Pediatric Neurosurgical Centers Research Articles

REUNION COMMUNE : SOCIETE FRANCAISE DE NEUROCHIRURGIE (SFNC) - SOCIETE DE NEUROCHIRURGIE DE LANGUE FRANCAISE (SNCLF)

Infantile hydrocephalus has various etiologies that may influence children's cognitive development and onset of neurological comorbidities such as epilepsy. However, few studies specifically analyzed etiologies encountered in this population. Here we report a 9-year retrospective analysis of the etiologies and short-term outcome of surgically treated patients in a major pediatric neurosurgical center in a high-income country.This was a single-center retrospective study for the period 2010 to 2018 using the database of the French medical classification for clinical procedures (CCAM) of the Necker Hospital, Paris. We included all the patients surgically treated for hydrocephalus before the age of 2 years and reviewed digital medical files and MRI.Four hundred and sixty seven patients were included, with a mean age at diagnosis of 4.8 months and male predominance (M/F ratio = 1/2). Etiologies comprised: intraventricular hemorrhage (27.8%), arachnoid cyst (13.9%), spinal dysraphism (12.4%), brain tumor (10.5%), associated brain malformation (8.6%), infection (7.5%), isolated aqueduct stenosis (5.1%), and other (14.1%). Epilepsy was more likely to occur in post-infection (40%) and post-hemorrhage (31%) hydrocephalus, and when brain malformation was associated (35%). Etiology, epileptic status and the number of dysfunctions influenced short-term school performance.This study identified various etiologies of infantile hydrocephalus, with different neurological outcomes. Specific follow-up is required according to these observations.

Idiopathic syrinx in the pediatric population: a combined center experience

Object Discovery of a syrinx in a child, without a readily identifiable proximate cause such as a Chiari malformation, tumor, or site of tethering, is often a cause of concern for families and a source of consternation for clinicians. There is a paucity of data describing the natural history of an idiopathic syrinx in the pediatric population. The authors present the combined data of 2 major pediatric neurosurgical centers to describe their experience with this condition. Methods Data were collected at Children's Hospital Boston and St. Louis Children's Hospital according to institutional review board–approved protocols and captured visits over a 2.5-year interval (October 2006–March 2009), with records reviewed if the patient had a preexisting diagnosis of syrinx. Patients were identified by ICD-9 codes derived from departmental databases. All pediatric patients (age < 19 years) in whom idiopathic syrinx had been diagnosed, as defined by MR imaging findings (dilated central canal in the spinal cord of ≥ 1 mm in axial dimension and extending over at least 2 vertebral levels), were included. Results Forty-eight patients met the criteria for idiopathic syrinx during this period, and in 32 of them detailed follow-up imaging was available. Discovery of a syrinx was incidental in 6 patients, whereas the others were referred for imaging because of the presence of pain, neurological symptoms, scoliosis, or skin markings. The average age at the first MR imaging session was 9.7 years, with a mean syrinx size of 4 mm (range 1.2–9.4 mm). The majority (52%) of patients had a thoracic syrinx, with the average lesion spanning 7.1 vertebral levels. The average follow-up was 23.8 months (range 2–64 months), and subgroups of patients with < 3 years and ≥ 3 years of follow-up were independently reviewed. Overall, symptoms improved in 34% and worsened in 9%; 57% of the patients remained asymptomatic or stable. Radiographically (in the subgroup of 32 patients with detailed follow-up imaging), syrinx size decreased in 25% of patients, increased in 12.5%, and remained unchanged in 62.5%, with no apparent correlation between change in syrinx size and clinical symptoms. Conclusions Clinically, children with an idiopathic syrinx remained asymptomatic, stable, or improved in 91% of cases. The majority of syringes (87.5%) remained stable or shrank over time, with no apparent correlation between changes in size and changes in symptoms. Although longer follow-up is needed, these data suggest that the natural history of an idiopathic syrinx in children is benign, and that repeated imaging may not be necessary.

Surgical morbidity and mortality of pediatric brain tumors: a single center audit

ObjectivesThe primary aim of this study is to perform an internal quality control of pediatric brain tumor surgery in the neurosurgical department of the VU University Medical Center Amsterdam (The Netherlands). Secondly, this study aims to contribute to the accumulating data concerning outcome in pediatric neurosurgery, in order to establish institutional practice benchmarks.MethodsWe report the surgical mortality and morbidity of 121 patients (0–18 years) surgically treated for a brain tumor from January 1999 to August 2007. Patients, in whom only a brain tumor biopsy was performed, were excluded.ResultsMean age at first surgery was 8.2 years. Of the 121 patients, 14 had a second surgery, and two underwent a third surgery (for a total of 137 operations). Of all 121 primary surgeries, 66% were total resections, 26% subtotal resections, and 8% partial resections. The overall surgical morbidity rate in this study was 69% after first surgery, 50% after second surgery, and one out of two after third surgery.ConclusionThese overall morbidity rates are comparable to other published mixed case series. The surgical mortality rate was 0.8%; this is comparable to the lowest rates reported for high-volume neurosurgical centers. We encourage other neurosurgical centers to collect, analyze, and publish their data. These data can then serve as a basis for comparison with other pediatric neurosurgical centers and will eventually lead to an improvement of pediatric neurosurgical practice and patient care.

Conservative Management of Acute Epidural Hematoma in a Pediatric Age Group

Objective: Craniotomy and evacuation is the standard treatment of acute epidural hematoma (EDH). Here, the authors report their experience in nonoperative management of acute EDH in children with mild head injury. Methods: The authors retrospectively reviewed charts of patients with conservatively treated EDH at the Department of Neurosurgery, King Abdulla University Hospital, Irbid, Jordan, between August 2003 and October 2007. All patients had a Glasgow Coma Scale score of 14 or 15, and an initial computerized tomography (CT) scan demonstrating an EDH with or without skull fractures. Follow-up included neurological examination and brain CT. Results: Six children (3 boys, 3 girls) with acute EDH were successfully managed at our department without surgical intervention. The Glasgow Outcome Scale score of all patients was 5, with no posttraumatic sequelae. Follow-up brain CT showed complete resolution of the EDH within 2–3 months. Conclusions: Our results demonstrated that pediatric EDH can be managed nonoperatively. The pronounced increase in the number of CT examinations for patients with head injuries has resulted in a greater proportion of EDH detected in conscious patients. We recommend such treatment be performed in specialized pediatric neurosurgical centers under close neurological observation.

Pediatric neurosurgical centers

Pediatric neurosurgical centers

Quality of life in children with hydrocephalus: results from the Hospital for Sick Children, Toronto

Children with hydrocephalus face several quality of life (QOL) issues that have been poorly studied. The authors' aim was to quantify the QOL for children with hydrocephalus and identify predictors of long-term outcome, using a reliable and validated outcome measure: the Hydrocephalus Outcome Questionnaire (HOQ). All children (5-18 years old) with treated hydrocephalus attending the neurosurgery outpatient clinic at the Hospital for Sick Children were asked to participate. The patient's QOL was measured by the parent-completed HOQ. Predictor variables were extracted from the medical records. Multivariable linear regression was used to identify those predictor variables that were significantly associated with outcome. There was an 89% participation rate, with a total of 346 children participating (mean age 11.7 years, mean duration since diagnosis 9.9 years). Their mean HOQ Overall Health score was 0.68 (on a scale of 0 [worst QOL] to 1.0 [best QOL]). On multivariable analysis, the following predictors were associated with a worse overall QOL: increased seizure frequency, increased length of stay (LOS) in the hospital for the initial treatment of hydrocephalus, increased LOS for treatment of shunt infection and shunt overdrainage, increased number of proximal shunt catheters in situ, and increased distance of the family residence from the pediatric neurosurgical center. For the first time, these results establish baseline QOL values for a typical large group of children many years after their diagnosis of hydrocephalus, by using a validated and reproducible outcome measure. Many of the factors that adversely impact QOL appear to be related to shunt complications and might, therefore, be modifiable.

External ventricular drains: is there a time limit in children?

The time limit for the use of external ventricular drains (EVDs) has always been controversial. The purpose of this study is to find out if there is a time limit with regard to infection of EVDs and their duration of use in children. The records of 28 patients who had a total of 46 EVDs over a 4-year period at the Regional Paediatric Neurosurgical Centre at the Royal Liverpool Children's Hospital, Alder Hey, Liverpool, UK, were retrieved. The cerebrospinal fluid (CSF) white cell counts, CSF Gram stains and the CSF culture results were analysed. There is no time limit for EVDs in children. They can be left as long as clinically indicated provided strict protocols are followed in the handling of the set.

Pediatric CNS Tumors

This multidisciplinary handbook of childhood brain and spinal cord tumours is well-produced, and illustrated with many high-quality neuro-radiological images demonstrating typical MRI and CT scan appearances, and colour photomicrographs to show tumour histopathology. The layout is modern and clear with two text colours and, many tables and summary boxes make for easy reading. Following a brief introduction reviewing the aetiology, epidemiology and classification of childhood central nervous system tumours, this book follows a traditional layout. Overall, 10 chapters cover the principal tumour types grouped by anatomical site and pathology. These cover tumour natural history, diagnosis and management principles. There is probably too much coverage of molecular biology observations which are of no current relevance, and too little on the practical detail of treatment. There follows an excellent chapter describing the most common neurocutaneous syndromes and the CNS tumours with which they can be associated. The final chapters cover imaging, surgery, chemotherapy and radiotherapy. These may be useful to inform practitioners in other specialties, but do not contain enough detail or practical applications to guide practitioners on the specialty they cover. There is tantalising mention of a number of technological innovations without citation of any evidence base to suggest that they are better than conventional treatment, or any words of caution that they should only be used in the context of a well-designed prospective study. The book essentially covers the tertiary and quaternary management of childhood CNS tumours in a paediatric neurosurgical and oncology centre. It has 28 contributors, all from the United States, of whom 19 are from San Francisco, California. Despite this restricted authorship, the evidence-based treatment recommendations are very largely those which would be given in any large paediatric neuro-oncology centre in Europe or America. It will be valuable to the specialist trainee or clinical nurse specialist in any relevant discipline. Sadly however, it contains nothing of relevance to the generalist. For example, nothing in this book will guide a district general hospital paediatrician towards making an early referral for specialist investigation of a previously well child who has developed symptoms or signs which could (but more probably do not) indicate the presence of a brain tumour. Similarly, there is nothing practical to help the general paediatrician in the shared care management of children known to have brain tumours. For example, it would have been useful to have guidance about complications may arise such as hydrocephalus or shunt blockage or infection, and when to refer a child back as an emergency to the neurosurgical centre. Passing mention is made in different chapters of the complications which may result from chemotherapy or radiotherapy. However, it would have been useful in addition to have a detailed section on supportive care, long-term follow-up and the management of a child who may have complex problems as a result of the tumour and its treatment. These include, but are not limited to, endocrine surveillance and hormone replacement therapy, psychometric testing and educational support, visual and audiological assessment and the support of sight- and hearing-impaired children, neuro-rehabilitation, relief of social and psychological distress in patients and their families, and management of the behaviourally disturbed child. Rightly, the focus of this book is on curative therapy, but unfortunately cure is not the only outcome. The book would have been enriched by guidance on symptom control and supportive care of the child with progressive, incurable disease, including the appropriate use of corticosteroids, anticonvulsant recommendations, analgesia and antiemetics and palliative interventions with surgery, radiotherapy and chemotherapy. Despite these clear reservations, this little book will still be useful for many people involved in the care of children with CNS tumours.

Ventriculoperitoneal shunt block: what are the best predictive clinical indicators?

Aims: To evaluate the predictive value of symptoms, signs, and radiographic findings accompanying presumed ventriculoperitoneal (VP) shunt malfunction, by comparing presentation with operative findings and subsequent clinical course. Methods: Prospective...

Latency between Symptom Onset and Diagnosis of Pediatric Brain Tumors: An Eastern Canadian Geographic Study

Tumors of the central nervous system are now thought to be the most common form of childhood malignancies. Previous studies suggested that delays might exist between symptom onset and the diagnosis of pediatric brain tumors. In the Maritime Provinces of Canada (New Brunswick, Nova Scotia, and Prince Edward Island), there are only two pediatric neurosurgical centers; therefore, the Maritime Provinces are ideal for study of the epidemiological features of pediatric brain tumors. The aim of this study was to examine the incidence rates of pediatric brain tumors in eastern Canada, as well as factors important in their diagnosis. We collected data on 104 cases during a 6-year period (1995-2000), both prospectively and retrospectively, for the Maritime pediatric neuro-oncology database. All < or =17-year-old patients in the Maritime Provinces with pediatric brain tumors were treated in one of two neurosurgical centers (St. John, New Brunswick, or Halifax, Nova Scotia). The incidence rate for pediatric brain tumors was 4.28/100,000 child-yr. Tumors were more common among male patients and involved the infratentorial compartment in 65% of the total cases. The two most common types of tumors were astrocytomas (37%) and medulloblastomas (21%). The mean time to diagnosis was 7.3 months (95% confidence interval [CI], 4.99-9.67 mo), and only 41% of our cases were correctly diagnosed within three visits to various physicians. Tumors located in the brainstem required significantly longer times for diagnosis, compared with those located elsewhere (mean, 11.76 mo [95% CI, 3.13-20.39 mo] versus 6.57 mo [95% CI, 4.20-8.95 mo]; P = 0.014). Medulloblastomas as a group exhibited significantly shorter diagnostic times, compared with other pathological subtypes (mean, 3.78 mo [95% CI, 1.97-5.59 mo] versus 8.35 mo [95% CI, 5.40-11.3 mo]; P = 0.006). The incidence rates for pediatric brain tumors in the Maritime Provinces are similar to those of other reported series. The correct diagnosis of pediatric brain tumors still generally requires a number of months and frequent visits to various physicians. The majority of pediatric brainstem tumors might no longer be of a diffuse malignant nature but might represent more-focal benign lesions.

Intratumoral Therapy with Bleomycin for Cystic Craniopharyngiomas in Children

Surgical removal of cystic craniopharyngiomas in children is associated with significant operative morbidity and recurrence rates. The purpose of this study was to review our experience with a less invasive therapy, namely, intratumoral bleomycin, in the treatment of predominantly cystic craniopharyngiomas. All children with craniopharyngiomas treated at a tertiary care pediatric neurosurgical center since 1994, when bleomycin was first used, were reviewed retrospectively. Seven patients received intratumoral bleomycin therapy. Patients received 2–5 mg bleomycin per dose, 3 times per week, for 3–5 weeks as an initial course. Mean follow-up of these patients was 3 years. In 4 patients, treatment resulted in a significant decrease (>50%) in tumor size, which has remained stable. Two patients’ tumors progressed and underwent resection, and 1 patient had surgical removal because of persistent headaches, although no growth of residual tumor had been noted. One patient developed peritumoral edema as a result of bleomycin therapy. Intratumoral bleomycin is a useful alternative therapy for cystic craniopharyngiomas, and may control tumor growth and delay potentially harmful resection and/or radiotherapy in young children.

The value of routine cultures of the cerebrospinal fluid in patients with external ventricular drains.

The purpose of this study was to determine whether routine cerebrospinal fluid (CSF) bacteriological cultures in patients with external ventricular drains (EVDs) can identify infections early and prevent complications related to bacterial ventriculitis. We retrospectively reviewed the microbiological reports and clinical data for all patients in whom an EVD was placed at a tertiary care pediatric neurosurgical center between 1984 and 1997. EVDs were inserted in the operating room or intensive care unit, and, in most patients whose EVD remained in place for more than 2 days, daily cultures of CSF were performed. One hundred fifty-seven patients in whom 160 EVDs had been placed were included in the study. Forty-eight positive cultures were identified, of which the majority were determined to be contaminants. Seven infections were identified on the basis of microbiological criteria (i.e., a gram-positive stain and positive culture) and a subsequent positive culture. In all patients in whom infections developed, routine daily cultures of CSF were performed, and, in each instance, these cultures failed to identify the infections before clinical changes occurred. All seven patients with infection had fever (>38.5 degrees C) and peripheral leukocytosis (>11 x 10(3)/mm3) on the day the infection was identified, and one had a change in CSF appearance. The results of this study suggest that routine culture of CSF in children with EVDs is not necessary, and that if CSF cultures are performed for new fever (>38.5 degrees C) or peripheral leukocytosis, neurological deterioration, or a change in CSF appearance, infections will be identified in a timely fashion. In situations in which these clinical indicators might be masked, routine cultures may be valuable.

Hospital ethics committees: a pediatric neurosurgical perspective.

The Pediatric Neurosurgical Section of the American Association of Neurological Surgeons was polled regarding the status of hospital ethics committees. Twenty-four committees were identified in 74 pediatric neurosurgical centers in the United States. (Thirteen of these were formed between October 1983 and February 1984.) In addition, 21 are in the planning stages. Physicians predominate, with nurses, hospital administrators, the clergy, social workers, attorneys and lay persons usually represented. Functions include review of ethical and patient care decisions and providing counsel and support primarily to physicians and nurses. Rarely do committees make policy, determine prognosis or make final decisions regarding life-support systems. Approximately one case per year is reviewed. Committee formation frequently appears to be a crisis response to highly publicized pediatric cases and recent positions of the Department of Health and Human Services. Their purpose, construct, function and utilization raise serious questions as to their usefulness and necessity.