Successful fetal surgery for spina bifida.
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Adult↗
Biomedical subjects
L N Sutton
Publications and source records attributed to L N Sutton.
Prognostic value of proton MR spectroscopy of cerebral hemisphere tumors in children.
We studied 14 young people with newly diagnosed hemisphere tumors, aged from 3 to 20 years (average 10 years). All underwent surgery following MR imaging (MRI) and spectroscopy (MRS). The tumors studied were three glioblastomas, one each of ganglio-glioblastoma, primitive neuroectodermal tumor (PNET), rhabdoid teratoid tumor, pilocytic astrocytoma, ependymoma, anaplastic ependymoma, and gliomatosis cerebri, and four gangliogliomas. Four patients died; ten patients are alive (five with stable residual tumor, five with no evident tumor). Images and spectra were acquired on a 1.5-T imager. Proton MRS was performed before gadolinium injection in all but one case. Single-voxel techniques were utilized in all cases, using a spin-echo or STEAM sequence with a long echo time (135 or 270 ms). Peak areas of N-acetyl aspartate (NAA), choline (Cho), and creatine and phosphocreatine (Cr) were assessed. The NAA/Cho peak-area ratio was very low in the patients who died (mean +/- s.d. 0.20 +/- 0.14), and higher in the patients who are alive (0.74 +/- 0.47; P = 0.007 by two-tailed t-test). The Cr/Cho peak-area ratio also followed a similar trend for the two groups (mean +/- s.d. 0.17 +/- 0.07 and 0.49 +/- 0.30, respectively; P = 0.01 by two-tailed t-test).
Brain↗
Molecular cytogenetic studies of pediatric ependymomas.
Cytogenetic and molecular studies of ependymomas have previously demonstrated deletions of chromosomes 17 and 22 as frequent abnormalities, implicating inactivation of tumor suppressor genes in the pathogenesis of these tumors. In the present study, we analyzed 22 childhood ependymomas by standard cytogenetic analysis, fluorescence in situ hybridization (FISH) and polymerase chain reaction (PCR)-based microsatellite analysis of chromosomes 17 and 22. Microsatellite analysis of chromosome 6 was performed to identify submicroscopic deletions implicated by the cytogenetic studies. Among the 22 cases, we demonstrated loss of chromosome 22 in 2 patients, deletion of chromosome 17 in 2 patients, and rearrangements or deletions of chromosome 6 in 5 patients. These data do not suggest that loss of a gene on chromosome 17p plays a primary role in the initiation of pediatric ependymomas. This is in contrast to what has been reported for pediatric CNS primitive neuroectodermal tumors and malignant astrocytomas, in which deletion of 17p is regarded as a primary event. Furthermore, loss of chromosome 22 may define a subset of ependymomas more commonly seen in adults. Cytogenetic studies in this series, however, suggest that a region on the long arm of chromosome may be involved in the development and/or progression of ependymomas in children.
Adolescent↗
Visual impairment associated with mutism after posterior fossa surgery in children.
OBJECTIVE: To report four children with visual impairment associated with mutism after posterior fossa surgery. Mutism after posterior fossa surgery is a well-described phenomena, but to our knowledge, visual impairment has not been reported in association with it. METHODS: Record review of four children (age range, 3-7 yr) who underwent posterior fossa surgery (via suboccipital craniotomies) for removal of a medulloblastoma (three patients) or ependymoma (one patient). Each presented with headache, ataxia, or nausea and vomiting, but none had preoperative visual complaints other than diplopia. Postoperatively, all patients were mute, and because of apparent visual loss, neuro-ophthalmic consultation was requested. Postoperative scans and examinations were also reviewed. RESULTS: Each child was awake but appeared withdrawn without verbal output. No child blinked to threat or fixed or followed. In each case, pupillary reactivity was normal, and funduscopic examinations revealed only papilledema. One child reached for money. Within weeks or months postoperatively, the mutism spontaneously resolved, and visual behavior in general improved, roughly in parallel. During the follow-up period, papilledema resolved and the disc color was normal in each case. Magnetic resonance images obtained postoperatively revealed nothing remarkable, except surgical defects, without lesions in the retrogeniculate pathway. CONCLUSION: Impaired visual behavior, mimicking cortical visual loss, may be associated with mutism after posterior fossa surgery in children. The prognosis for recovery is excellent and parallels the return of normal speech. The mechanism is unclear.
Brain Neoplasms↗
Surgical removal of a choroid plexus adenoma using the argon beam coagulator: technical case report.
OBJECTIVE AND IMPORTANCE: Choroid plexus tumors may be extremely vascular, limiting surgical resection. A case of a choroid plexus adenoma is presented in which the argon beam coagulator (System 6500; ConMed Corp., Utica, NY) was used as a surgical adjunct. CLINICAL PRESENTATION: A 12-year-old male patient with a giant tumor of the lateral ventricle presented with visual loss and was found to have a large intraventricular tumor. At surgery, the tumor was extremely vascular, and the pathological finding was consistent with a choroid plexus adenoma. TECHNIQUE: Because of its vascularity, the tumor was resected in stages with the aid of the argon beam coagulator. CONCLUSION: We conclude that this device may have application in selected neurosurgical operations.
Adenoma↗
Current neurosurgical management and the impact of the extent of resection in the treatment of malignant gliomas of childhood: a report of the Children's Cancer Group trial no. CCG-945.
OBJECT: One hundred seventy-two children with high-grade astrocytomas were treated by members of the Children's Cancer Group in a prospective randomized trial designed to evaluate the role of two chemotherapy regimens. Seventy-six percent of the patients (131 children) in whom a diagnosis of either anaplastic astrocytoma or glioblastoma multiforme was confirmed by central pathological review are the subject of this report. METHODS: Patients were stratified according to the extent of tumor resection (biopsy [< 10%], partial resection [10-50%], subtotal resection [51-90%], near-total resection [> 90%], and total resection) as determined by surgical observation and postoperative computerized tomography scanning. Information on contemporary neurosurgical management was obtained from the patient's operative records and standardized neurosurgical report forms. The vast majority of tumors were supratentorial: 63% (83 tumors) in the superficial cerebral hemisphere, 28% (37 tumors) in the deep or midline cerebrum, and only 8% (11 tumors) in the posterior fossa. A significant association was detected between the primary tumor site and the extent of resection (p < 0.0001). A radical resection (> 90%) was performed in 37% of the children: 49% of the tumors in the superficial hemisphere and 45% of tumors in the posterior fossa compared with 8% of midline tumors. Tumor location could also be used to predict the need for both temporary and permanent cerebrospinal fluid (CSF) diversion. Half of the deep tumors and 8% of the hemispheric astrocytomas ultimately required a permanent CSF shunt. Improvement in preoperative neurological deficits and level of consciousness was seen in 36% and 34% of the children, respectively. New or increased deficits were present in 14% of the children, with 6% experiencing a diminished sensorium after surgery. Postoperative nonneurological complications were rare: infection, hematoma, and CSF fistula each occurred in 1.7% of the children. Univariate and multivariate analyses demonstrated that radical tumor resection (> 90%) was the only therapeutic variable that significantly improved progression-free survival (PFS) rates. For all patients with malignant astrocytomas, the distributions of PFS rates were significantly different (p = 0.006) following radical resection compared with less extensive (< or = 90%) resection. The 5-year PFS rates were 35 +/- 7% and 17 +/- 4%, respectively. The differences in the distribution of PFS rate were significant for the subsets of patients with anaplastic astrocytoma (p = 0.055) and glioblastoma multiforme (p = 0.046). The 5-year PFS rates for anaplastic astrocytoma were 44 +/- 11% and 22 +/- 6% for cases in which the tumor was radically resected and less than radically resected, respectively; whereas the 5-year PFS rates for glioblastoma multiforme were 26 +/- 9% and 4 +/- 3% for cases in which the tumor was radically resected and less than radically resected, respectively. CONCLUSIONS: The demonstration of a survival advantage provided by radical resection should prompt neurosurgeons to treat malignant pediatric astrocytomas with aggressive surgical resection prior to initiation of radiotherapy or adjuvant chemotherapy.
Adolescent↗
Neurofibromatosis type 1: brain stem tumours.
We describe the clinical and imaging findings of brain stem tumours in patients with neurofibromatosis type 1 (NF1). The NF1 patients imaged between January 1984 and January 1996 were reviewed and 25 patients were identified with a brain stem tumour. Clinical, radiographical and pathological results were obtained by review of records and images. Brain stem tumour identification occurred much later than the clinical diagnosis of NF1. Medullary enlargement was most frequent (68%), followed by pontine (52%) and midbrain enlargement (44%). Patients were further subdivided into those with diffuse (12 patients) and those with focal (13 patients) tumours. Treatment for hydrocephalus was required in 67% of the first group and only 15% of the second group. Surgery was performed in four patients and revealed fibrillary astrocytomas, one of which progressed to an anaplastic astrocytoma. In 40% of patients both brain stem and optic pathway tumours were present. The biological behaviour of brain stem tumours in NF1 is unknown. Diffuse tumours in the patients with NF1 appear to have a much more favourable prognosis than patients with similar tumours without neurofibromatosis type 1.
Adolescent↗
Prognostic signs in the surgical management of plexiform neurofibroma: the Children's Hospital of Philadelphia experience, 1974-1994.
OBJECTIVES: To estimate the rate of progression of plexiform neurofibroma after surgery and to identify prognostic factors that predict progression. STUDY DESIGN: A retrospective review of the inpatient and outpatient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year period at a single large pediatric referral center. Data on age, location, indication for surgery, and extent of resection was analyzed for prognostic significance. RESULTS: The overall freedom from progression was 54%. Children < 10 years old had a shorter interval of tumor control than older children (p = 0.0004). Tumors of the head/neck/face fared worse than tumors of the extremities (p = 0.0003). Less extensive resection predicted shorter interval to progression (p < 0.0001). Indication for surgery was not of prognostic importance. In multivariable analysis older age and location in the extremities were predictors of a better outcome. CONCLUSIONS: Tumor progression is a serious problem for children with plexiform neurofibroma. Younger children, children with tumors of the head/neck/face, and tumors that cannot be nearly completely removed are at particular risk. These data may be useful in helping clinicians decide which patients and which tumors are most likely to benefit from surgical intervention.
Adolescent↗
Proton spectroscopy of suprasellar tumors in pediatric patients.
OBJECTIVE: Magnetic resonance imaging and computed tomography provide good anatomic detail of suprasellar tumors in pediatric patients but are not able to predict histology in many cases. Proton magnetic resonance spectroscopy provides metabolic data that may add to diagnostic specificity. We preoperatively performed localized proton magnetic resonance spectroscopy on pediatric patients with suprasellar tumors and correlated the results with the histological findings. Cyst fluid obtained from patients with craniopharyngiomas was studied with high-resolution magnetic resonance spectroscopy to better understand the in vivo data. METHODS: Nineteen patients aged 1 to 21 years underwent spectroscopy. Surgical pathological samples were obtained from 14 patients. In each of five patients, the presence of a solid chiasmatic mass in addition to clinical evidence of neurofibromatosis Type I allowed the presumptive diagnosis of chiasmatic astrocytoma. Thus, the study population included 6 patients with craniopharyngiomas, 10 with chiasmatic/hypothalamic astrocytomas, and 3 with pituitary adenomas. The data obtained were compared with those of healthy brain from age-matched participants. RESULTS: Spectroscopy was specific for the diagnosis. All craniopharyngiomas showed a dominant peak at 1 to 2 ppm, consistent with lactate or lipids, with trace amounts of other metabolites. This was confirmed using high-resolution spectroscopy. Chiasmatic gliomas showed a profile of choline, N-acetylaspartate, and creatine, and the choline:N-acetylaspartate ratio was 2.6 +/- 1.3, compared with 0.7 +/- 0.3 for samples of healthy brain (t test, P = 0.0003). Pituitary adenomas showed only a choline peak or no metabolites at all. CONCLUSION: Proton spectroscopy may be helpful in supplementing standard imaging for the preoperative diagnosis of three types of suprasellar tumors that are common in pediatric patients.
Adenoma↗
Primitive neuroectodermal tumors of the central nervous system.
Controversial issues relating to the pathobiology and classification of central nervous system primitive neuroectodermal tumors (PNETs) have plagued neuropathologists for more than 70 years. Hypotheses advanced in the mid-1920's have remained as fixed concepts in contemporary literature, largely consequent to repetitious support by a small number of neuropathologists despite a growing body of information discrediting these ideas from neuroembryologists, oncologists, neuroscientists and pathologists. Attention has largely focused upon PNETs arising in the cerebellum (commonly known as medulloblastomas ([MBs]), because about 80% of central nervous system (CNS) PNETs originate in this site. It has been asserted that the 20% which do not are biologically different, although most individuals agree that the histological features of PNETs that occur in different sites throughout the CNS are indistinguishable from those growing in the cerebellum. The historical aspects of this controversy are examined in the face of evidence that there is, in fact, a unique class of CNS tumors which should appropriately be regarded as primitive neuroectodermal in nature. Specifically, a number of different approaches to the problem have yielded data supporting this hypothesis. These approaches include the identification of patterns of expression among a variety of cellular antigens (demonstrated by the use of immunopathological techniques), molecular analyses of cell lines derived from these tumors, experimental production of PNETs and molecular genetic analyses. Differences of opinion among surgeons, oncologists and radiotherapists are typically resolved by conducting cooperative studies of patients with these tumors who are diagnosed and treated at multiple centers.
Central Nervous System Neoplasms↗
Progressive congenital kyphosis: report of five cases and review of the literature.
For over 60 years congenital kyphotic deformities of the spine have been categorized into two distinct groups, depending on the developmental defect. Those arising from a failure of formation of the vertebral bodies were classified as type 1, while those arising from a failure of segmentation were referred to as type 2. Recognition of the progressive and unstable nature of the type 1 defects alerted physicians to the need for early operative stabilization through decompression and stabilization through instrumentation. As the embryogenesis of the spinal column was further investigated, and as diagnostic imaging methods of the spine improved, unstable congenital kyphoses were further subdivided. Progressive congenital kyphotic deformities now may accompany a host of vertebral column developmental defects as well as genetically mediated mesenchymal tissue defect syndromes. This paper presents 5 patients from The Children's Hospital of Philadelphia with progressive and symptomatic congenital kyphotic deformities of the spine. Two of these lesions resulted from defects of formation of the vertebral bodies, while one resulted from segmental spinal dysgenesis, maldevelopment of both the anterior and posterior vertebral elements. One patient's kyphotic deformity was a result of caudal regression syndrome, and the final case presented experienced a high thoracic kyphosis from a syndrome associated diffuse midline mesenchymal tissue abnormalities known as cerebrocostomandibular syndrome. All patients showed evidence of progressive cord compression and required neural element decompression, fusion, and instrumentation. The cases are discussed individually, and the developmental and clinical aspects of each are explored.
Bone Transplantation↗
Thirteen-year-old female with hemangioblastoma.
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Adolescent↗
Two-year-old boy with seizures.
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Brain Neoplasms↗
Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years.
Gangliogliomas account for 1-4% of all pediatric CNS tumors. We reviewed the records of 123 patients treated at the Children's Hospital of Philadelphia between 1974 and 1995. Ninety-nine patients were eligible for review with complete medical records. The mean age was 9.5 years with 52% females. The mean follow-up was 3.7 years. The most common presenting symptom was seizures (49%). The mean length of symptoms to diagnosis was 24.4 months. Complex partial seizure was the most frequent seizure type (60%). Electroencephalograms were abnormal in 36%. The majority of gangliogliomas were in the temporal lobes (38%), with other sites in the parietal (30%) and frontal lobes (18%). All patients had abnormal nonspecific neuroimaging studies. Nine received chemotherapy, and 21 were treated with radiotherapy. Postoperatively, 78% were seizure free on no medication, 18% had improvement of seizure control, and 4% had stable seizure events. Nine died of disease. Gangliogliomas are benign tumors that frequently present with seizure disorders and nonspecific electroencephalographic and neuroimaging studies. Complete surgical extirpation of these tumors provides improved seizure control in most patients and a short course of antiepileptic medical therapy. Adjuvant therapy including radiotherapy and chemotherapy is limited to recurrent disease not amenable to reoperation.
Adolescent↗
Prediction of posterior fossa tumor type in children by means of magnetic resonance image properties, spectroscopy, and neural networks.
Recent studies have explored characteristics of brain tumors by means of magnetic resonance spectroscopy (MRS) to increase diagnostic accuracy and improve understanding of tumor biology. In this study, a computer-based neural network was developed to combine MRS data (ratios of N-acetyl-aspartate, choline, and creatine) with 10 characteristics of tumor tissue obtained from magnetic resonance (MR) studies, as well as tumor size and the patient's age and sex, in hopes of further improving diagnostic accuracy. Data were obtained in 33 children presenting with posterior fossa tumors. The cases were analyzed by a neuroradiologist, who then predicted the tumor type from among three categories (primitive neuroectodermal tumor, astrocytoma, or ependymoma/other) based only on the data obtained via MR imaging. These predictions were compared with those made by neural networks that had analyzed different combinations of the data. The neuroradiologist correctly predicted the tumor type in 73% of the cases, whereas four neural networks using different datasets as inputs were 58 to 95% correct. The neural network that used only the three spectroscopy ratios had the least predictive ability. With the addition of data including MR imaging characteristics, age, sex, and tumor size, the network's accuracy improved to 72%, consistent with the predictions of the neuroradiologist who was using the same information. Use of only the analog data (leaving out information obtained from MR imaging), resulted in 88% accuracy. A network that used all of the data was able to identify 95% of the tumors correctly. It is concluded that a neural network provided with imaging data, spectroscopic data, and a limited amount of clinical information can predict pediatric posterior fossa tumor type with remarkable accuracy.
Astrocytoma↗
Multifocal noninfectious granulomatous encephalitis in a child. Case report.
This 9-year-old boy presented with a multifocal, apparently noninfectious granulomatous process involving the central nervous system. Despite gross-total excision of the large left temporal lesion, it recurred after surgery. Serological studies and cultures failed to demonstrate an infectious agent, and histopathological investigation showed a nonspecific granulomatous process without vasculitis. The patient underwent a second craniotomy and was given corticosteroid therapy. There has been no recurrence during 3 years of follow-up review, the last year of which included no course of steroid medications. The case is presented as a noninfectious idiopathic granulomatous encephalitis that is responsive to surgery and corticosteroid therapy. To the authors' knowledge, it is the first reported case of its kind.
Brain↗
Ventriculofemoroatrial shunt: a viable alternative for the treatment of hydrocephalus. Technical note.
Children with shunted hydrocephalus often have a myriad of other medical conditions. When these concomitant problems involve the pleura, peritoneum, and/or the venous system, placement of the distal catheter may prove to be problematic. This report presents preliminary results in three hydrocephalic children following ventriculofemoroatrial shunt placement. The peritoneal and pleural cavities in each of these children were compromised and there was no vascular access into the superior vena cava due to intercurrent disease. An alternative technique for ventriculoperitoneal shunt placement was performed via the femoral vein. Fluoroscopic guidance was used to confirm the intraatrial position of the distal end of the shunt catheter. Follow-up review to date shows no complications. This newly described technique provides a feasible alternative to distal shunt catheter placement in patients in whom more traditional sites are unavailable.
Cerebrospinal Fluid Shunts↗
[Posterior fossa tumors in children].
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Adolescent↗