Barbara L. Shultz

Intracranial ependymomas of childhood: long-term outcome and prognostic factors.

A detailed outcome analysis was performed on 40 children with intracranial ependymomas treated at our institution between 1975 and 1993 to identify those factors that were predictive of overall and progression-free survival. Three patients (7.5%) who were treated in the first 5 years of the study died within 3 months of surgery and were excluded from further outcome assessments. Eight (22%) of the 37 patients who survived the perioperative period had evidence of leptomeningeal dissemination at presentation, on the basis of either imaging (three children) and/or cytological (six children) results. The 5- and 10-year progression-free survival rates among these 37 patients were 45.1 and 36.1%, respectively; overall survival rates were 57.1 and 45.0%, respectively. The site of progression was local in 17 of 19 patients with progressive disease. Three factors were found to have a significant association (P < or = 0.05) with the outcome on both univariate and multivariate analyses: 1) the extent of the resection, 2) the age of the patient at diagnosis, and 3) the duration of the symptoms before diagnosis. The 5-year progression-free and overall survivals were 8.9 and 22%, respectively, among patients who had evidence of residual disease on postoperative imaging studies, compared with 68 and 80% rates among patients with no apparent residual disease (P = 0.0001 and P < 0.0001, respectively). Patients younger than 3 years fared significantly worse than older children (5-year progression-free and overall survival rates of 12 and 22%, respectively, in the younger children versus 60 and 75% in older children (P = 0.003 and P = 0.01, respectively). In addition, patients with a duration of symptoms before diagnosis of < 1 month had a worse outcome than those with a more protracted course (5-year progression-free and overall survival rates of 33 and 33%, respectively, versus rates of 53 and 64%, respectively (P = 0.02 for both). Neither the finding of evidence for dissemination at presentation nor the detection of anaplastic histological features (e.g., dense cellularity or high numbers of mitoses) were associated with a significantly worse outcome in this series. The combination of variables that had the strongest association with both favorable and unfavorable outcomes was the combination of the age of the patient and the resection extent. Only 2 of 17 patients older than 3 years with gross total resections have died, whereas 13 of 20 children who were either younger than 3 years or had radiologically incomplete resections have died (P < 0.0001).(ABSTRACT TRUNCATED AT 400 WORDS)

Intracranial Ependymomas of ChildhoodLong-term Outcome and Prognostic Factors

A detailed outcome analysis was performed on 40 children with intracranial ependymomas treated at our institution between 1975 and 1993 to identify those factors that were predictive of overall and progression-free survival. Three patients (7.5%) who were treated in the first 5 years of the study died within 3 months of surgery and were excluded from further outcome assessments. Eight (22%) of the 37 patients who survived the perioperative period had evidence of leptomeningeal dissemination at presentation, on the basis of either imaging (three children) and/or cytological (six children) results. The 5- and 10-year progression-free survival rates among these 37 patients were 45.1 and 36.1%, respectively; overall survival rates were 57.1 and 45.0%, respectively. The site of progression was local in 17 of 19 patients with progressive disease. Three factors were found to have a significant association (P < or = 0.05) with the outcome on both univariate and multivariate analyses: 1) the extent of the resection, 2) the age of the patient at diagnosis, and 3) the duration of the symptoms before diagnosis. The 5-year progression-free and overall survivals were 8.9 and 22%, respectively, among patients who had evidence of residual disease on postoperative imaging studies, compared with 68 and 80% rates among patients with no apparent residual disease (P = 0.0001 and P < 0.0001, respectively). Patients younger than 3 years fared significantly worse than older children (5-year progression-free and overall survival rates of 12 and 22%, respectively, in the younger children versus 60 and 75% in older children (P = 0.003 and P = 0.01, respectively). In addition, patients with a duration of symptoms before diagnosis of < 1 month had a worse outcome than those with a more protracted course (5-year progression-free and overall survival rates of 33 and 33%, respectively, versus rates of 53 and 64%, respectively (P = 0.02 for both). Neither the finding of evidence for dissemination at presentation nor the detection of anaplastic histological features (e.g., dense cellularity or high numbers of mitoses) were associated with a significantly worse outcome in this series. The combination of variables that had the strongest association with both favorable and unfavorable outcomes was the combination of the age of the patient and the resection extent. Only 2 of 17 patients older than 3 years with gross total resections have died, whereas 13 of 20 children who were either younger than 3 years or had radiologically incomplete resections have died (P < 0.0001).(ABSTRACT TRUNCATED AT 400 WORDS)

High-grade Astrocytomas in Children: Radiologically Complete Resection Is Associated with an Excellent Long-term Prognosis

High-grade astrocytomas comprise approximately 10% of intracranial tumors in children. A better prognosis in children than in adults has been reported for patients with these neoplasms, although the reasons for this survival advantage are uncertain. To determine whether any consistent factors were associated with long-term survival, we reviewed the records of 31 children with high-grade non-brain stem gliomas who were treated at our institution between 1975 and 1992. Histology was reviewed and classified according to the World Health Organization scheme, and neuroimaging studies were examined to determine the extent of resection, in both instances by individuals who were unaware of the patients outcomes. The median overall survival for the 28 patients who survived the perioperative period was 18.5 months; 10 (36%) are currently alive, with a median follow-up of 70.5 months. The median progression-free survival (PFS) was 10.5 months; eight patients (29%) remain progression-free with a median follow-up of 78 months. The extent of resection at initial operation was associated most closely with PFS and overall survival as revealed by multivariate analysis. The 14 patients who underwent subtotal (< 90%) resection and the 7 who underwent near-total (90-99%) resection had median PFS of 5.5 and 11 months, respectively (P = 0.38), and overall survival of 10.5 and 25 months, respectively (P = 0.02). None of the seven patients who underwent gross total removal of tumor as confirmed by postoperative imaging had disease progression, with a median follow-up of 84 months (P < 0.0001). All of the tumors that underwent gross total resection were situated within the cerebral hemispheres; five of the seven patients had seizures as a presenting symptom. Both tumor location and seizures were significantly associated with outcome as determined by univariate analysis, but because of the overwhelming impact of resection extent on outcome, these factors were not independently associated with outcome as revealed by multivariate analysis. Histology was associated with outcome in the subgroup of patients with incompletely resected hemispheric tumors, in which children with anaplastic astrocytoma had a significantly better PFS than those with glioblastoma multiforme (P = 0.009). In summary, our results support the role of cytoreductive surgery in the treatment of cerebral hemispheric high-grade astrocytomas in children, which may encompass a biologically distinct group of tumors that, by virtue of their location and growth characteristics, are amenable to aggressive resection. The prognosis for children with deep-seated lesions and for those with subtotally resected hemispheric lesions is generally poor with conventional therapy.

The management of brainstem gliomas in patients with neurofibromatosis 1

The appropriate management of brainstem tumors in patients with neurofibromatosis 1 (NF1) has been problematic because the natural history of these lesions remains poorly defined.To formulate rational guidelines for the evaluation and treatment of these tumors, we reviewed the outcome of 21 patients with brainstem mass lesions followed in our NF clinic during the last 9 years. We subdivided the imaging features of these lesions into four groups: (1) diffuse enlargement of the brainstem with hypointensity on T1-weighted MR images and hyperintensity on T2-weighted images (n = 9); (2) focal enhancing masses (n = 7); (3) intrinsic tectal tumors (n = 5); and (4) focal nonenhancing areas of hypointensity on T1-weighted MR images (n = 2). Two cases exhibited two types of lesions. Twelve patients presented with, or developed, symptoms that were referable to the mass; in nine, the lesion was asymptomatic. A distinguishing feature of these tumors was their generally indolent biological behavior. With a median follow-up of 3.75 years, only 10 patients have had radiographic (n = 9) or clinical (n = 3) evidence of disease progression. In seven of these patients, the tumor subsequently stabilized in size or regressed without intervention. Only four patients, each with a focal enhancing tumor, received specific therapy for the tumor; this consisted of biopsy (n = 1), excision (n = 3), and adjuvant radiotherapy (n = 2). Each of these lesions was a low-grade glioma histologically and each remained stable in size after treatment (median follow-up = 4.25 years). Four patients with tectal tumors underwent insertion of a CSF shunt for hydrocephalus, but required no specific treatment for the tumor. None of the patients with diffuse brainstem lesions or focal areas of hypointensity required any intervention for the tumor. All 21 patients are presently alive and well. We conclude that the biological behavior of brainstem lesions in patients with NF1 differs significantly from that of lesions with a similar appearance in patients without this disorder.Although these lesions may at some time in their course exhibit clinical and radiographic progression, most do not require specific intervention. The lesions that are most likely to progress and require therapy are focal enhancing tumors; however, even lesions in this subgroup may stabilize in size or regress spontaneously without intervention. Based on these results, we recommend that intervention be limited to those lesions that exhibit rapid or unrelenting growth on serial images or that produce significant clinical deterioration. NEUROLOGY 1996;46: 1652-1660

Continuous intrathecal baclofen infusion for symptomatic generalized dystonia.

Five patients with generalized dystonia who were refractory to oral medications were treated by continuous intrathecal baclofen infusion. Dystonia was related to cerebral palsy in three patients and to Hallervorden-Spatz disease in two. Responsiveness to intrathecally administered baclofen was evaluated after bolus injections in one patient and during continuous infusions via an external micropump in four. Patients who responded to trial injections were subsequently implanted with a programmable pump for continuous infusion of baclofen. Dystonia in the three patients were cerebral palsy was substantially improved by continuous intrathecal baclofen infusion in doses of 500 to 800 micrograms/d. Benefit has persisted for > 19 months of continuous infusion. Dystonia in the two patients with Hallervorden-Spatz disease was not improved, although the screening trial was limited by side effects in one patient and by meningitis in the other. We conclude that continuous intrathecal baclofen infusion is beneficial therapy for some patients with generalized dystonia and that additional investigations are indicated.

All-terrain vehicle rules and regulations: impact on pediatric mortality

BACKGROUND/PURPOSEnAll-terrain vehicles (ATV) use by children leads to severe injury and death. Since the US Consumer Product Safety Commission consent decree expired in 1998, there has been little movement in regulating ATV use for children (<16 yr). The authors hypothesized that states with laws and regulations restricting pediatric ATV use may abrogate excess death compared with states without such restrictions.nnnMETHODSnPediatric mortality data reported to the consumer product safety commission from 1982 to 1998 were analyzed as well as state all-terrain vehicle requirements compiled by the Specialty Vehicle Institute of America in August 2001. The authors calculated ATV mortality rate by dividing ATV mortality frequency by 1980-2000 pediatric census results. They compared the top 26 states with the highest ATV mortality rates (TOP) with those of all other states (OTH) in terms of age, ATV type, ATV occupancy, and ATV laws. Chi-square analysis was performed.nnnRESULTSnThere were 1,342 ATV pediatric deaths during the 16-year period. The TOP states averaged approximately a 2-fold increase in adjusted ATV mortality rate compared with the national ATV pediatric mortality rate. Ninety-two percent of TOP states have no licensing laws compared with 73% of the OTH states (P <.07). There is no difference between groups with regard to minimum age requirements and safety certification.nnnCONCLUSIONSnCurrent legal and regulatory standards have low probability of decreasing ATV-related pediatric mortality. States should adopt laws that restrict the use of ATVs for children less than 16 years of age and potentially prevent excess ATV-related pediatric mortality.

Risk factors and predictors of mortality in children after ejection from motor vehicle crashes.

PURPOSEnMortality after ejection from a motor vehicle crash (MVC) has been studied extensively in adults. The magnitude of this problem in children is relatively unknown. We retrospectively examined fatalities resulting from ejection after MVC in the state of Pennsylvania to define risk factors and predictors of mortality in children.nnnMETHODSnThe records for all patients 0 to 16 years of age involved in an MVC and entered in the Pennsylvania Trauma Outcome Study between 1993 and 1997 were reviewed. We examined mortality, length of hospitalization, major injuries sustained, and impact of safety restraint devices. Significant differences were determined using chi2 test.nnnRESULTSnThere were 2,298 children involved in MVCs during this period; 189 were ejected. A total of 77% of the ejected passengers were greater than 10 years of age, 16% were 0 to 4 years of age, and 7% were 5 to 9 years of age. Overall, 88% of the ejected occupants were unrestrained. Ejection nearly tripled the overall mortality rate and significantly increased the Injury Severity Score for each age group. Infants and children 0 to 4 years of age had the highest fatality rate despite having a lower Injury Severity Score than all other age groups. Head injuries accounted for the majority of deaths in all age groups. Children older than 10 years of age had a higher incidence of associated chest, abdominal, and pelvic injuries.nnnCONCLUSIONnOur data show that most children ejected from MVCs were either unrestrained or improperly restrained. Head injuries were the most common cause of death in all age groups. Greater public awareness through educational programs targeting parents and children at risk may reduce this serious problem.

Long-Term Outcome after Sagittal Synostosis Operations

We evaluated 27 children who had been operated on 5-10 years previously for sagittal synostosis. The mean age at operation was 0.55 years. Twenty-two children were treated by the senior authors technique, 4 by the Pi craniectomy technique and 1 by parasagittal sagittal strip craniectomies. The mean age at follow-up was 9.6 years. The cosmetic appearance of the head was graded independently by 3 examiners. Skull radiographs were graded by 2 examiners according to the appearance and presence of beaten copper markings. The cephalic index was measured. Frequent headaches were reported in 7 of 27 children and were migrainous in 3 of the 7. No child had papilledema. The childrens appearance was considered to be normal or mildly abnormally by the parents in every case and by the medical observers in 25 of 27 (93%). The only 2 children with an unacceptable appearance were either operated late (3.3 years) or had a craniofacial syndrome. Skull radiographs revealed a normal or mildly abnormal appearance in 18-19 children, depending on the observer. Beaten copper markings were observed to some extent in 14 of 27 cases and did not correlate with the presence of headaches. The cranial index ranged from 62 to 78 with a mean of 70 (normal 81). Sagittal reconstructions resulted in an acceptable appearance 5-10 years postoperatively in all cases operated on at less than 1 year of age who had no syndrome. The need for long-term follow-up of children after sagittal synostosis operations cannot be determined from this sample size.

Analysis of pediatric all-terrain vehicle trauma data in Middle Tennessee: Implications for injury prevention

BACKGROUND Trauma registries capture data about injuries that can be used to objectively guide injury prevention initiatives. This article analyzes trauma registry data to describe the nature and distribution of all-terrain vehicle (ATV) injuries in Middle Tennessee. A community injury prevention effort, based on this analysis, is also presented. METHODS A retrospective analysis of data (2007–2009) from the trauma registry of a Level I pediatric trauma center in Middle Tennessee was conducted. Patients younger than 16 years with ATV-related injuries were included in the analysis (n = 163). The key variables examined were demographics, injury severity, helmet use, injury mechanism, length of stay, and patient’s county of residence. In addition, Geographic Information Systems software was used to examine the distribution of injuries and graphically represent counties with highest injury rates in the youth population. RESULTS ATV injuries were more prevalent among boys than girls (66% vs. 34%; p < 0.001). Approximately 64% of the ATV injuries were in the age group 10 years to 15 years. Most injuries were either moderately severe (44%) or severe (30%). Injury mechanism varied by age; younger children experienced more rollovers while older children tended to be injured from ejections (p < 0.05). Helmet use was low (33%). Data from this study suggest that helmet use resulted in fewer injuries to the head, neck, and face. Counties with high rates of ATV injuries were targeted for ATV training programs. 4-H agents trained by the ATV Safety Institute provided ATV training classes. CONCLUSION Rural youth are clearly at greater risk for ATV injuries than urban populations. Young ATV riders are often self-taught and lack the knowledge to ride ATVs safely. Organizations such as the 4-H, provide effective injury prevention outreach. LEVEL OF EVIDENCE Epidemiologic study, level III.

Intrathecal Baclofen Therapy and the Role of the Physical Therapist

The purpose of this article is t o describe intrathecal baclofen (ITB) therapy and the physical therapists role in identifying appropriate candidates and patient management. Baclofen is an antispasticity medication delivered intrathecally by an implanted programmable pump. The physical therapist, ideally on an interdisciplinary team, considers whether reducing moderate to severe hypertonia would benefit a patient and recommends appropriate intervention. Treatment goals include improved comfort, ease of care, and function. Advantages of ITB therapy include the capacity for minute dose adjustments, allowing hypertonus needed for posture and function, and reversibility of the procedure. Disadvantages of ITB therapy include risks of pump implantation, catheter complications, treatment costs, and adverse drug effects. Therapists see patients before and after pump implantation to promote functional gains with interventions such as stretching, strengthening, and functional training. In advising families and physicians regarding ITB therapy, therapists should consider the indications, potential benefits, risks, alternatives, and current evidence. (Pediatr Phys Ther 2000;12:77-86)