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Objective: Extent of resection (EOR) is the most important modifiable prognostic variable for pediatric patients with posterior fossa ependymoma. An understanding of primary and recurrent ependymoma complications is essential to inform clinical decision-making for providers, patients, and families. In this study, the authors characterize postsurgical complications following resection of primary and recurrent pediatric posterior fossa ependymoma in a molecularly defined cohort., Methods: The authors conducted a 20-year retrospective single-center review of pediatric patients undergoing resection of posterior fossa ependymoma at the Hospital for Sick Children in Toronto, Canada. Complications were dichotomized into major and minor groups; EOR was compared across complication categories. The association between complication occurrence with length of stay (LOS) and mortality was also assessed using multivariable regressions., Results: There were 60 patients with primary resection included, 41 (68%) of whom were alive at the time of data collection. Gross-total resection was achieved in 33 (58%) of 57 patients at primary resection. There were no 30-day mortality events following primary and recurrent ependymoma resection. Following primary resection, 6 patients (10%) had posterior fossa syndrome (PFS) and 36 (60%) developed cranial neuropathies, 56% of which recovered within 1 year. One patient (1.7%) required a tracheostomy and 9 patients (15%) required gastrostomy tubes. There were 14 ventriculoperitoneal shunts (23%) inserted for postoperative hydrocephalus. Among recurrent cases, there were 48 recurrent resections performed in 24 patients. Complications included new cranial neuropathy in 10 patients (21%), of which 5 neuropathies resolved within 1 year. There were no cases of PFS following resection of recurrent ependymoma. Gastrostomy tube insertion was required in 3 patients (6.3%), and 1 patient (2.0%) required a tracheostomy. Given the differences in the location of tumor recurrence, a direct comparison between primary and recurrent resection complications was not feasible. Following multivariate analysis adjusting for sex, age, molecular status, and EOR, occurrence of major complications was found to be associated with prolonged LOS but not mortality., Conclusions: These results detail the spectrum of postsurgical morbidity following primary and recurrent posterior fossa ependymoma resection. The crude complication rate following resection of infratentorial recurrent ependymoma was lower than that of primary ependymoma, although a statistical comparison revealed no significant differences between the groups. These results should serve to inform providers of the morbidity profile following surgical management of posterior fossa ependymoma and inform perioperative counseling of patients and their families.

Objective: The objective of this study was to describe the management of hydrocephalus in a cohort of pediatric patients with germinoma., Methods: The authors conducted a retrospective chart review of patients with germinoma and symptomatic hydrocephalus treated at the Hospital for Sick Children between 2002 and 2020. Descriptive data included tumor location, CSF diversion procedure (external ventricular drain [EVD], endoscopic third ventriculostomy [ETV], ventriculoperitoneal [VP] shunt) and outcomes. The frontooccipital horn ratio (FOR) method was used to determine the presence of ventriculomegaly., Results: Of 39 patients with germinoma, 22 (73% male) had symptomatic hydrocephalus at diagnosis (11 pineal, 4 suprasellar, and 7 bifocal). Management of hydrocephalus included EVD (n = 5, 22.7%), ETV (n = 5, 22.7%), and combination ETV and EVD (n = 7, 31.8%), whereas 5 patients (22.7%) did not undergo surgical intervention. The median FOR at diagnosis was 0.42 (range 0.38-0.58), which correlated with moderate to severe ventriculomegaly. Carboplatin and etoposide-based chemotherapy induced fast tumor shrinkage, avoiding CSF diversion (n = 5) and resolving hydrocephalus with a transient EVD (n = 5). The median duration until EVD removal was 7 days (range 2-10 days). Two of 12 patients with EVD ultimately required a VP shunt. Kaplan-Meier overall survival was 100% and progression-free survival was 96.4% at a median follow-up of 10.4 years., Conclusions: Timely initiation of chemotherapy is imperative to rapidly reduce tumor bulk in children with germinoma and limits the need for VP shunt insertions. In children in whom CSF diversion is required, hydrocephalus may be successfully managed with a temporary EVD ± ETV.

Objective: Torcular dural sinus malformations (tDSMs) are rare pediatric cerebrovascular malformations characterized by giant venous lakes localized to the midline confluence of sinuses. Historical clinical outcomes of patients with these lesions were poor, though better prognoses have been reported in the more recent literature. Long-term outcomes in children with tDSMs are uncertain and require further characterization. The goal of this study was to review a cohort of tDSM patients with an emphasis on long-term outcomes and to describe the treatment strategy., Methods: This study is a single-center retrospective review of a prospectively maintained data bank including patients referred to and cared for at The Hospital for Sick Children for tDSM from January 1996 to March 2019. Each patient's clinical, radiological, and demographic information, as well as their mother's demographic information, was collected for review., Results: Ten patients with tDSM, with a mean follow-up of 58 months, were included in the study. Diagnoses were made antenatally in 8 patients, and among those cases, 4 families opted for either elective termination (n = 1) or no further care following delivery (n = 3). Of the 6 patients treated, 5 had a favorable long-term neurological outcome, and follow-up imaging demonstrated a decrease or stability in the size of the tDSM over time. Staged embolization was performed in 3 patients, and anticoagulation was utilized in 5 treated patients., Conclusions: The authors add to a growing body of literature indicating that clinical outcomes in tDSM may not be as poor as initially perceived. Greater awareness of the lesion's natural history and pathophysiology, advancing endovascular techniques, and individualized anticoagulation regimens may lead to continued improvement in outcomes.

Objective: Pediatric rolandic arteriovenous malformations (AVMs) present a treatment challenge given the lifetime risk of hemorrhage, rehemorrhage, and associated long-term morbidity. Microsurgical resection has been recommended as the optimal treatment for AVMs in general, but there is no dedicated literature on the outcomes of resection of pediatric rolandic AVMs. Here, the study objective was to review the outcomes of microsurgical resection of pediatric rolandic AVMs in the modern era, together with the utilization of surgical adjuncts including navigation, intraoperative angiography, and neurophysiological monitoring., Methods: The authors performed a retrospective review of patients 18 years of age and younger with cerebral AVMs microsurgically treated between January 2000 and May 2016 at The Hospital for Sick Children. Only those patients with an AVM whose nidus was located within the rolandic region were analyzed. A descriptive analysis was performed to identify patient demographics, preoperative AVM characteristics, and postoperative obliteration rates and neurological complications., Results: A total of 279 AVMs were evaluated in the study period. Twenty-three of these AVMs were rolandic, and the median age in the 11 microsurgically treated cases was 11 years (range 1-17 years). AVM hemorrhage was the most common presentation, occurring in 8 patients (73%). Lesions were either Spetzler-Martin grade II (n = 8, 73%) or grade III (n = 3, 27%). The postoperative obliteration rate of AVMs was 100%. The mean imaging follow-up duration was 33 months (range 5-164 months). There was no documented recurrence of an AVM during follow-up. One patient developed a transient postoperative hemiparesis, while another patient developed right fingertip hyperesthesia., Conclusions: Microsurgical resection of rolandic pediatric AVMs yields excellent AVM obliteration with minimal neurological morbidity in selected patients. The incorporation of surgical adjuncts, including neurophysiological monitoring and neuronavigation, allows accurate demarcation of functional cortex and enables effective resection.

Objective: There are little data in the literature on the characteristics and natural history of unruptured intracranial aneurysms in children. The authors analyzed their experience with unruptured intracranial aneurysms in the pediatric population at their tertiary care pediatric institution over the last 18 years. The first objective was to assess the imaging characteristics and natural history of these aneurysms in order to help guide management strategies in the future. A second objective was to evaluate the frequency of an underlying condition when an incidental intracranial aneurysm was detected in a child., Methods: The authors conducted a Research Ethics Board-approved retrospective review of incidental intracranial aneurysms in patients younger than 18 years of age who had been treated at their institution in the period from 1998 to 2016. Clinical (age, sex, syndrome) and radiological (aneurysm location, type, size, thrombus, mass effect) data were recorded. Follow-up imaging was assessed for temporal changes., Results: Sixty intracranial aneurysms occurred in 51 patients (36 males, 15 females) with a mean age of 10.5 ± 0.5 years (range 9 months-17 years). Forty-five patients (88.2%) had a single aneurysm, while 2 and 3 aneurysms were found in 3 patients each (5.8%). Syndromic association was found in 22 patients (43.1%), most frequently sickle cell disease (10/22 [45.5%]). Aneurysms were saccular in 43 cases (71.7%; mean size 5.0 ± 5.7 mm) and fusiform in the remaining 17 (28.3%; mean size 6.5 ± 2.7 mm). Thirty-one aneurysms (51.7%) arose from the internal carotid artery (right/left 1.4), most commonly in the cavernous segment (10/31 [32.3%]). Mean size change over the entire follow-up of 109 patient-years was a decrease of 0.6 ± 4.2 mm (range -30.0 to +4.0 mm, rate -0.12 ± 9.9 mm/yr). Interval growth (2.0 ± 1.0 mm) was seen in 8 aneurysms (13.3%; 4 saccular, 4 fusiform). An interval decrease in size (8.3 ± 10.7 mm) was seen in 6 aneurysms (10%). There was an inverse relationship between aneurysm size and growth rate (r = -0.82, p < 0.00001). One aneurysm was treated endovascularly with internal carotid artery sacrifice., Conclusions: Unruptured pediatric intracranial aneurysms are most frequently single but can occur in multiples in a syndromic setting. None of the cases from the study period showed clinical or imaging signs of rupture. Growth over time, although unusual and slow, can occur in a proportion of these patients, who should be identified for short-term imaging surveillance.

OBJECTIVE The etiology of jugular bulb stenosis (JBS) or occlusion in the context of vein of Galen aneurysmal malformations (VGAMs) is unknown. It can lead to decompensation of a lesion that was previously clinically stable. The aim of this study was to describe the natural history of JBS or occlusion in VGAM and to determine whether there is an association with bony remodeling of the jugular foramina. METHODS The authors identified all cases of JBS greater than 70% bilaterally involving patients seen at The Hospital for Sick Children between January 2007 and June 2014. The foramen diameters were measured on sagittal CT imaging, on a slice passing at the level of the jugular vein. The jugular foramen diameters were also compared to measurements obtained in a matched population of the same age group who had no VGAM and had undergone cerebral CT for a reason other than vascular disease. RESULTS Eight patients (6 male and 2 female) with bilateral JBS were included in this series. The median duration of clinical follow-up was 2.5 years (IQR 1.7-4.2 years). JBS was associated with bony narrowing the jugular foramina in 7 of the 8 patients over time. Between 1 and 2 years of age, patients with a VGAM demonstrated jugular foramen narrowing in comparison with a matched population (p = 0.015). CONCLUSIONS Jugular bulb stenosis or occlusion in VGAM may be associated with narrowing of the jugular foramina. These conditions seem to have a male predominance. If treatment is required, bony narrowing of the jugular foramina should be taken into account when deciding whether angioplasty and stent placement or surgical bypass might be appropriate therapeutic options.

Clinical and imaging manifestations of the so-called partially thrombosed aneurysm (PTA) are different from those of the classic intracranial saccular aneurysm. Given some of their peculiar imaging features, it had been hypothesized that some PTAs occur due to repeated intramural hemorrhages. The authors present a case of PTA that evolved from an acute dissecting aneurysm as shown by serial imaging. A previously healthy 5-year-old boy had a sudden onset of left hemiparesis. Initial MRI sequences showed a perforating vessel infarction in the right basal ganglia area secondary to an acute distal middle cerebral artery (MCA) dissection as demonstrated on conventional angiography. Conservative management with close observation of this dissection was chosen, and serial MRI studies revealed layering of blood of various ages within the wall of an aneurysmal outpouching of the MCA, thereby leading to the imaging appearance of a PTA. The findings in this case indicate that some PTAs may be caused by repeated or chronic dissections, with blood entering the wall through an endothelial defect. Understanding the pathological mechanism underlying the formation of these aneurysms will help inform appropriate treatment strategies.

Traumatic brain injuries afflict a large number of pediatric patients. The most severe injuries lead to increased intracranial pressure and herniation, with resultant changes in the brainstem. Traumatic brainstem hemorrhages have previously been associated with poor neurological outcome and fatality. However, this report discusses 2 pediatric patients who sustained severe head trauma with subsequent brainstem hemorrhages, and yet experienced good neurological outcome; the possible mechanism is described.

Object: Neurenteric cysts (NCs) are endothelium-lined structures of presumed endodermal origin. There have been few pediatric series of intracranial NCs reported previously. The authors present their experience in the management of these lesions., Methods: A retrospective chart review of all cases of NCs identified between 1977 and 2007 was carried out. Demographics, details of clinical presentation, surgical therapy, and outcome data were extracted., Results: Eleven cases were identified, involving 6 girls and 5 boys. The patients' average age was 4.6 years (range 1 day-14 years). Limb weakness was the most common presenting symptom. The location of the cysts was cervical in 2 cases, cervicothoracic in 4 cases, and thoracic in 3 cases. One cyst was anterior to the pons with extension to the left cerebellopontine angle and 1 cyst was at the craniocervical junction. Five patients had an apparently complete initial excision, with 1 recurrence. Four patients were initially treated with incomplete excision and/or cyst drainage, usually into an Ommaya reservoir. A neonate with a large cervicothoracic cyst died of multiple congenital anomalies without any intervention. All other patients were alive at last follow-up. One patient with a radiologically identified presumed cyst, which remained stable, was simply followed up. Several patients required multiple procedures and 1 patient developed hydrocephalus after aseptic meningitis and was treated with CSF shunting., Conclusions: Neurenteric cysts are rare in the pediatric population. Total resection, if possible, provides the best long-term outcome. Incomplete excision may lead to multiple procedures, including cyst drainage, but can lead to long-term stability.

Arteriovenous malformations (AVMs) can lead to distortion or reorganization of functional brain anatomy, making localization of eloquent white matter tracts challenging. To improve the accuracy of corticospinal tract (CST) mapping, recent studies have examined the use of functional imaging techniques to help localize cortical motor activations and use these as seed points to reconstruct CSTs using diffusion-tensor imaging (DTI). The authors examined the role of pretreatment functionally guided DTI CST mapping in 3 children with ruptured AVMs. In 2 patients, magnetoencephalography motor activations were adjacent to the nidus and/or hemorrhagic cavity. However, in 1 child, functional MRI motor activations were detected in both hemispheres, suggestive of partial transfer of cortical motor function. In all children, quantitative analysis showed that fractional anisotropy values and fiber density indices were reduced in the CSTs of the hemisphere harboring the AVM compared with the unaffected side. In 2 children, CST caliber was slightly diminished, corresponding to no motor deficit in 1 patient and a temporary motor deficit in the other. In contrast, 1 child demonstrated marked reduction and displacement of the CSTs, correlating with severe motor deficit. Preoperative motor tractography data were loaded onto the intraoperative neuronavigation platform to guide complete resection of the AVM in 2 cases without permanent neurological deficits. These preliminary results confirm the feasibility of CST mapping in children with ruptured AVMs using functionally guided DTI tractography. Prospective studies are needed to assess the full value of this technique in the risk stratification, prognosis, and multimodality management of pediatric AVMs.

Object: Extraaxial infections of the CNS, including subdural empyema and epidural abscess, are rare but potentially life-threatening conditions. Symptoms are usually progressive, and early diagnosis is therefore important. Early intervention with appropriate treatment offers the best opportunity for eradicating the infection and promoting maximal neurological recovery., Methods: The medical records of children with extraaxial CNS infection over the last 24 years at the Hospital for Sick Children were analyzed. Only those patients with radiological and/or operative confirmation of the diagnosis of subdural empyema or epidural abscess were included in the study. Demographic and clinical data were collected to determine the outcomes after such infections and factors that predict for such outcomes., Results: The authors identified 70 children who fulfilled the inclusion criteria. Sinusitis was the most common etiology and was seen in 38 patients. All of these patients were older than 7 years of age at diagnosis. Subdural empyemas were diagnosed in 13 patients following bacterial meningitis, and they were found primarily in infants within the 1st year of life. Other etiological factors included otogenic infection (4 cases), postneurosurgical infection (7 cases), and hematogenous spread of infection (7 cases including 6 cases of spinal epidural abscess). Streptococcus anginosus and Staphylococcus aureus were the most common pathogens identified. Sixty-four patients (91.4%) underwent at least 1 neurosurgical procedure. Seizures and cerebral edema from cortical vein thrombosis were the most common complications., Conclusions: Due to variable etiology, identification of the responsible microorganism through neurosurgical drainage followed by long-term intravenous antibiotics remains the mainstay in treating extraaxial CNS infections. Optimal outcome is achieved with early diagnosis and therapy.

The management of large and giant arteriovenous malformations (AVMs) in patients presenting with nonhemorrhagic neurological deficits secondary to vascular steal phenomenon is challenging and controversial. In many cases, large AVMs cannot be completely excised or cured, leaving patients with residual or partially treated AVMs, the natural history of which is unknown. Additionally, large, diffuse vascular malformations with multiple, small feeders, slow flow, or so-called cerebral proliferative angiopathy represent a related but distinct clinical and angiographic entity that may require a different therapeutic approach than traditional brain AVMs. The current management of children with other conditions of chronic cerebral hypoperfusion, such as moyamoya disease, involves consideration of surgical revascularization to enhance blood flow to the compromised hemisphere. Here, the authors present the case of a young child with a large thalamic vascular malformation who presented with clinical and radiological features of vascular steal and ischemia. In an effort to augment flow to the hypoperfused brain and protect against future ischemia, the authors treated the child with unilateral pial synangiosis. At 12 months, postoperative angiography demonstrated robust neovascularization, and the child has not sustained any further ischemic events. The authors discuss concept of vascular malformation-related hypoperfusion and the utility of indirect revascularization for inoperable vascular malformations presenting with ischemic symptoms.

Object: Confirming the successful management of pediatric arteriovenous malformations (AVMs) requires high-quality postoperative digital subtraction angiography. Although the role of intraoperative angiography during the microsurgical removal of AVMs is well established in adults, the technique has several limitations including poor image quality, uniplanar image acquisition, and absent full heparin protection. Here, the authors report on their experience with high-quality intraoperative angiography during the surgical management of pediatric AVMs in their image-guided therapy (IGT) facility., Methods: The authors retrospectively reviewed the demographic, clinical, and radiological characteristics of 22 patients who underwent the surgical management of AVMs at the Hospital for Sick Children in Toronto, with the aid of high-quality intraoperative or immediate postresection cerebral angiography via a transfemoral approach., Results: Between January 2000 and August 2009, 18 children (mean age 13.05 ± 4.04 years, range 4-21 years) underwent both surgical management of an AVM and intraoperative cerebral angiography at an IGT facility. An additional 4 children underwent angiography immediately after surgery in the regular operating room while under the same anesthesia. The mean AVM size was 2.55 ± 1.43 cm (range 1-6 cm) with a mean Spetzler-Martin grade of 2.27 (range 1-4). Intraoperative angiography in 4 of the 18 patients demonstrated residual AVM requiring additional resection. One patient demonstrated residual AVM on immediate postoperative angiography and underwent immediate reoperation. Successful excision of the residual AVM was confirmed on angiography the following day in that case. Procedural complications occurred in connection with 1 (3.3%) of 30 angiograms, including asymptomatic transient nonfilling of an ophthalmic artery, which was resolved on follow-up angiography. Negative intraoperative angiograms were confirmed with follow-up angiograms in 15 (93.75%) of 16 patients at a mean of 9.93 ± 5.70 months. One patient with a negative intraoperative angiogram demonstrated a tiny residual AVM on follow-up angiography at 8 months (false-negative rate 6.25%), but had a negative preoperative angiogram 1 year later in the IGT facility. No patient with a negative intraoperative angiogram required further AVM-directed treatment., Conclusions: Intraoperative angiography is a safe and effective adjunct to the surgical management of AVMs in children. This novel approach allows the pre-, intra-, and postoperative acquisition of high-quality images, which can help guide the resection of AVMs, especially those that are small, diffuse, or of a complex angioarchitecture.

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is occasionally seen after hypothalamic injury or dysfunction, although it typically occurs in association with other endocrine disturbances. It is has never been described as a presenting feature of a suprasellar arachnoid cyst (SAC) in the pediatric population. The authors describe the case of an enlarging SAC resulting in SIADH as the only presenting feature, with an otherwise normal hypothalamic-pituitary axis. An SAC was diagnosed in utero in this 5-month-old girl who had a normal functioning hypothalamic-pituitary axis on presentation. Because of cyst enlargement and hydrocephalus, the patient was scheduled for surgery; however, preoperative labs revealed SIADH. After stabilizing the serum sodium concentration with fluid restriction and the administration of 3% sodium chloride, the patient underwent endoscopic cyst fenestration. Postoperatively, she had complete resolution of the SIADH. Syndrome of inappropriate antidiuretic hormone secretion as the presenting symptom of an SAC has not been previously described. In the aforementioned patient, the proposed mechanism for SIADH was enlargement of the suprasellar arachnoid cyst causing compression of the supraoptic and paraventricular nuclei and thus overstimulating the secretion of arginine vasopressin, which resulted in SIADH. The association of SIADH with an SAC is reportable, as is the resolution of the SIADH via cyst fenestration. The authors suggest that SIADH is an uncommon presenting feature of SACs and that syndrome resolution is possible with cyst decompression.

Object: The authors used postoperative superselective angiography to assess the relative contributions of the middle meningeal artery (MMA) and the superficial temporal artery (STA) to revascularization following surgery for moyamoya syndrome in children., Methods: Using the neurosurgical database at the Hospital for Sick Children, the authors reviewed the clinical and pre- and postoperative angiographic records obtained in patients with moyamoya syndrome undergoing superselective angiography. Patients were 16 years of age or younger and were undergoing revascularization surgery for moyamoya syndrome during the study period. Lateral internal carotid artery, external carotid artery, STA, and MMA angiograms were analyzed in the late arterial phase to assess the relative contributions of the STA and MMA to overall revascularization as determined by the external carotid artery injection., Results: The total moyamoya surgical revascularization experience at the Hospital for Sick Children over a 12-year period (May 1996-December 2008) comprised 33 patients (20 girls and 13 boys) undergoing a total of 50 craniotomies. A decision was made in 2001 to perform superselective angiography postoperatively in patients with moyamoya syndrome. Superselective angiography was identified to have been performed postoperatively in 12 patients and 18 treated hemispheres, and it demonstrated that the MMA contributed more significantly than the STA in 11 (61%) of the 18 hemispheres. Seven patients were Asian, 3 patients had neurofibromatosis Type 1, 1 had Down syndrome, and 2 had no apparent risk factors (1 patient was Asian and had neurofibromatosis Type 1). Stroke had occurred in 58% of patients and transient ischemic attacks in 50% prior to surgery. Within the first 30 days of surgery, there were 2 episodes of stroke (11.7% per surgically treated hemisphere and 18.2% per patient). Seventy-eight percent of hemispheres surgically treated exhibited excellent revascularization (Matsushima Grade A) on follow-up angiography, and there were no strokes documented in any patients more than 1 month after surgery, in a long-term follow-up of mean 4.1 years., Conclusions: The contributions of the MMA to revascularization after pial synangiosis for moyamoya syndrome are significant and may frequently exceed the contribution of the STA when surgery is performed with preservation of dural vasculature and dural inversion.

Object: Because of their location and biological behavior, brainstem cavernous malformations (CMs) pose a formidable clinical challenge to the neurosurgeon. The optimal management of these lesions requires considerable neurosurgical judgment. Accordingly, the authors reviewed their experience with the management of pediatric brainstem CMs at the Hospital for Sick Children., Methods: The authors performed a retrospective chart review of pediatric patients who had received diagnoses of a brainstem CM at the Hospital for Sick Children over the past 20 years., Results: Twenty patients were diagnosed with brainstem CMs. The mean age at diagnosis was 10.1 +/- 5.4 years, and the patients included 13 boys and 7 girls. The mean maximal diameter of the CM was 14.3 +/- 11.2 mm. The lesions were evenly distributed on the right and left sides of the brainstem with 4 midbrain, 13 pontine, and 3 medullary lesions. Seven patients underwent surgery for the management of their CMs, with a mean age at presentation of 5.2 years, and a mean CM size of 21.0 mm. Of note from the surgical group, 2 patients had a family history of CMs, 2 lesions were medullary, the CM reached a pial surface in 6 of 7 patients, and 6 of 7 lesions were located on the right side. The mean age at presentation among the 13 patients in the nonsurgical group was 12.7 years, and the mean CM size was 10.6 mm. Seven of these patients had a prior history of radiation for tumor, and only 3 had lesions that reached a pial surface., Conclusions: The management of brainstem CMs in children is influenced by multiple factors. The majority of patients received conservative management and tended to be asymptomatic with smaller lesions. Patients with larger lesions and direct pial contact, in whom symptoms arose at a younger age were more likely to undergo surgical management. A history of familial CM was also a predictor for receiving surgical treatment. No patients with a prior history of radiation therapy underwent surgery for CMs. The presence of multiple lesions seemed to have no impact on the type of management chosen. Patients who underwent surgery did suffer morbidity related to the procedure, and tended to improve clinically over time. Conservative management was associated with new deficits arising in children, some of which improved with time. Consideration of many clinical and radiological parameters is thus prudent when managing the care of children with brainstem CMs.

Object: Epidermoid tumors are benign lesions representing 1% of all intracranial tumors. There have been few pediatric series of intracranial epidermoid tumors reported previously. The authors present their experience in the management of these lesions., Methods: The neurosurgical database at the Hospital for Sick Children was searched for children with surgically managed intracranial epidermoid tumors. The patients' charts were reviewed for demographic data, details of clinical presentation, surgical therapy, and follow-up. Ethics board approval was obtained for this study., Results: Seven children, all girls, were identified who met the inclusion criteria between 1980 and 2007. The average age at surgery was 11.2 years (range 8-15 years), and the mean maximal tumor diameter was 2.1 cm. Headache was the most common presenting symptom, and 1 tumor was found incidentally. Most patients had normal neurological examinations, but meningism was found in 2 cases. There were 3 cerebellopontine angle lesions, 1 pontomedullary lesion, and 3 supratentorial tumors. Hydrocephalus developed in 1 patient after aseptic meningitis, and she underwent shunt placement. There were no operative deaths. Complete resection could be performed in 2 patients. One patient experienced a small recurrence that did not require a repeated operation, while 1 subtotally resected lesion recurred and the patient underwent a second operation., Conclusions: Intracranial epidermoid tumors are rare in the pediatric population. Total resection is desirable to minimize the risk of postoperative aseptic meningitis, hydrocephalus, and tumor recurrence. Aggressive neurosurgical resection may be associated with cranial nerve or ischemic deficits, however. In these cases, neurosurgical judgment at the time of surgery is warranted to ensure maximum resection while minimizing postoperative neurological deficits.

Object: Cephaloceles represent primary axial mesodermal defects, occurring in 0.8-4 per 10,000 live births. Prior studies have reported posterior location, hydrocephalus, microcephaly, seizure, and presence of brain tissue as poor prognostic markers for neurological outcome. However, these studies were small and the results were analyzed using univariate tests. The purpose of this study was to investigate the potential risk factors for the occurrence of developmental delay in patients with cephaloceles, using both univariate and multivariable regression techniques., Methods: This is a retrospective cohort study of cephalocele cases treated at the Hospital for Sick Children between 1990 and 2006. Two independent investigators collected the data from the Hospital for Sick Children Encephalocele Database and hospital charts. Developmental assessments were made by general pediatricians and neuropsychologists. Both univariate analysis (alpha=0.10) and multivariable logistic regression analysis (alpha=0.05) were performed., Results: Eighty-five cases of cephaloceles were identified. The patient group consisted of 48 boys and 37 girls. Sixty-eight lesions were encephaloceles and 17 were meningoceles. The distribution was as follows: frontal (40 lesions), occipital (33), and parietal (12). Associated conditions included hydrocephalus (23), seizure disorder (17), microcephaly (6), corpus callosal abnormalities (15), heterotopias (9), cerebral dysgenesis (11), and myelomeningocele (1). Evaluation of long-term development revealed that 41 patients (48%) had normal development, 9 (11%) had mild delay, 14 (16%) had moderate delay, and 21 (25%) had severe delay. Hydrocephalus, seizure disorder, microcephaly, presence of associated intracranial abnormalities, and presence of brain tissue were significantly associated with poor outcome on univariate analysis. Multivariable analysis revealed hydrocephalus and presence of intracranial abnormalities to be statistically significant predictors of developmental delay., Conclusions: To the authors' knowledge, this is one of the largest North American cephalocele series documented. Unlike prior studies, location of the cephaloceles is not a significant predictor of outcome. The multivariable regression analysis demonstrates hydrocephalus and the presence of associated intracranial abnormalities as variables with cumulative predictive effects for developmental delay.

Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by disruption of normal collagen formation resulting in varying degrees of skeletal vulnerability, ligamentous laxity, and scleral discoloration. Children with OI may suffer from complex neurosurgical problems affecting the brain and spine. The authors sought to determine the neurosurgical implications of OI in a cohort of patients treated at a quaternary care center for pediatrics. The authors reviewed the case histories of 10 children with OI treated by the neurosurgical service at the Hospital for Sick Children in Toronto between January 1988 and March 2007. The cases of 4 of these children are highlighted in the article. The most common neurosurgical conditions encountered in this cohort included macrocephaly in 5 patients, subdural hematoma in 3 patients, epidural hematoma in 2 patients, and hydrocephalus in 3 patients. Basilar invagination and spinal fractures were observed in 20% of the cohort. Although some patients could be treated nonoperatively, several required craniotomy for clot evacuation, decompression, and spinal fixation for fracture or basilar invagination, and cerebrospinal fluid shunt insertion. Neurosurgical conditions affecting patients with OI include macrocephaly, the development of an acute intracranial hematoma after often minimal trauma, the development of chronic subdural fluid collections that may require drainage, hydrocephalus (both communicating and noncommunicating), basilar invagination, and subaxial spinal fractures. Surgery may be complicated in some children because of the underlying bone fragility and bleeding diathesis commonly observed in patients with OI.

The authors describe the novel use of C-1 lateral mass screws in four children 8 years of age or younger, in whom occipitocervical or atlantoaxial fusion was performed for trauma or os odontoideum. The authors retrospectively reviewed the demographics and procedural data of four children, ranging in age from 2 to 8 years, who required and underwent surgical fixation. Although C1-2 screw/rod constructs involving individual C-1 lateral mass screws and C-2 pars interarticularis or pedicle screws have been widely applied in adults, only C1-2 transarticular screw fixation has been reported in children less than 8 years of age. This report demonstrates the successful results of rigid occipitocervical and atlantoaxial fusion in four children in whom C-1 lateral mass screws were placed as part of a screw/rod construct. There was one instance of a vertebral artery injury, and the lessons learned from this complication are discussed.

Object: Endoscopy is a useful technique for obtaining biopsy samples of intraventricular tumors, and it offers the advantage of simultaneous treatment of obstructive hydrocephalus through endoscopic third ventriculostomy (ETV). Figures in the literature on the diagnostic yield of endoscopic biopsy, its complication rate, and the success rate of ETV in this context vary significantly. The authors performed a retrospective analysis of biopsy accuracy, complication rate, and success rate of ETV in a series of 31 endoscopic biopsy procedures., Methods: All data regarding tissue and cerebrospinal fluid sampling, endoscopy-related complications, procedures performed for hydrocephalus treatment, tissue diagnosis, and further management were reviewed. The accuracy of the biopsy findings was graded on a four-level scale: Level I, fully diagnostic; Level II, diagnostic with some reservation; Level III, pathological categorization problematic; and Level IV, not interpretable. Failure of ETV was defined as the need for any further operation for the treatment of hydrocephalus. Tissue diagnosis was graded as Level I or II in 23 cases. One Level I diagnosis differed from the diagnosis made following craniotomy. During the study period one patient underwent endoscopy without biopsy because the tumor could not be visualized. Consequently, the percentage of successful biopsies was 69% (22 of 32 endoscopic procedures). Complications occurred in six cases (19%), of which two (6%) were significant. Three patients (10%) suffered hemorrhagic complications. When combined with biopsy, the ETV procedure was successful in nine (64%) of 14 patients., Conclusions: Endoscopic biopsy of intraventricular tumors is a useful technique for establishing a tissue diagnosis and is associated with an acceptable complication rate. The biopsy does not affect the success rate of simultaneous ETV.

Object: The authors' aim in conducting this study was to investigate retrospectively the prognostic significance of angiogenic features in optic pathway/hypothalamic gliomas (OPHGs) in children., Methods: Patients were identified in whom a diagnosis of OPHG was made using pathological analysis at the Toronto Hospital for Sick Children between 1985 and 2002. Tumor specimens were reviewed for diagnostic accuracy and adequacy of the specimen. Sections were immunostained with factor VIII to assess microvessel density (MVD). A ratio of alpha-smooth muscle actin to factor VIII immunostaining was calculated to arrive at a vascular maturity index (VMI). Vascular endothelial growth factor (VEGF) and VEGF receptor (VEGFR) immunostaining were performed to evaluate angiogenic factors. In addition, the MIB-1 labeling index (LI) was used to assess proliferation. These factors were evaluated with respect to progression-free survival (PFS). Forty-one of 60 patients originally identified had adequate samples and follow up for inclusion in the study. Of these, eight patients had coexisting neurofibromatosis Type 1. Twenty-eight patients experienced tumor progression after the initial treatment (surgery with or without adjuvant treatment). Thirty-eight patients are still alive. A high MVD (> 21 vessels/1.2 mm2) was associated with a significantly higher rate of progression compared with a low MVD (< 21 vessels/1.2 mm2; p = 0.017). Microvessel density was also predictive of reduced PFS on multivariate analysis stratified for extent of resection (p = 0.04), and VMI as well as intensity and distribution of VEGF and VEGFR staining and the MIB-1 LI were not significantly associated with PFS., Conclusions: These findings suggest that MVD is the best current predictor of PFS in incompletely resected OPHGs. This information highlights the importance of angiogenesis in regard to low-grade gliomas.

Object: Television tipover has recently been recognized as a significant cause of injury in children, and head injury accounts for most of the associated deaths. The authors reviewed their experience with children who sustained head injury from falling televisions., Methods: Children admitted with the diagnosis of head injury related to falling televisions since 1992 were identified from the authors' trauma database, and a retrospective review of the medical records was performed. Eighteen patients were identified: 13 boys and five girls whose ages ranged from 12 months to 10 years (mean 44 months). The admission Glasgow Coma Scale (GCS) score was 15 in 10 patients. Only three patients had a GCS score of 8 or less (one patient had a score of 4). Neurological examinations were normal in 10 patients; three had cranial nerve deficits and three had otorrhea, otorrhagia, or hemotympanum. Radiological abnormalities included 16 skull fractures, three epidural hematomas, three small subdural hematomas, one intracranial hemorrhage, and three venous obstructions of the transverse-sigmoid sinus. The mean hospital stay was 8.9 days (range 2-39 days). Follow up at 0.2 to 68 months (mean 13.4 months) revealed severe neurological deficits in one patient and cranial nerve deficits of cranial nerves six, seven, or eight in six patients., Conclusions: Falling televisions result in significant head injuries in children, with substantial short- and long-term sequelae. This injury is easily preventable through simple measures taken by both the manufacturers and caregivers.

Object: The ability to map cerebrovascular reactivity (CVR) at the tissue level in patients with moyamoya disease could have considerable impact on patient management, especially in guiding surgical intervention and assessing the effectiveness of surgical revascularization. This paper introduces a new noninvasive magnetic resonance (MR) imaging-based method to map CVR. Preoperative and postoperative results are reported in three cases to demonstrate the efficacy of this technique in assessing vascular reserve at the microvascular level., Methods: Three patients with angiographically confirmed moyamoya disease were evaluated before and after surgical revascularization. Measurements of CVR were obtained by rapidly manipulating end-tidal PCO2 between hypercapnic and hypocapnic states during MR imaging. The CVR maps were then calculated by comparing the percentage of changes in MR signal with changes in end-tidal PCO2. Presurgical CVR maps showed distinct regions of positive and negative reactivity that correlated precisely with the vascular territories supplied by severely narrowed vessels. Postsurgical reactivity maps demonstrated improvement in the two patients with positive clinical outcome and no change in the patient in whom a failed superficial temporal artery-middle cerebral artery bypass was performed., Conclusions: Magnetic imaging-based CVR mapping during rapid manipulation of end-tidal PCO2 is an exciting new method for determining the location and extent of abnormal vascular reactivity secondary to proximal large-vessel stenoses in moyamoya disease. Although the study group is small, there seems to be considerable potential for guiding preoperative decisions and monitoring efficacy of surgical revascularization.

Object: In the preceding article, the authors described the Hydrocephalus Outcome Questionnaire (HOQ), a simple, reliable, and valid measure of health status in children with hydrocephalus. In the present study, they present their initial experience in using the HOQ to quantify the health status in a typical cohort of children with hydrocephalus., Methods: The mothers of children with hydrocephalus completed the HOQ and, with the child's attending surgeon, provided a global rating of their children's health. An exploratory analysis was performed using a multivariate analysis of variance (ANOVA) to determine which variables might be associated with worse health status. The mothers of 80 children, ranging in age from 5 to 17 years, participated in the study. The mean HOQ Overall Health score was 0.68, a value estimated to be equivalent to a mean health utility score of 0.77. The global health ratings provided by the mothers and the surgeons were moderately correlated with the HOQ scores (Pearson correlations 0.58 and 0.57, respectively). Results of the multivariate ANOVA indicated that the presence of epilepsy was strongly associated with a worse health status (p < 0.0001, F-test)., Conclusions: The health status of a typical sample of children with hydrocephalus was measured using the HOQ. The only consistently significant association with health status found was the presence of epilepsy.

Neck injuries in children most commonly affect the upper cervical spine. Injuries of the transverse ligament and its attachments may result in C1-2 instability, but the optimum form of treatment is unknown. Three patients, who ranged in age from 5 to 11 years, sustained transverse atlantal ligament injuries with unilateral avulsion fracture of the osseous tubercle of C-1. One child was injured in a fall and two were involved in motor vehicle accidents. Although all patients presented with neck pain, none exhibited neurological deficits. Plain radiography demonstrated no evidence of osseous injury, but an increased predental interval was noted in each case. Computerized tomography (CT) scanning demonstrated the avulsion fractures, and magnetic resonance imaging revealed evidence of soft-tissue injuries in the occipital-C2 ligamentous complex. All children were managed with external immobilization (halo vest in two and a Sterno-Occipito-Mandibular Immobilizer brace in one), for 6 to 12 weeks. Follow-up CT scanning demonstrated reattachment of the avulsed osseous tubercle, and dynamic cervical spine radiographs revealed the absence of C1-2 instability. The results of these cases suggest a role for external immobilization in the treatment of osseous avulsion injuries of the transverse atlantal ligament in children.