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Spring-assisted posterior vault expansion has been adopted at the London Great Ormond Street Hospital for Children to treat raised intracranial pressure in patients affected by syndromic craniosynostosis, a congenital calvarial anomaly causing the premature fusion of skull sutures. This procedure involves elastic distractors used to dynamically reshape the skull and increase the intracranial volume (ICV). In this study, we developed and validated a patient-specific model able to predict the ICV increase and carried out a parametric study to investigate the effect of surgical parameters on that final volume. Pre- and post-operative computed tomography data relative to 18 patients were processed to extract simplified patient-specific skull shape, replicate surgical cuts, and simulate spring expansion. A parametric study was performed to quantify each parameter’s impact on the surgical outcome: for each patient, the osteotomy location was varied in a pre-defined range; local sensitivity of the predicted ICV to each parameter was analysed and compared. Results showed that the finite element model performed well in terms of post-operative ICV prediction and allowed for parametric optimization of surgical cuts. The study indicates how to optimize the ICV increase according to the type of procedure and provides indication on the most robust surgical strategy.

Objective Expanded endonasal approaches (EEAs) to the skull base have increased the scope and extent of pathologies that can be treated endoscopically. The trade‐off is creation of large skull base bone defects requiring reconstruction to re‐establish barriers between the sino‐nasal mucosa and subarachnoid space to prevent CSF leak and infection. A popular reconstructive technique is the local vascularized pedicled naso‐septal flap, an option that may not always be possible when there is disruption of the vascular pedicle from multiple previous surgeries, adjuvant radiotherapy or extensive tumor infiltration. An alternative is the regional temporo‐parietal fascial flap (TPFF) transposed via the trans‐pterygoid route. We implemented a modification of this technique incorporating contralateral temporalis muscle at the tip of this flap and deeper vascularised pericranial layers within the pedicle to provide a more robust flap in selected cases. Study design/methods A retrospective review of two cases is presented with both patients having undergone multiple EEAs to resect skull base tumors with adjuvant radiotherapy, their postoperative courses complicated by recalcitrant CSF leaks resistant to multiple surgeries. Results Our patients had their persistent CSF fistulae repaired using infra‐temporal transposition of the TPFF modified to include some of the contralateral temporalis muscle with optimisation of a vascular pedicle: a temporo‐parietal temporalis myo‐fascial flap (TPTMFF). Both CSF leaks resolved without further complication. Conclusion In situations where local flap repair to reconstruct skull‐base defects following EEA may not be viable or has failed, a modified regional flap incorporating temporo‐parietal fascia with a preserved vascular pedicle along with attached temporalis muscle plug may provide a robust alternative option.

Introduction There is no clear consensus regarding the technique of surgical revascularization for moyamoya disease and syndrome (MMD/MMS) in the pediatric population. Previous meta-analyses have attempted to address this gap in literature but with methodological limitations that affect the reliability of their pooled estimates. This meta-analysis aimed to report an accurate and transparent comparison between studies of indirect (IB), direct (DB), and combined bypasses (CB) in pediatric patients with MMD/MMS. Methods In accordance with PRISMA guidelines, systematic searches of Medline, Embase, and Cochrane Central were undertaken from database inception to 7 October 2022. Perioperative adverse events were the primary outcome measure. Secondary outcomes were rates of long-term revascularization, stroke recurrence, morbidity, and mortality. Results Thirty-seven studies reporting 2460 patients and 4432 hemispheres were included in the meta-analysis. The overall pooled mean age was 8.6 years (95% CI: 7.7; 9.5), and 45.0% were male. Pooled proportions of perioperative adverse events were similar between the DB/CB and IB groups except for wound complication which was higher in the former group (RR = 2.54 (95% CI: 1.82; 3.55)). Proportions of post-surgical Matsushima Grade A/B revascularization favored DB/CB over IB (RR = 1.12 (95% CI 1.02; 1.24)). There was no significant difference in stroke recurrence, morbidity, and mortality. After meta-regression analysis, year of publication and age were significant predictors of outcomes. Conclusions IB, DB/CB are relatively effective and safe revascularization options for pediatric MMD/MMS. Low-quality GRADE evidence suggests that DB/CB was associated with better long-term angiographic revascularization outcomes when compared with IB, although this did not translate to long-term stroke and mortality benefits.

ObjectiveLow grade intramedullary spinal cord tumours (IMSCTs) are rare tumours of childhood with potential for significant late morbidity following surgery.We present our institutional experience with surgical treatment of these complex lesions emphasizing consistent definition of extent of resection (EOR), techniques to maximise surgical safety and a novel stratification of residual disease to guide post-operative strategy.MethodsA retrospective review of low-grade IMSCTs treated at GOSH between 2000 and 2019 was conducted. All surgery carried out by a single surgeon with intent of safe maximal resection guided by intra-operative neurophysiological monitoring (IONM). Pre and post-operative MRI were reviewed by neuro-radiologists.EOR was recorded as:Gross Total Resection (GTR) –100% resectionNear Total Resection (NTR) – at least 95% resectionSub Total Resection (STR) – 90% tumour resectionPartial Resection (PR) – less than 90% tumour resectionFurther outcome measures were time to recurrence, need for adjuvant therapy and mobility at last follow-up.ResultsA total of 30 patients underwent surgery. IONM parameters (Motor evoked potentials,D-wave) were used to guide EOR. EORs achieved: GTR = 8, NTR = 4, STR = 9, PR = 9.All patients were alive at last follow up with eighteen patients (60%) remaining radiologically and clinically stable. Twelve patients developed recurrence/progressive disease during surveillance (40%) requiring adjuvant treatment. Progression free survival was significantly better in cases with GTR+NTR in comparison to either STR or PR.Following surgery, 26/30 patients were independently mobile, 1/30 required crutches and 3/30 required a wheelchair.9/30 patients were treated with adjuvant therapy following surgery.ConclusionSurvival rates for low grade IMSCT are excellent. Radical micro-surgical resection, guided by IONM provides an effective means of balancing the objectives of maximal safe resection, functional outcome and tumour control. Small volume residual disease does not compromise long-term oncological outcome.

ObjectiveEndoscopic suturectomy for craniosynostosis with helmet therapy (ESCH) has emerged as a successful treatment for craniosynostosis initially in North America. We present early outcomes from the first cohort of ESCH patients treated in the United Kingdom.MethodsRetrospective cohort study with review of electronic records.Results18 consecutive patients from the first procedure in the UK (May 2017) until January 2020 were identified. Our cohort consisted of 12 male and 6 female infants with craniosynostosis, with mean age of 4.6 months (range: 2.5–7.8 months) and weight of 6.8 kg (range 4.8–9.8 kg). The most commonly operated diagnosis was metopic synostosis (n=8) followed by unicoronal (n=7), sagittal (n=2) and multi-sutural (n=1) synostoses. Median length of skin incision was 3 cm (range 2–10 cm), with 15 patients having a single skin incision. 16/18 received no blood products with 2 requiring transfusion (1 donor exposure).Mean operative time (including anaesthesia) was 96 mins (range 40–127 mins). The median length of hospital stay was 1 night with no patients staying more than 2 nights (n=3). Only 1 surgical complication was noted (a suture abscess requiring oral antibiotics). All patients are currently undergoing helmet orthosis with 100% compliance so far. No patients have required revisional surgery.ConclusionEarly peri-operative experience from the first UK cohort of ESCH patients suggests this is a safe and well tolerated technique with low morbidity, need for transfusion and short hospital stay. We hope the success of these results leads to developing discussions to facilitate funding of the helmet orthotic therapy as part of NHS highly specialised commissioning services at Great Ormond Street.

Introduction Brain arteriovenous malformations (bAVMs) present complex challenges in neurosurgery, requiring precise pre-surgical planning. In this context, 3D printing technology has emerged as a promising tool to aid in understanding bAVM morphology and enhance surgical outcomes, particularly in pediatric patients. This study aims to assess the feasibility and effectiveness of using 3D AVM models in pediatric bAVM surgery. Methodology The study was conducted at Great Ormond Street Hospital, and cases were selected sequentially between October 2021 and February 2023. Eight pediatric bAVM cases with 3D models were compared to eight cases treated before the introduction of 3D printing models. The 3D modelling fidelity and clinical outcomes were assessed and compared between the two cohorts. Results The study demonstrated excellent fidelity between 3D models and actual operative anatomy, with a median difference of only 0.31 mm. There was no statistically significant difference in angiographic cure rates or complications between the 3D model group and the non-3D model group. Surgical time showed a non-significant increase in cases involving 3D models. Furthermore, the 3D model cohort included higher-grade bAVMs, indicating increased surgical confidence. Conclusion This study demonstrates the feasibility and efficacy of utilizing 3D AVM models in pediatric bAVM surgery. The high fidelity between the models and actual operative anatomy suggests that 3D modelling can enhance pre-surgical planning and intraoperative guidance without significantly increasing surgical times or complications. Further research with larger cohorts is warranted to confirm and refine the application of 3D modelling in clinical practice.

Paediatric intracranial aneurysms are rare entities accounting for less than 5% of all age intracranial aneurysms. Traumatic aneurysms are more common in children and have an association with anatomical variations such as arterial fenestrations. Here, we present a case of a child initially presenting with traumatic subarachnoid haemorrhage who returned to baseline and was discharged home only to return within 2 weeks with diffuse subarachnoid and intraventricular re-haemorrhage. A dissecting aneurysm of a duplicated (fenestrated) V4 vertebral artery segment was identified as a rare cause of rebleeding. We describe a course complicated by severe vasospasm delaying aneurysm detection and treatment. Dissecting aneurysms in children should be considered in all cases of delayed post-traumatic cranial rebleeding, particularly where there is anomalous arterial anatomy.

Purpose Assess the effects of selective dorsal rhizotomy (SDR) on motor function and quality of life in children with a Gross Motor Function Classification System (GMFCS) level of IV or V (non-ambulatory). Methods This is a prospective, observational study in three tertiary neurosurgery units in England, UK, performing SDR on children aged 3–18 with spastic diplegic cerebral palsy, and a GMFCS level of IV or V, between 2012 and 2019. The primary outcome measure was the change in the 66-item Gross Motor Function Measure (GMFM-66) from baseline to 24 months after SDR, using a linear mixed effects model. Secondary outcomes included spasticity, bladder function, quality of life, and pain scores. Results Between 2012 and 2019, 144 children who satisfied these inclusion criteria underwent SDR. The mean age was 8.2 years. Fifty-two percent were female. Mean GMFM-66 score was available in 77 patients (53.5%) and in 39 patients (27.1%) at 24 months after SDR. The mean increase between baseline and 24 months post-SDR was 2.4 units (95% CI 1.7–3.1, p  < 0.001, annual change 1.2 units). Of the 67 patients with a GMFM-66 measurement available, a documented increase in gross motor function was seen in 77.6% ( n  = 52). Of 101 patients with spasticity data available, mean Ashworth scale decreased after surgery (2.74 to 0.30). Of patients’ pain scores, 60.7% ( n  = 34) improved, and 96.4% ( n  = 56) of patients’ pain scores remained the same or improved. Bladder function improved in 30.9% of patients. Conclusions SDR improved gross motor function and reduced pain in most patients at 24 months after surgery, although the improvement is less pronounced than in children with GMFCS levels II and III. SDR should be considered in non-ambulant patients.

Introduction Intracranial infection is often associated with contiguous sinus infection, with Streptococcus intermedius being the most common pathogen. Microbiological assessment is possible via sinus or intracranial sampling. While a sinus approach is minimally invasive, it is not clear whether this yields definitive microbiological diagnosis leading to optimized antimicrobial therapy and avoidance of intracranial surgery. Methods A retrospective review of a prospectively collected electronic departmental database identified patients between 2019 and 2022. Further demographic and microbiological information was obtained from electronic patient records and laboratory management systems. Results Thirty-one patients were identified with intracranial subdural and/or epidural empyema and concurrent sinus involvement during the 3-year study period. The median age of onset was 10 years with a slight male predominance (55%). All patients had intracranial sampling with 15 patients undergoing sinus sampling in addition. Only 1 patient (7%) demonstrated identical organism(s) grown from both samples. Streptococcus intermedius was the most common pathogen in intracranial samples. Thirteen patients (42%) had mixed organisms from their intracranial cultures and 57% of samples undergoing bacterial PCR identified additional organisms, predominantly anaerobes. Sinus samples had a significant addition of nasal flora and Staphylococcus aureus which was rarely grown from intracranial samples. Of concern, 7/14 (50%) of sinus samples did not identify the main intracranial pathogen diagnosed on intracranial culture and additional PCR. Literature review identified 21 studies where sinus drainage was used to treat intracranial empyemas, with only 6 authors reporting concurrent microbiology results. This confirmed our cohort to be the largest comparative study in the current literature. No center has observed a greater than 50% concordance in microbiological diagnoses. Conclusion Endoscopic sinus surgery may have therapeutic benefit, but it is not an appropriate approach for microbiological diagnosis in pediatric subdural empyemas. High rates of contaminating nasal flora can lead to misdiagnosis and inappropriate treatment. Routine addition of 16S rRNA PCR to intracranial samples is recommended.

Intracardiac migration is a rare complication of ventriculoperitoneal shunt insertion. Only 15 cases have been reported, 7 of which were paediatric cases, treated with techniques including interventional radiography, open thoracotomies and direct extraction through the initial shunt incision. The authors report the youngest case of intracardiac shunt migration complicated by significant coiling and knotting within the cardiac chambers and pulmonary vasculature. Migration likely began when the SVC was pierced during initial shunt placement and progressed due to negative intrathoracic pressure. Extrusion was achieved combining thoracoscopic endoscopy, interventional fluoroscopy screening and a posterolateral neck incision with uncoiling of the shunt via a Seldinger guide wire. This offered a minimally invasive solution with rapid post-operative recovery.