Intracardiac migration of distal catheter-a rare complication of VP shunt insertion: case report and literature review.

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.

The Current Status of the Surgical Management of Complex Spinal Cord Lipomas: Still Navigating the Labyrinth?

This review summarises the classification, anatomy and embryogenesis of complex spinal cord lipomas and describes in some detail the technique of total lipoma resection and radical reconstruction of the affected neural placode. Its specific mission is to tackle two main issues surrounding the management of complex dysraphic lipomas: whether total resection confers better long-term benefits than partial resection and whether total resection does better than conservative treatment, i.e. no surgery, for asymptomatic lipomas. Accordingly, the 24-year progression-free survival data of the senior author and colleagues' series of over 300 cases of total resection are compared with historical data from multiple series (including our own) of partial resection, and total resection data specifically for asymptomatic lesions are compared with the two known series of non-surgical treatment of equivalent patients. These comparisons so far amply support the author's recommendation of total resection for most complex lipomas, with or without symptoms. The notable exception is the asymptomatic chaotic lipoma, whose peculiar anatomical relationship with the neural tissue defies even our aggressive surgical approach, and consequently projects worse results (admittedly of small number of cases) than for the other two lipoma subtypes of dorsal and transitional lesions. Prophylactic resection of asymptomatic chaotic lipomas is therefore not currently endorsed. We have also recently found that some dorsal lipomas with clear outline of the conus on preoperative imaging had a significantly better long-term prognosis of preserving neurourological functions without surgery. Whether this subset of lipomas should be managed conservatively until symptoms arise is now an open question awaiting a longer follow-up of a larger cohort of such patients.

Fetal Surgery for Myelomeningocele: Neurosurgical Perspectives.

More than 30 years have elapsed since it was recognised that folic acid supplementation could substantially reduce the risk of open neural tube defects (ONTDs). During that time, many countries have adopted policies of food fortification with demonstrable reduction in the incidence of both cranial and spinal ONTDs. Improved prenatal detection and termination has also resulted in a reduction in the number of affected live births. Nonetheless, in the USA about 1500 children, and in the UK around 500 children are born each year with myelomeningocele (MMC) and so the management of MMC and its complications continues to constitute a significant clinical workload for many paediatric neurosurgical units around the world.Until recently, the options available following antenatal diagnosis of MMC were termination of pregnancy or postnatal repair. As a result of the MOMS trial, prenatal repair has become an additional option in selected cases (Adzick et al., N Engl J Med 364(11):993-1004, 2011). Fetal surgery for myelomeningocele is now offered in more than 30 centres worldwide. The aim of this chapter is to review the experimental basis of prenatal repair of MMC, to critically evaluate the neurosurgical implications of this intervention and to describe the technique of 'open' repair, comparing this with emerging minimally invasive alternatives.

A topographical analysis of encephalocele locations: generation of a standardised atlas and cluster analysis.

OBJECTIVE: Encephaloceles are considered to result from defects in the developing skull through which meninges, and potentially brain tissue, herniate. The pathological mechanism underlying this process is incompletely understood. We aimed to describe the location of encephaloceles through the generation of a group atlas to determine whether they occur at random sites or clusters within distinct anatomical regions. METHODS: Patients diagnosed with cranial encephaloceles or meningoceles were identified from a prospectively maintained database between 1984 and 2021. Images were transformed to atlas space using non-linear registration. The bone defect, encephalocele and herniated brain contents were manually segmented allowing for a 3-dimensional heat map of encephalocele locations to be generated. The centroids of the bone defects were clustered utilising a K-mean clustering machine learning algorithm in which the elbow method was used to identify the optimal number of clusters. RESULTS: Of the 124 patients identified, 55 had volumetric imaging in the form of MRI (48/55) or CT (7/55) that could be used for atlas generation. Median encephalocele volume was 14,704 (IQR 3655-86,746) mm3 and the median surface area of the skull defect was 679 (IQR 374-765) mm2. Brain herniation into the encephalocele was found in 45% (25/55) with a median volume of 7433 (IQR 3123-14,237) mm3. Application of the elbow method revealed 3 discrete clusters: (1) anterior skull base (22%; 12/55), (2) parieto-occipital junction (45%; 25/55) and (3) peri-torcular (33%; 18/55). Cluster analysis revealed no correlation between the location of the encephalocele with gender (χ2 (2, n = 91) = 3.86, p = 0.15). Compared to expected population frequencies, encephaloceles were relatively more common in Black, Asian and Other compared to White ethnicities. A falcine sinus was identified in 51% (28/55) of cases. Falcine sinuses were more common (χ2 (2, n = 55) = 6.09, p = 0.05) whilst brain herniation was less common (χ2 (2, n = 55) = .16.24, p < 0.0003) in the parieto-occipital location. CONCLUSION: This analysis revealed three predominant clusters for the location of encephaloceles, with the parieto-occipital junction being the most common. The stereotypic location of encephaloceles into anatomically distinct clusters and the coexistence of distinct venous malformations at certain sites suggests that their location is not random and raises the possibility of distinct pathogenic mechanisms unique to each of these regions.

Torticollis in childhood-a practical guide for initial assessment.

Torticollis is encountered often in the paediatric setting and should be considered a presenting symptom, rather than a diagnosis. Aetiologies of torticollis are numerous, and the nomenclature describing underlying diagnosis can be confusing. Furthermore, children with torticollis typically present in the first instance to primary or secondary care rather than to the subspecialist. These factors can contribute to erroneous treatment of this patient-group which could be time critical in some instances. In this review, we discuss the common causes for torticollis and propose a simple clinical assessment tool and early management scheme that will assist in the differential-diagnosis and treatment pathway of this challenging condition.Conclusion: Torticollis can be the initial presentation of various conditions. The diagnosis and management tools provided in this article can aid in guiding paediatricians as to the correct initial management, imaging, and specialist referral. What is Known: • Torticollis in childhood is a very common presenting symptom with numerous aetiologies. • Management is complex, requires multiple clinical and imaging examinations, and is usually performed by non-specialized professionals. What is New: • A new, simple clinical-assessment tool under the acronym PINCH designed to aid paediatric general practitioners in diagnosing correctly the aetiology of torticollis. • A practical management scheme to aid in the treatment pathway of children with torticollis.

Radical resection of lumbosacral lipomas in children: the Great Ormond Street Hospital experience.

PURPOSE: In 2009, Pang described a radical resection technique for congenital lumbosacral lipomas, with lower long-term symptomatic re-tethering rates compared with partial resections and conservative management, and low surgical morbidity. We adopted this technique in 2011, and aim to describe our first results. METHODS: In this monocentric retrospective audit, we included dorsal, transitional, chaotic, and caudal-type lumbosacral lipomas. Exclusion criteria were previously operated lipomas, pure filar lipomas, and concomitant major congenital anatomical urogenital/gastrointestinal abnormalities. Neuro-uro-orthopaedic status at presentation and at three months, one year and last postoperative follow-up, intraoperative electrophysiology, and extent of resection were collected. RESULTS: From January 2011 to September 2019, 91 patients were operated (median age 2y2m; 63 transitional; 14 caudal; 8 dorsal; 6 chaotic). Preoperatively, 67% were symptomatic. Preoperative and one-year postoperative rates of impaired ambulation (44% to 43%), hypoesthesia (8% to 5%), urodynamic/uroradiological abnormalities (49% to 37%), and foot/ankle deformities (8% to 5%) were comparable, whilst pain improved (25% to 5%) but catheterisation rates increased (21% to 36%). 23/92 (25%) suffered wound-related complications. 2/91 (2%) developed symptomatic re-tethering requiring second surgery. Mean cord/sac ratio was 0.47. 43% had > 20 mm3 residual fat, which improved with increasing surgical experience. CONCLUSION: Radical lipoma resection, guided by intraoperative neuromonitoring, with reconstruction of the neural placode and expansion duraplasty is technically feasible and results in low rates of late deterioration and re-tethering. Lipoma-type and pre-operative status are important outcome predictors. Operative risks are not insignificant. Future studies need to determine appropriate selection criteria for surgery.

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.

Craniovertebral junction fixation in children less than 5 years.

PURPOSE: Whilst rigid fixation for craniovertebral instability is the gold standard, in very young, small children conventional management may have to be modified. We present a single-centre experience of craniocervical fixation in children under 5 years. METHODS: A retrospective review of cases that had undergone atlantoaxial (AA) or occipitocervical (OC) fixation aged under 5 years. Fusion was assessed using computerised tomography or flexion extension X-rays. RESULTS: Twenty-six children (median age 2.3, range 0.8-4.9 years, 19 under the age of 3) underwent OC (n = 19) or AA (n = 7) fusion between 1999 and 2016. Pathology comprised 17 congenital, five trauma, two tumour and two post-infection cases. Twenty-one patients underwent sublaminar cable fixation with calvarial, autologous bone graft and halo-body orthosis immobilisation. An occipital plate and rods to sublaminar wire construct were used in four cases. A rigid instrumented fixation with occipital plate and C2 pedicle screws was utilised in one case. Follow-up was for a median of 2.8 years (range 0.03-16.3 years). Initial fusion rate was 91%, reaching 100% following two re-operations. Ninety-two per cent of patients were neurologically stable or improved following surgery. Twenty-one patients had a good overall outcome. Two patients had post-operative neurological deteriorations, and four died due to non-procedure related causes. Pin site morbidity secondary to halo use occurred in five cases. CONCLUSION: High fusion rates with good outcomes are achievable using semi-rigid fixation in the under 5-year-olds. Full thickness, autologous calvarial bone graft secured with wire cables and halo external orthosis offers a safe and effective alternative technique when traditional screw instrumentation is not feasible. These slides can be retrieved under Electronic Supplementary Material.

Chiari I-a 'not so' congenital malformation?

The term Chiari I malformation (CIM) is imbedded in the paediatric neurosurgical lexicon; however, the diagnostic criteria for this entity are imprecise, its pathophysiology variable, and the treatment options diverse. Until recently, CIM has been considered to be a discrete congenital malformation requiring a uniform approach to treatment. Increasingly, it is recognised that this is an oversimplification and that a more critical, etiologically based approach to the evaluation of children with this diagnosis is essential, not only to select those children who might be suitable for surgical treatment (and, of course those who might be better served by conservative management) but also to determine the most appropriate surgical strategy. Whilst good outcomes can be anticipated in the majority of children with CIM following foramen magnum decompression, treatment failures and complication rates are not insignificant. Arguably, poor or suboptimal outcomes following treatment for CIM reflect, not only a failure of surgical technique, but incorrect patient selection and failure to acknowledge the diverse pathophysiology underlying the phenomenon of CIM. The investigation of the child with 'hindbrain herniation' should be aimed at better understanding the mechanisms underlying the herniation so that these may be addressed by an appropriate choice of treatment.

Intraoperative neurophysiological monitoring in paediatric Chiari surgery-help or hindrance?

INTRODUCTION: The role of intraoperative neurophysiological monitoring (IONM) during surgery for Chiari I malformation has not been fully elucidated. Questions remain regarding its utility as an adjunct to foramen magnum decompression surgery, specifically, does IONM improve the safety profile of foramen magnum decompression surgery and can IONM parameters help in intraoperative surgical decision-making. This study aimed to describe a single institution experience of IOM during paediatric Chiari I surgery. METHODS: The methodology comprised a retrospective review of prospectively collected electronic neurosurgical departmental operative database. Inclusion criteria were children under 16 years of age who had undergone foramen magnum decompression for Chiari I malformation with IONM. In addition to basic demographic data, details pertaining to presenting features and post-operative outcomes were obtained. These included primary symptoms of Chiari I malformation and indications for surgery. MRI findings, including the presence of syringomyelia on pre-and post-operative imaging, were reviewed. Details of the surgical technique for each patient were recorded. Only patients with either serial brainstem auditory evoked potential (BAEP) and/or upper limb somatosensory evoked potential (SSEP) recordings were included. Two time points were used for the purposes of analysing IONM data; initial baseline before skin incision and final at the time of skin closure. RESULTS: Thirty-seven children underwent foramen magnum decompression (FMD) with IONM. Mean age was 10.5 years (range 1-16 years) with a male:female ratio 13:24. The commonest clinical features on presentation included headaches (15) and scoliosis (13). Twenty-four patients had evidence of associated syringomyelia (24/37 = 64.9%). A reduction in the SSEP latency was observed in all patients. SSEP amplitude was more variable, with a decrease seen in 18 patients and an increase observed in 12 patients. BAEP recordings decreased in 13 patients and increased in 4 patients. There were no adverse neurological events following surgery; the primary symptom was resolved or improved in all patients at 3-month follow-up. Resolution or improvement in syringomyelia was observed in 19/24 cases. CONCLUSIONS: Our data shows that FMD for Chiari malformation (CM) is associated with changes in SSEPs and BAEPs. However, we did not identify a definite link between clinical outcomes and IONM, nor did syrinx outcome correlate with IONM. There may be a role for IONM in CM surgery but more robust data with better-defined parameters are required to further understand the impact of IONM in CM surgery.

Evaluation and management of tethered cord syndrome in occult spinal dysraphism: Recommendations from the international children's continence society.

AIMS: As awareness and frequency of tethered spinal cord (TSC) related to occult spinal dysraphism (OSD) has increased with magnetic resonance imaging (MRI), variability exists in its evaluation and management. Due to no published level I data, we summarize the current International Children's Continence Society (ICCS) recommendations for diagnosis and treatment of OSD. METHODS: Guidelines were formulated based on analysis of pertinent literature and consensus among authors. This document was vetted by the multidisciplinary members of the ICCS via its website before submission for peer review publication. RESULTS: The more frequent diagnosis of OSD is associated with increased operative intervention. Spinal cord untethering (SCU) has a highly variable risk profile, largely dependent on the specific form of OSD. Progressive neurological deterioration attributed to "tethered cord" may occur, with or without surgery, in selected forms of OSD whereas other cohorts do well. CONCLUSION: Infants with classic cutaneous markers of OSD, with progressive neurologic, skeletal, and/or urologic findings, present no diagnostic or therapeutic dilemma: they routinely undergo MRI and SCU. Conversely, in asymptomatic patients or those with fixed, minor abnormalities, the risk profile of these OSD cohorts should be carefully considered before SCU is performed. Irrespective of whether or not SCU is performed, patients at risk for progression should be followed carefully throughout childhood and adolescence by a multidisciplinary team.

William Harkness, president of the ISPN 2016-2017.

William Harkness was educated at Eton College near Windsor, an illustrious educational establishment that has been responsible for the education of no less than 19 British Prime Ministers. Notable recent alumini include David Cameron and Boris Johnson; fortunately for paediatric neurosurgery, William's leadership qualities have been more successful than the former and his international diplomacy more skilled than the latter.

Ultrasound guided placement of the distal catheter in paediatric ventriculoatrial shunts-an appraisal of efficacy and complications.

PURPOSE: Ventriculoatrial (VA) shunts are commonly used as a second-line treatment of hydrocephalus when the peritoneum is an unsuitable site for the distal catheter. Many centres now utilise ultrasound and interventional radiology techniques to aid placement of the distal catheter. The purpose of this study was to conduct a contemporary audit of VA shunting in children using interventional radiology techniques for placement of the distal catheter. METHODS: A retrospective analysis of all patients who had VA shunts inserted between June 2000 and June 2010 was conducted using a prospectively updated surgical database and case notes review. RESULTS: Ninety-four VA shunts were inserted in 38 patients. Thirty-seven patients had been treated initially with ventriculoperitoneal (VP) shunts. Twenty-two patients required at least 1 shunt revision (58 %). The 6-month, 1- and 2-year shunt survival rates were 53, 43 and 27 %, respectively. Blockage was the commonest reason for shunt failure (68 %). The site of failure was proximal (ventricular catheter +/- valve) in 32 % and distal (atrial catheter) in 21 % of cases. The overall infection rate was 6 % per procedure and 11 % per patient. There were 7 deaths, of which 3 were shunt related. CONCLUSIONS: VA shunting provides a viable second-line option for shunt placement in complex hydrocephalus. The causes of shunt failure (blockage, infection and equipment failure) are similar to VP shunting though shunt survival rates are inferior to VP shunts. Ultrasound guided VA shunt placement provides a relatively safe, second-line alternative to the placement of a ventriculoperitoneal shunt when this route is unsuitable.

The use of de-epithelialized skin flap in the surgical repair of terminal myelocystoceles.

BACKGROUND: Terminal myelocystocele is a severe form of spinal dysraphism characterized by cystic expansion of the terminal spinal cord that herniates through a deficiency of the posterior sacral spinal elements to fuse with the subcutaneous fat. Postnatal enlargement of the subcutaneous fluid-filled sac may result in progressive neurological deficit and threaten the viability of the overlying skin. Surgical repair entails spinal cord untethering, resection of nonfunctional neural elements and watertight reconstruction of the terminal thecal sac. Young age at the time of surgery, large dural defect, attenuated tissues and locally altered CSF dynamics frequently mean that wound complications including CSF leakage and pseudomeningocele formation are common. TECHNIQUE: With consideration of these requirements, we describe our surgical technique in terminal myelocystocele repair, which combines a novel surgical incision and for the first time in a neurosurgical setting, the use of a de-epithelialized skin flap to augment the closure. We report successful operative outcomes in three infant patients with terminal myelocystocele.

Embryology, classification, and surgical management of bony malformations of the craniovertebral junction.

The embryology of the bony craniovertebral junction (CVJ) is reviewed with the purpose of explaining the genesis and unusual configurations of the numerous congenital malformations in this region. Functionally, the bony CVJ can be divided into a central pillar consisting of the basiocciput and dental pivot; and a two-tiered ring revolving round the central pivot, comprising the foramen magnum rim and occipital condyles above, and the atlantal ring below. Embryologically, the central pillar and the surrounding rings descend from different primordia, and accordingly, developmental anomalies at the CVJ can also be segregated into those affecting the central pillar and the surrounding rings, respectively. A logical classification of this seemingly unwieldy group of malformations is thus possible based on their ontogenetic lineage, morbid anatomy, and clinical relevance. Representative examples of the main constituents of this classification scheme are given, and their surgical treatments are selectively discussed.

Long subcutaneous tunnelling reduces infection rates in paediatric external ventricular drains.

PURPOSE: The aim of this study is to report the efficacy of long subcutaneous tunnelling of external ventricular drains in reducing rates of infection and catheter displacement in a paediatric population. METHODS: In children requiring external ventricular drainage, a long-tunnelled drain was placed and managed according to a locally agreed guideline. End points were novel CSF infection incurred during the time of drainage and re-operation to re-site displaced catheters. Data were compared to other published series. RESULTS: One hundred eighty-one long-tunnelled external ventricular drains (LTEVDs) were inserted. The mean age was 6.6 years (range 0-15.5 years). Reasons for insertion included intraventricular haemorrhage (47 %), infection (27 %), tumour-related hydrocephalus (7.2 %), as a temporising measure (17 %) and trauma (2.2 %). The overall new infection rate for LTEVD was 2.76 %. If the 48 cases where LTEVDs were inserted to treat an existing infection are excluded, the infection rate was 3.8 % (5/133). The mean duration of insertion was 10 days (range 0-42 days). Four LTEVDs (2.2 %) were inadvertently dislodged, requiring reinsertion. Thirteen patients required removal of EVD alone. There was a significant difference (p < 0.05) when comparing our infection rate to 14 publications of infection rates in short-tunnelled EVDs; however, there was no difference when comparing our data to three publications using LTEVDs. CONCLUSION: The use of an antibiotic-impregnated LTEVD, managed according to a predefined guideline, is associated with significantly reduced infection and displacement rates when compared with contemporary series. It is suggested that this reduction is of both clinical and economic benefits.

Case Report: Two cases of multiples and atypical dermal sinus tracts.

Dermal sinus tracts (DSTs) are congenital lesions that connect the cutaneous ectoderm with the underlying neuroectodermal tissues. They are typically midline, solitary lesions. Multiple, and atypically located DSTs have been only rarely described. We present two cases of multiple and laterally located DSTs. The first presented with bacterial meningitis and two tracts in the right buttock, one of which entered the spinal canal through the S3 neural foramen. The second child had three midline lumbar DSTs, one subcutaneous dermoid cyst and one intradural epidermoid cyst. Complete surgical excision was achieved in both cases with good late follow up. Multiple or atypically located DSTs appear to carry the same risks of infection as the more common, midline, single tracts. Complete surgical excision is recommended to avoid the risks of neurological deterioration, in particular due to infection. Incomplete disjunction is the proposed developmental anomaly for DSTs, however the location of the cases presented here requires an alternative explanation.

Early Intervention and Use of Autologous Grafts in Growing Skull Fractures Results in Better Outcomes: Experience From a Tertiary Pediatric Neurosurgery Center.

BACKGROUND AND OBJECTIVES: Growing skull fracture (GSF) is a rare complication of pediatric head trauma. Definitive treatment is surgical repair. We have attempted to assess whether use of autologous grafts for duraplasty and cranioplasty leads to better outcomes. We have also attempted to understand how timing of surgery might affect the degree of underlying damage to cortical tissue. METHODS: This is a single-center retrospective observational study based on review from the Great Ormond Street Hospital Neurosurgery prospective surgical database. All patients undergoing surgery for GSF repair between 1991 and 2015 were included. Surgical techniques included split calvarial grafts in 4 patients, whereas rest had full-thickness bone grafting. In all cases with full-thickness graft, the donor site was covered with morselized bone chips mixed with fibrin glue (Salami technique). RESULTS: Twenty-eight patients were identified (16 males, 12 females). The average age at the time of injury was 13 months. The mean duration of onset of symptoms from the time of injury was 4.4 months. The time interval from symptom onset to surgical repair was 5.92 months. Seven patients had Type I GSF (leptomeningeal cyst with minimal brain parenchyma), 13 had type II (hernia containing gliotic brain), and 8 had type III (porencephalic cyst extending through the skull defect into subgaleal space). Patients with delayed presentation had severe brain injury (Type III) and had more long-term complications (refractory epilepsy requiring temporo-occipito-parietal disconnection and development of hydrocephalus requiring ventriculoperitoneal shunt insertion). CONCLUSION: Autologous pericranium for duraplasty and split-thickness bone graft or the Salami technique are recommended for cranioplasty. Synthetic materials should be used if the index operation fails or there are complications. Patients with high-risk findings should be identified at the time of initial presentation and followed up in clinic early to prevent onset of neurological deficit. Early repair is associated with better neurological outcomes.