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Undifferentiated spindle cell sarcoma (UDS) is a poorly defined or understood entity, essentially a waste-basket for cases failing to fulfill criteria for better-established diagnoses based on combined histology, immunohistochemistry, and tumor genetic assays. We identified a novel chromosomal translocation t(17;19)(p13;q13) in a pediatric UDS and have characterized this alteration to show rearrangement of the MLL4 and GPS2 genes, resulting in an in-frame fusion gene MLL4-GPS2, the expression of which promotes anchorage-independent growth. MLL4 was previously reported to be similarly rearranged in hepatocellular carcinomas, notably those positive for hepatitis B virus. Isolated reports of individual rearrangements of GPS2 in a prostate carcinoma cell line and in glioblastoma multiforme, each with different partner genes, recently emerged from high-throughput sequencing studies but have not been further evaluated for biological effect.

Visual loss following surgery for craniopharyngioma is usually the result of operative injury or tumor recurrence. The authors present the case of a 12-year-old boy who developed progressive visual field constriction 11 years after gross-total resection of a solid and cystic craniopharyngioma. No tumor recurrence was evident on multiple MRI studies, and it was only at surgical exploration that the diagnosis of optochiasmatic arachnoiditis was established. Lysis of the adhesions around the optic nerves and chiasm resulted in substantial and sustained visual improvement.

Cerebrospinal fluid (CSF) leakage is a complication of intradural spinal surgery and is associated with poor wound healing and infection. The incidence of CSF leak is reported at ∼16 % in adults, but little information is available in children. The aim of this study is to determine the CSF leak rate and predisposing factors after intradural pediatric spinal surgeries. This study was a retrospective chart review of 638 intradural spinal operations at BC Children’s Hospital. CSF leak was defined as pseudomeningocele or CSF leak through incision. Primary operations to untether lipomyelomeningoceles, myelomeningocele/meningocele closure, and Chiari decompressions were excluded. CSF leaks occurred in 7.1 %, with 3 % having overt CSF leaks through skin (OCSF leak). CSF leaks, specifically OCSF leaks, were associated with postoperative wound infection (P = 0.0016). Sixteen of 45 cases of CSF leak required reoperation. The type of dural suture used, site of operation, or use of fibrin glue did not affect CSF leak rates. Previous spinal surgery (P

Background/Aims: Lumbar disc herniation (LDH) can be associated with ring apophysis fracture (RAF), which is found in 6% of adult cases. However, the incidence and management of RAF in pediatric LDH is not well documented. The purpose of this study is to determine the prevalence of RAF in pediatric LDH, identify risk factors and explore the influence of RAF on the surgical management and outcome of LDH patients. Methods: Hospital records and images were retrospectively reviewed for all pediatric patients (Results: Forty-two patients met the study inclusion criteria. RAF was present in 38% of the pediatric patients with LDH. There was a significant correlation with gender (p = 0.021; 55% of the males had RAF, and 20% of the females) and association with central herniations (p = 0.003). At the last follow-up, 58% of the patients with RAF were symptom free, compared with 68% of the patients with no RAF. Conclusion: RAF is more frequently associated with LDH in children than in adults. Gender and central disc herniation are associated with RAF. Given the frequency of RAF, to properly identify these fractures and anticipate their treatment, preoperative CT would be necessary.

The BC Patient Safety & Quality Council has a mandate to bring health system stakeholders together in a collaborative partnership to improve quality of care. Our experience has demonstrated the value of networks to provide a forum for individuals to “think like a system,” considering the perspectives of others in addressing system issues. This transition from silo-based thinking is important as we move to improve the quality of care at the pace that is required.

Object In general, patients who present with low Glasgow Coma Scale (GCS) scores and/or fixed and dilated pupils are not expected to do well following arteriovenous malformation (AVM) hemorrhage. However, there is a sense among neurosurgeons that pediatric patients may make a better recovery than adults following such an event. There have been few studies focusing on the outcome of pediatric patients with poor neurological status following AVM hemorrhage. The purpose of this study was to characterize functional outcome in pediatric patients with severe disability after AVM hemorrhage. Methods This was a retrospective analysis of clinical presentation and outcome in 15 patients seen at the authors' pediatric hospital presenting with low GCS scores (defined as GCS ≤ 8) following AVM hemorrhage. Results Initial GCS scores ranged from 3 to 6, and 11 of 14 patients had fixed pupils on clinical examination (data were not available in 1 patient). Eight of 15 patients suffered primarily a lobar hemorrhage, 3 suffered primarily infratentorial bleeding, 2 suffered primarily hemorrhages of the basal ganglia, and 2 suffered intraventricular hemorrhage. The overall mortality rate was 20% (3 of 15 patients). The clinical outcome of survivors was defined by the Pediatric Cerebral Performance Category (PCPC) and Pediatric Overall Performance Category (POPC) scores at follow-up. One year after AVM hemorrhage, 7 (58%) of the 12 surviving patients showed normal or mild disability (PCPC Score 1 or 2), whereas 5 (42%) of 12 patients had moderate or severe disability (PCPC Score 3 or 4). No patients were in a coma or vegetative state, and 11 (92%) of the 12 patients were functioning independently (POPC Score 1, 2, or 3) 1 year after AVM hemorrhage. All patients were functionally independent by last follow-up, with 8 patients (67%) in the normal or mild disability PCPC category, and 4 in the moderate category (PCPC Score 3). All 12 survivors made a meaningful recovery and went on to live independent lives. Conclusions Pediatric patients suffering AVM hemorrhage have a good outcome and are able to function independently, despite a poor neurological state initially.

Object Microfibrillar collagen hemostat (MCH; trade name Avitene) is a partially water-insoluble acid salt of purified bovine corium collagen. This agent has been widely used to control hemorrhage at surgery, and especially during pediatric neurosurgeries at the authors' institution. Despite its effectiveness, rare case reports detailing adverse inflammatory reactions to MCH have been documented. Based primarily on MR imaging, postoperative reactions have most commonly elicited clinical differential diagnoses of tumor recurrence or abscess. According to the literature, MCH induces a very characteristic mixed inflammatory response that is rich in eosinophils; in light of these observations, many authors have suggested an allergy-based pathogenesis. Methods The authors retrospectively reviewed 3 pediatric neurosurgical cases treated at their institution, wherein a common histomorphological inflammatory reaction to MCH was elicited at the site of prior craniotomy. Results Case 1 is that of a 10-year-old girl whose diagnosis was a right temporal lobe ganglioglioma, classified as WHO Grade I. Case 2 is that of a 9-year-old boy whose diagnosis was a left parietal lobe anaplastic ependymoma, classified as WHO Grade III. Finally, Case 3 is that of a 15-year-old girl whose diagnosis was focal cortical dysplasia Type IIA affecting the left occipital lobe. Each patient presented with new or recurrent seizures 5–6 weeks after the initial resection. The postsurgical reactions incited by MCH mimicked the radiological appearance of either an abscess (Cases 2 and 3) or recurrent tumor (Case 1). Histologically, the mixed inflammatory infiltrate was typified by the presence of MCH-centric necrotizing granulomas that were surrounded by a palisade of macrophages and often several eosinophils. Conclusions The findings are in keeping with previous case reports describing the clinicopathological features of adverse reactions occurring due to MCH. Based on the authors' observations, the possibility of an idiopathic inflammatory reaction to MCH should be considered when either seizures, a typical radiological appearance (that is, consistent with tumor recurrence or abscess formation), or both arise shortly after initial surgery. A conservative treatment approach to this type of inflammatory lesion appears to be the most appropriate management strategy.

An effective safety event reporting system is an essential part of a comprehensive patient safety program. In British Columbia, we are implementing a provincial web-based event reporting tool and learning system called the BC Patient Safety and Learning System (PSLS). In this paper, we describe and report the results of our pilot study in a neonatal intensive care unit at BC Women's Hospital in Vancouver. Our approach aimed to foster a culture of safety by using the technology implementation to facilitate organizational learning about patient safety and to promote sustainable reporting behaviours. Results showed that PSLS was enthusiastically adopted by staff and enabled efficient reporting, promoted timely and complete follow-up activities and facilitated quality improvement. Our lessons learned laid the foundation for the provincial rollout of PSLS and may be of interest to those implementing similar systems elsewhere.

Undifferentiated spindle cell sarcoma (UDS) is a poorly defined or understood entity, essentially a waste-basket for cases failing to fulfill criteria for better-established diagnoses based on combined histology, immunohistochemistry, and tumor genetic assays. We identified a novel chromosomal translocation t(17;19)(p13;q13) in a pediatric UDS and have characterized this alteration to show rearrangement of the MLL4 and GPS2 genes, resulting in an in-frame fusion gene MLL4-GPS2, the expression of which promotes anchorage-independent growth. MLL4 was previously reported to be similarly rearranged in hepatocellular carcinomas, notably those positive for hepatitis B virus. Isolated reports of individual rearrangements of GPS2 in a prostate carcinoma cell line and in glioblastoma multiforme, each with different partner genes, recently emerged from high-throughput sequencing studies but have not been further evaluated for biological effect.

Visual loss following surgery for craniopharyngioma is usually the result of operative injury or tumor recurrence. The authors present the case of a 12-year-old boy who developed progressive visual field constriction 11 years after gross-total resection of a solid and cystic craniopharyngioma. No tumor recurrence was evident on multiple MRI studies, and it was only at surgical exploration that the diagnosis of optochiasmatic arachnoiditis was established. Lysis of the adhesions around the optic nerves and chiasm resulted in substantial and sustained visual improvement.

Review of children with low-grade cerebellar astrocytoma (LGCA) prior to 1992 showed a 98 % rate of gross total resection (GTR) but a concerning incidence of permanent neurological dysfunction. The purpose of this study was to determine the rate of GTR of LGCA since 1992 and frequency of neurologic injury. Retrospective review of children with LGCA was performed. CT/MR scans were rereviewed to assess extent of resection. Primary outcomes included incidence of GTR and incidence of permanent new neurological deficits. Other outcomes included late effects severity score (LESS), Bloom score for functional status, and educational assessment. Of 50 LGCA, GTR was achieved in 38 (76 %) compared to 43 of 44 (98 %) prior to 1992 (p

Object The natural history of syringomyelia in pediatric patients remains uncertain. Although symptomatic and operative cases of syringomyelia are well studied, there are fewer articles in the literature on the nonoperative syrinx and its clinical and radiological course. The purpose of this research was to analyze the natural history of untreated syringomyelia in pediatric patients presenting with minimal neurological symptoms. Methods A review of the neurosurgery database at British Columbia's Children's Hospital identified all pediatric patients (< 18 years of age) with syringes identified on MR imaging. Patients were included in this study if they had at least 2 MR images of the spine, at least 1 year apart, while receiving nonoperative treatment. Magnetic resonance imaging was used to determine changes in the size of the syrinx over time. Clinic notes were analyzed to establish demographic and clinical features and to determine any clinical changes over time. Results A total of 17 patients were included in the study. Symptoms at presentation were often mild and included limb numbness (3 cases), headaches (2 cases), mild sensory deficits (2 cases), mild motor deficits (3 cases), and intermittent incontinence (7 cases). The consultant neurosurgeon believed that the syrinx was not contributing to the symptoms in these 17 patients. The syrinx either remained unchanged (7 cases) or diminished in size (8 cases) in a total of 15 patients (88%). In the remaining 2 patients the authors noted an increase in syrinx size, in 1 of whom the clinical course also worsened. Both of these patients had a Chiari malformation and subsequently underwent craniocervical decompression. Overall, the mean change was −0.7 mm of maximal axial diameter (range −2.6 to +2.7 mm). Sixteen patients (94%) exhibited no worsening of symptoms over time. Conclusions Syringomyelia often remains stable in patients receiving nonoperative treatment. However, given that 2 (12%) of 17 syringes in this series enlarged, it is likely appropriate to include periodic imaging in the follow-up of these cases.

revision was 1.5 years). In 29 control subjects, 34% (10 patients) never required subsequent revision (mean follow-up 8.9 years), and 66% required revisions (mean time to first revision was 1.5 years). The shunt after ETV was significantly more likely to survive (p=0.023) than the shunt in the non-ETV group. Conclusions VPS in patients with previous “failed” ETV appear to have better survival than VPS in patients who have never had ETV. This has interesting implications in considering the potential benefit of ETV, even when a VPS is subsequently necessary.

We present a case report of a 21-month-old female patient to highlight magnetic resonance (MR) imaging findings associated with papilledema in a pediatric patient with an intracranial tumor. The MR findings included optic disc elevation, dilated perioptic subarachnoid spaces, optic nerve tortuosity and restricted diffusion in the optic nerve heads, all of which resolved upon resolution of raised intracranial pressure (ICP) and papilledema. The case demonstrates that both conventional and diffusion-weighted MR imaging findings can detect the presence, and follow posttreatment resolution, of increased ICP and papilledema in a pediatric tumor patient. The postoperative resolution of optic disc elevation clearly indicates the intracranial tumor etiology.

Objectives Acute respiratory tract infections represent a significant burden on pediatric emergency departments (ED) and families. We hypothesized that early and rapid diagnosis of a viral infection alleviates the need for ancillary testing and antibiotic treatment. Study design We conducted a randomized, controlled trial of children 3 to 36 months of age with febrile acute respiratory tract infections at a pediatric ED. Two hundred four subjects were randomly assigned to receive rapid respiratory viral testing on admission or a routine ED admission protocol. Outcome measures were: mean length of visits, rate of ancillary tests, and antibiotic prescription in the ED. A follow-up call was made to all study subjects to inquire about further healthcare visits, ancillary testing, and antibiotic prescription after ED discharge. Results We did not find a statistically significant difference in ED length of visits, rate of ancillary testing, or antibiotic prescription rate in the ED between the study groups. There was, however, a significant reduction in antibiotic prescription after ED discharge (in the group who had rapid viral testing RR = 0.36; 95% CI = 0.14, 0.95). Conclusions Rapid multi-viral testing in the ED did not significantly affect ED patient treatment but may reduce antibiotic prescription in the community after discharge from the ED, suggesting a novel strategy to alter community physician antibiotic prescription patterns.

To study cerebral blood flow and cerebral oxygen consumption in severe head-injured children and also to assess the effect of hyperventilation on regional cerebral blood flow.Prospective cohort study.Pediatric intensive care unit at a tertiary-level university children's hospital.Twenty-three children with isolated severe brain injury, whose admission Glasgow Coma Scores were8.PaCO2 was adjusted by altering minute ventilation. Cerebral metabolic measurements were made at three levels of PaCO2 (35, 25 to 35, and25 torr [4.7, 3.3 to 4.7, and3.3 kPa]) after allowing 15 mins for equilibrium.Thirty-eight studies (each study consisting of three sets of measurements at different levels of PaCO2) were performed on 23 patients. At each level of PaCO2, the following measurements were made: xenon-enhanced computed tomography scans; cerebral blood flow; intracranial pressure; jugular venous bulb oxygen saturation; mean arterial pressure; and arterial oxygen saturation. Derived variables included: cerebral oxygen consumption; cerebral perfusion pressure; and oxygen extraction ratio. Cerebral blood flow decreased below normal after head injury (mean 49.6 +/- 14.6 mL/min/100 g). Cerebral oxygen consumption decreased out of proportion to the decrease in cerebral blood flow; cerebral oxygen consumption was only a third of the normal range (mean 1.02 +/- 0.59 mL/min/100 g). Neither cerebral blood flow nor cerebral oxygen consumption showed any relationship to time after injury, Glasgow Coma Score at the time of presentation, or intracranial pressure. The frequency of one or more regions of ischemia (defined as cerebral blood flow of18 mL/min/100 g) was 28.9% during normocapnia. This value increased to 73.1% for PaCO2 at25 torr.Severe head injury in children produced a modest decrease in cerebral blood flow but a much larger decrease in cerebral oxygen consumption. Absolute hyperemia was uncommon at any time, but measured cerebral blood flow rates were still above the metabolic requirements of most children. The clear relationship between the frequency of cerebral ischemia and hypocarbia, combined with the rarity of hyperemia, suggests that hyperventilation should be used with caution and monitored carefully in children with severe head injuries.

✓ At British Columbia's Children's Hospital, the criteria used in selective functional posterior rhizotomy (SFPR) evolved in three distinct phases. In Phase 1 the electrophysiological criteria for abnormality included a low threshold to a single stimulation, a sustained response to 50-Hz stimulation, and spread outside the segmental level being stimulated. In Phase 2 the electrophysiological criteria were unchanged, but fewer L3–4 nerve roots were cut. In Phase 3, fewer L3–4 nerve roots were cut, as in Phase 2, but based on the results of posterior nerve root stimulation in nonspastic controls, the only electrophysiological criterion used was contralateral and suprasegmental spread. The present study examined the relationship between the criteria used in each phase and patient outcome. The records of 77 consecutive children who underwent SFPR and had a minium follow-up period of 1 year were reviewed, comprising 25, 19, and 33 patients in Phases 1, 2, and 3, respectively. Outcome parameters included quantitative assessments of lower-limb spasticity and range of motion, and qualitative assessments of lower-limb function. In Phase 3, 52% of the nerve roots were cut, compared to 66% in Phases 1 and 2. In all three phases there was a significant decrease in lower-limb spasticity and an increase in range of movement, with the smallest decrease in spasticity in Phase 3. Over 90% of children in each phase improved with respect to lower-limb function, and excluding independent walkers and quadriplegics confined to a wheelchair, improvement in the level of ambulation occurred in 87.5%, 71.4%, and 73.7% of patients, in Phases 1, 2, and 3, respectively.

SUMMARY This study reports the use of intrathecal baclofen in two ventilator-dependent children with severe spasms secondary to spinal-cord injury. Baclofen was delivered via a subcutaneously implanted, programmable pump. The children were followed for 12 and 24 months. Baclofen dramatically reduced spasms, resulting in more stable ventilation, improved ease of care, reduced distress and better integration into the community. Although effective, intrathecal baclofen represents a significant intervention; careful consideration must be given to potential complications and the need for long-term management. Full effectiveness was dependent on free CSF flow. RESUME Traitement de spasmes severes par baclofene intrarachidien continu chez deux enfants presentant des lesions medullaires L'article rapporte l'utilisation de baclofene intrarachidien chez deux enfants en assistance respiratoire avec des spasmes severes secondares a des lesions medullaires. Le baclofene fut donne par une pompe programmable implantee en sous-cutane. Les enfants furent suivis durant 12 et 24 mois. Le baclofene diminua considerablement les spasmes, favorisant une respiration plus stable, facilitant les soins, diminuant l'anxiete et permettant une meilleure integration sociale. Bien qu'efficace, le baclofene intra-rachidien represente une intervention serieuse: une prise en compte soigneuse des complications possibles est indispensable, ainsi que la necessite d'une prise en charge a long terme. Le plein effet du traitement dependait d'une libre circulation du L.C.R. ZUSAMMENFASSUNG Kontinuierliche intrathekale Baclofentherapie bei zwei Kindern mit schweren Spasmen durch Ruckenmarksverletzungen Diese Studie berichtet uber intrathekale Backlofengaben bei zwei beatmeten Kindern mit schweren Spasmen durch Ruckenmarksverletzungen. Baclofen wurde uber eine subkutan implantierte programmierbare Pumpe appliziert. Die Kinder wurden 12 und 24 Monate kontrolliert. Durch Baclofen wurden die Spasmen dramatisch vermindert, sodas die Ventilation stabilisiert, die Pflege verbessert, die Atemnotsanfalle vermindert und die Integration in die Gemeinschaft gefordert wurden. Trotz seiner Effektivitat ist die intrathekale Applikation von Baclofen ein signifikanter Eingriff: mogliche Komplikationen und die Notwendigkeit einer langfristigen Behandlung mussen sorgfaltig bedacht werden. Die voile Wirkungsentfaltung konnte nur bei freier Liquorpassage erreicht werden. RESUMEN Tratamiento continuo con baclofen intratecal de los espasmos graves en dos ninos con lesion medular Este estudio aporta el uso de baclofen en dos ninos dependientes de ventilacion artificial, con espasmos graves secundarios a una lesion espinal. El baclofen se administro con una bomba programable de implantacion subcutanea. Los ninos fueron seguidos durante 12 y 24 meses. El baclofen redujo teatralment los espasmos, proporcionando una ventilacion mas estable, una mejoria en la comodidad de los cuidados y una reduccion del estress, con una mejor integracion en la comunidad. Aunque eficaz, el baclofen intratecal significa una intervencion importante. Deben considerarse con cuidado las eventuales complicaciones y la necesidad de un tratamiento largo. La eficacia completa dependia de la libertad de flujo del LCR.