Bassel Zebian

• MBBS, BSc (Hons), MRCS (Eng), FRCS (Neuro.Surg)
• Consultant at King's College Hospital NHS Foundation Trust

Purpose High-grade gliomas (HGG) occur in any central nervous system location and at any age. HGGs in teenagers/young adults (TYA) are understudied. This project aimed to characterize these tumors to support accurate stratification of patients. Experimental Design 207 histone/IDH wild-type tumors from patients aged 13 to 30 years were collected. D...

Introduction: We describe four cases of open spina bifida initially presumed to be "closed" defects in which serial prenatal ultrasound examinations documented the occurrence of sac rupture and development of Chiari II malformation later in the pregnancy. Case presentation: In each case, the ultrasound examinations starting from 19-24 weeks' ges...

Malignant tumours arising at the site of closed neural tube defects are extremely rare and have only been described three times in the literature. We report the case of a child with a congenital lumbosacral lipoma, a dermal sinus tract and low-lying spinal cord who was initially treated non-operatively due in part to non-attendance and who then pre...

BACKGROUND AND OBJECTIVES Despite growing interest in neuroendoscopy, endoscopic resection of intraventricular and paraventricular brain tumors is still hindered by a lack of appropriate instrumentation. The Söring ultrasonic aspirator holds significant promise but is limited because of its original design allowing sole use with the GAAB® endoscope...

Minimally invasive parafascicular surgery (MIPS) with the use of tubular retractors achieve a safe resection in deep seated tumours. Diffusion changes noted on postoperative imaging; the significance and clinical correlation of this remains poorly understood. Single centre retrospective cohort study of neuro-oncology patients undergoing MIPS. The i...

Histone mutations (H3 K27M, H3 G34R/V) are molecular features defining subtypes of paediatric-type diffuse high-grade gliomas (HGG) (diffuse midline glioma (DMG), H3 K27-altered, diffuse hemispheric glioma (DHG), H3 G34-mutant). The WHO classification recognises in exceptional cases, these mutations co-occur. We report one such case of a 2-year-old...

BACKGROUND Bithalamic gliomas (BTG) are rare brain tumors with dismal prognosis. Optimal management and role of novel therapies have not been established yet. This study aimed to improve our understanding of BTG to guide adapted treatments. METHODS Retrospective case series across 6 Pediatric Neuro-Oncology Units (United Kingdom, Spain) of ≤18-yea...

BACKGROUND Histone mutations (H3 K27M, H3 G34R/V) define subtypes of paediatric-type diffuse high-grade gliomas (HGG) (diffuse midline glioma (DMG), H3 K27-altered, diffuse hemispheric glioma (DHG), H3 G34-mutant). The WHO classification recognises exceptional cases where these mutations co-occur. We report one such case of a 2-year-old female pres...

BACKGROUND High-grade gliomas (HGG) arise in any CNS location with a poor prognosis. HGGs in teenagers/young adults (TYA) are understudied; this project aimed to characterise these tumours and identify therapeutic targets. METHODS HGG samples (histone/IDH-wildtype, n=207, FFPE/FF, 13-30 years) were collected from national/international collaborato...

Purpose To this day there is no consensus regarding evidence of usefulness of Intraoperative Neurophysiological Monitoring (IONM). Randomized controlled trials have not been performed in the past mainly because of difficulties in recruitment control subjects. In this study, we propose the use of Bayesian Networks to assess evidence in IONM. Method...

Non-germinomatous germ cell tumours of the central nervous system (CNS NG-GCT) have no standardized treatment at relapse and prognosis is dismal. Additionally, limited patient numbers preclude any clinical trials in this setting. Here we report the case of an adolescent with relapsed metastatic pineal yolk sac tumour treated with induction chemothe...

CSF-venous fistulas (CVFs) are increasingly recognised as a cause of spontaneous intracranial hypotension. They may present atypically including with brain sagging pseudo-dementia. Cervical CVFs are rare and their management can be difficult due to associated eloquent nerve roots. We report the case of a 49-year-old woman who presented with cogniti...

Objective CSF dynamics and their disorders are at the heart of understanding and managing neurosurgical conditions. Routine imaging protocols have so far fallen short of providing an accurate assessment of CSF flow. We present our 9-year experience of using T2-weighted 3D spin echo sequences with variable flip angle (Cube/SPACE) to demonstrate how...

Pediatric diencephalic tumors represent a histopathologically and molecularly diverse group of neoplasms arising in the central part of the brain and involving eloquent structures, including the hypothalamic-pituitary axis (HPA), optic pathway, thalamus, and pineal gland. Presenting symptoms can include significant neurological, endocrine, or visua...

AIMS Whole genome sequencing is currently being offered to paediatric, teenage and young adult neuro-oncology patients and this was being managed by a genomics practitioner who works cross-site. We found that some patients are being discharged from the hospital without 1 component of the test or the consent. Our aim is to create a solid pathway in...

AIMS The aim of this review is to analyse the need for a specialist nurse role for the TYA patients. Having a new dedicated TYA developmental role implemented can help by identifying areas for improvement from the wider team to ensure optimal support for patient group. METHOD Clinic attendance over a 1-year period has been measured. Looking specif...

Introduction Germinal matrix / intraventricular haemorrhage (GMIVH) remains a significant complication of prematurity. The more severe grades are associated with parenchymal haemorrhagic infarction (PHI) and hydrocephalus. A temporising procedure is usually the first line in management of neonatal post-haemorrhagic hydrocephalus (nPHH) as the risk...

Although rare, craniopharyngiomas constitute up to 80% of tumours in the hypothalamic-pituitary region in childhood. Despite being benign, the close proximity of these tumours to the visual pathways, hypothalamus, and pituitary gland means that both treatment of the tumour and the tumour itself can cause pronounced long-term neuroendocrine morbidit...

Purpose To this day there is no consensus regarding evidence of usefulness of Intraoperative Neurophysiological Monitoring (IONM). Randomized controlled trials have not been performed in the past mainly because of difficulties in recruitment control subjects. In this study, we propose the use of Bayesian Networks to assess evidence in IONM. Methods...

3D T2-weighted spin echo sequences with variable flip angle allow excellent visualisation of CSF flow, at least equivalent to phase contrast CINE-MRI studies. • They have the added advantage of being suitable for direct upload to neuronavigation systems. • Promising results from our in vitro studies suggest that we may be able to use them to better...

Background Diffuse hemispheric glioma, H3 G34-mutant, is a novel paediatric tumour type in the fifth edition of the WHO classification of CNS tumours associated with an invariably poor outcome. We present a comprehensive clinical, imaging and pathological review of this entity. Methods Patients with confirmed H3 G34R-mutant high-grade glioma were...

The massa intermedia (MI) or interthalamic adhesion (ITA) is a band of tissue connecting the medial surfaces of the thalami and is present in the majority of healthy individuals. Its enlargement as well as its absence have been associated with some pathological states. We describe the first case report of a 3-year-old child presenting with obstruct...

Understanding CSF flow dynamics on imaging is essential for management of many neurosurgical conditions. We have 8 years' experience using T2-weighted 3D spin echo sequences with variable flip angle (SPACE/Cube) coupled with high resolution 3D-balanced steady-state free precession sequences (CISS/FIESTA). Areas of CSF flow appear as regions of sign...

Letter to the editor

Infected cephalhaematomas are rare and can lead to complications such as sepsis, meningitis and osteomyelitis. We present an infected cephalhaematoma in a neonate with resultant underlying osteomyelitis and a review of the literature. Our patient presented 6 days following birth with a fever and a swelling consistent with cephalhaematoma. He was ma...

The survival of children with diffuse intrinsic pontine glioma (DIPG) remains dismal, with new treatments desperately needed. In a prospective biopsy-stratified clinical trial, we combined detailed molecular profiling and drug screening in newly established patient-derived models in vitro and in vivo. We identified in vitro sensitivity to MEK inhib...

Somatic mutations in ACVR1 are found in a quarter of children with diffuse intrinsic pontine glioma (DIPG), but there are no ACVR1 inhibitors licensed for the disease. Using an artificial intelligence–based platform to search for approved compounds for ACVR1-mutant DIPG, the combination of vandetanib and everolimus was identified as a possible ther...

High grade gliomas (HGG) have a dismal prognosis with survival rates of 15-35%. Approximately 10-12% of pediatric HGG occur in young children and their molecular biology and clinical outcomes differ from those arising at older ages. We report on four children aged <5 years newly diagnosed with non-brainstem HGG between 2011 and 2018 who were treate...

Abstract Objective: To describe the endoscopic management of a septum pellucidum cavernoma. Design A case study of a 16-year female who presented with a septal haematoma whose repeat imaging demonstrated an underlying lesion most in keeping with a cavernoma. Methods All the patient data including imaging were collected from hospital records. The...

Objectives Having previously published some of our initiatives to increase exposure to neurosurgery at the undergraduate level as well as their positive impact, we set out to assess the impact of a neurosurgical shadowing scheme open to medical students. We assessed the efficacy of the scheme at harnessing the medical students’ interest in the spec...

Introduction and objectives The novel severe acute respiratory syndrome coronavirus 2 (COVID-19) pandemic has had drastic effects on global healthcare with the UK amongst the countries most severely impacted. The aim of this study was to examine how COVID-19 challenged the neurosurgical delivery of care in a busy tertiary unit serving a socio-econo...

Paediatric high grade glioma and diffuse midline glioma (including DIPG) are comprised of multiple biological and clinical subgroups, the majority of which urgently require novel therapies. Patient-derived in vitro primary cell cultures represent potentially useful tools for mechanistic and preclinical investigation based upon their retention of ke...

Objectives IgG4-related hypophysitis is a novel clinical disease entity, which is typically seen in the sixth decade of life and is typically complicated by hypopituitarism. We describe an adolescent female with IgG4-related hypophysitis with normal pituitary function and summarize the relevant literature. Case presentation A 11.8-year-old girl pr...

Background Neurocysticercosis is a helminthic infestation of the brain by the encysted larval form of the human tape worm Taenia Solium. It is a common cause of focal epilepsy in countries with inadequate sanitation following oro-faecal transmission and ingestion of food contaminated with TaeniaSolium eggs. The larvae can lodge in different parts o...

Little is known about pseudoprogression in brain tumours other than gliomas. A 9‐year‐old male child with a pineal teratoma/germinoma underwent surgical resection followed by adjuvant chemo‐radiotherapy. The magnetic resonance imaging scan 4 months post‐radiotherapy showed a contrast‐enhancing lesion within the surgical cavity suspicious of recurre...

Infant high-grade gliomas appear clinically distinct from their counterparts in older children, indicating that histopathologic grading may not accurately reflect the biology of these tumors. We have collected 241 cases under 4 years of age, and carried out histologic review, methylation profiling, and custom panel, genome, or exome sequencing. Aft...

We present a case-based review of the first five percutaneous fetoscopic in-utero spina bifida repair procedures undertaken in the UK. Our focus is on implications of anaesthesia and analgesia for the mother and fetus, provision of uterine relaxation and fetal immobilisation while providing conditions conducive to surgical access. Minimizing risks...

Gangliogliomas are relatively uncommon accounting for less than 1 percent of intracranial neoplasms. We report a case of endoscopic complete resection of a paraventricular ganglioglioma through the tumour cyst in an 18 years female: Recurrence-free and symptom-free at 24 months follow up.

Gliomas often present clinically with seizures. Tumour-associated seizures can be difficult to control with medication. A deeper understanding of the cellular mechanisms underlying tumour-associated seizures would provide a basis for developing new treatments. Here, we investigate epileptic discharges in peritumoral cortex using living human brain...

There are no established non-invasive methods for accurately assessing shunt patency. Invasive methods such as shunt tapping and radioisotope techniques are not without risk. A potential non-invasive method for assessing patency is a flow-sensitive magnetic resonance image (MRI) sequences. Studies dating back to the late 1980s evaluated the sensiti...

BACKGROUND Invading glioma cells affect the physiological function of the peritumoural cortex. This may manifest clinically as seizures. Here, we investigate the effect the invading glioma cells on the electrophysiological signalling of the peritumoral cortex using living human brain tissue donated by people having a craniotomy for glioma resection...

Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder characterised by premature ageing. We report a case of a 13-year-old girl with HGPS who presented with an extradural haematoma following head injury. A conservative approach was successfully employed in this case, avoiding the risks of general anaesthesia and surgery.

Objectives Virtual reality (VR) simulators are one of the training models allowing the practice of surgical skills for trainees. We aim to assess the benefit of VR training in neurosurgery. Design A single-blinded randomised controlled trial and a literature review. Subjects Trainees (n=12), Fellows and Consultant Neurosurgeons (n=4) and Medical...

Objectives Research remains a foundation in advancing the field of neurosurgery. We explore attitudes and perceived barriers to conducting research in neurosurgery globally. Design A 29-item questionnaire, consisting of multiple-choice questions, Likert scales and short answers. Subjects Clinicians at various stages of the Neurosurgical career....

Introduction Arachnoid cysts (ACs) account for a small proportion of all intracranial lesions. They are often incidental but can become symptomatic and even cause a threat to life. Symptoms are usually due to direct compression of neural elements and/or raised intracranial pressure. Case report We report the case of an infant with an enlarging pos...

Introduction: Undergraduate student conferences provide networking opportunities for students interested in exploring a speciality. The ability to meet with like-minded students, engage with senior clinicians and experience the practical side of a speciality are valuable in shaping the career trajectory of undergraduates. We report our experience o...

Background: Exposure to neurosurgery at the undergraduate level is an important factor in determining future interest in, and applications to, the specialty. Given the high competition for training posts, this exposure is also becoming an almost essential addition to an applicant's portfolio. For this reason, we organised a neurosurgical conferenc...

INTRODUCTION The use of the endoscopic ultrasonic aspirator for the management of intraventricular tumours is still in its infancy. We share our early experience using the aspirator in the management of intraventricular, paraventricular and suprasellar tumours. SUBJECTS 19 patients (10 males, 9 females) underwent 22 operations with the endoscopic...

BACKGROUND Craniopharyngiomas are the commonest suprasellar tumour in childhood. Despite high overall survival, children with craniopharyngiomas are at risk of multiple relapses and long-term tumour- and treatment-related morbidity. We sought to provide, for the first time, a national evidence-based standard for best practice for the assessment, tr...

INTRODUCTION Citation number can be considered a measure of a publication’s academic influence. Previous studies have suggested a citation number greater than 50 and a citation rate higher than 5 per year constitute a high impact in neurosurgery. We set out to determine and categorise the most highly influential papers related to the neurosurgical...

BACKGROUND. Molecular alterations: point mutations, amplifications and deletions give cells the ability to bypass therapeutic approaches. Molecular profiling facilitates access to personalised strategies to treat patients harbouring such alterations. METHODS. A Next Generation Sequencing (NGS) panel comprising 78 genes (version1) or 92 genes (versi...

Infant gliomas have been considered as early-onset examples of tumour types found throughout paediatric populations. Interestingly, infant high-grade gliomas (HGG) have a better overall survival compared to older children, indicating that grading may not accurately reflect the biology of these tumours. We have to-date collected 181 cases of diffuse...

BACKGROUND 5-aminolevulinic acid (5-ALA, Gliolan) is widely used in adults for facilitating the resection of high-grade gliomas. However, its safety and efficacy in the paediatric population remains to be established. We present the UK’s first case series of 10 patients, undergoing 11 operations with 5-ALA. METHODS 10 consecutive patients (aged 1....

Paediatric high grade glioma and diffuse midline glioma (including DIPG) are comprised of multiple biological and clinical subgroups, the majority of which urgently require novel therapies. Patient-derived in vitro primary cell cultures represent potentially useful tools for mechanistic and preclinical investigation based upon their retention of ke...

INTRODUCTION In patients presenting with obstructive hydrocephalus secondary to pineal region lesions, a combined endoscopic third ventriculostomy (ETV) and tumour biopsy is an increasingly common procedure. Performing it through a monoportal approach with a rigid scope is especially challenging given the anatomy of the third ventricle. Here we rep...

Use of endoscopic ultrasonic aspirator for management of adult and pediatric brain tumors

OBJECTIVE Thalamic tumours are uncommon lesions and their management uncertain. They are predominantly astrocytic and can be low or high grade, unilateral or bilateral. One of the challenges they pose is their relative inaccessibility. We present a case of a unilateral thalamic tumour causing obstructive hydrocephalus which we managed endoscopicall...

OBJECTIVE Combined endoscopic third ventriculostomy (ETV) and tumour biopsy is a common approach to the management of patients presenting with hydrocephalus secondary to a pineal region lesion. We report our experience in performing an ETV and limited debulking of a pineal tumour using a rigid scope through a single burr hole. This was made possibl...

Introduction Traumatic brain injury requiring operative intervention in a neonate as a result of motor vehicle collision (MVC) whilst in utero is extremely rare. There have been only a few reported cases of brain injury in infants following in utero trauma as a result of MVC in the literature. The infants sustain significant injuries such as intrav...

X-rays and ventriculograms were the first imaging modalities used to localize intracranial lesions including brain tumors as far back as the 1880s. Subsequent advances in preoperative radiological localization included computed tomography (CT; 1971) and MRI (1977). Since then, other imaging modalities have been developed for clinical application al...

OBJECTIVE: Tectal plate ‘gliomas’ are uncommon, indolent lesions. Presentation is usually with hydrocephalus and diagnosis is typically made radiologically without histological confirmation. Long term data guiding management is lacking. METHOD: Retrospective review of all paediatric tectal plate lesions in our unit over the last 10 years. RESULTS:...

BACKGROUND: 5-aminolevulinic acid (5-ALA, Gliolan) use is well documented and established in the resection of adult high-grade gliomas. There is growing interest in its safety and usefulness in the paediatric populations. The main challenge in the latter is the diverse range of histopathological tumour types that can occur. We present the UK’s firs...

OBJECTIVE: Pilocytic astrocytomas are extremely rare in octagenarians especially those occurring in the third ventricle. They are similar histopathologically to pilomyxoid astrocytomas but the latter are more aggressive. We report the case of an 86-year old patient presenting with confusion and lethargy secondary to a third ventricular lesion causi...

Objectives: The management of neonatal post-haemorrhagic hydrocephalus (nPHH) varies considerably between centres and even between neurosurgeons in the same centre. We performed a nationwide survey to evaluate current practice in the UK and reviewed the evidence for each intervention. Methods: An online semi-structured questionnaire was emailed...

Objective Pilocytic astrocytomas are common glial neoplasms in children. They are less common in adults and extremely rare in octagenarians especially when they occur in the third ventricle. They are similar histopathologically to pilomyxoid astrocytomas but the latter are more aggressive. We report a case of an 86-year old lady who presented with...

Objective Pilocytic astrocytomas are common glial neoplasms in children. They are less common in adults and extremely rare in octagenarians especially when they occur in the third ventricle. They are similar histopathologically to pilomyxoid astrocytomas but the latter are more aggressive. We report a case of an 86-year old lady who presented with...

Aim: To compare cochlear implant (CI) neuromodulation with deep brain stimulation (DBS) for dystonia in childhood and explore relations between age and duration of symptoms at implantation and outcome. Methods: Comparison of published annual UK CI figures for 1985-2009 with a retrospective cohort of the first 9 years of DBS for dystonia in child...

Intracranial germinomas are rare primary central nervous system tumours that are highly sensitive to radiotherapy and chemotherapy. Recurrences are infrequent, with the majority occurring within 5years. Although multidisciplinary treatments have resulted in good event-free survival, long-term outcomes, over decades, are relatively poorly reported....

Yawning is thought to be a behavior regulated by the brainstem. Although excessive yawning has been reported in brainstem strokes, demyelination, and tumors, the cases presented here are the first reports of excessive yawning in patients with Chiari malformation Type I (CM-I). The authors believe that brainstem compression at the craniocervical jun...

INTRODUCTION: Intra-operative MRI (ioMRI) is increasingly being used by some neurosurgical units to ensure maximal tumour resection and therefore improved outcome. Its use has also extended to other non-oncological neurosurgical procedures. METHOD: We report our ioMRI experience in Alder Hey over a period of 53 months and compare the results with a...

INTRODUCTION: Indication for resection of paediatric thalamic tumours has always been debated, varying from biopsy only strategy to total resection. Thalamic tumours are considered unfavourable for resection because of high morbidity and a lack of data between extent of resection and survival. In this study we reviewed our experience with thalamic...