Maria Lamberti-Pasculli

The changing epidemiology of paediatric brain tumours: a review from the Hospital for Sick Children

PurposeThis study examines the changing epidemiology of paediatric brain tumours over the past three decades (1980–2008) in a single institution, SickKids, Toronto, Canada.MethodsWe classified 1,866 surgical pathology cases of brain tumours in children under the age of 19 according to the World Health Organization 2007 consensus and analysed them by gender, histological tumour type, age distribution and decade.ResultsMales showed a slightly higher predominance with 56.8% of cases overall. The main histological tumour types were low-grade (I/II) astrocytomas (26.4%), medulloblastoma (10.6%), anaplastic astrocytoma/glioblastoma multiforme (7.1%) and ependymoma (7.0%). Over three decades, an increasing proportion of certain tumour types, including pilocytic astroctoma, atypical teratoma/rhabdoid tumours and neuronal/mixed neuronal-glial tumours was seen.ConclusionsOur results are consistent with those published with similar methodologies in other countries. Any changes in the epidemiology of childhood central nervous system tumours over the past three decades may be attributed in part to changing classification systems, improved imaging technologies and developments in epilepsy surgery; however, continued surveillance remains important.

Spontaneous intraparenchymal hemorrhage in full-term neonates.

OBJECTIVE Spontaneous intraparenchymal hemorrhage is extremely rare in full-term newborns. Reports to date have been limited to descriptions of individual cases, small groups within larger studies of intracranial hemorrhage, and one series of four patients. Structural lesions are rarely identified, and the majority of patients described have been managed without surgical intervention. METHODS Analysis of a computerized database of pediatric neurosurgical patients from January 1960 to February 2000 identified full-term newborns younger than 3 months of age with nontraumatic intraparenchymal hemorrhages. Prenatal histories, labor and delivery histories, clinical presentations, imaging studies, management, and outcomes were reviewed. RESULTS Eleven full-term newborns with spontaneous intraparenchymal hemorrhages were identified. The majority had normal prenatal courses. Most presented within the first 2 days of life (6 of 11 patients), and the most common presenting sign was seizure (7 of 11 patients). No cause was identified in 6 of 11 patients; the remainder were attributed to coagulopathy (n = 3), ruptured intracranial aneurysm (n = 1), or hemorrhagic infarction (n = 1). Eight patients underwent surgical hematoma evacuation on the basis of radiographic evidence of significant mass effect, evidence of signs of elevated intracranial pressure, or both. Three patients did not receive surgical intervention. There were no subsequent hemorrhages or deaths during a mean follow-up period of 4.5 years (range, 1–16 yr). Four patients had normal neurological outcomes, four had motor deficits (one of whom additionally demonstrated cognitive delay), and three had delayed speech. CONCLUSION No cause is identified in most newborns with spontaneous intraparenchymal hemorrhage. Radiographic evidence of mass effect or signs of elevated intracranial pressure may necessitate surgical hematoma evacuation. Outcome varies widely and may be normal, even in patients with sizeable intraparenchymal hemorrhages.

Repeat Cerebrospinal Fluid Shunt Infection in Children

Background: In this study, we investigated the treatment of cerebrospinal fluid (CSF) shunt infection and the risk factors for repeat shunt infection (RSI) in a cohort of children treated at the Hospital for Sick Children, Toronto, Canada. Methods: Between 1996 and 2000, a total of 51 children were identified with shunt infection (mean age 5.8 years). The medical records of these children were reviewed to identify cases of RSI within 6 months of the initial shunt infection (ISI). Results: In the 51 ISIs, the infecting organisms were coagulase-negative Staphylococcus (43.1%), Staphylococcus aureus (37.3%) and others (19.6%). The initial mode of treatment of the shunt infection was using an external ventricular drain (EVD) with removal of the shunt apparatus (54.9%), externalization of the shunt (37.3%) or shunt removal only (7.8%). The mean number of days of external CSF drainage (either EVD or externalized shunt) was 11.2 days. Ten patients (19.6%) developed RSI. The actuarial risk of RSI plateaued after 90 days at 24.4%. The following variables were tested as risk factors for RSI using survival analysis, although none reached statistical significance: initial organism (p = 0.09), age (p = 0.42), etiology of hydrocephalus (p = 0.45), number of days of CSF drainage (p = 0.45), type of surgical treatment of the ISI (p = 0.58) and the presence of bacteriologically positive CSF at ISI (p = 0.85). Conclusions: The risk of RSI is substantial and greater effort needs to be directed towards understanding the risk factors. Such studies will need a greater sample size in order to obtain sufficient statistical power.

Predicting postresection hydrocephalus in pediatric patients with posterior fossa tumors

OBJECT Approximately 30% of children with posterior fossa tumors exhibit hydrocephalus after tumor resection. Recent literature has suggested that prophylactic endoscopic third ventriculostomy diminishes the risk of this event. Because the majority of patients will not have postoperative hydrocephalus, a preoperative clinical prediction rule that identifies patients at high or low risk for postresection hydrocephalus would be helpful to optimize the care of these children. METHODS The authors evaluated a derivation cohort of 343 consecutive children with posterior fossa tumors who underwent treatment between 1989 and 2003. Multivariate methods were used on these data to generate the Canadian Preoperative Prediction Rule for Hydrocephalus. The rules estimated risk of postresection hydrocephalus was compared with risk observed in 111 independent patients in the validation cohort. RESULTS Variables identified as significant in predicting postresection hydrocephalus were age < 2 years (score of 3), papilledema (score of 1), moderate to severe hydrocephalus (score of 2), cerebral metastases (score of 3), and specific estimated tumor pathologies (score of 1). Patients with scores > or = 5 were deemed as high risk. Predicted probabilities for the high- and low-risk groups were 0.73 and 0.25, respectively, from the derivation cohort, and 0.59 and 0.14 after prevalence adjustment compared with the observed values of 0.42 and 0.17 in the validation cohort. CONCLUSIONS A patients score on the Preoperative Prediction Rule for Hydrocephalus will allow improved patient counseling and surgical planning by identifying patients at high risk of developing postresection hydrocephalus. These patients might selectively be exposed to the risks of preresection CSF diversion to improve outcome.

Prospective surveillance of complications in a pediatric neurosurgery unit: Clinical article

OBJECT Complications of specific pediatric neurosurgical procedures are well recognized. However, focused surveillance on a specific neurosurgical unit, for all procedures, may lead to better understanding of the most important complications, and allow targeted strategies for quality improvement. METHODS The authors prospectively recorded the morbidity and mortality events at a large pediatric neurosurgical unit over a 2-year period. Morbidity was defined as any significant adverse outcome or death (for obstructive shunt failure, within 30 days). Multiple and unrelated complications in the same patient were recorded as separate events. RESULTS There were 1082 surgical procedures performed during the evaluation period. One hundred seventy-seven complications (16.4%) occurred in 147 patients. By procedure, the most common complications occurred in vascular surgery (41.7%) and brain tumor surgery (27.9%). The most common complications were CSF leakage (31 cases), a new neurological deficit (27 cases), early shunt or endoscopic third ventriculostomy obstruction (27 cases), and shunt infection (24 cases). Meningitis occurred in 19 cases: in 58% of shunt infections, 13% of CSF leaks, and 10% of wound infections. Sixty-four percent of adverse events required a second procedure, most commonly an external ventricular drain placement or shunt revision. CONCLUSIONS Complications in pediatric neurosurgical procedures are common, result in significant morbidity, and more than half the time require a repeat surgical procedure. Targeted strategies to prevent common complications, such as shunt infections or CSF leaks, might significantly reduce this burden.

Rotatory subluxation: experience from the Hospital for Sick Children

OBJECT Diagnosis and management of atlantoaxial rotatory subluxation (AARS) is challenging because of its variability in clinical presentation. Although several treatment modalities have been employed, there remains no consensus on the most appropriate therapy. The authors explore this issue in their 9-year series on AARS. METHODS Records of patients diagnosed radiologically and clinically with AARS between May 2001 and March 2010 were retrospectively reviewed. Of 40 patients identified, 24 were male and were on average 8.5 years of age (range 15 months-16 years). Causes of AARS included trauma, congenital abnormalities, juvenile rheumatoid arthritis, infection, postsurgical event, and cryptogenic disease. Four patients had dual etiologies. Symptom duration varied: 29 patients had symptoms for less than 4 weeks, 5 patients had symptoms between 1 and 3 months, and 6 patients had symptoms for 3 months or more. RESULTS Treatment with a cervical collar was sufficient in 21 patients. In 1 patient collar management failed and halter traction was used to reduce the subluxation. Seven patients underwent initial halter traction, but in 4 the subluxation progressed and the patients required halo traction. A halo vest was placed in 2 patients on presentation because the rotatory subluxation was severe; both patients required subsequent operative fusion. One patient required decompression and fusion due to severe canal compromise and myelopathy. All patients requiring fusion presented with subacute symptoms. CONCLUSIONS Management of AARS varies due to the spectrum of clinical presentations. Patients presenting acutely without neurological deficits can likely undergo collar therapy; those in whom the subluxation cannot be reduced or who present with a neurological deficit may require traction and/or surgical fixation. Patients presenting subacutely may be more prone to requiring operative intervention.

Risk factors for repeated cerebrospinal shunt failures in pediatric patients with hydrocephalus

OBJECT Repeated cerebrospinal fluid (CSF) shunt failures in pediatric patients are common, and they are a significant cause of morbidity and, occasionally, of death. To date, the risk factors for repeated failure have not been established. By performing survival analysis for repeated events, the authors examined the effects of patient characteristics, shunt hardware, and surgical details in a large cohort of patients. METHODS During a 10-year period all pediatric patients with hydrocephalus requiring CSF diversion procedures were included in a prospective single-institution observational study. Patient characteristics were defined as age, gender, weight, head circumference, American Society of Anesthesiology class, and cause of hydrocephalus. Surgical details included whether the procedure was performed on an emergency or nonemergency basis, use of antibiotic agents, concurrent surgical procedures, and duration of the surgical procedure. Details on shunt hardware included: the type of shunt, the valve system, whether the shunt system included multiple or complex components, the type of distal catheter, the site of the shunt, and the side on which the shunt was placed. Repeated shunt failures were assessed using multivariable time-to-event analysis (by using the Cox regression model). Conditional models (as established by Prentice, et al.) were formulated for gap times (that is, times between successive shunt failures). There were 1183 shunt failures in 839 patients. Failure time from the first shunt procedure was an important predictor for the second and third episodes of failure, thus establishing an association between the times to failure within individual patients. An age younger than 40 weeks gestation at the time of the first shunt implantation carried a hazard ratio (HR) of 2.49 (95% confidence interval [CI] 1.68-3.68) for the first failure, which remained high for subsequent episodes of failure. An age from 40 weeks gestation to 1 year (at the time of the initial surgery) also proved to be an important predictor of first shunt malfunctions (HR 1.77, 95% CI 1.29-2.44). The cause of hydrocephalus was significantly associated with the risk of initial failure and, to a lesser extent, later failures. Concurrent other surgical procedures were associated with an increased risk of failure. CONCLUSIONS The patients age at the time of initial shunt placement and the time interval since previous surgical revision are important predictors of repeated shunt failures in the multivariable model. Even after adjusting for age at first shunt insertion as well as the cause of hydrocephalus, there is significant association between repeated failure times for individual patients.

Laparoscopic insertion of ventriculoperitoneal shunts in pediatric patients — A retrospective cohort study

INTRODUCTION Ventriculoperitoneal shunts (VPSs) are the mainstay of treatment of hydrocephalus but have frequent complications including shunt failure and infection. There has been no comparison of laparoscopic versus open primary VPS insertion in children. We hypothesized that laparoscopic VP shunt insertion may improve patient outcomes. METHODS A prospectively-maintained, externally-validated database of pediatric patients who underwent VPS insertion at a single center between 2012 and 2016 was reviewed. Outcomes including subsequent revisions, shunt infections, operative time, and hospital stay between open and laparoscopic groups were compared. RESULTS 210 patients underwent VPS insertion - 41 laparoscopically and 169 open. Operative time was longer for laparoscopic insertions. There was no difference in shunt infections, complications or length of stay. There was no difference between overall revisions or in confirmed peritoneal obstructions in the laparoscopic (12%) versus open VPS insertions (5%), p = 0.13. CONCLUSIONS This first cohort analysis of laparoscopic versus open VPS insertion in pediatric patients indicates no difference in confirmed peritoneal obstructions. With increasing use of laparoscopic placement in some centers, it remains important to elucidate if there is a subset of pediatric patients who might benefit from this technique; possible candidates may be those who are overweight/obese or have had previous intra-abdominal surgery. LEVEL OF EVIDENCE III - Retrospective cohort study.

Laparoscopic Revision of Ventriculoperitoneal Shunts in Pediatric Patients May Result in Fewer Subsequent Peritoneal Revisions

INTRODUCTION Ventriculoperitoneal shunts (VPSs) are the mainstay of treatment of hydrocephalus but frequently need revision. We sought to directly compare the impact of laparoscopic versus open peritoneal shunt revision on the need for subsequent VPS revisions in pediatric patients. MATERIALS AND METHODS A prospectively maintained, externally validated database of pediatric patients who underwent a first peritoneal VPS revision at a single center between 2008 and 2016 was reviewed. Outcomes, including subsequent revisions, shunt infections, operative time, and hospital stay between open and laparoscopic groups, were compared. RESULTS A total of 148 patients underwent a first peritoneal VPS revision during the time period-40 laparoscopically and 108 open-with no significant difference in age or gender between the groups. Operative time, length of stay after shunt revision, and shunt infection rates did not vary between laparoscopic versus open revisions. There was no significant difference between need for subsequent overall (peritoneal or ventricular) shunt revisions in the laparoscopic (20%) versus the open group (34%), P = .07. However, there were significantly fewer frequent peritoneal revisions in the laparoscopic group (3% versus 15%, P = .04). CONCLUSIONS This first cohort analysis of laparoscopic versus open VPS revision in pediatric patients suggests that laparoscopic peritoneal VPS revision may reduce the rate of subsequent peritoneal revisions without increasing shunt infections or operative time in pediatric patients.