs in the pre-symptomatic phase. For pathophysiological investigative purposes we conducted a descriptive image analysis study on pre-symptomatic patients. Methods: Patients that...

Background: The flow pattern of the cerebrospinal fluid is probably the most important factor related to obstruction of ventricular catheters during the normal treatment of hydrocephalus. To better comprehend the flow pattern, we have carried out a parametric study via numerical models of ventricular catheters. In previous studies, the flow was st...

Objective: Although infants following major surgery frequently require RBC transfusions, there is still controversy concerning the best definition for requirement of transfusion in the individual patient. The aim of this study was to determine the impact of RBC transfusion on cerebral oxygen metabolism in noncardiac and cardiac postsurgical infant...

Objective: The aim of this study is to assess visually the impact of duration and intensity of cerebrovascular autoregulation insults on 6-month neurological outcome in severe traumatic brain injury. Material and methods: Retrospective analysis of prospectively collected minute-by-minute intracranial pressure (ICP) and mean arterial blood pressu...

Objective: A drawback in the use of an external ventricular drain (EVD) originates in the fact that draining cerebrospinal fluid (CSF) (open system) and intracranial pressure (ICP) monitoring can be done at the same time but is considered to be unreliable regarding the ICP trace. Furthermore, with the more widespread use of autoregulation monitori...

Pilocytic astrocytoma (PA) is the most frequent pediatric brain tumor. This single-pathway disease exhibits aberrant activation of the MAPK pathway driving the tumor into oncogene-induced senescence (OIS). OIS is proposed to be the source of the unstable however benign growth observed in PA patients. Senescence is thought to be regulated by the sen...

Background: It could be shown in traumatic brain injury (TBI) in adults that the functional status of cerebrovascular autoregulation (AR), determined by the pressure reactivity index (PRx), correlates to and even predicts outcome. We investigated PRx, cerebral perfusion pressure (CPP) and intracranial pressure (ICP) and their correlation to outcom...