bluuebunny/crossref_metadata_2025 huggingface dataset:
Symptomatic Calcified Subdural Hematomas
Authors: Sharma Rewati Raman , Mahapatra Ashok , Pawar Sanjay J. , Sousa Jesus , Athale Sanjeev D.
Published: April 2003

Two unique cases of chronic calcified subdural hematomas are reported in children as a long-term complication of a ventriculoperitoneal shunt. Both the patients had undergone shunt procedures in infancy for congenital hydrocephalus. In one patient, the cause of the hydrocephalus was aqueduct stenosis, while in the second patient, a lumbar meningomyelocele was associated with hydrocephalus. In both these patients, a ventriculoperitoneal shunt was done in infancy. In one of them, following the shunt surgery, a bilateral subdural collection was noticed which required burr hole evacuation. Both the patients remained asymptomatic for 9 years, when they presented to our center with acute raised intracranial pressure and contralateral hemiparesis. Both the patients had a relatively short history and had altered sensorium at admission. Surprisingly, in both the patients, the CT scan showed significant mass effect producing calcified subdural hematomas. The shunt systems were found to be working well at surgery. Craniotomy and excision of the calcified subdural hematomas was undertaken. Postoperatively, the patients showed satisfactory recovery, and at discharge the patients were doing well. At the follow-up at the outpatient clinic, the patients were asymptomatic.
Multiple Recurrent Gram-Negative Cerebrospinal Fluid Shunt Infections Associated with a Patient with a Retained Ventricular Foreign Body
Authors: Shingadia Delane , Grant John , Bendet Moshe , Shulman Stanford T. , Yogev Ram
Published: April 2003

A 3-year-old boy experienced 10 recurrent gram-negative ventriculoperitoneal shunt (VPS) infections with identical strains over a 17-month period. Multiple cranial MRI and CT scans to identify a retained foreign body were negative. CT myelography and pressure infusion radionuclide cisternography were also unhelpful. Ventriculoscopy revealed a small retained foreign body which was successfully removed, and cultures subsequently yielded gram-negative organisms identical to the previously identified bacteria by pulsed field gel electrophoresis. No further infections were noted after removal of the retained fragment. Exploratory ventriculostomy should be considered in patients with recurrent VPS infections where other techniques fail to reveal a cause.
Management of Intrinsic Gliomas of the Tectal Plate in Children
Authors: Grant Gerald A. , Avellino Anthony M. , Loeser John D. , Ellenbogen Richard G. , Berger Mitchel S. , Roberts Theodore S.
Published: April 2003

The natural history, management, and long-term outcome for patients with benign, intrinsic tectal plate gliomas remain controversial in spite of their propensity to cause late-onset hydrocephalus. A 10-year retrospective review has identified 11 consecutive children with tectal plate lesions. Headache, vomiting, a decline in school performance, tremor, and complex partial seizures were common presenting symptoms. All patients presented with signs and symptoms of hydrocephalus. Magnetic resonance (MR) imaging delineated an intra-axial mass lesion of the midbrain primarily localized to the tectal plate which uniformly was hyperintense on T2-weighted imaging and had a more variable appearance on T1-weighted imaging and rare enhancement with gadolinium. No patient underwent surgical resection, chemotherapy, or radiotherapy. Three of 11 patients (27%) showed evidence of progression in size or a new focus of enhancement on MR imaging, which was clinically asymptomatic. In this series, no patient with a tectal plate lesion less than 1.5 cm in maximal diameter and without gadolinium enhancement showed any evidence of clinical or radiological progression. Although intrinsic tectal lesions in children are clinically indolent and the initial management consists of CSF diversion, these lesions may eventually progress and still warrant long-term follow-up with serial MR imaging.
Spinal Cord Tethering Associated with Amniotic Band Syndrome
Authors: Prabhu Vikram C. , Tomita Tadanori
Published: April 2003

Amniotic band syndrome (ABS) comprises fetal morphological abnormalities that may be associated with fibrous amniotic bands that damage developing fetal parts resulting in cutaneous scars, erosions and ulcerations, digital constricting bands, craniofacial and visceral anomalies. Multiple asymmetric encephaloceles and anencephaly are neural-tube-like defects previously reported with ABS. This is the first report of spinal dysraphism with dorsal spinal cord tethering associated with ABS. We examine the pathogenetic theories of ABS in light of this report.
Is Indomethacin Harmful in Spinal Cord Injury Treatment?
Authors: Guven Mehmet Bahadir , Cirak Bayram , Yuceer Nurullah , Ozveren Faik
Published: April 2003

This study was designed to analyze the effect of early indomethacin on the lipid peroxidation after spinal cord injury in rats. The use of anti-inflammatory drugs to affect delayed and secondary injury after trauma to the spinal cord has now become a matter of standard clinical practice. However, spinal cord injury remains an enormous clinical problem and research that may lead to improved treatment is to be encouraged and commended. Three experimental groups consisting of 40 rats each were formed. Using microsurgical technique, total laminectomy between T5 and T10 was performed. Spinal cord injury was achieved with an epidural aneurysm clip, and pharmacological treatment immediate after the injury was performed by injecting indomethacin intraperitoneally (i.p.) at a dose of 3 mg/kg to indomethacin-treated group. The three main groups were divided into subgroups of 8 rats each. It was planned to stop the biochemical reactions at a different time in each of these subgroups, by the application of liquid nitrogen to the spinal cord and paravertebral structures at the end of the 1st, 15th, 30th, 60th, and 90th minutes. All the spinal cords were removed and protected from further reactions by immersing in the liquid nitrogen tank. The lipid peroxidation levels were assessed by determining thiobarbituric acid reactive substances formation. The results of the study showed that the administration of 3 mg/kg indomethacin immediately after spinal cord injury induces lipid peroxidation to a significant degree (p < 0.05 one-way ANOVA and Tukey HSD tests) when compared to the saline-treated group. This result suggests that early posttraumatic indomethacin treatment may be harmful in spinal cord injury.
Propofol for Sedation and Control of Intracranial Pressure in Children
Authors: Spitzfaden Andrew C. , Jimenez David F. , Tobias Joseph D.
Published: April 2003

Following central nervous system insults, control of intracranial pressure may lessen the incidence of morbidity and mortality. Therapies to control intracranial pressure include osmolar agents, prevention of and control of seizures, drainage of cerebrospinal fluid, hypothermia, and barbiturates. Control of agitation and excessive patient movement are additional components in the management of ICP. Although opioids and benzodiazepines are generally effective, in a small subset of patients, alternative agents may be necessary. The authors present 2 children with increased ICP in whom propofol was used to provide sedation and control ICP. The use of propofol in this setting and its possible applications in the children with increased ICP are discussed.
Thalamic Glioblastoma with Cerebrospinal Fluid Dissemination in the Peritoneal Cavity
Authors: Kumar Raj , Jain Rajan , Tandon Vini
Published: April 2003

Glioblastoma multiforme is one of the commonest primary malignant tumours of the brain with rare incidence of extracranial metastases. Systemic dissemination via the CSF or CSF diversionary shunt procedures is also rare. The reported 9-year-old child was a case of thalamic glioblastoma with hydrocephalus who underwent biventriculoperitoneal shunting before tumour decompression and radiotherapy. The child developed incapacitating ascitis 8 months following surgical decompression and 9 months after the shunt diversion which was found to be caused by CSF dissemination of the glioblastoma via the ventriculoperitoneal shunt. The child ultimately succumbed to his disease.
Delayed Repair of Open Depressed Skull Fracture
Authors: Curry Daniel J. , Frim David M.
Published: April 2003

Introduction: Elevation and repair of an open depressed skull fracture is often thought of as an emergency procedure. Common indications for emergent elevation of a depressed skull fracture have been dural tear, seizure, gross contamination or mass effect from bone or a sizable underlying intracerebral hematoma. As treatment of head injury moves towards management of cerebral perfusion pressure (CPP) rather than intracranial pressure (ICP), we sought a way to maximize CPP in the initial treatment of head-injured patients with depressed skull fractures that would eventually require surgery by delaying surgery, when possible, until after the initial period of elevated ICP. Methods: Over a 12-month period, 7 patients (all male, ages 1–15 years) were admitted to our institution with the diagnosis of open depressed skull fracture without significant mass effect requiring urgent decompression. All had significant head trauma with altered mental status and a Glasgow Coma Score of 3–12. Patients were treated with antibiotic prophylaxis (nafcillin, ceftriaxone, metronidazole), seizure prophylaxis (phenytoin) and underwent CPP management in an intensive care unit setting as indicated by intracranial pressure monitoring or clinical assessment. Length of medical management of CPP ranged from 4 to 12 days. Upon stabilization of CPP, patients were operated for repair of their dural, bone and scalp injuries. Results: All 7 patients treated in the above manner suffered no ill effects from their delayed surgery: there was no meningitis, no late seizures, and no cerebrospinal fluid leak. Complications attributable to delay were not present at follow-up ranging from 12 to 24 months. Conclusions: We have delayed surgery for repair of open depressed skull fractures in order to maximize medical management of CPP in the setting of acute trauma. Among other considerations, the risk of intraoperative hypotension occurring at a time of acutely raised ICP was avoided by this delay. We conclude that there is a role, in this specifically defined subset of head trauma patients, for delayed surgical repair of open depressed skull fractures.
Current Patterns of Inflicted Head Injury in Children
Authors: Dashti Shervin R. , Decker Debra D. , Razzaq Ashfaq , Cohen Alan R.
Published: April 2003

The purpose of this study was to examine the current patterns of head trauma associated with child abuse. We reviewed the records of all patients admitted to our medical center between 1995 and 1997 with a primary diagnosis of head trauma, and analyzed the clinical presentation, mechanism of injury, socioeconomic status and outcome for these patients. Head trauma was deliberately inflicted in 38/405 children (9%). There were 25 boys and 13 girls, with a median age of 5.5 months. Two thirds of the families lived in the inner city. Of the 99 children under the age of 2 years admitted for head trauma, the injury was inflicted in 32 (32%). Acute subdural hematoma was present in 22/32 (69%) of children with inflicted trauma, but in only 5/68 (7%) with accidental trauma. Retinal hemorrhages were present in 17/32 (53%) abused children, but in no cases of accidental trauma (0/68). Deliberately inflicted injury is a frequent cause of serious head trauma in young children. Head injury is a major cause of morbidity and mortality in the abused child. Child abuse cases correlated strongly with low socioeconomic status. Nonaccidental trauma must be considered strongly in children under 2 years of age who present with acute subdural hematoma in the absence of a history of a motor vehicle accident.
Genetic Studies in Neural Tube Defects
Authors: Melvin Elizabeth C. , George Timothy M. , Worley Gordon , Franklin Amy , Mackey Joanne , Viles Kristi , Shah Nishu , Drake Courtney R. , Enterline David S. , McLone David , Nye Jeffrey , Oakes W. Jerry , McLaughlin Colleen , Walker Marion L. , Peterson Paula , Brei Timothy , Buran Connie , Aben Joanna , Ohm Bonnie , Bermans Iskandar , Qumsiyeh Mazin , Vance Jeffrey , Pericak-Vance Margaret A. , Speer Marcy C.
Published: April 2003

Neural tube defects (NTD) are one of the most common birth defects and are caused by both environmental and genetic factors. The approach to identifying the genes predisposing to NTD, through linkage analysis and candidate gene analysis, is reviewed along with characteristics of a large, nationally ascertained cohort of families. Results from specific assessments of p53, PAX3 and MTHFR failed to suggest that these genes play a major role in NTD development in these families. Advances in genetic laboratory and statistical techniques have made this a prime opportunity for investigation into the causes of complex disorders, such as NTD. However, traditional approaches may prove to be challenging due to the difficulty of ascertaining samplable multiplex families.
Ectopic Choroid Plexus within a Juvenile Arachnoid Cyst of the Cerebellopontine Angle: Cause of Cyst Formation or Reason of Cyst Growth
Authors: Schuhmann Martin U. , Tatagiba Marcos , Hader Claudia , Brandis Almuth , Samii Madjid
Published: April 2003

The unusual and rare case of a 6-year-old boy is reported who presented with an arachnoid cyst located in the cerebellopontine angle incorporating an ectopic piece of choroid plexus tissue. A microneurosurgical cyst wall resection was performed and the plexus tissue identified and removed. The rare occurrence of ectopic choroid plexus tissue within cysts of the CNS is discussed.
Long-Term Outcome of Terminal Myelocystocele Patients
Authors: Choi SooHo , McComb J. Gordon
Published: April 2003

Introduction: A terminal myelocystocele, a closed form of a neural tube defect (NTD), can present as a large, fully epithelialized, cystic lumbosacral mass containing fat, cerebrospinal fluid (CSF) and neural tissue. The spinal cord terminates at a neural placode wherein the central canal opens into a CSF-filled cavity that is distinct from fluid in the subarachnoid space surrounding the spinal cord. This form of NTD, in our experience, was only associated with major caudal cell mass abnormalities, as these patients often have maldevelopment of the lower spine, pelvis, genitalia, bowel, bladder, kidney and the abdominal wall. This study will describe the clinical manifestations, surgical management and long-term outcome of our terminal myelocystocele patients. Methods: To characterize this rare entity, a 13-year retrospective review was undertaken at our institution. Results: Nine patients with terminal myelocystoceles were identified. In all cases, there were multiple congenital defects including cloacal exstrophy, imperforate anus, omphalocele, pelvic deformity, equinovarus or renal abnormality. Only 1 out of 9 patients has required a shunt for hydrocephalus. The main goal of neurosurgical intervention was to reduce the size of the mass, which can slowly enlarge over time. The spinal cord was also untethered, although these patients have no chance of bowel or bladder control. With a mean follow-up of 63 months, all patients remained neurologically stable. Impairment of lower extremity function is usually severe. However, some patients were ambulatory with the aid of a walker or orthotic device. All patients required a prolonged hospital stay as well as multiple operations prior to initial discharge. Conclusion: Experienced, multispecialty care is needed to optimize the long-term outcome of these complex patients.
Nonselective Partial Dorsal Rhizotomy: A Clinical Experience with 1-Year Follow-Up
Authors: Sacco David J. , Tylkowski Chester M. , Warf Benjamin C.
Published: April 2003

The ability to perform a ‘selective’ dorsal rhizotomy has been challenged. EMG responses are inconsistent and often do not represent reflex responses. We perform nonselective partial dorsal rhizotomy (NSPDR) when reflex response is not evident. Ten children undergoing primarily NSPDR were evaluated preoperatively and postoperatively with the Modified Ashworth Scale, gait lab analyses gross motor function measure and the NSPDR was performed by nonselectively sectioning 50–75% of the dorsal roots not demonstrating a reflex response. Standard selective rhizotomy was performed in the remainder. Only 17 of 106 (16%) dorsal roots demonstrated reflex responses. The results reported in this study demonstrate a benefit to patients undergoing primarily NSPDR which is similar to that reported for patients in whom a selective procedure was intended.
Arachnoid Cyst Rupture with Concurrent Subdural Hygroma
Authors: Donaldson Jill W. , Edwards-Brown Mary , Luerssen Thomas G.
Published: April 2003

Arachnoid cysts (ACs) are relatively common intracranial mass lesions, which occur most often in the middle cranial fossa. While these lesions can present as a mass lesion, many are asymptomatic. Rarely, posttraumatic or spontaneous rupture of ACs can result in intracystic hemorrhage, subdural hematoma or subdural hygroma. We have encountered two cases of ruptured arachnoid cysts that resulted in subdural hygromas. Both patients harbored middle cranial fossa cysts and suffered mild closed head injuries. The presentation, radiographic findings and surgical management of these patients as well as the association between ACs and subdural hygromas are described.
Spinal Atypical Teratoid/Rhabdoid Tumor in an Infant
Authors: Tamiya Takashi , Nakashima Hiroyuki , Ono Yasuhiro , Kawada Sanami , Hamazaki Shuji , Furuta Tomohisa , Matsumoto Kengo , Ohmoto Takashi
Published: April 2003

Atypical teratoid/rhabdoid tumor of the central nervous system in infancy and childhood was established as an entity based on histological, immunohistochemical, and cytogenetic studies. We report the case of a 7-month-old girl who presented with progressive paraplegia and hypesthesia of her legs. Imaging studies revealed a spinal cord mass occupying the entire spinal canal below the T7 level. Through a T12-L3 laminectomy, the intramedullary tumor was partially debulked. Histologically, the tumor specimen had rhabdoid cells, and immunostaining showed vimentin and cytokeratin positivity. No abnormality of chromosome 22q was detected with the fluorescence in situ hybridization method.
Initial Management of Children with Hypothalamic and Thalamic Tumors and the Modifying Role of Neurofibromatosis-1
Authors: Allen Jeffrey C.
Published: April 2003

Diencephalic gliomas may be grouped into 2 clinical categories. Optic pathway/hypothalamus gliomas (OPG) arise primarily from a slower-growing juvenile pilocytic astrocytoma, and thalamic gliomas arise primarily from a fibrillary astrocytoma which can become clinically and histologically more aggressive. Children with OPG have an excellent long-term prognosis with a 10-year survival of over 85%. The major therapeutic challenge for these patients is to maximize their quality of life by preserving visual and endocrine function while minimizing treatment-related morbidity. Treatment is often initiated at diagnosis in infants and toddlers who have a major visual impairment or the diencephalic syndrome. The judicious application of chemotherapy may serve to forestall the need for radiotherapy or surgery. Children with neurofibromatosis-1 (NF-1) usually have a more indolent course. Tumors may grow more slowly or occasionally regress spontaneously. However, over 90% of children with OPG without NF-1 will require some form of therapy. Patients with thalamic gliomas present with a shorter history, often with hydrocephalus. Surgical intervention is often required to relieve intracranial pressure and establish the histologic identity of the tumor. Over 75% of these tumors will become locally aggressive. Current multimodality therapy is relatively ineffective. The bithalamic variant behaves similarly to a pontine glioma.
Evaluation of Shunt Malfunction Using Shunt Site Reservoir
Authors: Sood S. , Canady A.I. , Ham Steven D.
Published: April 2003

Objective: To determine the usefulness of a separate reservoir placed at the site of the shunt in evaluation of shunt malfunction. Methods and Materials: A ventricular catheter was placed alongside the proximal catheter of the shunt and connected to a subgaleal reservoir in 17 patients, in 9 a double-lumen catheter with integrated reservoir and in 13 patients a dual catheter with a double-port reservoir was used. At presentation of suspected shunt malfunction, a standard shunt function evaluation using shunt tap, CT scan or shunt injection was performed, and subsequently, the pressure from the tap of the reservoir was obtained. Results: Thirty-three patients presented with symptoms of malfunction at an interval of 2.3 ± 3 months (range 2–429 days). The pretest probability of shunt malfunction in this population was 73%. Posttest probability of shunt malfunction was 82.5% with standard evaluation and improved to 100% by the separate reservoir tap pressure measurement. In 4 patients in whom the shunt tap was dry, shunt infection was diagnosed prior to revision using CSF obtained at the reservoir tap. In 5 patients with proximal malfunction and bradycardia, the reservoir tap allowed early ventricular decompression. Conclusion: This study shows that a reservoir placed at the site of the shunt remains patient even when the shunt malfunctions, suggesting that flow rather than catheter position is important in proximal malfunction. It is superior to shunt tap for detection of shunt malfunction and infection, and it allows early ventricular decompression in a sick patient awaiting surgery for shunt revision.
Multiple Genomic Alterations Including N-myc Amplification in a Primary Large Cell Medulloblastoma
Authors: Reardon David A. , Jenkins Jesse J. , Sublett Jack E. , Burger Peter C. , Kun Larry E.
Published: April 2003

The large cell (LC) subtype is a recently described histologic variant of medulloblastoma (Mb) associated with a rapid and aggressive clinical course. We describe the genomic profile of a LC-Mb tumor obtained from a patient who developed recurrent and fulminant disease despite ‘good-risk’ features at diagnosis and state- of-the-art multidisciplinary therapy. The tumor sample was analyzed using comparative genomic hybridization (CGH) and complementary molecular approaches. CGH revealed amplicons at chromosome bands 2p24–25, 2q12–22, and 17p11; losses of chromosomes 11q and 18; and low-level gains of 3q, 11p, 13q and 14q. Southern blot analysis confirmed N-myc amplification. No evidence of p53 mutation was detected. The genomic profile of this LC-Mb tumor sample revealed a distinctive pattern of genetic alterations including amplification of N-myc and anonymous oncogenes at chromosome bands 2q12–22 and 17p11. These genomic abnormalities are uncommon in other subtypes of Mb.
Pathology of Diencephalic Astrocytomas
Authors: Burger Peter C. , Cohen Kenneth J. , Rosenblum Marc K. , Tihan Tarik
Published: April 2003

Pediatric gliomas span a spectrum of neoplasms ranging from the well-circumscribed, slowly growing lesions that can be totally removed by surgery to highly infiltrating, nonexcisable, rapidly growing tumors that resist any form of postoperative therapy. As part of this symposium, we discuss selected members of this group in the diencephalon, where lesions of both extremes are common in children. The report illustrates and contrasts the clinical, radiographic and pathological features of the two classic regional astrocytomas, pilocytic and fibrillary, as well as two less common but distinctive lesions, pilomyxoid astrocytoma and bithalamic astrocytoma.
Axillary Nerve Injuries in Children
Authors: Tyagi A. , Drake J. , Midha R. , Kestle J.
Published: April 2003

Isolated axillary nerve injury is uncommon, particularly in children. The motor deficit of shoulder abduction may not recover spontaneously and can be a substantial handicap. Detection may be difficult initially, as the injury is masked by trauma such as head injury, and concomitant shoulder injury requiring immobilization. After mobilization, patients learn to partially compensate by using alternate muscles. There are few reports of surgical management of this nerve injury. Most concern predominantly adults, and the results are mixed with on average slightly greater than half having a good recovery (defined as grade 4–5 Medical Research Council muscle power). We present our experience with 4 pediatric patients who had axillary nerve injury. Three patients had an interposition nerve graft, and 1 patient underwent neurolysis. All patients recovered to grade 4–5 deltoid muscle power. Children with an axillary nerve injury which fails to recover spontaneously by 4–6 months should strongly be considered for surgical exploration.
Neonatal Cervical Osteomyelitis with Paraspinal Abscess and Erb’s Palsy
Authors: Sharma Rewati Raman , Sethu A.U. , Mahapatra A.K. , Pawar Sanjay J. , Nath Ashok
Published: April 2003

An unusual case of pyogenic cervical osteomyelitis is reported in a newborn who immediately after birth had no movements in the left shoulder. There was a fullness in the left cervical region. Left Erb’s palsy due to an unrecognized birth trauma was diagnosed in a peripheral hospital. Later, the child developed fever and a significant swelling in the left cervical region. On transfer to our institution, the X-rays of the cervical spine, ultrasound and computed tomography (CT) established the diagnosis of C6 cervical osteomyelitis and paraspinal abscess which extended to the posterior triangle of the neck. The abscess was drained, and the lamina and lateral mass of the C6 vertebra were debrided. Staphylococcus aureus was grown from the pus. The patient was put on long-term antibiotics to which he responded very well, and he became asymptomatic. In the immediate post-operative period, some movements at the left shoulder were noted, and at 6-month follow-up in the out-patient clinic, the child was virtually normal with near-complete regeneration of the C6 lamina and lateral mass.
A Modified Burr-Hole Method ‘Galeoduroencephalosynangiosis’ in a Young Child with Moyamoya Disease
Authors: Kawamoto Hitoshi , Kiya Katsuzo , Mizoue Tatsuya , Ohbayashi Naohiko
Published: April 2003

We performed combined indirect surgical procedures using encephaloduroarteriomyosynangiosis and a burr-hole method according to descriptions by Suzuki et al. and Kawaguchi et al. with some modifications in a 4-year-old child with moyamoya disease. Transient ischemic attacks on both sides completely disappeared within 1 month and never occurred during 2.5 years follow-up period. This surgical procedure may have enough value as the first operation to prevent ischemic damage in the territories of both the anterior cerebral artery and middle cerebral artery in children with moyamoya disease.
Spinal Lipomas in Children
Authors: Xenos Chris , Sgouros Spiros , Walsh Richard , Hockley Anthony
Published: April 2003

Spinal cord lipomas are a common cause of cord tethering that can lead to progressive neurological defects. The role of prophylactic surgery for spinal lipomas has recently been questioned. Between 1985 and 1999, 59 children underwent a total of 69 surgical procedures at the Birmingham Children’s Hospital in Birmingham, UK. The spinal lipomas were classified into: 18 terminal, 17 transitional, 6 dorsal and 18 filum lipomas – including 12 who had a typical thickened filum terminale. At the first operation, 19 patients (32%) were asymptomatic, and 40 patients (68%) presented with symptoms. Surgical indications in the asymptomatic group included the presence of a dermal sinus tract or syrinx. Prophylactic surgery was undertaken in selected cases. The mean total follow-up for the group since the first surgical procedure was 61.8 months (range: 7.0–203.0 months). In the asymptomatic group, 26% of the patients had late neurological deterioration. Of the 14 patients with asymptomatic conus lipomas, 3 (21%) developed sphincter dysfunction and motor problems at long-term follow-up. In the symptomatic group, 68% improved, 20% remained unchanged, and 12% had late neurological deterioration. None of the 18 patients with symptomatic filum lipoma deteriorated postoperatively. However, 39% had bladder dysfunction, 54% had neuro-orthopaedic deformity, and only 15% returned to overall normal function at latest follow-up. Of the 27 patients with symptomatic conus lipomas, 67% improved, 15% remained stable, and 18% had late neurological deterioration. However, 74% had bladder dysfunction, 67% had neuro-orthopaedic deformity, and 45% had motor problems at long-term follow-up. Spinal lipomas can cause progressive neurological deficits irrespective of spinal untethering surgery. This study demonstrates that filum and conus lipomas have similar clinical presentation, but differ in their outcome following surgery. Filum lipomas are ‘benign’, for which surgery is safe and effective. Conus lipomas are more difficult to manage. When asymptomatic, our results suggest that prophylactic surgery does provide some protection from future neurological deterioration. When symptomatic, conus lipoma surgery is effective in stopping further deterioration. Improvement in neurological function can occur, but few patients return to normal overall function, and pre-existing sphincter dysfunction is not significantly altered by surgery.
What Causes Childhood Brain Tumors? Limited Knowledge, Many Clues
Authors: Bunin Greta
Published: April 2003

Little is known about the causes of brain tumors in children. Children with one of several genetic disorders including tuberous sclerosis and Li-Fraumeni syndrome are at increased risk, as are children who have received therapeutic irradiation to their head. The multifactorial causation of brain tumors, the inaccuracies of recall of past exposures, and the study of all pediatric brain tumors as a single etiologic entity may be contributing to the difficulty in identifying additional risk factors. The evidence that frequent cured meat consumption by the mother during pregnancy increases the risk is suggestive but not conclusive. For other potential risk factors, the evidence is limited and/or conflicting. These exposures and characteristics include pesticides, carcinogen metabolizing genes, and polyomaviruses.
The Prognosis of Children with Hydrocephalus and Congenital Heart Disease
Authors: Crawford Todd S. , Olivero William C. , Hanigan William C.
Published: April 2003

The association of congenital hydrocephalus and heart disease in children is infrequent, but may present considerable dilemmas in management. This report describes the treatment and prognosis of 11 children with both clinical problems. There were 5 males and 6 females. Hydrocephalus occurred following aqueductal stenosis in 5 children and the Dandy-Walker malformation in 3. Three children were diagnosed with idiopathic hydrocephalus. Ten children underwent cerebrospinal fluid diversion procedures for control of hydrocephalus. Five children received pharmacological therapy for cardiac disease; 4 children required surgical correction. Two children died from medical conditions; 2 families declined treatment. Follow-up from 2 to 7 years in the remaining 7 children demonstrated moderate or severe neurodevelopmental disability in 5. One child at 2 years of age showed borderline developmental disability while 1 child is developing normally at 10 years of age. Overall the occurrence of symptomatic hydrocephalus and heart disease in the perinatal period resulted in mortality or neurodevelopmental disability in 9/11 children.
Interhemispheric Neuroepithelial Cyst Associated with Agenesis of the Corpus callosum
Authors: Uematsu Yuji , Kubo Kenji , Nishibayashi Toshiki , Ozaki Fuminori , Nakai Kunio , Itakura Toru
Published: April 2003

This report describes a neonatal case in whom a large interhemispheric cyst associated with agenesis of the corpus callosum was revealed by fetal ultrasonography and demonstrated by MRI to be multilobulated. Endoscopic fenestration of cysts was initially designed in view of the development of the patient’s brain and surgical invasiveness. One year later, when motor paresis of the left arm and progressive enlargement of the cyst on MRI were revealed, open surgery was performed. The histological diagnosis was a neuroepithelial cyst with the feature of choroid plexus epithelia. The clinicopathological features of interhemispheric epithelial cysts associated with agenesis of the corpus callosum are reviewed in the light of differential diagnosis and therapeutic considerations.
Critical Shunt-Induced Subdural Hematoma Treated with Combined Pressure-Programmable Valve Implantation and Endoscopic Third Ventriculostomy
Authors: Fukuhara Toru , Vorster Sarel J. , Luciano Mark G.
Published: April 2003

The authors present 2 patients with VP shunt-induced subdural hematomas (SDH) treated with pressure-programmable valve implantation and endoscopic third ventriculostomies (TV). The first patient is an 11-year-old girl who developed a shunt-induced SDH. Revision of the shunt valve with a higher-pressure valve resulted in a prolonged deterioration of her consciousness. External ventricular drainage at low pressure led to clinical improvement. A pressure-programmable valve set at 50 mm H2O was implanted, and the pressure gradually increased. At a pressure of 120 mm H2O symptoms recurred, even though the subdural collection was beginning to decrease in size. An endoscopic TV was performed, and the valve pressure was then increased to 200 mm H2O without any neurological symptoms. The second patient, a 7-year-old boy with shunt-induced SDH, had recurrent SDH, even after shunt revision with placement of a higher-pressure valve, which resulted in prolonged lethargy. A pressure-programmable valve was implanted with concurrent endoscopic TV. Gradual valve pressure increases up to 200 mm H2O could be performed without recurrent symptoms. Eventually, the shunt system was ligated to resolve residual positional headache, and the TV has been patent for more than 3 years. In both patients, the pressure-programmable valve was useful, since the optimal CSF drainage pressure changed during the period of recovery from symptomatic subdural collections. Concurrent TV appeared to enable increasing the valve pressure gradually without any neurological symptoms. The advantages of this combined approach are discussed.
Monobloc and Midface Distraction Osteogenesis in Pediatric Patients with Severe Syndromal Craniosynostosis
Authors: Meling Torstein R. , Tveten Stein , Due-Tønnessen Bernt J. , Skjelbred Per , Helseth Eirik
Published: April 2003

We present 2 children with severe syndromal craniosynostosis who were in need of urgent midface advancement surgery due to recurrent ocular dislocations (Pfeiffer’s syndrome type II) or severe upper respiratory obstruction (Crouzon’s syndrome). They were operated using distraction osteogenesis, with gradual midface or monobloc advancements. In the Pfeiffer patient, a maxillary distraction of 25 mm achieved effective cessation of ocular dislocations, whereas a 23-mm monobloc advancement in the Crouzon patient achieved cessation of nocturnal arterial desaturations. No major postoperative complications were recorded. Distraction osteogenesis has become a versatile and safe technique that allows for large skeletal advancements.
Late Effects of Therapy of Thalamic and Hypothalamic Tumors in Childhood: Vascular, Neurobehavioral and Neoplastic
Authors: Siffert Joao , Allen Jeffrey C.
Published: April 2003

The late effects in children with hypothalamic and thalamic tumors relate to the effects of the tumor on the surrounding brain, the effects of surgery, radiotherapy (RT) and, to a lesser extent, chemotherapy. The clinical manifestations of late effects include endocrinologic dysfunction, neurocognitive sequelae, behavioral problems and second neoplasia. The prevention of late effects is an integral part of current treatment strategies. Early diagnosis, a rational use of surgery, and deferral of RT are the mainstays of the modern treatment in these patients. The improvement of RT techniques and the use of radioprotective compounds may further help spare normal brain tissue. A better understanding of chemotherapy use and the development of newer agents may increase efficacy, reduce side effects and allow deferral of RT in a greater percentage of patients. Finally, an aggressive management of endocrinological problems, physical and cognitive rehabilitation as well as psychological and school support help provide these children with maximal function within their potential.
Cervical Myelocystoceles and Meningoceles: Long-Term Follow-Up
Authors: Sun John C.L. , Steinbok Paul , Cochrane D. Douglas
Published: April 2003

Little is known about the long-term prognosis of patients with cervical myelocystoceles and meningoceles. In this study, we report the long-term follow-up (average 10 years and 8 months) of 8 patients with cervical myelocystoceles and meningoceles. Neurologic, orthopedic, urologic and psychosocial status was assessed on long-term follow-up. The results indicate that in patients with cervical myelocystoceles, neurologic deficits become noticeable in the first year of life as the infant matures. Motor deficit was common in these patients, whereas sensory and urologic deficits did not occur. We believe that this is related to underlying myelodysplasia. In addition, patients with cervical myelocystoceles tend to have significant orthopedic problems. In contrast, patients with cervical meningoceles do not have neurologic deficits, but do develop mild orthopedic problems.
Synergistic Action of Genistein and Cisplatin on Growth Inhibition and Cytotoxicity of Human Medulloblastoma Cells
Authors: Khoshyomn Sami , Manske Gregory C. , Lew Sean M. , Wald Steven L. , Penar Paul L.
Published: April 2003

Objective: Recent experimental data have shown that dietary soy isoflavones such as genistein can significantly suppress invasiveness and growth of a number of human malignancies. In this study we examined whether genistein, at a concentration typical of plasma levels following soy formula intake, in combination with cisplatin or vincristine exhibited an additive or synergistic inhibitory effect on the growth of medulloblastoma cells. Methods: Three human medulloblastomas cell lines (HTB-186, CRL-8805 and MED-1) were treated with genistein at 6 μM, the maximum reported dietary plasma level in children, combined with cisplatin (0–10 μM) or vincristine (0–1 μM). Monolayer cell growth and cytotoxicity, as measured by colonigenic survival in soft agarose, were then compared in control and drug-treated cultures. Presence of apoptosis, using the DNA ladder assay and laser scanning cytometry, was investigated in all cell lines at those concentrations at which an enhancement of antiproliferative effect of cisplatin and vincristine in presence of genistein was observed. Results: Genistein at 6 μM led to a 2.8-fold increase in the monolayer growth inhibitory effect of cisplatin (0.05 μM) in HTB-186 cells (p = 4.5 × 10–4 by one-tailed t test). Genistein increased colonigenic survival inhibition of HTB-186 2.6-fold at the same cisplatin concentration (p = 1.5 × 10–4). Genistein caused a 1.3-fold increase in antiproliferative effect of cisplatin (0.5 μM) in CRL-8805 cells (p = 3.1 × 10–4). Similarly the inhibition of colonigenic survival was enhanced 2.0-fold in CRL-8805 (p = 1.22 × 10–5). The addition of genistein to 0.5 μM cisplatin led to a 1.7-fold increase in monolayer growth inhibition and 2.4-fold increase in colonigenic survival inhibition of MED-1 cells (p = 8.3 × 10–4 and p = 1.1 × 10–4 respectively). These effects were primarily synergistic but also additive in nature. The combination of genistein and vincristine, as compared to vincristine alone, caused a minimal-to-modest increase in antiproliferative effect on medulloblastoma cells studied here. We were unable to detect apoptosis by two methodologies in any of the medulloblastoma lines when genistein was combined with cisplatin or vincristine. Conclusion: These results indicate that genistein at typical dietary plasma levels can significantly enhance the antiproliferative and cytotoxic action of cisplatin and, to a lesser extent, vincristine. The implication for treatment of medulloblastomas of early childhood may be a reduction in the chemotherapeutic dose recommendations of these agents and subsequently a decrease in the risk of treatment sequelae for these patients.
Endoscopic Biopsy for Tumors of the Third Ventricle
Authors: Souweidane Mark M. , Sandberg David I. , Bilsky Mark H. , Gutin Philip H.
Published: April 2003

Twelve patients underwent endoscopic biopsy of a tumor involving the third ventricle. Nine patients had no significant medical history while 3 had a history of cancer. Unique characteristics of each case dictated the optimal surgical technique. Endoscopic tumor biopsy was combined with additional procedures in 9 cases; shunt insertion (3), shunt insertion with endoscopic septostomy (5), and transcallosal craniotomy (1). Diagnosis was established in 11 patients (92%); 6 primary brain tumors, 3 metastatic central nervous system tumors, 1 metastatic systemic cancer, and 1 region of post-treatment gliosis. One case was aborted due to poor visualization. Therapy was directly influenced by endoscopic biopsy in 11/12 cases (92%) and craniotomy for tumor resection was avoided in 10/12 patients (83%). Of the 5 patients who underwent endoscopic septostomy, 4 required no subsequent procedures for hydrocephalus. There were no complications, and hospital stay averaged 1.78 days for patients who underwent successful endoscopic biopsy. Tumors of the third ventricle are amenable to endoscopic biopsy with excellent diagnostic yield and low morbidity. The procedure must be tailored depending upon the tumor location within the third ventricle, the degree of ventriculomegaly, and the need to perform a septostomy. Singularly or combined with other endoscopic procedures, patients can be spared multiple and more invasive surgical procedures.
Intracranial Ependymomas of Childhood: Current Management Strategies
Authors: Smyth Matthew D. , Horn Biljana N. , Russo Carolyn , Berger Mitchel S.
Published: April 2003

Children with intracranial ependymomas have relatively poor outcomes despite the low-grade histology of these tumors and recent advances in diagnosis, microneurosurgical resection, and adjuvant therapy. Aggressive surgical resection and postoperative adjuvant therapy result in only a 5-year survival rate of 50%. In this paper, we provide a review of the current technical aspects of surgical resection, and focus on recent studies of the epidemiology, molecular markers, prognostic factors and adjuvant therapy for childhood intracranial ependymomas and discuss their implications for current and future management strategies.
Optic Pathway Hypothalamic Gliomas in Children under Three Years of Age: The Role of Chemotherapy
Authors: Silva Michele Madeira , Goldman Stewart , Keating Gesina , Marymont Mary Anne , Kalapurakal John , Tomita Tadanori
Published: April 2003

Objectives: Optic pathway/hypothalamic gliomas (OPHGs) tend to occur in young children. Treatment options consist of surgical resection, radiation therapy (RT) and chemotherapy. Due to complications induced by surgery and RT, chemotherapy has gained significant recognition for the treatment of OPHG in young children. Chemosensitivity of OPHG in very young children under 3 years of age has not been well documented. We analyzed 14 patients who were treated with chemotherapy with or without surgery. Materials and Methods: Fourteen children younger than 3 years (median age of 10 months) with OPHG were treated between 1988 and 1998. Magnetic resonance imaging was obtained in all cases. Hydrocephalus was present in 8 patients and diencephalic syndrome was noted in 6. Only 3 of these had evidence of neurofibromatosis-1. Five patients had partial tumor resection and 4 had endoscopic biopsy at the time of ventriculoperitoneal shunt placement. Pathological examination revealed low-grade astrocytoma in 5 and juvenile pilocytic astrocytoma in 4. All patients received chemotherapy: carboplatin in 8, a combination of carboplatin and vincristine in 4 and a combination of other agents in 2. Results: Eight (57%) of 14 patients had a sustained reduction of tumor during the follow-up time between 15 months and 8 years. The 5-year progression-free survival was 63%. These tumor reductions were often accompanied by clinical improvements. Diencephalic syndrome responded to chemotherapy alone in 4 of 6 patients. However, 5 others had progressive disease; 3 during the treatment and 2 following the treatment (9 months and 2 years, respectively). All these 5 patients had a partial tumor resection prior to chemotherapy. Conclusion: A majority of OPHGs responds to chemotherapy. Due to slow progression of these tumors and adverse effects of other therapeutic modalities, we recommend chemotherapy as a primary treatment for OPHGs. Our present data indicates that partial surgical resection does not enhance chemotherapy effectiveness for OPHGs in infants or children younger than 3 years.
Personality Patterns and Outcome in Depressive and Bipolar Disorders
Authors: Heerlein Andrés , Richter Paul , Gonzalez Matías , Santander Jaime
Published: April 2003

Personality traits and disorders have a strong influence on the course and outcome of depressive and bipolar disorders. Studies of the influence of personality disorders (PD) and some PD clusters on outcome of mood disorders are controversial and suggest that more specific assessment of underlying traits or dimensions is needed. Utilizing the Munich Personality test (MP-T) scales of von Zerssen, this study tries to identify specific personality traits that may influence the outcome and clinical course of unipolar endogenous depression and bipolar disorder. Six unipolar depressives and 6 bipolar patients, according to DSM III-R and ICD 10 criteria, were assessed with the MP-T self- and family-reporting scales. Three years later, their outcome scores were correlated with the corresponding premorbid personality profile. Preliminary results show that introversion has a negative effect on outcome of unipolar melancholic depression, while extraversion, esoteric tendencies and rigidity have a positive influence. Neuroticism has a negative influence on outcome of bipolar disorder, but not on unipolar endogenous depression. Data from the literature suggest that neuroticism, hostility and social dysfunction seem to have a negative prognostic value only for nonendogenous depressives and bipolar disorder, thus supporting the notion that the diagnostic distinction between bipolar disorder, endogenous and nonendogenous depression is relevant to prognostic discussions. These observations help to understand the differences between depressive syndromes and their relationship to prognosis, but also to comprehend the role of personality in clinical and theoretical research of mood disorders.
The Influence of Psychopathology, Personality, and Marital Interaction on the Short-Term Course of Major Depression
Authors: Mundt Ch. , Kronmüller K.-T. , Backenstrass M. , Reck C. , Fiedler P.
Published: April 2003

By means of an aggregated variables design the domains of ‘psychopathology’, ‘personality’, and ‘social interaction’ were weighted according to their impact on the 2-year course of 50 patients with unipolar major depression. Preindex course ranked highest, asthenic personality second. Variables of social interaction disappeared in the second-order logistic regression. These findings fit in well with the recent literature. They are interpreted in terms of habituation processes and residual personality changes which lower the vulnerability threshold for relapse and unfavorable course. As a consequence of this conclusion early relapse prevention would be most important.