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. METHODS:  This is a retrospective study of all children less than 16 years of age with suspected or confirmed trampoline-associated cranial or spinal injuries, managed by a tertiary pediatric neurosurgery unit from 2010 to 2020. Data collected included the patient's age at the time of injury, gender, neurological deficits, radiological findings, management, and clinical outcome. The data were analyzed to highlight any trends in the pattern of injuries. RESULTS:  A total of 44 patients with a mean age of 8 years (ranging from one year and five months to 15 years and five months) were identified. 52% patients were male. 10 patients (23%) had a reduced Glasgow Coma Scale (GCS) score. In terms of imaging findings, 19 patients (43%) had a radiologically positive head injury, nine (20%) had a craniovertebral junction (CVJ) injury, including the first (C1) and second (C2) cervical vertebrae, and six (14%) had an injury involving other parts of the spine. No patient sustained concurrent head and spinal injuries. Eight (18%) patients had normal radiological findings. Two (5%) had incidental findings on radiology that required subsequent surgery. A total of 31 patients (70%) were managed conservatively. 11 patients (25%) underwent surgery for their trauma, of which seven were cranial. Two further patients underwent surgery for their incidental intracranial diagnoses. One child died from an acute subdural hemorrhage. CONCLUSIONS:  This study is the first to focus on trampoline-associated neurosurgical trauma and report the pattern and severity of cranial and spinal injuries. Younger children (less than five years of age) are more likely to develop a head injury, whereas older children (more than 11 years of age) are more likely to develop a spinal injury following the use of a trampoline. Although uncommon, some injuries are severe and require surgical intervention. Therefore, trampolines should be used prudently with the appropriate safety precautions and measures.

Spondylodiscitis following ingestion of button battery in an infant-a case-based update.

Button battery ingestion in infants is an increasingly common surgical emergency which can lead to oesophageal perforation, mediastinitis, trachea-oesophageal fistulation, airway compromise and death. One exceedingly rare complication of battery ingestion is discitis and osteomyelitis in the cervical and upper thoracic spine. Diagnosis is normally delayed due to the non-specific presentation, delayed imaging findings and the initial clinical focus on dealing with the immediate, and potentially life-threatening, complications. We describe a case of a 1-year-old girl who presented with haematemesis and an oesophageal injury, secondary to button battery ingestion. Sagittal reconstruction of the CT chest demonstrated a suspicious area of vertebral erosion in the cervicothoracic spine which prompted a further evaluation with MRI demonstrating spondylodiscitis of C7-T2 with vertebral erosion and collapse. The child was successfully treated with long course of antibiotics. We wish to highlight the importance of clinical and radiological spinal assessment in children with button battery ingestion to avoid delayed diagnosis and complications of spinal osteomyelitis.

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. Various scoring systems have been developed to predict the risk for CSF diversion following posterior fossa surgery. However, no accurate tool exists regarding which pathological subset or group of medulloblastoma patients will require a shunt post-operatively. In this study we investigated the impact of molecular subgroup of medulloblastoma on shunt dependency post-operatively in paediatric patients. METHODS: We undertook a retrospective multi-centre study of children with medulloblastoma who underwent tumour resection. Those with available molecular subgroup were identified. Demographic data and clinical parameters including age, sex, presence of pre-operative hydrocephalus, extent of surgical resection, evidence of metastasis/leptomeningeal disease and need for CSF diversion post-operatively were further analysed. RESULTS: Sixty-nine children with medulloblastoma with available molecular data were identified during the study period with male to female ratio of 1.5:1 (42M:27F). Twelve patients (17.4%) belonged to SHH, 10 (14.5%) Wnt, 19 (27.5%) Group 3 and 15 (21.7%) Group 4; 13 (18.8%) were non-specified Group 3 or 4. A total of 18 (26%) patients had evidence of leptomeningeal disease at presentation (20% of Wnt, 42% of Group 3, 33% of group 4, 23% of group 3/4, and 0% of SHH). Fifteen patients (22%) underwent post-operative ventriculoperitoneal (VP) shunt insertion. No patient in the Wnt group required ventriculoperitoneal (VP) shunt post-operatively in this cohort. Need for shunt was associated with pre-operative hydrocephalus, leptomeningeal disease, with molecular group 3 or 4 demonstrating higher rate of leptomeningeal disease, and pre-operative hydrocephalus. Age, extent of resection and pre-operative EVD were not associated with need for shunt in this cohort. Regression analysis identified only pre-operative hydrocephalus and leptomeningeal disease as independent predictors of need for shunt post-resection in this cohort. CONCLUSION: All patients requiring permanent post-operative VP shunt belonged to non-Wnt groups, particularly group 3 and 4. Although medulloblastoma subgroup does not independently predict need for post-operative shunt, presence of leptomeningeal disease and pre-operative hydrocephalus, and their higher prevalence in group 3 and 4, likely account for observed higher rate of shunting in these groups.

The role of surgery in relieving calcified shunt site-related pain in patients with functioning VP shunt.

PURPOSE: Shunt calcification is a known late sequela of ventriculoperitoneal (VP) shunt insertion and is associated with shunt malfunction. However, in some patients, while shunt functionality is preserved despite calcification of the catheters, they experience nociceptive symptoms. In this paper, the authors present their surgical experience in managing patients with a functional VP shunt and experiencing pain secondary to shunt calcification. METHODS: We analysed outcomes of patients presenting with pain at the level of a calcified shunt who underwent surgical untethering of the calcified catheter from the soft tissues. This procedure was commenced by the senior author in 2015. Patients were collected prospectively from the databases of two institutions. Evidence of shunt calcification was confirmed on neuroimaging. RESULTS: Seven patients, two male and five female, were included. The mean age at untethering was 13.5 years. The mean time interval between primary shunt surgery and symptom onset was 12 years (range 6-16 years). The commonest site of tethering was the neck (50%) followed by abdomen and chest (both 25%). Six patients underwent untethering of the catheter from soft tissues. One patient had removal of a redundant segment of calcified shunt left in situ during a previous revision. All patients experienced pain relief following shunt untethering. CONCLUSION: Untethering of calcified VP shunt catheters from soft tissue can be considered an effective treatment of shunt site pain and offered to patients presenting with a functional VP shunt.

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. RESULTS: A total of 50 children were identified with median age of 11 (range 1-16) years. The mean follow-up was 7.6 years. Forty-one children presented as an emergency and of those, 40 had hemorrhage identified on initial brain imaging. The average nidus size was 25 mm, drainage was superficial in 51% of cases, and located in eloquent cortex in 56%. The supplemental Spetzler-Martin grading indicated 78% (39/50) were grade 4 and above (moderate to high risk). Primary treatment modalities included embolization in 50% (25) or SRS in 30% (15) and surgery in 20% (10).The AVM was obliterated on follow-up DSA in 66% children. Three children had post-treatment hemorrhage, two related to embolization and one the day following SRS, giving a re-bleed rate of 6%. The GOSE was available for 32 children at long term follow and 94% had a good outcome (GOSE 5-8). Two children died due to acute hemorrhage (4%). CONCLUSION: The majority of children with AVM present with hemorrhage. The rebleed rate during definitive treatment is low at 6% over the study period. The selective use of the 3 modalities of treatment has significantly reduced mortality and severe disability.

Functional hemispherotomy for epilepsy in the very young.

OBJECTIVE: Epilepsy is one of the most common neurological disorders in children. Among very young children, one-third are resistant to medical treatment, and lack of effective treatment may result in adverse outcomes. Although functional hemispherotomy is an established treatment for epilepsy, its outcome in the very young child has not been widely reported. In this study the authors investigated seizure and developmental results after hemispherotomy in children younger than 3 years. METHODS: The authors reviewed a prospective database of all children younger than 3 years with medically intractable epilepsy who underwent functional hemispherotomy at the authors' institution during the period between 2012 and 2020. Demographic data, epilepsy history, underlying etiology, operative and transfusion details, and seizure and developmental outcomes were analyzed. RESULTS: Twelve patients were included in this study. The mean age (± SD) at seizure onset was 3 ± 2.6 months and at surgery was 1.3 ± 0.77 years, with a mean follow-up of 4 years. Diagnoses included hemimegalencephaly (n = 5), hemidysplasia (n = 2), hypoxic/hemorrhagic (n = 2), traumatic (n = 1), Sturge-Weber syndrome (n = 1), and mild hemispheric structural abnormality with EEG/PET correlates (n = 1). Eleven patients achieved an Engel class I outcome, and 1 patient achieved Engel class IV at last follow-up. No deaths, infections, cerebrovascular events, or unexpected long-term neurological deficits were recorded. All children progressed neurodevelopmentally following surgery, but their developmental levels remained behind their chronological age, with an overall mean composite Vineland Adaptive Behavior Scale score of 58 (normal: 86-114, low: < 70). One patient required insertion of a subdural peritoneal shunt, 1 patient required dural repair for a CSF fluid leak, and 1 patient required aspiration of a pseudomeningocele. In 2 patients, both of whom weighed less than 5.7 kg, the first operation was incomplete due to blood loss. CONCLUSIONS: Hemispherotomy in children younger than 3 years offers excellent seizure control and an acceptable risk-to-benefit ratio in well-selected patients. Families of children weighing less than 6 kg should be counseled regarding the possibility of staged surgery. Postoperatively, children continue to make appropriate, despite delayed, developmental progress.

Tectal Plate Gliomas Masquerading as Idiopathic Aqueduct Stenosis.

Aqueduct stenosis is a recognized cause of obstructive hydrocephalus in children and can be treated effectively with endoscopic third ventriculostomy. Preoperative magnetic resonance imaging is often diagnostic of the cause of aqueduct stenosis. We describe 2 pediatric cases with obstructive hydrocephalus secondary to a working diagnosis of idiopathic aqueduct stenosis. Following successful endoscopic third ventriculostomy, repeat magnetic resonance brain imaging revealed tectal plate glioma as the primary cause of obstruction. We believe these 2 reported cases demonstrate a previously unreported phenomenon whereby concealed tectal gliomas presenting with hydrocephalus are only unmasked following relief of hydrocephalus and decompression and normalization of the ventricular system. We aim to raise awareness about this unusual phenomenon and recommend routine postoperative interval imaging following endoscopic third ventriculostomy to avoid missing underlying pathology masquerading as aqueduct stenosis.

A rare case of inferior vena cava occlusion secondary to abdominal pseudocyst associated with ventriculoperitoneal shunt-case report and review of literature.

BACKGROUND AND IMPORTANCE: Abdominal pseudocyst (APC) is an uncommon but well-recognised complication of ventriculo-peritoneal (VP) shunt. Diagnosis is based on clinical features of shunt malfunction, including headaches, vomiting and drowsiness, and abdominal swelling. APCs can grow to large sizes resulting in compression of abdominal viscera; however, inferior vena cava (IVC) compression is extremely rare, and only one other case associated with VP shunt had been reported. CLINICAL PRESENTATION: We report a case of a 12-year-old girl with a background of open myelomeningocoele repair, kyphoscoliosis and right-sided VP shunt in situ who presented with bilateral lower limb swelling and abdominal distension. She exhibited no features of raised intracranial pressure but had bilateral pitting oedema up to the groin. Abdominal ultrasound and CT scan showed a massive septated pseudocyst (20 × 18 × 8 cm) compressing the IVC. The APC was drained, and the shunt was externalised, with conversion to a ventriculo-atrial (VA) shunt 1 week later after cultures of the cystic fluid, cerebrospinal fluid and shunt tube came back sterile. The patient remained well with no recurrence of the abdominal fluid and no VA shunt complication at 36-month follow-up. CONCLUSION: This patient had specific risk factors for development of a massive APC and the subsequent IVC compression, including prior surgeries, spinal deformity, abnormal abdominal anatomy and poor abdominal muscle tone and sensation. Early recognition of this complication and prompt alleviation of the mass effect of IVC compression can prevent long-term neurological and vascular sequalae.

Infantile Macrocephaly: Complicated Familial Benign Enlargement of Subarachnoid Space in Twins of Suspected Nonaccidental Injury.

A 3-month-old first of dizygotic male twins, born at 34 + 5 weeks gestational age, presented with a 4-week history of increasing head circumference and vomiting. He had a tense anterior fontanelle and a head circumference above the 97th percentile. Magnetic resonance imaging showed large bilateral subdural collection with hemosiderin deposits suggestive of hemorrhage. Nine days of bilateral subdural drainage reduced the collection size and blood load. On postoperative day 16, magnetic resonance imaging confirmed persistent but smaller subdural collections, unmasking the underlying subarachnoid space enlargement. On day 18, a right subdural-peritoneal valveless shunt was inserted as definitive treatment. As part of a nonaccidental injury investigation, Twin 2 was also found to have macrocephaly secondary to benign enlargement of subarachnoid space, which was managed conservatively. Benign enlargement of subarachnoid space has an assumed autosomal/multifactorial inheritance and predisposes to subdural hemorrhage. Ultimately, no safeguarding issues were raised. Both twins continued to be neurologically stable at 2-year follow-up with head circumferences between the 98th and 99th percentiles.

Optic Nerve Germinoma and Transient Spontaneous Regression-More Than Meets the Eye.

Infiltration of the optic pathway by germ cell tumors is exceptional and can lead to confusion with glioma or inflammatory conditions. We present the case of a 14-year-old girl with an optic nerve germinoma extending to the hypothalamus and manifesting as panhypopituitarism and visual loss. The patient experienced spontaneous regression of the lesion followed by secondary deterioration requiring treatment. Four other cases of spontaneously regressing intracranial germinoma followed by regrowth have been reported in the literature. This report highlights the importance of clinical and radiologic monitoring of intracranial germinoma, even in the event of initial spontaneous improvement.

Clinical effectiveness of and family experience with telephone consultation in a regional pediatric neurosurgery center in the United Kingdom.

OBJECTIVE: Pediatric neurosurgery outpatient consultation is conducted face-to-face (FTF) conventionally. Reasons for not using telemedicine include the perceived difficulty with obtaining a reliable history and an inability to perform a physical examination. However, FTF consultation can cause distress and inconvenience to the child and family. In 2018, the authors' department piloted a clinical nurse specialist-led telephone consultation (TC) for follow-up appointments. This was extended to the routine neurosurgery clinics in 2020. In this study, the authors evaluate 1) the effectiveness of TC, 2) families' experience with TC compared with traditional FTF appointments, and 3) the factors associated with their preferences. METHODS: In this prospective study using a survey methodology, TCs carried out by 2 consultant neurosurgeons and 1 nurse specialist over 8 weeks were evaluated. Based on clinical background, each patient was assigned to a TC or FTF appointment. Clinical and surgical details and home postal code were recorded. At the end of each TC, the clinician recorded whether the child required an FTF appointment within 3 months. In addition, patients/families answered 1) how the current TC compared with FTF consultation, and 2) their preference of TC or FTF for the next consultation. RESULTS: A total of 114 TCs were included. No child required an FTF appointment within 3 months. Overall, compared with an FTF appointment, the TC was the "same/better/much better" for 101 families (89%), and "worse/much worse" for 13 (11%). Two-thirds of families preferred the next appointment to be a TC. Families attending a TC for new appointments preferred the next appointment to be FTF compared with those attending a follow-up TC (6/8 [75%] vs 31/106 [29%], p = 0.006). A high rating of the current TC was associated with a preference for a TC as the next appointment (p < 0.0001). Families preferring TC over FTF lived farther from the hospital (mean 38 vs 27 km) (p = 0.029). CONCLUSIONS: From the clinicians' perspective, TC is adequate in appropriately selected patients as either the primary mode of consultation or as a triage system. From a service users' perspective, the majority of families felt that the appointment was the same/better than traditional FTF appointments. The findings suggested that 1) new patients should be offered FTF appointments; 2) follow-up TCs should be offered to families when possible; and 3) clinicians should develop their skills in conducting TCs. The authors' results have led to a modification of our algorithm in delivering traditional outpatient service and telemedicine with telephone.

Pediatric stereo-electroencephalography: effects of robot assistance and other variables on seizure outcome and complications.

OBJECTIVE: The safety of stereo-electroencephalography (SEEG) has been investigated; however, most studies have not differentiated pediatric and adult populations, which have different anatomy and physiology. The purpose of this study was to assess SEEG safety in the pediatric setting, focusing on surgical complications and the identification of patient and surgical risk factors, if any. The authors also aimed to determine whether robot assistance in SEEG was associated with a change in practice, surgical parameters, and clinical outcomes. METHODS: The authors retrospectively studied all SEEG cases performed in their department from December 2014 to March 2020. They analyzed both demographic and surgical variables and noted the types of surgery-related complications and their management. They also studied the clinical outcomes of a subset of the patients in relation to robot-assisted and non-robot-assisted SEEG. RESULTS: Sixty-three children had undergone 64 SEEG procedures. Girls were on average 3 years younger than the boys (mean age 11.1 vs 14.1 years, p < 0.01). The overall complication rate was 6.3%, and the complication rate for patients with left-sided electrodes was higher than that for patients with right-sided electrodes (11.1% vs 3.3%), although the difference between the two groups was not statistically significant. The duration of recording was positively correlated to the number of implanted electrodes (r = 0.296, p < 0.05). Robot assistance was associated with a higher number of implanted electrodes (mean 12.6 vs 7.6 electrodes, p < 0.0001). Robot-assisted implantations were more accurate, with a mean error of 1.51 mm at the target compared to 2.98 mm in nonrobot implantations (p < 0.001). Clinical outcomes were assessed in the first 32 patients treated (16 in the nonrobot group and 16 in the robot group), 23 of whom proceeded to further resective surgery. The children who had undergone robot-assisted SEEG had better eventual seizure control following subsequent epilepsy surgery. Of the children who had undergone resective epilepsy surgery, 42% (5/12) in the nonrobot group and 82% (9/11) in the robot group obtained an Engel class IA outcome at 1 year (χ2 = 3.885, p = 0.049). Based on Kaplan-Meier survival analysis, the robot group had a higher seizure-free rate than the nonrobot group at 30 months postoperation (7/11 vs 2/12, p = 0.063). Two complications, whose causes were attributed to the implantation and head-bandaging steps, required surgical intervention. All complications were either transient or reversible. CONCLUSIONS: This is the largest single-center, exclusively pediatric SEEG series that includes robot assistance so far. SEEG complications are uncommon and usually transient or treatable. Robot assistance enabled implantation of more electrodes and improved epilepsy surgery outcomes, as compared to those in the non-robot-assisted cases.

UK Chiari 1 Study: protocol for a prospective, observational, multicentre study.

INTRODUCTION: Chiari 1 malformation (CM1) is a structural abnormality of the hindbrain characterised by the descent of the cerebellar tonsils through the foramen magnum. The management of patients with CM1 remains contentious since there are currently no UK or international guidelines for clinicians. We therefore propose a collaborative, prospective, multicentre study on the investigation, management and outcome of CM1 in the UK: the UK Chiari 1 Study (UKC1S). Our primary objective is to determine the health-related quality of life (HRQoL) in patients with a new diagnosis of CM1 managed either conservatively or surgically at 12 months of follow-up. We also aim to: (A) determine HRQoL 12 months following surgery; (B) measure complications 12 months following surgery; (C) determine the natural history of patients with CM1 treated conservatively without surgery; (D) determine the radiological correlates of presenting symptoms, signs and outcomes; and (E) determine the scope and variation within UK practice in referral patterns, patient pathways, investigations and surgical decisions. METHODS AND ANALYSIS: The UKC1S will be a prospective, multicentre and observational study that will follow the British Neurosurgical Trainee Research Collaborative model of collaborative research. Patients will be recruited after attending their first neurosurgical outpatient clinic appointment. Follow-up data will be collected from all patients at 12 months from baseline regardless of whether they are treated surgically or not. A further 12-month postoperative follow-up timepoint will be added for patients treated with decompressive surgery. The study is expected to last three years. ETHICS AND DISSEMINATION: The UKC1S received a favourable ethical opinion from the East Midlands Leicester South Research Ethics Committee (REC reference: 20/EM/0053; IRAS 269739) and the Health Research Authority. The results of the study will be published in peer-reviewed medical journals, presented at scientific conferences, shared with collaborating sites and shared with participant patients if they so wish.

Seizure improvement following vagus nerve stimulator (VNS) battery change with cardiac-based seizure detection automatic stimulation (AutoStim): early experience in a regional paediatric unit.

PURPOSE: The recent VNS models (AspireSR® Model 106, SenTiva™ Model 1000 (VNS Therapy®, LivaNova)) include a new function of cardiac-based seizure detection (CBSD) automatic stimulation, known as 'AutoStim'. This algorithm uses tachycardia as a proxy to a seizure, and the battery delivers a closed-loop electrical current in addition to its programmed stimulation. This function leads to further seizure reduction in adults, but this advantage has not been reported in the paediatric population. This study aims to investigate whether battery change with AutoStim leads to further seizure reduction in children. METHODS: This observational study included the first 10 cases of VNS battery change from non-AutoStim to AutoStim function. During the battery change operation, the new VNS was switched on, with the same normal and magnet mode settings as the previous VNS. The AutoStim mode was activated at the same time. Data on seizure burden were collected at 3 time points: (1) before the first VNS insertion, (2) before battery replacement (post-1st VNS) and (3) 12 months post-battery change (post-AutoStim). The net effect of AutoStim, the only changed parameter, was evaluated by comparing the seizure burden prior to and 12 months following battery change in each child. RESULTS: The seizure reduction improved significantly from 60 to 83% following battery change with AutoStim. Categorising the outcome according the McHugh classification, children achieving class I and II outcome (≥ 50% seizure reduction) improved from 70 to 90%. CONCLUSION: This is the first study to demonstrate the additional efficacy of AutoStim in children treated with VNS.

Atlantoaxial limited dorsal myeloschisis: A report of two cases and review of literature.

Introduction: Limited dorsal myeloschisis (LDM) is a rare form of spinal dysraphism that is characterised by a distinctive fibroneural stalk connecting the spinal cord to the overlying skin lesion. The skin lesions associated with LDM can appear benign clinically and careful evaluation with an MRI scan is essential for diagnosing LDM and to differentiate this entity from other forms of spinal dysraphism and benign causes of skin lesions. Research question: There is a lack of reported atlantoaxial LDM in the literature. We sought to report the clinical presentation, radiological features and surgical management of the first two reported atlantoaxial LDM. Material and methods: Clinical findings and radiological images of the two cases of atlantoaxial LDM that underwent surgical intervention at our institution were retrieved from the medical notes, operative records and imaging system. Results: Both cases of atlantoaxial LDM (C0-1 and C1-2 respectively) underwent successful resection of the overlying cutaneous lesions and stalks to release the tethered spinal cords. Discussion: The surgical management of LDM have been associated with good outcomes and consists of resecting the fibroneural stalk close to the underlying cord, releasing the tethered spinal cord and removing the overlying cutaneous lesion. Conclusion: These are the first two reported cases of atlantoaxial LDM in the literature. We aim to raise awareness of this pathological entity and highlight the importance of establishing the correct diagnosis to guide definitive management, and report the favourable neurological outcome in these cases despite the rostral location.