ABSTRACT
Raman amplification arising from the excitation of a density echelon in plasma could lead to amplifiers that significantly exceed current power limits of conventional laser media. Here we show that 1-100 J pump pulses can amplify picojoule seed pulses to nearly joule level. The extremely high gain also leads to significant amplification of backscattered radiation from "noise", arising from stochastic plasma fluctuations that competes with externally injected seed pulses, which are amplified to similar levels at the highest pump energies. The pump energy is scattered into the seed at an oblique angle with 14 J sr-1, and net gains of more than eight orders of magnitude. The maximum gain coefficient, of 180 cm-1, exceeds high-power solid-state amplifying media by orders of magnitude. The observation of a minimum of 640 J sr-1 directly backscattered from noise, corresponding to ≈10% of the pump energy in the observation solid angle, implies potential overall efficiencies greater than 10%.
ABSTRACT
INTRODUCTION: Primary tethered spinal cord (TSC) refers to a group of abnormalities associated with a posterior bony spinal defect that develops beneath an intact dermis and epidermis. There is relative agreement that patients with symptomatic TSC will require surgical intervention. However, it is still debatable as to how to approach asymptomatic patients with primary TSC. OBJECTIVE: To study the clinical and urodynamic (UDS) outcomes of patients with primary TSC after spinal cord untethering (SCU). STUDY DESIGN: Charts of patients with primary TSC between 1998 and 2010 were retrospectively reviewed. Patients that underwent before and after SCU clinical and UDS evaluation with minimum of 5-years follow-up were included. Continence status was assessed in children ≥5 years. Patients with dry intervals of ≥4 h were considered continent. Urologic and neuro-orthopedic manifestations, as well as UDS parameters, were compared before and after SCU. Categorical data were compared using Fisher-Exact test and continuous variables were compared using Wilcoxon-Signed-Rank test. A P-value <0.05 was considered significant. RESULTS: Twenty-two patients met the inclusion criteria. The median age at time of SCU was 11.5 months (range 3-211). The median age at time of follow-up UDS after SCU was 22 months (range 9-218). The median age at time of last follow-up was 153.5 months (range 65-228). The median follow-up time was 71 months (range 60-192). A total of 14/22 patients had clinical manifestation before SCU, while 8/22 were asymptomatic and diagnosed based on magnetic resonance imaging/UDS findings. Of the symptomatic patients, 86% had symptom improvement after SCU. The UDS parameters showed statistically significant improvement in the median percentage of change of actual bladder capacity (P = 0.01), median intravesical pressure for patients with pre-operative pressure ≥40 cm/H2O at total cystometric bladder capacity (P = 0.012), and median bladder compliance at 75% bladder capacity (P = 0.01) (Table). DISCUSSION: Tethered spinal cord syndrome (TSCS) is a clinical entity that presents with neurological, urological, and/or orthopedic symptoms caused by primary or secondary tethering of the spinal cord, which may result in ischemic damage of the neural tissue and symptom development. While some authors believe that surgical management should be reserved for symptomatic patients, others prefer prophylactic surgery to avoid possible irreversible neurological damage. The present study provides detailed discussion of the clinical and UDS outcomes for patients with primary TSC that underwent SCU. CONCLUSION: For patients with primary TSC, spinal cord untethering is beneficial in terms of clinical and UDS outcomes. A prospective long-term study with large numbers could further highlight outcomes for this particular group of patients.
Subject(s)
Neural Tube Defects/physiopathology , Neural Tube Defects/surgery , Urodynamics , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Retrospective Studies , Treatment Outcome , Young AdultABSTRACT
Stimulated Raman backscattering in plasma is potentially an efficient method of amplifying laser pulses to reach exawatt powers because plasma is fully broken down and withstands extremely high electric fields. Plasma also has unique nonlinear optical properties that allow simultaneous compression of optical pulses to ultra-short durations. However, current measured efficiencies are limited to several percent. Here we investigate Raman amplification of short duration seed pulses with different chirp rates using a chirped pump pulse in a preformed plasma waveguide. We identify electron trapping and wavebreaking as the main saturation mechanisms, which lead to spectral broadening and gain saturation when the seed reaches several millijoules for durations of 10's - 100's fs for 250 ps, 800 nm chirped pump pulses. We show that this prevents access to the nonlinear regime and limits the efficiency, and interpret the experimental results using slowly-varying-amplitude, current-averaged particle-in-cell simulations. We also propose methods for achieving higher efficiencies.
ABSTRACT
In regimes far beyond the wave-breaking threshold of Raman amplification, we show that significant amplification can occur after the onset of wave breaking, before phase mixing destroys the coherent coupling between pump, probe, and plasma wave. Amplification in this regime is therefore a transient effect, with the higher-efficiency "coherent wave-breaking" (CWB) regime accessed by using a short, intense probe. Parameter scans illustrate the marked difference in behavior between below wave breaking, in which the energy-transfer efficiency is high but total energy transfer is low, wave breaking, in which efficiency is low, and CWB, in which moderate efficiencies allow the highest total energy transfer.
ABSTRACT
We present Leap, a simulation model for Raman amplification in plasma, combining an envelope treatment of the laser fields with an electrostatic particle-in-cell solver. The code is fully two dimensional, with the model readily extendible to three dimensions, and includes dispersive and refractive effects. Simulations carried out for Raman amplification in a plasma channel show that guiding of both the pump and the probe contribute to the evolution of the probe, resulting in a shorter, more intense pulse.
ABSTRACT
BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive malignant brain tumor that, since it was first identified, has been treated with aggressive treatment regimens, e.g. high-dose chemotherapy with stem cell rescue and early radiotherapy. We reviewed our experience because of concerns with respect to treatment-related toxicity in our patients. METHODS: Seven patients with a median age at presentation of 18 months were diagnosed with AT/RT between 1996 and 2006. Tumor location was supratentorial in 2 patients, in the posterior fossa in 4 and spinal in 1. Gross total resection was performed in 1 patient, subtotal resection in 5 and biopsy only in 1. Adjuvant treatment consisted of chemotherapy and radiotherapy in 5 patients. RESULTS: Median progression-free survival was 4 months, and median overall survival was 7 months. Two children are alive at 44 and 102 months. Significant surgical and chemotherapy-related morbidity was seen. Biopsy-proven multifocal necrotizing leukoencephalopathy (MNL) was seen in one patient who is alive 44 months after diagnosis. Another patient who was thought to have recurrent tumor in the brainstem 9 months after diagnosis had imaging findings compatible with MNL. CONCLUSION: Although improving results are reported for AT/RT using intensive treatment regimens, treatment-related morbidity is considerable in this young patient population.
Subject(s)
Leukoencephalopathy, Progressive Multifocal/epidemiology , Leukoencephalopathy, Progressive Multifocal/surgery , Rhabdoid Tumor/epidemiology , Rhabdoid Tumor/surgery , Teratoma/epidemiology , Teratoma/surgery , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Leukoencephalopathy, Progressive Multifocal/diagnosis , Male , Morbidity , Necrosis , Retrospective Studies , Rhabdoid Tumor/diagnosis , Teratoma/diagnosis , Treatment OutcomeABSTRACT
BACKGROUND: Juvenile pilocytic astrocytomas (JPA), a subgroup of low-grade astrocytomas (LGA), are common, heterogeneous and poorly understood subset of brain tumours in children. Chromosomal 7q34 duplication leading to fusion genes formed between KIAA1549 and BRAF and subsequent constitutive activation of BRAF was recently identified in a proportion of LGA, and may be involved in their pathogenesis. Our aim was to investigate additional chromosomal unbalances in LGA and whether incidence of 7q34 duplication is associated with tumour type or location. METHODS AND RESULTS: Using Illumina-Human-Hap300-Duo and 610-Quad high-resolution-SNP-based arrays and quantitative PCR on genes of interest, we investigated 84 paediatric LGA. We demonstrate that 7q34 duplication is specific to sporadic JPA (35 of 53 - 66%) and does not occur in other LGA subtypes (0 of 27) or NF1-associated-JPA (0 of 4). We also establish that it is site specific as it occurs in the majority of cerebellar JPA (24 of 30 - 80%) followed by brainstem, hypothalamic/optic pathway JPA (10 of 16 - 62.5%) and is rare in hemispheric JPA (1 of 7 - 14%). The MAP-kinase pathway, assessed through ERK phosphorylation, was active in all tumours regardless of 7q34 duplication. Gain of function studies performed on hTERT-immortalised astrocytes show that overexpression of wild-type BRAF does not increase cell proliferation or baseline MAPK signalling even if it sensitises cells to EGFR stimulation. CONCLUSIONS AND INTERPRETATION: Our results suggest that variants of JPA might arise from a unique site-restricted progenitor cell where 7q34 duplication, a hallmark of this tumour-type in association to MAPK-kinase pathway activation, potentially plays a site-specific role in their pathogenesis. Importantly, gain of function abnormalities in components of MAP-Kinase signalling are potentially present in all JPA making this tumour amenable to therapeutic targeting of this pathway.
Subject(s)
Astrocytoma/genetics , Biomarkers, Tumor/genetics , Brain Neoplasms/genetics , Chromosomes, Human, Pair 7/genetics , Adolescent , Astrocytoma/metabolism , Astrocytoma/pathology , Blotting, Western , Brain Neoplasms/metabolism , Brain Neoplasms/pathology , Carrier Proteins/genetics , Child , Child, Preschool , Female , Fluorescent Antibody Technique , Gene Dosage , Gene Duplication , Humans , Immunohistochemistry , Male , Mitogen-Activated Protein Kinases/metabolism , Polymorphism, Single Nucleotide , Protein Serine-Threonine Kinases/genetics , Proto-Oncogene Proteins B-raf/genetics , Reverse Transcriptase Polymerase Chain Reaction , Signal Transduction/physiologyABSTRACT
BACKGROUND AND PURPOSE: Patients who have benign enlargement of the subarachnoid spaces (BESS) have long been suspected of having an increased propensity for subdural hematomas either spontaneously or as a result of accidental injury. Subdural hematomas in infants are often equated with nonaccidental trauma (NAT). A better understanding of the clinical and imaging characteristics of subdural hematomas that occur either spontaneously or as a result of accidental trauma may help distinguish this group of patients from those who suffer subdural hematomas as a result of NAT. The purpose of this study is to describe the clinical and imaging characteristics of subdural hematomas that occur either spontaneously or as a result of accidental injury in infants with BESS. METHODS: We conducted a retrospective review of all patients with BESS complicated by subdural hematomas evaluated at a single institution from 1998 to 2004. Data concerning the patient's clinical presentation, physical findings, imaging, and management are described. RESULTS: During the study period, 7 patients with BESS complicated by subdural hematoma were identified. Their mean age at identification of the subdural hematoma was 7.4 months of age. In 5 cases, there was no recognized trauma before identification of the subdural hematoma. In 3 cases, baseline CT or MR imaging was available, showing prominent subarachnoid spaces without any evidence of subdural hemorrhage. CONCLUSION: Although suspicious for NAT, subdural hematomas can occur in children either spontaneously or as a result of accidental trauma. Caution must be exercised when investigating for NAT based on the sole presence of subdural hematomas, especially in children who are otherwise well and who have BESS.
Subject(s)
Battered Child Syndrome/diagnosis , Hematoma, Subdural/diagnosis , Image Processing, Computer-Assisted , Magnetic Resonance Imaging , Subarachnoid Space/pathology , Tomography, X-Ray Computed , Accidents, Traffic , Diagnosis, Differential , Dilatation, Pathologic , Dominance, Cerebral/physiology , Female , Head Injuries, Closed/diagnosis , Humans , Infant , Male , Retrospective Studies , Subdural Space/pathologyABSTRACT
BACKGROUND: Patients with hypothalamic hamartomas (HH) often have severe refractory epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Attempts to control the seizure disorder by resection of apparently epileptogenic mesial temporal or other cortical structures have failed consistently. OBJECTIVE: To report a series of 13 patients in whom the hamartoma itself was resected. METHODS: All patients underwent preoperative evaluation between ages 2 and 33 years and had subtotal or complete resection of the hamartoma. Follow-up ranged from 1 to 5.5 years (mean: 2.8 y). RESULTS: Preoperatively, all patients had variable combinations of gelastic, complex partial, and generalized seizures. Eight had drop attacks. In addition, all had marked behavior abnormalities and cognitive impairment. Postoperatively, two patients are completely seizure-free and 11 are either seizure-free or have achieved a greater than 90% reduction of drop attacks and generalized tonic-clonic seizures. However, minor gelastic, complex partial, and atypical absence seizures have persisted in 11 patients, although at significantly reduced rates. In addition, there has been a dramatic improvement in behavior and cognition. Three patients had an anterior thalamic and one a capsular infarct, which left only minimal long-term deficits. Exact location of the lesion in relation to the interpeduncular fossa and the walls of the third ventricle correlated with extent of excision, seizure control, and complication rate. CONCLUSION: Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.
Subject(s)
Epilepsy/etiology , Hamartoma/complications , Hamartoma/surgery , Hypothalamic Neoplasms/complications , Hypothalamic Neoplasms/surgery , Adolescent , Adult , Brain/diagnostic imaging , Brain/pathology , Brain/surgery , Child , Child, Preschool , Cognition Disorders/etiology , Electroencephalography , Epilepsy/therapy , Female , Follow-Up Studies , Hamartoma/diagnosis , Humans , Hypothalamic Neoplasms/diagnosis , Lamotrigine , Magnetic Resonance Imaging , Male , Neurosurgical Procedures/adverse effects , Paresis/etiology , Recovery of Function , Remission Induction , Tomography, Emission-Computed , Treatment Outcome , Triazines/therapeutic useABSTRACT
OBJECT: Selective posterior rhizotomy is a well-established treatment for spasticity associated with cerebral palsy. At most medical centers, responses of dorsal rootlets to electrical stimulation are used to determine ablation sites; however, there has been some controversy regarding the reliability of intraoperative stimulation. The authors analyzed data obtained from the McGill Rhizotomy Database to determine whether motor responses to dorsal root stimulation were reproducible. METHODS: A series of 77 patients underwent selective dorsal rhizotomy at a single medical center. The dorsal roots from L-2 to S-2 were stimulated to determine the threshold amplitude. The roots were then stimulated at 2 to 4 times the highest threshold with a 1-second 50-Hz train. A second stimulation run of the entire dorsal root was performed before it was divided into rootlets. Rootlets were individually stimulated and sectioned according to the extent of abnormal electrophysiological propagation. Motor responses were recorded by electromyography and were also assessed by a physiotherapist, and grades of 0 to 4+ were assigned. The difference in grades between the first and second stimulation trains was determined for 752 roots. Statistical analysis demonstrated a clear consistency in motor responses between the two stimulation runs, both in the electromyographic readings and the physiotherapist's assessment. More than 93% of dorsal roots had either no change or a difference of only one grade between the two trials. Furthermore, the vast majority of dorsal roots assigned a grade of 4+ at the first trial maintained the same maximally abnormal electrophysiological response during the second stimulation run. CONCLUSIONS: This study indicates that currently used techniques are reproducible and reliable for selection of abnormal rootlets. Intraoperative electrophysiological stimulation can be valuable in achieving a balance between elimination of spasticity and preservation of underlying strength.
Subject(s)
Cerebral Palsy/surgery , Electromyography , Monitoring, Intraoperative , Muscle Spasticity/surgery , Rhizotomy/methods , Cerebral Palsy/physiopathology , Child , Child, Preschool , Electric Stimulation , Female , Ganglia, Spinal/physiopathology , Ganglia, Spinal/surgery , Humans , Isometric Contraction/physiology , Male , Motor Neurons/physiology , Muscle Spasticity/physiopathology , Muscle, Skeletal/innervation , Prospective Studies , Treatment OutcomeABSTRACT
Case records of 37 patients with a diagnosis of brainstem glioma treated at the Montreal Children's Hospital from June 1989 to June 1999 were reviewed. 15 patients had diffuse pontine gliomas and 22 patients had focal forms of brainstem gliomas. The two groups were compared with respect to age, clinical evolution, radiological appearance, type of surgery practised, histological diagnosis, adjuvant treatments and survival. A non-pontine brainstem location, a cystic or exophytic component, bright enhancement with gadolinium injection, a histological diagnosis of pilocytic astrocytoma or ganglioglioma were favourable prognostic factors. Progression-free survival and overall survival were significantly worse in the group of patients with diffuse pontine gliomas. The relative impact of radical surgery and/or radiotherapy is analysed. Surgery coupled to adjuncts such as navigation, ultrasound and monitoring plays an important role for focal brainstem lesions. Focal/conformal radiotherapy has an adjuvant role but better treatments are needed for the diffuse pontine brainstem lesions.
Subject(s)
Brain Stem/radiation effects , Brain Stem/surgery , Glioma/radiotherapy , Glioma/surgery , Adolescent , Brain Stem/pathology , Child , Child, Preschool , Female , Follow-Up Studies , Gadolinium , Glioma/pathology , Humans , Magnetic Resonance Imaging , Male , Microsurgery , Monitoring, Intraoperative , Radiopharmaceuticals , Radiotherapy Dosage , Retrospective Studies , Time FactorsABSTRACT
Residual seizures after functional hemispherectomy occur in approximately 20% of patients with catastrophic epilepsy. These episodes are traditionally attributed to incomplete disconnection, persistent epileptogenic activity in the ipsilateral insular cortex, or bilateral independent epileptogenic activity. The authors report on the case of an 8-year-old boy with an intractable seizure disorder who had classic frontal adversive seizures related to extensive unilateral left hemispheric cortical dysplasia. The initial intervention consisted of extensive removal of the epileptic frontal and precentral dysplastic tissue and multiple subpial transections of the dysplastic motor strip, guided by intraoperative electrocorticography, Subsequently, functional hemispherectomy including insular cortex resection was performed for persistent attacks. After a seizure-free period of 6 months, a new pattern ensued, consisting of an aura of fear, dystonic posturing of the right arm, and unusual postictal hyperphagia coupled with an interictal diencephalic-like syndrome. Electroencephalography and ictal/interictal single-photon emission computerized tomography were used to localize the residual epileptic discharges to deep ipsilateral structures. Results of magnetic resonance imaging indicated a complete disconnection except for a strip of residual frontobasal tissue. Therefore, a volumetric resection of the epileptogenic frontal basal tissue up to the anterior commissure was completed. The child has remained free of seizures during 21 months of follow-up review. Standard hemispherectomy methods provide extensive disconnection, despite the presence of residual frontal basal cortex. However, rarely, and especially if it is dysplastic, this tissue can represent a focus for refractory seizures. This is an important consideration in determining the source of ongoing seizures posthemispherectomy in patients with extensive cortical dysplasia. It remains important to assess them fully before considering their disease refractory to surgical treatment.
Subject(s)
Cerebral Cortex/abnormalities , Cerebral Cortex/surgery , Cerebral Decortication , Epilepsy, Generalized/surgery , Postoperative Complications/pathology , Child , Epilepsy, Generalized/diagnostic imaging , Epilepsy, Generalized/pathology , Humans , Magnetic Resonance Imaging , Male , Postoperative Complications/diagnostic imaging , Seizures/pathology , Seizures/surgery , Tomography, Emission-Computed, Single-PhotonABSTRACT
OBJECTIVE: We discuss the clinical aspects, pathology, and molecular genetics of 7 patients with primitive neuroectodermal tumors (PNETs) arising in the brainstem that were treated at our institution from 1986 through 1995. Most neuro-oncologists avoid performing biopsies in children with pontine tumors. This article raises the question as to whether biopsies should be performed, because treatment recommendations might differ if a PNET was diagnosed rather than a pontine glioma. PATIENTS AND METHODS: We reviewed the clinical neuro-oncology database and the files of the Division of Neuropathology at New York University Medical Center from 1986 through 1995 and identified 7 histologically confirmed PNETs arising in the brainstem among 146 pediatric brainstem tumors. The clinical, neuroradiological, and neuropathological data were reviewed. Postmortem examinations were performed in 2 cases. Formalin-fixed, paraffin-embedded tumor tissues were also available in 6 of 7 patients that were tested for p53 gene mutations using single-strand conformation polymorphism analysis. We also tested 9 cerebellar PNETs, 9 brainstem gliomas, and 3 normal brains for p53 gene mutations as controls. RESULTS: All 7 patients presented with focal cranial nerve deficits, and 2 were also hemiparetic. The median age at diagnosis was 2.7 (1-8 years). Magnetic resonance imaging (MRI) characteristics included a focal intrinsic exophytic nonenhancing brainstem lesion that had low T1-weighted and high T2-weighted signals. Hydrocephalus was present in 5 patients at diagnosis, 3 of whom had leptomeningeal dissemination. Meningeal dissemination occurred later in the course of the disease in 3 other patients. Five children required shunts at diagnosis and another 2 at recurrence. Despite therapy, all 7 PNET patients died within 17 months of diagnosis with a mean survival of 8 (4-17) months. No mutation in the p53 gene was detected. CONCLUSIONS: Brainstem PNETs tend to arise at a younger age than brainstem gliomas and medulloblastomas. The MRI pattern suggests a localized rather than a diffuse intrinsic nonenhancing brainstem tumor. Like other PNETs, brainstem PNETs have a high predilection to disseminate within the central nervous system. The absence of p53 mutations is similar to other PNETs. Despite their origin close to the cerebellum, brainstem PNETs exhibit a more aggressive behavior and result in worse clinical outcomes than do cerebellar PNETs.
Subject(s)
Brain Neoplasms/diagnosis , Brain Stem/pathology , Neuroectodermal Tumors/diagnosis , Brain Neoplasms/genetics , Brain Neoplasms/pathology , Brain Stem/chemistry , Child , Child, Preschool , Female , Genes, p53/genetics , Glioma/diagnosis , Glioma/genetics , Glioma/pathology , Humans , Immunoenzyme Techniques , Infant , Male , Mutation , Neuroectodermal Tumors/genetics , Neuroectodermal Tumors/pathology , Polymorphism, GeneticABSTRACT
Mesial temporal sclerosis (MTS) is the most frequently encountered lesion in adult patients with intractable temporal epilepsy; it is found in isolation in approximately two-thirds of surgically treated cases. Whereas the exact etiology of MTS is still controversial, several reports suggest that this pathologic lesion is both the cause and the consequence of chronic seizures and develops progressively during childhood secondary to recurrent seizures. In order to evaluate the clinical importance of MTS in children, we retrospectively reviewed the clinical charts of children who underwent surgery for medically intractable temporal epilepsy and report cases presenting an amygdalo-hippocampic dual pathology. Six children aged 1.5-16 years (mean +/- SD: 7.5 +/- 3 years) presenting with partial complex seizures (5 cases) or extension spasms (1 case), with onset from 6 months to the age of 8.5 years (mean seizure onset +/- SD: 3 +/- 5 years) underwent anterior temporal lobectomy including resection of the amygdala and hippocampus. All patients exhibited variable degrees of severity of neuronal loss and gliosis in the amygdala and/or hippocampus. The pathological picture of MTS was not isolated, however. Careful pathological examination has thus shown foci of amygdalo-hippocampic neuronal dysplasia in six patients, with concomitant bilaminated fascia dentata in two cases. Postoperatively, no mortality or morbidity was encountered. After a mean follow-up of 2.5 years, four patients are seizure free. One patient had a 80% rate of improvement in seizure frequency, though still having occasional febrile convulsions. In another patient, complex partial seizures resolved, but rare episodes of absence were still observed. These data are in keeping with the hypothesis that MTS could be secondary to repeated seizures. The analysis of this series of patients could suggest that mesiotemporal dysplastic lesions within the amygdalo-hippocampic structures induce seizures, which, in turn, will favor the development of MTS during childhood. MTS could then lead to synaptic reorganization, which can express abnormal hyperexcitability and result in more recurrent seizures. In this way a vicious circle is set up, which may explain the progression of seizures in some patients.
Subject(s)
Epilepsy, Complex Partial/surgery , Epilepsy, Temporal Lobe/surgery , Temporal Lobe/surgery , Adolescent , Adult , Amygdala/pathology , Amygdala/surgery , Child , Child, Preschool , Epilepsy, Complex Partial/pathology , Epilepsy, Temporal Lobe/pathology , Female , Follow-Up Studies , Gliosis/pathology , Gliosis/surgery , Hippocampus/pathology , Hippocampus/surgery , Humans , Male , Neurons/pathology , Psychosurgery , Sclerosis , Temporal Lobe/pathologySubject(s)
Craniopharyngioma/surgery , Neurosurgical Procedures/methods , Pituitary Neoplasms/surgery , Adolescent , Adult , Child , Child, Preschool , Craniopharyngioma/pathology , Female , Humans , Male , Neurosurgical Procedures/adverse effects , Pituitary Neoplasms/pathology , Preoperative Care , Retrospective Studies , Sphenoid Bone/surgery , Survival Analysis , Treatment OutcomeABSTRACT
Developments in imaging and in neurosurgical techniques over the past decade have substantially altered the management of children with low-grade astrocytoma. Indications for surgery have become more clearly defined, and a larger proportion of children undergo complete or subtotal resection than in the past. Fewer receive adjuvant therapy, even though the options in this regard are more numerous now and theoretically likely to result in less morbidity than conventional external beam radiotherapy. This review will address in particular the correlations between location, imaging appearance, and behavior that need to be more widely appreciated, and present recommendations regarding the management of these tumors.
Subject(s)
Astrocytoma/therapy , Brain Neoplasms/therapy , Astrocytoma/complications , Astrocytoma/diagnostic imaging , Astrocytoma/pathology , Astrocytoma/radiotherapy , Astrocytoma/surgery , Brain Neoplasms/complications , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/pathology , Brain Neoplasms/radiotherapy , Brain Neoplasms/surgery , Child , Combined Modality Therapy , Humans , Radiography , Survival RateABSTRACT
PURPOSE: We evaluated the role of magnetic resonance imaging (MRI) of the lumbosacral spinal cord in children with complicated voiding dysfunction and normal neuro-orthopedic examination. MATERIALS AND METHODS: We reviewed the records of 32 consecutive children with complicated enuresis who were referred for neurosurgical evaluation, including those with a history of refractory voiding dysfunction or incontinence associated with persistent vesicoureteral reflux, encopresis, or associated leg or back pain. Nine patients were excluded from study because of urethral or anorectal anomalies, or failure to meet the inclusion criteria. Eligible for study inclusion were 23 children with a mean age of 8.9 years. Complete neurological and orthopedic examinations were normal in all patients except 1 with mild scoliosis and 1 with congenital facial palsy. RESULTS: Urodynamic studies revealed instability in 14 cases, hypertonia in 7, hyporeflexia in 2 and detrusor-sphincter dyssynergia in 4. Skeletal abnormalities, mostly spina bifida occulta, were detected in 16 of the 23 children (70%). Spinal MRI was normal in 21 patients (91.3%), including 1 with a tethered cord and lipoma associated with a complex skeletal abnormality, and 1 with a nonprogressive, nonsurgical T7 to T9 syrinx. Only the case of lipoma required neurosurgical intervention. CONCLUSIONS: The value of MRI is limited in children with voiding dysfunction and a normal neuro-orthopedic assessment. This study should be reserved for patients with associated neuroorthopedic findings or complex skeletal deformity on plain x-ray.
Subject(s)
Enuresis/pathology , Magnetic Resonance Imaging , Urinary Incontinence/pathology , Adolescent , Child , Child, Preschool , Coccyx/pathology , Female , Humans , Lumbar Vertebrae/pathology , Male , Sacrum/pathologyABSTRACT
PURPOSE: Approximately a third of children with cerebral palsy are expected to present with dysfunctional voiding symptoms. Selective dorsal rhizotomy, which is indicated for managing lower extremity spasticity in children with cerebral palsy, also has the potential of affecting bladder function. We evaluate the impact of selective dorsal rhizotomy on bladder function by comparing preoperative and postoperative symptoms, and urodynamic parameters in children undergoing selective dorsal rhizotomy for spasticity. MATERIALS AND METHODS: We reviewed urodynamic studies in 25 boys and 15 girls with a mean age plus or minus standard deviation of 5.43+/-2.1 years who underwent selective dorsal rhizotomy for spasticity at our institution between January 1992 and September 1995. Urodynamics were performed preoperatively only in 22 patients, preoperative and postoperatively in 13, and postoperatively only in 5. Preoperative urodynamic studies were done within 2 weeks of surgery and postoperative studies were done at least 6 months after surgery (mean 1.32+/-0.65 years). RESULTS: Of the 35 patients with preoperative urodynamic studies total bladder capacity, pressure specific volumes and full resting pressure were abnormal for age in 23 (65.7%). In addition, 17 of the 23 children (74%) were completely asymptomatic. In the group that underwent preoperative and postoperative urodynamic studies there was significant improvement in total bladder capacity (p <0.005) and pressure specific volumes (p <0.005) using the paired Student t test. All children had neurological improvement postoperatively, 5 of 7 (71%) who were incontinent preoperatively became continent and none had deterioration on urodynamics. CONCLUSIONS: At least half of the children with spastic cerebral palsy have clinically silent bladder dysfunction. Selective dorsal rhizotomy improves spasticity and significantly improves bladder storage characteristics. We propose that urodynamic studies be included in the evaluation of children with spastic cerebral palsy who are possible candidates for selective dorsal rhizotomy to treat lower limb spasticity.
Subject(s)
Cerebral Palsy/complications , Rhizotomy , Urinary Bladder Diseases/surgery , Urinary Bladder/physiopathology , Cerebral Palsy/physiopathology , Child , Child, Preschool , Female , Humans , Male , Muscle Spasticity , Postoperative Period , Preoperative Care , Rhizotomy/methods , Urinary Bladder Diseases/etiologyABSTRACT
PURPOSE: To assess the efficacy of functional hemispherectomy in promoting language acquisition in a child with severe language delay and continuous left hemispheric epileptiform discharge in the absence of clinical seizures. We report a 6-month-old girl who initially presented with a right hemiparesis secondary to a lesion of probable prenatal origin in the distribution of the left middle cerebral artery. Weeks later, she developed infantile spasms. At 1.5 years of age, because of intractable seizures, the patient had fenestration of the left porencephalic cyst and anterior temporal lobectomy. The seizures ceased; however, language development remained limited to 35 words at 3.5 years of age. The EEG showed almost continuous epileptiform activity over the left hemisphere with no independent epileptiform potentials contralaterally. She underwent a functional left hemispherectomy. METHODS: Imaging, electroencephalographic investigation, pre- and postoperative psychological and speech assessment were carried out. RESULTS: Considerable language, speech, and behavior improvement was noted within 2 months of surgery. CONCLUSIONS: Interictal epileptiform discharges can interfere with the development of contralateral hemispheric function in the absence of clinical seizures. Early functional hemispherectomy may have a role in promoting optimal language development in appropriately selected patients. Although the primary indication for functional hemispherectomy is to control intractable seizures, a secondary proposed indication is to reduce functional impairment of the other hemisphere by electrical interference.
Subject(s)
Brain/surgery , Epilepsy/surgery , Functional Laterality/physiology , Language Development Disorders/surgery , Brain/physiopathology , Brain Diseases/surgery , Child, Preschool , Cysts/surgery , Electroencephalography , Epilepsy/diagnosis , Epilepsy/physiopathology , Female , Humans , Infant , Language Development Disorders/physiopathology , Temporal Lobe/surgery , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Tumors arising in the brain stem, comprising the midbrain, pons, and medulla oblongata, are now recognized as distinct clinico-pathological entities. Advances in neurosurgical techniques have made surgery not only feasible but the treatment of choice for some of these tumor types. Previously the mainstay of treatment, radiotherapy is now used more selectively. This article reviews the current state of knowledge with regard to tumors arising in the brain stem, the therapeutic options available for each, and provides recommendations with regard to management.