Cranial vault expansion in treatment of paediatric idiopathic intracranial hypertension.

Introduction: Idiopathic intracranial hypertension (IIH) is a rare clinical entity in the paediatric population. Clinical presentation is mostly similar to adult counterpart and can include headaches, vomiting, papilloedema, deterioration in visual acuity or fields, and 6th cranial nerve palsy, leading to significant morbidity. Therapeutic lumbar puncture and medical treatment with acetazolamide are usually the first-line treatments.

Intraoperative neuromonitoring and mapping during spinal cord untethering surgery; a single-centre paediatric neurosurgery unit experience.

Purpose: A review of intraoperative neuromonitoring (IONM) and mapping (IONMa) utility during paediatric tethered cord surgery with particular attention to feasibility, measures to prevent injury, and postoperative outcome.

Methods: A retrospective analysis of spinal cord untethering surgery between 2015 and 2022 was carried out. Cohort demographics, IONM and IONMa data, and procedural details were summarised and associations between variables explored.

Pseudoachondroplasia associated with os odontoideum and retro-odontoid mass: case-based update.

Purpose: Pseudoachondroplasia is a rare skeletal dysplasia caused by a mutation in the COMP gene. Infants with pseudoachondroplasia present with rhizomelic dwarfism. Pseudoachondroplasia can resemble achondroplasia, which also presents with a phenotype of rhizomelic dwarfism.

Rathke Cleft Cysts in Identical Twins.

Rathke cleft cysts are congenital, non-neoplastic sellar cysts derived from remnants of embryonic Rathke pouch. Presentation of Rathke cleft cysts can be incidental; however, in larger cysts, presentation can be with headaches, visual deterioration, and pituitary and endocrine dysfunction. Here we report a rare case of identical twin boys with Rathke cleft cysts, highlighting the likely genetically driven development of cyst in this identical twin and need for cranial imaging in identical twins with anomalies of the brain.

Evaluation of incidence and outcomes of compressive extradural haematoma-related infarcts in the paediatric population.

Introduction: Traumatic extradural haematoma (EDH) is one of the neurosurgical emergencies in the paediatric population. One of the rare complications of extradural haematoma is adjacent parenchymal infarct from the compressive effect of haematoma leading to further morbidity. We aimed to evaluate the incidence and outcomes of this rare complication in paediatric trauma patients.

Middle fossa arachnoid cyst fenestration for ruptured cysts associated with subdural collections: paediatric neurosurgery tertiary unit experience.

Introduction: Arachnoid cysts are commonly encountered benign cystic structures and often come to attention as incidental findings following cranial imaging. Surgical intervention rates vary in different studies; however, rupture of cyst and subdural collection with mass effect are some of the indications for surgical intervention. In this study, we aimed to evaluate our operated cohort of middle fossa arachnoid cysts to determine the rate of traumatic subdural collection in this cohort and further assess outcomes.

Dens Growth After Surgical Fixation in Children with Morquio-Brailsford Disease.

Mucopolysaccharidosis type IVA is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase. Mucopolysaccharidosis type IVA is multisystemic disease with significant spinal involvement and atlantoaxial instability leading to neural compression and significant morbidity. Dens hypoplasia is a common feature of this condition.

Retroflexed dens in paediatric Chiari 1 patients and implications: Single centre retrospective study.

Introduction: Chiari 1 malformation and hind brain hernia can be associated with skull base and craniocervical anomalies. One of the more recently associated anomalies is a retroverted or retroflexed odontoid process or dens. We conducted a retrospective study of our operated symptomatic and conservatively managed asymptomatic Chiari cohort to assess the impact of dens retroflexion on rate of revision or cerebrospinal fluid diversion following primary foramen magnum decompression (FMD).

Medulloblastomas, CNS embryonal tumors, and cerebellar mutism syndrome: advances in care and future directions.

Central nervous system (CNS) embryonal tumors, commonly found in pediatric patients, represent a heterogeneous mix of lesions with an overall poor (though improving) prognosis. Medulloblastomas are by far the most frequently encountered and most widely studied subtype, though others include atypical teratoid/rhabdoid tumors (AT/RTs), embryonal tumor with multilayered rosettes (ETMRs), and CNS neuroblastomas, FOX-R2 activated. The classification, diagnosis, and treatment of these lesions have evolved drastically over the years as their molecular underpinnings have been elucidated.

Trampoline-Associated Cranial and Spinal Injuries: A 10-Year Study in a Pediatric Neurosurgery Center.

Objective:  There has been an increasing use of trampolines for recreation by children in recent years. Many studies have explored the different types of injuries sustained due to falls from trampolines, but so far none have focused specifically on cranial and spinal injuries. In this study, we describe the pattern of cranial and spinal injuries sustained by pediatric patients that were associated with the use of trampolines and their management in a tertiary pediatric neurosurgery unit over a period of 10 years.

Molecular subgroup of medulloblastoma: evaluation of contribution to CSF diversion following tumour resection.

Introduction: Medulloblastoma is the commonest malignant brain tumour in children. Pre-operative hydrocephalus is present in up to 90% of these patients at presentation. Following posterior fossa surgery, despite resolution of fourth ventricular obstruction, a proportion of these children will still require cerebrospinal fluid (CSF) diversion for management of persistent or new hydrocephalus.

Clinical features and outcome in pediatric arteriovenous malformation: institutional multimodality treatment.

Purpose: Intracranial arteriovenous-malformation (AVM) is a relatively rare condition in pediatrics, yet is a major cause of spontaneous intracranial hemorrhage with a risk of fatal hemorrhage reported to be between 4 and 29%. Little is known about vessel morphology and optimum treatment modalities including multimodality combination therapy and prognosis in children.

Methods: A retrospective review of all children presenting to our institution from 2006 to 2020 that had an AVM was undertaken.

Amplitude-reduction alert criteria and intervention during complex paediatric cervical spine surgery.

Objective: To determine the utility of widely used intraoperative neuromonitoring (IONM) alert criteria and intervention for predicting postoperative outcome following paediatric spinal surgery.

Methods: Retrospective analysis of somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP) in consecutive cervical spine fixations. An intervention protocol followed amplitude-reductions in SSEPs (≥50 %) and/or MEPs (≥80 %).

Myasthenia gravis complicating the surgical management of achondroplasia: a case-based update.

Background: Achondroplasia is the commonest skeletal dysplasia of autosomal dominant inheritance caused by "gain of function" mutations in the fibroblast growth factor receptor 3 (FGFR3) gene. Foramen magnum compression due to accelerated ossification and spinal canal stenosis secondary to reduced interpedicular distance is a hallmark of achondroplasia, driven by G380R nucleotide pair substitution. In severe cases, limb weakness and neurogenic claudication will require surgical decompression.

Spinal canal stenosis in children with achondroplasia: the role of augmentation laminoplasty-a 15-year single institution experience.

Purpose: Achondroplasia typically results in compressive spinal canal stenosis in one-third of children, but rare under the age of 15 years. Laminectomy is the mainstay of treatment but this leads to instability and progressive deformity requiring complex fixation. In order to reduce that risk, we developed a novel modified augmented laminoplasty that increases spinal canal diameter while preserving the posterior column stability.

Posterior Calvarial Distraction in older paediatric population: single centre paediatric neurosurgery craniofacial unit outcomes.

Purpose: Posterior calvarial distraction (PCD) is a safe and effective technique used to increase cranial vault volume and therefore reduce intracranial pressure in children with complex craniosynostosis. Optimal timing and method used for PCD is controversial. This procedure is usually performed in children younger than 2 years.

Posterior Calvarial Augmentation for Chiari Malformation Type 1 Refractory to Foramen Magnum Decompression.

Background: Chiari 1 malformation is a structural abnormality of the hindbrain and posterior fossa characterized by herniation of the cerebellar tonsils through the foramen magnum. Although asymptomatic in some cases, hindbrain herniation can be associated with disruption of cerebrospinal fluid flow dynamics at the craniovertebral junction and syrinx formation, leading to symptoms. Foramen magnum decompression with or without duraplasty has been the most commonly performed surgical procedure in the management of this condition.

Paediatric developmental venous anomalies (DVAs): how often do they bleed and where?

Introduction: Developmental venous anomalies (DVAs) are anomalies of venous drainage and considered a low-flow malformation. Studies evaluating natural history and risk factors for intracranial haemorrhage in the paediatric population are rare. We evaluate clinical and radiological features, risk factors and outcomes of paediatric DVAs.

Paediatric spondylodiscitis: a 10-year single institution experience in management and clinical outcomes.

Purpose: Discitis in the paediatric population poses diagnostic challenges due to non-specific presenting symptoms and difficulty with expressing pain in non-communicating children. Discitis remains a relatively rare condition in the paediatric population and previous reports are limited to small cohorts. In this article, we report our experience in management of discitis over a 10-year period and review the literature on this topic.

Consent in paediatric neurosurgery: adequacy of documentation and parental perspectives.

Introduction: Consenting paediatric patients for surgical procedures remains inherently unique in that it is underpinned by principles such as parental responsibility, assessment of the child's capacity to consent, and adherence to national/legal guidelines. Quality record keeping is an important objective evidence to demonstrate the highest standards of medical care provided to our patients. The consent form is a crucial medical record encapsulating the attainment of informed consent from a parent/guardian for performing a procedure on their child.

Paediatric intracranial aneurysms: a British institutional review.

Introduction: Paediatric intracranial aneurysms are rare, with a differing natural history and thought to account for only up to 7% of all intracranial aneurysms. There is much uncertainty that surrounds the prevalence of unruptured intracranial aneurysms and it is estimated to be anywhere between 2 and 90 per 1000. This is the largest British single-centre analysis of paediatric intracranial aneurysms.

Biological material collection to advance translational research and treatment of children with CNS tumours: position paper from the SIOPE Brain Tumour Group.

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 characteristics have been identified, and these characteristics are increasingly used to facilitate accurate diagnoses and therapeutic recommendations.