An inexpensive foramen magnum decompression training tool: Feasibility and validation study.

Foramen magnum decompression (FMD) is a standard neurosurgical procedure, typically utilized to treat Chiari malformation. The aim of this educational project was to develop and validate a low-cost FMD simulation training model. Mold-based methods were used to develop a prototype. Feasibility was tested during an FMD training session for 17 neurosurgery trainees. Face and content validity were assessed through a Likert Scale. The perceived training benefit was determined using the Physician Performance Diagnostic Inventory Scale (PPDIS). A total of 87.5% successfully removed the C1 arch, 81.3% successfully performed an FMD, and 68.8% avoided injury to the underlying structures. The model scored highly for visual and tactile realism. The median confidence rating on PPDIS significantly improved from early learner to competent. We demonstrate feasibility, content, and face validity. Furthermore, this is a low-cost, portable model that can be easily replicated and used for simulation training.

Association of the Achondroplasia Foramen Magnum Score and intraoperative neuromonitoring.

INTRODUCTION: Foramen magnum stenosis in achondroplasia carries a risk of sudden death. A proportion of these patients benefit from foramen magnum decompression (FMD). The Achondroplasia Foramen Magnum Score (AFMS) was developed to stratify those most at risk. We hypothesise that this score may be reflected in neurophysiological findings. METHODS: Patients with achondroplasia who had undergone FMD (n=20) were retrospectively grouped into AFMS 2, 3 and 4. Amplitude from tibialis anterior (TA) and the percentage change in somatosensory evoked potential (SSEP) latency after FMD were reported. RESULTS: Baseline motor evoked potential amplitudes for patients with AFMS=4 were significantly lower left (p=0.0017 and p=0.02 for right and left TA, respectively) compared with AFMS grades 2 and 3. Median reduction (% change) in SSEP latency (ms) after surgery was not significantly different in any of the patients. CONCLUSIONS: This short report cross-references AFMS to intraoperative neuromonitoring. Baseline amplitudes were noticeably lower in the most severe AFMS group. This observation supports the notion that AFMS can help risk stratify patients and aid in surgical selection.

Chemical angioplasty vs. balloon plus chemical angioplasty for delayed cerebral ischemia: a pilot study of PbtO2 outcomes.

BACKGROUND: Delayed cerebral ischaemia (DCI) is a major cause of morbidity and mortality after aneurysmal subarachnoid haemorrhage (aSAH). Chemical angioplasty (CA) and transluminal balloon angioplasty (TBA) are used to treat patients with refractory vasospasm causing DCI. Multi-modal monitoring including brain tissue oxygenation (PbtO2) is routinely used at this centre for early detection and management of DCI following aSAH. In this single-centre pilot study, we are comparing these two treatment modalities and their effects on PbtO2. METHODS: Retrospective case series of patients with DCI who had PbtO2 monitoring as part of their multimodality monitoring and underwent either CA or TBA combined with CA. PbtO2 values were recorded from intra-parenchymal Raumedic NEUROVENT-PTO® probes. Data were continuously collected and downloaded as second-by-second data. Comparisons were made between pre-angioplasty PbtO2 and post-angioplasty PbtO2 median values (4 h before angioplasty, 4 h after and 12 h after). RESULTS: There were immediate significant improvements in PbtO2 at the start of intervention in both groups. PbtO2 then increased by 13 mmHg in the CA group and 15 mmHg in the TBA plus CA group in the first 4 h post-intervention. This improvement in PbtO2 was sustained for the TBA plus CA group but not the CA group. CONCLUSION: Combined balloon plus chemical angioplasty results in more sustained improvement in brain tissue oxygenation compared with chemical angioplasty alone. Our findings suggest that PbtO2 is a useful tool for monitoring the response to angioplasty in vasospasm.

Ventriculo-atrial shunt in idiopathic intracranial hypertension.

PURPOSE: CSF diversion  is a recognised intervention in idiopathic intracranial hypertension (IIH), particularly in the presence of vision-threatening papilledema. Although ventriculo-atrial (VA) shunt insertion is a routine neurosurgical procedure, ventriculoperitoneal and lumboperitoneal shunts have been mostly used in this particular indication. This study aims to look at a single centre's experience with VA shunts in idiopathic intracranial hypertension (IIH). METHODS: Retrospective case series with a review of electronic records over a 10-year period; exclusion criteria were duplication of same shunt insertion, no VA shunt insertion, paediatric patients and indication other than IIH. Notes were reviewed for demographics, shunt survival (defined by time prior to revision) and reasons for revision. RESULTS: Eight VA shunt procedures were identified in 6 patients (mean age at insertion 34 ± 10 years) with a mean follow-up of 58 ± 25 months. All shunts were secondary procedures; 2 revisions from lumbo-pleural, 2 from ventriculopleural, 2 from ventriculoatrial and one each from ventriculoperitoneal and combined lumbo-/ventriculoperitoneal. At 50 months, 75% of VA shunts had survived, compared to only 58.3% of VPleural shunts in patients with IIH. Revisions were required due to acute intracranial bleed (1 case)-revised at day 1, and thrombus at distal site (1 case)-revised at day 57. Both shunts were later reinserted. From the latest clinic letters, all patients had their treatment optimised with this procedure, although only two patients had documented resolved papilloedema post-procedure. CONCLUSIONS: Ventriculo-atrial shunts are a safe and efficacious alternative option for CSF diversion in IIH. In this series, only 1 shunt was revised for a VA shunt-specific complication.

Lumbosacral lipoma in childhood, how strong is the evidence base? A systematic review.

OBJECTIVE: Conus region lumbosacral lipomas (LSLs) are highly heterogeneous in their morphology, clinical presentation, and outcome, with an incompletely understood natural history and often treacherous surgical anatomy. This systematic review aims to critically evaluate and assess the strength of the current LSL evidence base to guide management strategies. METHODS: According to a systematic review following PRISMA guidelines, a search was conducted using the key term "lumbosacral lipoma" across MEDLINE (OVID), Embase, Cochrane Library, and PubMed databases from January 1951 to April 2021. All studies containing ten or more paediatric conus lipomas were included. Data heterogeneity and bias were assessed. RESULTS: A total of 13 studies were included, containing 913 LSLs (predominantly transitional type-58.5%). Two-thirds (67.5%) of all patients (treated and non-treated) remained clinically stable and 17.6% deteriorated. Neuropathic bladder was present in 8.6% at final follow-up. Of patients managed surgically, near-total resection vs. subtotal resection deterioration-free survival rates were 77.2-98.4% and 10-67% respectively. 4.5% (0.0-27.3%) required re-do untethering surgery. Outcomes varied according to lipoma type. Most publications contained heterogeneous populations and used variable terminology. There was a lack of consistency in reported outcomes. CONCLUSION: Amongst published series, there is wide variability in patient factors such as lipoma type, patient age, and methods of (particularly urological) assessment. Currently, there is insufficient evidence base upon which to make clear recommendations for the management of children with LSL. There is an imperative for neurosurgeons, neuroradiologists, and urologists to collaborate to better standardise the terminology, assessment tools, and surgical interventions for this challenging group of conditions.

Subtemporal Decompression in Resistant Slit Ventricle Syndrome in Children: An Observational Study and Survival Analysis.

INTRODUCTION: Slit ventricle syndrome (SVS) remains a challenging problem in the early-shunted paediatric population. Various surgical and non-surgical treatments have been devised for this condition. However, there is currently no gold standard for its optimal management. Among various treatment modalities, subtemporal decompression (STD) is often performed as a last resort. We present our experience of STD in paediatric patients with SVS in whom initial treatment with programmable valves and anti-syphon device were not successful. METHODS: This is a single-centre retrospective observational study and survival analysis. Patients who underwent STD for SVS were included. Pre- and post-operative imaging data and clinical outcomes were collected. RESULTS: There were 20 patients (12 M, 8 F) with a mean age of 9 years (SD: 4) at first STD. 90% (n = 18) of patients had multiple shunt revisions pre-STD. At first STD, 70% (n = 14) and 30% (n = 6) of patients had unilateral or bilateral STD, respectively. STD led to a reduction in the frequency of shunt revisions in 60% (n = 12) of patients. The median time required before further STD, shunt surgery, or cranial vault surgery was 14 months. The median time before a further STD was required (either revision or contralateral side) was 89 months. At a median follow-up of 66.5 months (range: 1-159), 65% (n = 13) of patients had improvement in symptoms. CONCLUSIONS: A large proportion of patients with persistent SVS symptoms, refractory to multiple shunt revisions, benefitted from STD in combination with shunt optimization. It was also safe and well-tolerated. Therefore, in patients who have multiple failed shunts, STD may reduce the morbidity associated with further shunt revisions and can significantly improve symptomatology.

Resolution of Large Cervico-Thoracic Syringomyelia Following Treatment of Thyrotoxicosis: A Case Report.

Treatment for a large symptomatic syrinx associated with a Chiari 1 is predominately surgical, via a foramen magnum decompression (FMD), with the aim to normalise cerebrospinal fluid (CSF) movement. Whilst theories of underlying hyperdynamic states in Chiari 1 and Syringomyelia exist, to date there is no effective medical treatment to reverse Syringomyelia. A 17-year-old female was referred with a seven-month history of gradually progressive impaired temperature sensation in her left upper limb. She had also been concomitantly diagnosed with thyrotoxicosis. Magnetic resonance imaging (MRI) confirmed a Chiari 1 with a large syrinx. The patient preferred to avoid surgery in the first instance. She underwent treatment for her thyrotoxicosis. The eight-month, 20- and 36-month follow-up MRI scans demonstrated a gradual resolution of the Chiari 1 malformation and the syrinx. Whilst there have been reports of Chiari 1 malformation association with hyperthyroidism, this is the first report describing syrinx resolution following treatment of thyrotoxicosis. Hyperdynamic circulation can result in syrinx formation through various mechanisms. We hypothesise that the treatment of thyrotoxicosis resulted in normalisation of CSF pulse amplitude and subsequent syrinx resolution. Hyperthyroidism evaluation may be explored in studies of CM1 and Syrinx or other CSF disorders.

Gastrulation and Split Cord Malformation.

Split cord malformation (SCM) is a rare form of closed spinal dysraphism, in which two hemi-cords are present, instead of a single spinal cord. SCM is categorised into type 1 and type 2. Type 1 SCM is defined by the presence of a bony or osseocartilaginous spur between the hemi-cords, whereas type 2 SCM has no bony spur, and the two hemi-cords are contained within a single dura. In this chapter, we present the putative mechanisms by which SCM arises, including gastrulation defects and Pang's unified theory. The typical and rare clinical presentations and variations are described. Finally, we outline the step-by-step surgical approach to both SCM 1 and 2 and the overall prognosis of both conditions.

Neurosarcoidosis Mimicking Normal Pressure Hydrocephalus.

Two female patients, aged 46 and 51, were referred to the National Hospital for Neurology and Neurosurgery with symptoms resembling normal pressure hydrocephalus (NPH) and with ventriculomegaly on the MRI. Both had a definite diagnosis of neurosarcoidosis (NS) on biopsy, and they underwent the medical and surgical management reserved for NPH. At follow-up, their presenting symptoms had resolved and they had clinically improved. Neurosarcoidosis can mimic NPH, and it should be excluded in patients presenting with NPH.

Distal fusion in Duchenne scoliosis: the relevance of preoperative pelvic obliquity. A case series study.

PURPOSE: The aim of this study was to determine clinical and radiographic outcomes for Duchenne Muscular Dystrophy (DMD) patients who underwent posterior spinal fusion from T2/3 to L5 (without pelvic fixation), at this single centre. METHODS: From January 2012 to January 2020, 29 consecutive DMD scoliosis patients underwent posterior spinal fusion using pedicle screws from T2/3 to L5 in a single center with a minimum of 3 years follow-up (FU). Radiologic measurements and chart review were performed. RESULTS: Twenty nine patients aged 14 ± 1.5 years were included. No patient was lost to FU. All patients had significant correction in Cobb angle, pelvic obliquity (PO) and lumbar lordosis (LL), without loss of correction at last FU. The mean values for preoperative, immediate postoperative and last FU were CA 62o, 15o and 17o, PO: 21o, 8o and 9o; and LL 10o, -41o and -41o respectively. Correction in CA was independent of any variable analysed including implant density, rod diameter, traction, or bone density. Regarding PO, it was inversely related to age and independent of all other variables. Factors associated with postoperative complications were age and respiratory function. CONCLUSIONS: It appears from our results that pelvic fixation might not always be required in DMD scoliosis surgery, when using pedicle screws with lowest instrumented vertebra at L5. However, larger preoperative PO values can be related with residual PO. It seems that probably related to the underlying condition, early surgery may decrease incidence of complications. LEVEL OF EVIDENCE: IV.

A case of recurrent flight-induced cerebrospinal fluid shunt overdrainage.

Shunted patients often complain of headaches after flights. The effect of air travel on shunt systems is unknown. We describe the case of a patient with longstanding hydrocephalus, who suffered flight-induced clinical deterioration and shunt overdrainage in two independent occasions. The patient, clinically stable for 1.5 and 5 years before each episode, reported severe headaches starting during the descent stages of the air travel. On both occasions, brain MRI imaging demonstrated pronounced ventricular size reduction. This case suggests that flight-induced shunt overdrainage can occur and should be suspected in patients with prolonged headaches and/or clinical deterioration triggered by air travel.

Regional Scalp Blockade for Painless Removal ofIntracranial Pressure Bolts: Technical Note and Patient-Reported Outcomes.

OBJECTIVE: Elective insertion of intracranial pressure (ICP) monitoring bolts is useful for the diagnosis and treatment of disorders of cerebrospinal fluid dynamics. Patients typically report severe discomfort on bolt removal, which negatively impacts overall patient satisfaction of ICP monitoring. We assessed the efficacy and safety of using supratrochlear and supraorbital nerve block-a commonly used form of scalp anesthesia-alongside oral analgesia before bolt removal. METHODS: We compared the efficacy and safety of regional scalp block anesthesia alongside oral analgesia versus oral analgesia alone in a cohort of 85 patients undergoing removal of diagnostic ICP bolts between June 2017 and April 2019 using retrospective patient questionnaires, as well as electronic admission documentation. RESULTS: We found that scalp block alongside oral analgesia improved bolt removal experience (4/5 vs. 3/5 on a 1-5 point Likert scale) and that a majority (70.6%) of patients would have preferred local anesthetic before removal in the oral analgesia-only group. Only 2 patients experienced mild and localized side effects: mild bruising and temporary facial and eyelid swelling following regional scalp block. CONCLUSIONS: We conclude that regional supraorbital and supratrochlear nerve block is a simple, safe, and effective adjunct to minimizing discomfort during bolt removal and improving overall patient satisfaction of ICP monitoring.

A survival analysis of ventricular access devices for delivery of cerliponase alfa.

OBJECTIVE: Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is a rare autosomal recessive disease caused by tripeptidyl peptidase 1 enzyme deficiency. At the authors' center, the medication cerliponase alfa is administered every 2 weeks via the intracerebroventricular (ICV) route. This requires the placement of a ventricular access device (VAD) or reservoir and frequent percutaneous punctures of this device over the child's lifetime. In this study, the authors audited the longevity and survival of these VADs and examined the causes of device failure. METHODS: A single-center survival analysis of VAD insertions and revisions (January 2014 through June 2020) was conducted. All children received cerliponase alfa infusions through a VAD. Patient characteristics and complications were determined from a prospectively maintained surgical database and patient records. For the VAD survival analysis, the defined endpoint was when the device was removed or changed. Reservoir survival was assessed using Kaplan-Meier curves and the log-rank (Cox-Mantel) test. RESULTS: A total of 17 patients had VADs inserted for drug delivery; median (range) age at first surgery was 4 years 4 months (1 year 8 months to 15 years). Twenty-six VAD operations (17 primary insertions and 9 revisions) were required among these 17 patients. Twelve VAD operations had an associated complication, including CSF infection (n = 6) with Propionibacterium and Staphylococcus species being the most prevalent organisms, significant surgical site swelling preventing infusion (n = 3), leakage/wound breakdown (n = 2), and catheter obstruction (n = 1). There were no complications or deaths associated with VAD insertion. The median (interquartile range) number of punctures was 59.5 (7.5-82.0) for unrevised VADs (n = 17) versus 2 (6-87.5) for revised VADs (n = 9) (p = 0.70). The median survival was 301 days for revisional reservoirs (n = 9) versus 2317 days for primary inserted reservoirs (n = 17) (p = 0.019). CONCLUSIONS: In the context of the current interest in intrathecal drug delivery for rare metabolic disorders, the need for VADs is likely to increase. Auditing the medium- to long-term outcomes associated with these devices will hopefully result in their wider application and may have potential implications on the development of new VAD technologies. These results could also be used to counsel parents prior to commencement of therapy and VAD implantation.

Effect of position on intracranial pressure and compliance: a cross-sectional study including 101 patients.

OBJECTIVE: A better understanding of the effect of position on intracranial pressure (ICP) and compliance is important for the development of treatment strategies that can restore normal cerebrospinal fluid (CSF) dynamics. There is limited knowledge on the effect of position on intracranial compliance. In this cross-sectional study the authors tested the association of pulse amplitude (PA) with position and the day/night cycle. Additionally, they describe the postural ICP and PA changes of patients with "normal" ICP dynamics. METHODS: This single-center retrospective study included patients with suspected and/or confirmed CSF dynamics abnormalities who had been examined with elective 24-hour ICP monitoring between October 2017 and September 2019. Patients had been enrolled in a short exercise battery including four positions: supine, lumbar puncture position in the left lateral decubitus position, sitting, and standing. Each position was maintained for 2 minutes, and mean ICP and PA were calculated for each position. The 24-hour day and night median ICP and PA data were also collected. Linear regression models were used to test the correlation of PA with position and day/night cycle. All linear regressions were corrected for confounders. The postural ICP monitoring results of patients without obvious ICP dynamics abnormality were summarized. RESULTS: One hundred one patients (24 males and 77 females) with a mean age of 39 ± 13years (mean ± standard deviation) were included in the study. The adjusted linear regression models demonstrated a significant association of ICP with position and day/night cycle, with upright (sitting and standing) and day ICP values lower than supine and night ICP values. The adjusted linear regression model was also significant for the association of PA with position and day/night cycle, with upright and day PA values higher than supine and night PA results. These associations were confirmed for patients with and without shunts. Patients without clear ICP dynamics abnormality had tighter control of their postural ICP changes than the other patients; however, the difference among groups was not statistically significant. CONCLUSIONS: This is the largest study investigating the effect of postural changes on intracranial compliance. The results of this study suggest that PA, as well as ICP, is significantly associated with posture, increasing in upright positions compared to that while supine. Further studies will be needed to investigate the mechanism behind this association.

Brain MRI and Ophthalmic Biomarkers of Intracranial Pressure.

OBJECTIVE: To evaluate the utility of brain MRI and ophthalmic biomarkers for the prediction of intracranial hypertension, we have studied the association between six biomarkers and 24-hour intracranial pressure (ICP) monitoring results in 45 patients. METHODS: This single-centre observational study includes patients who underwent 24-hour ICP monitoring, brain MRI (within three months) and ophthalmic assessment (during ICP monitoring). Six biomarkers were investigated: pituitary gland shape, vertical tortuosity of the optic nerve, distension of the optic nerve sheath, optic disc protrusion (MRI), papilloedema (slit lamp biomicroscopy) and spontaneous venous pulsations (SVP, infrared video recordings). RESULTS: Forty-five patients (mean age 39±14SD, 38 females) met the inclusion criteria. All 6 biomarkers had a significant association with 24-hour ICP. Concave pituitary gland was observed with moderately elevated median ICP. Protrusion of the optic disc (MRI), papilloedema and absence of SVP were associated with the highest median ICP values. Twenty patients had raised ICP (median 24-hour ICP>5.96 mmHg, cut-off obtained through Youden index calculation). Patients with all normal biomarkers had normal median ICP in 94% (St.Err.=6%) of the cases. All the patients with 3 or more abnormal biomarkers had intracranial hypertension. The combination of at least one abnormal biomarker in MRI and ophthalmic assessments was highly suggestive of intracranial hypertension (AUC 0.94, 95% CI 0.93-0.94) CONCLUSIONS: Brain MRI and ophthalmic biomarkers can non-invasively guide the management of patients with suspected CSF dynamics abnormalities. Patients with multiple abnormal biomarkers (≥3) or a combination of abnormal MRI and ophthalmic biomarkers are likely to have intracranial hypertension and should be managed promptly.

Does conus morphology have implications for outcome in lumbosacral lipoma?

OBJECTIVE: Lumbosacral lipomas (LSL) of the 'dorsal' type have been associated with more favourable outcomes compared with other conus region lipomas. We hypothesised that integrity of the conus on MRI underpins the improved prognosis in this subgroup of LSL patients. METHODS: The definition of 'dorsal lipomas' included lipomas with attachment to the conus, but where the conus could be delineated on MRI (Morota type 1) as reported by Morota et al. (J Neurosurg Pediatr 19:428-439, 2017). Additional inclusion criteria included asymptomatic status at presentation, age >3 years at follow-up, and neurological and urological evaluation at presentation and at last follow-up. Lipoma extent and conus level were recorded. Outcome measures were the need for untethering surgery and neuro-urological status at last follow-up. Urological outcomes were defined by continence and efficacy of bladder emptying. RESULTS: Twenty-six children were included (median age 8.7 years). Conus level was low (at or below L2) in 92%. Nine required untethering surgery: 5 prophylactic, 4 because of clinical deterioration. Twenty-five children were continent at last follow-up, one had stress incontinence, and none required catheterisation. One had persisting ankle weakness after surgery requiring orthotic support. CONCLUSIONS: In LSL of the conus, visualisation of the conus on MRI is associated with good urological and motor outcomes. The integrity of the conus appears to be a more important prognostic factor than anatomical level. An observational approach to this group of LSL patients does not appear to compromise outcomes. These findings support a selective approach to untethering surgery.

Accuracy of bolt external ventricular drain insertion by neurosurgeons of different experience.

OBJECTIVE: The accuracy of tunneled external ventricular drain (EVD) placement has been shown to be similar among practitioners of varying experience, but this has not yet been investigated for bolt EVDs. Tunneled and bolt EVDs are distinct techniques, and it is unclear if conclusions regarding accuracy can be inferred from one method to the other. The goal of this study was to determine whether neurosurgical experience influences the accuracy of bolt EVD placement. METHODS: We performed a single-center retrospective analysis of accuracy of bolt EVD placement between 1st December 2018 and 31st May 2020, comparing the accuracy outcomes between three levels of training (junior trainees (JT); mid-grade trainees (MT); senior trainees/fellows (ST)). Accuracy was determined radiologically by two methods: Kakarla grade and by measuring the distance of the catheter tip to its optimal position (DTOP) at the foramen of Monro. RESULTS: Eighty-seven patients underwent insertion of bolt EVDs, of which n = 19 by JT, n = 40 by MT and n = 28 by ST, with a significant difference found between training grades in the median Kakarla grade (p = 0.0055) and in the accuracy of placement as per DTOP (p = 0.0168). CONCLUSIONS: In contrast to previous published results on tunneled EVDs, we demonstrate that the accuracy of bolt EVD placement is dependent on neurosurgical experience. Our results draw awareness to the fact that the bolt EVD technique can represent a challenge for less experienced practitioners and underline the importance of dedicated training to support the safe insertion of bolt ventricular catheters.