Fatal Second Impact Syndrome in Rowan Stringer, A 17-Year-Old Rugby Player.

Second impact syndrome (SIS) is associated with malignant brain swelling and usually occurs in young athletes with one or more prior, recent concussions. SIS is rare and some dispute its existence. We report a case of SIS in Rowan Stringer, age 17, a rugby player who sustained a fatal brain injury despite prompt medical therapy including decompression surgery. The cause of the massive brain swelling was initially unknown. An inquest revealed Rowan's text messages to friends describing symptoms from two prior, recent rugby brain injuries, likely concussions, within 5 days of the fatal blow and confirming the diagnosis of SIS.

Infantile Myofibromatosis With Intracranial Extradural Involvement and PDGFRB Mutation: A Case Report and Review of the Literature.

Infantile myofibroma is a rare benign mesenchymal tumor that presents as solitary or multiple lesions (myofibromatosis) in the skin, soft tissue, bone, or internal organs. It most commonly affects the head and neck of infants and young children, but it can also affect adults. Intracranial involvement is reported to be extremely rare, and its clinical picture has been poorly characterized. Recently, it has been demonstrated that germline and somatic mutations in the platelet-derived growth factor receptor beta (PDGFRB) are associated with familial infantile myofibromatosis. We report a case of infantile myofibromatosis with predominant posterior fossa extradural involvement in a 14-year-old adolescent girl with a confirmed mutation in the PDGFRB gene.

Cranioplasties following craniectomies in children-a multicenter, retrospective cohort study.

OBJECTIVE: Complications following pediatric cranioplasty after craniectomy with either autologous bone flaps or cranial implants are reported to be common, particularly bone flap resorption. However, only sparse data are available regarding cranioplasty strategies, complications, and outcomes. This manuscript describes a Canadian-Dutch multicenter pediatric cohort study with autografts and cranial implant cranioplasties following craniectomies for a variety of indications. METHODS: The study included all children (< 18 years) who underwent craniectomy and subsequent cranioplasty surgeries from 2008 to 2014 (with a minimum of 1-year follow-up) at four academic hospitals with a dedicated pediatric neurosurgical service. Data were collected regarding initial diagnosis, age, time interval between craniectomy and cranioplasty, bone flap storage method, type of cranioplasty for initial procedure (and redo if applicable), and the postoperative outcome including surgical site infection, wound breakdowns, bone flap resorption, and inadequate fit/disfigurement. RESULTS: Sixty-four patients (46 males, average age 9.7 ± 5.5 years) were eligible for inclusion, with mean follow-up of 82.3 ± 31.2 months after craniectomy. Forty cranioplasties (62.5%) used autologous bone re-implant, 23 (57.5%) of which showed resorption. On average, resorption was documented at 434 days (range 62-2796 days) after reimplantation. In 20 cases, a revision cranioplasty was needed. In 24 of the post-craniectomy cases (37.5%), a cranial implant was used with one of ten different implant types. Implant loosening prompted a complete revision cranioplasty in 2 cases (8.3%). Cranial implants were associated with low morbidity and lower reoperation dates compared to the autologous cranioplasties. CONCLUSION: The most prominent finding in this multicenter cohort study was that bone flap resorption in children remains a common and widespread problem following craniectomy. Cranioplasty strategies varied between centers and evolved over time within centers. Cranial implants were associated with low morbidity and low reoperation rates. Still, longer term and prospective multicenter cohort studies are needed to optimize cranioplasty strategies in children after craniectomies.

Complications following pediatric cranioplasty after decompressive craniectomy: a multicenter retrospective study.

OBJECTIVE In children, the repair of skull defects arising from decompressive craniectomy presents a unique set of challenges. Single-center studies have identified different risk factors for the common complications of cranioplasty resorption and infection. The goal of the present study was to determine the risk factors for bone resorption and infection after pediatric cranioplasty. METHODS The authors conducted a multicenter retrospective case study that included all patients who underwent cranioplasty to correct a skull defect arising from a decompressive craniectomy at 13 centers between 2000 and 2011 and were less than 19 years old at the time of cranioplasty. Prior systematic review of the literature along with expert opinion guided the selection of variables to be collected. These included: indication for craniectomy; history of abusive head trauma; method of bone storage; method of bone fixation; use of drains; size of bone graft; presence of other implants, including ventriculoperitoneal (VP) shunt; presence of fluid collections; age at craniectomy; and time between craniectomy and cranioplasty. RESULTS A total of 359 patients met the inclusion criteria. The patients' mean age was 8.4 years, and 51.5% were female. Thirty-eight cases (10.5%) were complicated by infection. In multivariate analysis, presence of a cranial implant (primarily VP shunt) (OR 2.41, 95% CI 1.17-4.98), presence of gastrostomy (OR 2.44, 95% CI 1.03-5.79), and ventilator dependence (OR 8.45, 95% CI 1.10-65.08) were significant risk factors for cranioplasty infection. No other variable was associated with infection. Of the 240 patients who underwent a cranioplasty with bone graft, 21.7% showed bone resorption significant enough to warrant repeat surgical intervention. The most important predictor of cranioplasty bone resorption was age at the time of cranioplasty. For every month of increased age the risk of bone flap resorption decreased by 1% (OR 0.99, 95% CI 0.98-0.99, p < 0.001). Other risk factors for resorption in multivariate models were the use of external ventricular drains and lumbar shunts. CONCLUSIONS This is the largest study of pediatric cranioplasty outcomes performed to date. Analysis included variables found to be significant in previous retrospective reports. Presence of a cranial implant such as VP shunt is the most significant risk factor for cranioplasty infection, whereas younger age at cranioplasty is the dominant risk factor for bone resorption.

Endovascular glue embolization of a radiation-induced lenticulostriate artery pseudoaneurysm in a pediatric patient with optic pathway glioma: Case report and review of literature.

Radiation-associated vascular changes most commonly present in the form of stenosis, thrombosis and occlusion. However, development of intracranial aneurysms secondary to radiation is far less common and often manifests with rupture. These aneurysms are difficult to treat and associated with high morbidity and mortality when ruptured compared with saccular aneurysms unrelated to radiation treatment. Both surgical and endovascular options are available for treatment of these aneurysms. We present a young patient with a radiation-induced intracranial pseudoaneurysm arising from the lenticulostriate branch of the left middle cerebral artery (MCA); this developed 1 year 4 months after 59.4 Gy of focused radiation to the suprasellar pilomyxoid astrocytoma. The patient successfully underwent endovascular glue embolization of the aneurysm and occlusion of the lenticulostriate artery after unsuccessful trapping of the aneurysm and occlusion of the parent artery using coils. She developed transient hemiparesis of the right side following the procedure, which was managed conservatively. We performed a complete review of the literature dealing with the radiation-induced intracranial aneurysms, their presentation, treatment and outcome.

Traumatic spinal injuries in children at a single level 1 pediatric trauma centre: report of a 23-year experience.

BACKGROUND: With a reported incidence of up to 10% compared to all spinal trauma, spinal injuries in children are less common than in adults. Children can have spine fractures with or without myelopathy, or spinal cord injuries without radiological abnormalities (SCIWORA). METHODS: We retrospectively reviewed the cases of children with spinal injuries treated at a level 1 pediatric trauma centre between 1990 and 2013. RESULTS: A total of 275 children were treated during the study period. The mean age at admission was 12 ± 4.5 years, and the male:female ratio was 1.4:1. Spinal injuries were more common in children of ages 12-16 years, with most injuries among ages 15-16 years. The top 3 mechanisms of spinal injury were motor vehicle-related trauma (53%), sports (28%) and falls (13%). Myelopathy occurred in 12% and SCIWORA occurred in 6%. The most common spine levels injured were L2-sacrum, followed by O-C2. Associated injuries, including head injuries (29%), and fractures/dislocations (27%) occurred in 55% of children. Overall mortality was 3%. Surgical intervention was required in 14%. CONCLUSION: The creation of a pediatric spinal injury database using this 23-year retrospective review helped identify important clinical concepts; we found that active adolescent boys had the highest risk of spine injury, that noncontiguous spine injuries occured at a rate higher than reported previously and that nonaccidental spine injuries in children are underreported. Our findings also emphasize the importance of maintaining a higher index of suspicion with trauma patients with multiple injuries and of conducting detailed clinical and radiographic examinations of the entire spine in children with a known spinal injury.

CONTEXTE: Les traumatismes médullaires sont moins fréquents chez l'enfant que chez l'adulte, avec une incidence d'au plus 10 % de tous les cas déclarés. Les enfants peuvent subir des fractures de la colonne vertébrale avec ou sans myélopathie, ou un traumatisme médullaire sans anomalie radiologique visible (SCIWORA). MÉTHODES: Nous avons effectué une analyse rétrospective des cas d'enfants atteints d'un traumatisme médullaire admis dans un centre de traumatologie spécialisé en pédiatrie de niveau 1 entre 1990 et 2013. RÉSULTATS: Au total, 275 enfants ont été traités durant la période de l'étude. L'âge moyen au moment de l'hospitalisation était de 12 ± 4,5 ans, et le ratio garçons:filles était de 1,4:1. Les adolescents de 12 à 16 ans formaient le groupe le plus représenté, et l'incidence de traumatisme la plus élevée a été observée chez les jeunes de 15 et 16 ans. Les 3 mécanismes lésionnels les plus fréquents étaient les accidents de la route (53 %), la pratique d'un sport (28 %) et les chutes (13 %). Le taux d'incidence de la myélopathie était de 12 %, et celui du SCIWORA, de 6 %. La section du rachis touchée le plus fréquemment s'étendait de L2 au sacrum, les vertèbres entre l'occiput et C2 arrivant au deuxième rang. Des lésions concomitantes, dont des blessures à la tête (29 %) et des fractures et luxations (27 %), ont été observées chez 55 % des enfants. Le taux global de mortalité était de 3 %. L'intervention chirurgicale a été nécessaire chez 14%. CONCLUSION: La création à partir de cette étude rétrospective d'une base de données sur les traumatismes médullaires chez l'enfant a mis en évidence quelques constatations cliniques dignes d'intérêt : les garçons adolescents actifs présentent le risque le plus élevé de traumatisme médullaire; les lésions médullaires non contiguës surviennent plus fréquemment que ce qui avait été rapporté précédemment; les traumatismes médullaires non accidentels chez l'enfant sont sous-déclarés. Nos conclusions viennent aussi souligner l'importance de maintenir un indice de suspicion élevé dans le cas de patients atteints de lésions multiples et d'effectuer un examen clinique et radiographique détaillé de la totalité de la colonne vertébrale des enfants ayant une lésion médullaire connue.

Pediatric thalamic tumors in the MRI era: a Canadian perspective.

BACKGROUND: Thalamic gliomas are rare. The natural history is unpredictable, and the optimal management of these tumors in children is poorly defined. The aim was to identify outcomes, prognostic factors, and response to various modalities of treatment in a relatively large population of pediatric thalamic tumors from many centers within a fairly homogeneous health care system. METHODS: We performed a Canadian multicenter retrospective review of pediatric thalamic tumors presenting during the MRI era (1989-2012). Radiology and pathology were reviewed by central independent reviewers. Paraffin shavings for RNA extraction were taken and tested for fusion events involving KIAA1549:BRAF. Tumors were classified as unilateral or bithalamic based on their origin on imaging. Univariate and multivariate analyses on factors influencing survival were performed. RESULTS: Seventy-two thalamic tumors were identified from 11 institutions. Females represented 53% of the study population, and the mean age at presentation was 8.9 years. Sixty-two tumors were unilateral and 10 bithalamic. Unilateral tumors had a greater propensity to grow inferiorly towards the brainstem. These tumors were predominantly low grade in comparison to bithalamic tumors which were high-grade astrocytomas. The 5-year overall survival was 61 ± 13% for unithalamic tumors compared to 37 ± 32% for bithalamic tumors (p = 0.097). Multivariate analysis indicated tumor grade as the only significant prognostic factor for unithalamic tumors. Six unilateral tumors, all low grade, were BRAF fusion positive. CONCLUSION: Unilateral and bilateral thalamic tumors behave differently. Surgical resection is an appropriate treatment option in unilateral tumors, most of which are low grade, but outcome is not related to extent of resection (EOR). Bilateral thalamic tumors have a poorer prognosis, but the occasional patient does remarkably well. The efficacy of chemotherapy and radiotherapy has not been clearly demonstrated. Novel therapeutic approaches are required to improve the prognosis for malignant unilateral thalamic tumors and bilateral thalamic tumors.

Management of abdominal pseudocyst in shunt-dependent hydrocephalus.

BACKGROUND: Abdominal pseudocyst (APC) is an uncommon manifestation of a ventriculoperitoneal (VP) shunt that is attributed to an inflammatory response, usually the result of infection. CASE DESCRIPTION: A 13-year-old girl with a VP shunt presented with progressive abdominal distention, pain and vomiting. The shunt was inserted at infancy for congenital hydrocephalus. A shunt infection was treated with externalization of the shunt, antibiotics and subsequent shunt replacement. At the age of four, the shunt was revised for a distal malfunction. Nine years later, abdominal CT and ultrasound demonstrated large multiseptated cysts. The shunt was externalized and 1.8 L of sterile, xanthochromic peritoneal fluid was drained. The cerebrospinal fluid was clear, colorless, acellular and sterile with normal protein and glucose levels. Two days later, the distal portion of the shunt was replaced back into the pleural cavity. Five months later a pleural effusion formed. Thoracentesis was performed and there was no evidence of infection. The shunt was subsequently converted to a ventriculoatrial system. The patient has remained well for over 3.5 years. CONCLUSION: APC represents an important complication of VP shunts, with an unclear etiology that can occur nine years after shunt surgery. This paper presents an update on the management of APCs.

Retethering in children after sectioning of the filum terminale.

BACKGROUND/AIM: Sectioning of the filum terminale is performed when spinal cord tethering is suspected, sometimes without clinical symptoms. Retethering can occur and require reoperation due to the presentation of either recurrent or new symptoms. The purpose of this institutional review was to identify the retethering rate in children, especially in those who were initially asymptomatic, and to discuss the role of surgery. METHODS: The medical records of all children at the Children's Hospital of Eastern Ontario (CHEO) who underwent tethered cord surgery between 1978 and 2009 for a thickened filum terminale were retrospectively reviewed, as well as those who retethered. RESULTS: A total of 146 patients with a mean age of 4.3 years underwent a low lumbar single or partial laminectomy for sectioning of the filum terminale; 44 patients (30.1%) were asymptomatic at the time of surgery, 51.4% had bladder and bowel dysfunction, 26.7% had neuroorthopedic findings, 15.8% had pain and 6.2% had progressive scoliosis; 11 children with a median age of 8.9 years had symptoms of retethering requiring reoperation (median time to retether was 4.3 years) and 4 were initially asymptomatic. Repeat surgery was successful at alleviating the new symptoms that occurred as a result of retethering. CONCLUSIONS: Of the 146 patients at CHEO who underwent surgery, 7.5% retethered, with 36% being initially asymptomatic. Those operated in the first year of life were not found to be at a higher risk. The level of the conus medullaris did not influence the rate or retethering or urological dysfunction. Children who were initially asymptomatic improved after surgery for retethering, but may not have required surgery in the first place.

Extensive central nervous system involvement in optic pathway gliomas in neurofibromatosis type 1.

Optic pathway gliomas (OPG) in neurofibromatosis type 1 (NF1) usually remain localized to the anterior visual pathway. However, a small number can demonstrate widespread dissemination. We describe three children with NF1 OPGs and extensive central nervous system involvement. In one case, a postmortem examination revealed tumor cells extending continuously from the optic nerves to the conus medullaris. This is the most widespread NF1 OPG reported in the literature. We suggest that rapid visual deterioration in the absence of radiographic changes or increased intracranial pressure can be caused by increasing tumor infiltration within the central nervous system.

The necessity of shunt series.

OBJECT: This work assessed the value of shunt series in the evaluation of children with CSF shunts, a test that is frequently ordered as part of the assessment of shunt integrity. METHODS: The medical records of all children who underwent shunt series at Children's Hospital of Eastern Ontario between 1975 and 2007 were reviewed. Ancillary studies that were performed within 2 days of the shunt series (brain CT, MR imaging, ultrasonography, and radionuclide shuntography) were noted, as well as the subsequent requirement for a shunt revision. Shunt series and ancillary studies were categorized as either positive (revealing an observable sign that a shunt-related problem was present, such as shunt discontinuity on the shunt series or enlargement of the ventricles on the brain CT scan) or negative (no clear sign of a shunt-related problem). Shunt series were further grouped into shunt series performed for diagnostic reasons, shunt series performed on a routine basis during follow-up in clinic, and shunt series performed postoperatively. The length of time between shunt insertion and shunt fracture was noted. Statistical analyses were performed, including the derivation of sensitivities and specificities. RESULTS: There were 3416 shunt series in 394 patients, of which 3004 were performed with ancillary studies. On average, patients underwent 9 shunt series, with a minimum of 1 and a maximum of 43, during the follow-up period (range 3 weeks to 19 years). A total of 2493 shunt series and ancillary studies (83%) yielded negative results and no surgery was performed. One hundred thirty shunts series were negative with a positive ancillary study (4.3%) and no surgery was required. In 8.7% (261 shunt series negative, ancillary studies positive) shunt revision surgery was necessary. The opposite trend was far less prevalent (17 shunt series positive, ancillary test negative; 0.6%) in which surgery was required. There were 87 patients with 96 shunt fractures (2.8% of shunt series). The average time between shunt insertion and shunt fracture was 7.9 years (range 3 months to 18 years). Shunt series had a sensitivity of 18% and a specificity of 97%. The ancillary studies had a sensitivity of 84% and a specificity of 85%. CONCLUSIONS: The routine utilization of shunt series in the evaluation of a child with a CSF shunt is not necessary. This study showed that a very small number (0.6%) of shunt series helped in surgical decision making. Shunt series can be performed in selected cases, especially preoperatively in the absence of a baseline study to obtain information necessary for surgical planning.

Classification of symptomatic Chiari I malformation to guide surgical strategy.

BACKGROUND: Treatment options for Chiari I malformations include posterior fossa decompression (PFD) with additional techniques including laminectomy, intradural exploration, and duraplasty. Neuroimaging findings of cisterna magna volume, syringomyelia, and intraoperative ultrasonography may tailor surgical intervention. METHODS: We developed an algorithm classifying symptomatic Chiari I patients into three groups to define minimum operation. Without syringomyelia, the presence of cisterna magna defined Group A and the absence defined Group B. Patients with syrinx formed Group C. Mild structural pathology (Group A) or adequate space following PFD (Group B, normal intraoperative ultrasound (IOUS)) should be treated by PFD alone. Conversely, presence of syringomyelia (Group C) or inadequate space following PFD (Group B, abnormal IOUS) should additionally have duraplasty. We applied this algorithm to patients treated at a single institution over 16 years. RESULTS: Twenty-four symptomatic Chiari I malformation patients were divided into three groups that did not differ by age, gender, or extent of tonsillar ectopia. All patients treated by this algorithm experienced clinical and radiographic improvement. This included eight Group B patients who underwent PFD only (n=6) or additional duraplasty (n=2) decided by IOUS. CONCLUSION: Treatment of symptomatic Chiari I malformation may have inadequate outcome with conservative strategy or complications with aggressive strategy. This algorithm utilizes preoperative neuroimaging and intraoperative ultrasound to tailor intervention, with excellent clinical outcome and radiographic syrinx resolution on application to 24 patients. Further validation requires prospective multicenter evaluation with larger patient population.

Postoperative surveillance magnetic resonance imaging for cerebellar astrocytoma.

INTRODUCTION: Patients with low grade astrocytomas generally have good prognosis when total resection can be achieved, but surveillance neuroimaging is commonly performed to detect recurrence or progression. This study evaluated the utility and yield of such strategy for pilocytic and non-pilocytic cerebellar astrocytomas. METHODS: A 20-year retrospective review was performed of patients undergoing resection of cerebellar astrocytoma at a single institution. A negative MRI string (NMS) ratio was computed as the fraction of total follow-up period over which surveillance neuroimaging was negative for recurrence or progression. Chi-squared analysis differentiated NMS ratio by resection extent and lesion histopathology. RESULTS: Twenty-eight patients with pilocytic (n=15) and non-pilocytic (n=13) astrocytoma underwent 34 craniotomies, with total resection in 19 cases. Surveillance MRIs (n=167) among total resection patients were uniformly negative for recurrent disease at average seven years follow-up (NMS ratio = 1.0). The 43 surveillance MRIs among subtotal resection patients revealed disease progression in two patients within six months of operation (NMS ratio = 0.78, p<0.05). No differences in NMS ratio were observed between pilocytic and non-pilocytic astrocytoma subtypes. DISCUSSION: This study illustrates pediatric patients with low-grade cerebellar astrocytomas undergoing total resection may not benefit from routine surveillance neuroimaging, primarily because of low recurrence likelihood. Patients with subtotal resection may benefit from surveillance of residual disease, with further work aimed at exploring the schedule of such follow-up.

Pediatric traumatic dural arteriovenous fistula.

BACKGROUND: Dural arteriovenous fistulas are uncommon lesions in children, with traumatic lesions suspected to arise from incomplete arterial injury in proximity to a vein or draining sinus. Management of symptomatic acquired lesions requires evaluation of patient presentation, neurological status, and pathoanatomic configuration, with special consideration required for surgery secondary to failed endovascular technique. CASE REPORT: A 12-year-old male sustained a bicycle fall causing a right temporo-parietal skull fracture associated with non-surgical right epidural hematoma and left contre-coup parietal contusion. Six-weeks later, he complained of a right temporal bruit with subsequent cerebral angiography demonstrating a dural-based fistula between the right middle meningeal artery and a dural vein draining into the sigmoid sinus. INTERVENTION: Endovascular treatment of this lesion with glue embolization and coiling was unsuccessful, with angiographic illustration of previously unobserved collateral vessels and coils occupying the sigmoid sinus. A right temporo-parietal craniectomy was required to excise the dural-based fistula, followed by dural defect repair with bovine pericardium and subsequent cranioplasty. Six years later the patient remains neurologically intact with no headaches or bruit. CONCLUSIONS: Dural arteriovenous fistula can uncommonly occur following traumatic injury in children. Partial injury to the middle mengineal artery may have established arterial communication with the draining vein that became ectatic and tortuous under high pressure. Failure of primary endovascular treatment may complicate secondary surgical intervention.

Congenital tumors of the central nervous system: the MCH experience.

INTRODUCTION: Congenital brain tumors in the younger pediatric population are rare lesions that are histologically distinct from those in the older pediatric population. Malignant histology is common, with persistently poor outcomes despite accessible neuroimaging and evolving adjuvant therapy. There remains scant literature about the natural history of these patients because of rarity and varied institutional experiences. METHODS: A retrospective review was performed of congenital brain tumor patients surgically treated at the Montreal Children's Hospital (MCH) over a 22-year period. Patients presenting in the first year of life were evaluated for demographic information, presenting symptoms, lesion location, and management. Analysis was by median rank test and chi(2) statistics. RESULTS: 13 cases of congenital brain tumors were identified: 5 supratentorial and 8 infratentorial. Median age (p = 0.93) and gender (p = 0.57) did not differ by location, and predominant histologies were choroid plexus papilloma and primitive neuroectodermal tumor. Seizure activity was exclusive to supratentorial lesions (40%, p = 0.03), with hypotonia observed only among infratentorial lesions (50%, p = 0.02). There was equal incidence of hydrocephalus (69%, p = 0.57) and increasing head circumference (38%, p = 0.27) by lesion location. Supratentorial lesions were treated by total resection (n = 3), subtotal resection (n = 1), and biopsy (n = 1). Infratentorial lesions were treated by total resection (n = 1), subtotal resection (n = 2), biopsy (n = 1), no operation (n = 2), and decompressive laminectomy for two spinal lesions. CONCLUSIONS: Congenital brain tumor patients represent fewer than 2% of patients treated at MCH. An evolving understanding of management objectives for these lesions requires understanding institutional experiences. Patients with supratentorial lesions frequently present with seizures, hydrocephalus, and macrocrania, and more frequently underwent total resection at surgery.

Intracranial tumors in first year of life: the CHEO experience.

INTRODUCTION: One seventh of pediatric brain tumors are diagnosed in the first year of life. With more widespread and accessible neuroimaging, these lesions are being diagnosed earlier, but there remains scant literature about their natural history. METHODS: A retrospective review was performed of brain tumor patients presenting to the Children's Hospital of Eastern Ontario (CHEO) through the last 34 years. Patients presenting in the first year of life, including symptoms, management features, and functional outcome, were analyzed using ANOVA and chi (2) statistics. RESULTS: Eighteen cases of brain tumors in the first year of life were identified: 12 suptratentorial, eight with benign histology, and six infratentorial all with malignant histology. Median age of presentation differed by lesion location (p = 0.05) and glial tumors were most common. Raised intracranial pressure was more than twice as prevalent with posterior fossa lesions (p < 0.01) with equivalent likelihood of increasing head circumference (p = 0.74), whereas seizures were more frequent with supratentorial tumors (p = 0.04). Gross total resection was achieved in 47% of patients, cerebrospinal fluid diversion was more frequently necessary among infratentorial lesions (p = 0.02), and adjuvant therapy was more utilized for infratentorial lesions (p < 0.01). Among eight surviving infants, seven had supratentorial tumors, five survived to adulthood, and six are functionally independent. CONCLUSIONS: Brain tumors in the first year of life represent 4.8% of patients treated at CHEO. Mode of presentation, utilization of adjuvant therapy, and survival depend on tumor location and histology, with worse prognosis for infratentorial lesions. One third of patients had acceptable functional outcome requiring no special assistance.

Vascular complications of cranial radiation.

OBJECTIVES: Cerebral vascular disease has been reported as a long-term complication of cranial radiotherapy. The purpose of this study was to examine the frequency and risk factors associated with development of cerebral vascular disease in children after cranial radiation. MATERIALS AND METHODS: A retrospective chart review of all cancer patients treated between 1985 and 2003 who were under the age of 18 years at the time of initial radiotherapy was performed. Variables examined include diagnosis and site of malignancy, age at the time of radiotherapy, sex, total radiation dosage, number of fractions, duration, and whether the patient had proven cerebral vascular event. RESULTS: Two hundred and forty-four patients met the study criteria. One hundred and 13 cases involved tumors of the central nervous system. The remaining patients had systemic neoplastic disease. Post radiation cerebral vascular disease occurred in 11 (5%) patients, and all but one patient had a tumor involving the central nervous system (mainly in the posterior fossa and supratentorial midline). CONCLUSION: There is an increased risk of cerebral vascular disease after radiation therapy in childhood, especially in children who received high dose radiation at the posterior fossa and supratentorial axial region.