bluuebunny/crossref_metadata_2025 huggingface dataset:
A Novel Embryogenetic Mechanism for Currarino’s Triad: Inadequate Dorsoventral Separation of the Caudal Eminence from Hindgut Endoderm
Authors: Dias Mark S. , Azizkhan Richard G.
Published: April 2003

Currarino’s triad is a congenital malformation involving the combination of anorectal stenosis, a presacral mass (most often a teratoma or ventral menigocele) and an anterior sacral bony defect (scimitar sacrum). Current theories regarding its embryogenesis are difficult to reconcile with our current understanding of caudal neuraxial and hindgut development. Caudal neuraxial structures develop from the caudal eminence (or tail bud), which normally separates from the hindgut endoderm concurrent with ingrowth of the posterior notochord during late gastrulation. We describe the first reported association of Currarino’s triad with a caudal split cord malformation. It has previously been proposed that split cord malformations and related ‘complex dysraphic malformations’ involving abnormalities of one or more of the three primary germ layers arise through disordered midline axial integration during gastrulation. The presence of a split cord malformation in a patient with Currarino’s triad suggests that the two disorders share a common embryogenetic pathway. We propose that the malformations of Currarino’s triad arise through a failure of dorsoventral separation of the caudal eminence from the hindgut endoderm during late gastrulation.
Treatment and Long-Term Outcome of Pineal Nongerminomatous Germ Cell Tumors
Authors: Knappe Ulrich J. , Bentele Karl , Horstmann Martin , Herrmann Hans-Dietrich
Published: April 2003

In 1994 we reported on a small series of 3 children with malignant, marker-positive pineal nongerminomatous germ cell tumors treated with a ‘sandwich’ protocol. Here, we report on the long-term survival of these children. Preoperative chemotherapy consisted of two courses of bleomycin, etoposide, and cisplatin. En bloc resection of the tumors via the supracerebellar, infratentorial route was performed immediately after decline of tumor marker levels. Postoperatively, two courses of vinblastine, ifosfamide, and cisplatin were applied, followed by craniospinal irradiation. The patients showed no major neurological deficits and no evidence (neuroradiologically or with regard to tumor marker levels) of recurrence of disease after 66, 71, and 78 months, respectively. We propose this regimen for children with tumors of the pineal region in whom the tumor markers are positive. It should be started without histological classification of the tumor to avoid possible spillage of malignant tumor cells to the cerebrospinal fluid.
Cranial Vault Expansion Using Transcutaneously Activated Magnetic Implants
Authors: Rinehart Gregory , Forget Thomas , Zografakis John , Manepalli Anantha N. , Pittman Thomas
Published: April 2003

The technique of distraction osteogenesis has not been widely used in the treatment of problems of the head and face because of the need for external fixators. By using magnetic, rather than mechanical, forces to drive bone movement we hope to expand the applications of the technique to include the treatment of cranial vault deformities. Thirty immature rabbits were studied. Twenty-six of the rabbits underwent operations. Each of the 26 had 2 magnets fixed to its skull: one on the left parietal bone and one on the right parietal bone. Incomplete circumferential osteotomies were then performed around each magnet on 13 of the rabbits. A head frame was attached to each animal. Head frames without magnets were placed on 6 of the rabbits (3 with osteotomy, 3 without osteotomy) while 20 of the animals (10 with osteotomy, 10 without osteotomy) had 2 magnets mounted on the frames which were of opposite polarity to those implanted. The remaining 4 rabbits served as nonoperative controls. Six weeks later all of the animals were sacrificed. There were significant differences in the cranial volumes and contours between the groups of animals. Many of the differences were increased by coincident osteotomy. Associated histologic findings are described.
Growth Factor Receptors as Targets for Therapy in Pediatric Brain Tumors
Authors: Meuillet Emmanuelle J. , Bremer Eric G.
Published: April 2003

Growth factor receptors (GFRs) have been described as overexpressed in several types of brain tumors. Overexpression of these transmembrane proteins is considered to be an important part of tumorigenesis. Genetic as well as epigenetic modulation of the receptors have to be considered when trying to understand the role of GFRs in tumors or as targets for tumor therapy. GFR function can be modulated by membrane components (e.g. gangliosides) or by the change in receptor glycosylation. These types of changes and the occurrence of the expression of mutated receptor expressed in tumor cell can result in altered signaling. In this review, we have focused on GFRs, their expression and mutations in brain tumors. Recently the correlation between GFR expression and patient outcome has suggested that these tyrosine kinases and their signaling might play a decisive role in the course of patients with brain tumors. The importance of GFRs as possible targets for brain tumor therapy is also discussed.
An Unusual Variant of a Growing Skull Fracture in an Adolescent
Authors: Çolak Ahmet , Akbaşak Aytaç , Biliciler Bülent , Fuat Erten Serhat , Koçak Ayhan
Published: April 2003

A great majority of growing skull fractures occur in infancy and earlychildhood. Since the growth of brain is necessary as a driving force for these lesions to occur, almost all reported cases have been before the first 3 years of life. Although a number of uncommon locations, such as basiooccipital and skull base areas, have been reported, they are commonly located on calvaria. The authors report a growing skull fracture on the orbital roof in a 16-year-old female admitted to hospital with complaints of headache and seizures. She had had an orbital trauma 8 years before. CT scan revealed a hypodense lesion in the right frontal lobe and a diastatic fracture line on the right orbital roof. A right craniotomy was performed. Excision of arachnoid loculations and duraplasty were carried out. This is an unusual condition with respect to the location of the lesion, as well as the age of the patient.
Management of Uncomplicated Skull Fractures in Children:Is Hospital Admission Necessary?
Authors: Vogelbaum M.A. , Kaufman B.A. , Park T.S. , Winthrop A.L.
Published: April 2003

Objective: This study was undertaken to determine the necessity for routine hospital admission of children with skull fractures, a normal neurological exam, a normal head CT, and no other injuries (‘uncomplicated skull fracture’). Methods: A prospective study of closed-head injuries in children was done over a 2-year period at St. Louis Children’s Hospital. All patients with closed head injuries underwent skull radiographs and a head CT scan. From this cohort, children with uncomplicated skull fractures were identified and studied. For comparison, a retrospective analysis was also performed of the hospital admission records of children admitted over a 5-year period (1990–1994) with the diagnosis of epidural hematoma (EDH) to identify the typical time intervals between injury and documentation of the lesion in these cases. Results: Forty-four patients with uncomplicated skull fractures were identified; all had been admitted for observation. Mean age was 1.8 years. Average time between injury and hospital admission was 6.35 h with half of this time being spent in the emergency room. Average LOS was 35 h, but 50% of patients were hospitalized less than 24 h. No patient in this study group suffered a complication related to their inury. Twenty-three patients with EDH had been admitted during the 5-year review period. Slightly more than one-half of patients had their EDH detected within 6 h of injury. The others were diagnosed more than 6 h after injury due to a delay in medical evaluation or a delay in obtaining a computed tomographic (CT) scan after an initial medical evaluation. Conclusions: Patients with uncomplicated skull fractures, in the absence of recurrent emesis and/or evidence of child abuse, can be considered for discharge home. The definition of an uncomplicated skull fracture requires that a head CT be performed on these patients.
Midcervical Postinfectious Ligamentous Instability: A Variant of Grisel’s Syndrome
Authors: Lopes Demetrius K. , Li Veetai
Published: April 2003

Objective and Importance: We report our recent experience with two cases of C3–C4 ligamentous instability that developed after the onset of an upper airway infection. To our knowledge, this is the first report in the literature of a variant of Grisel’s syndrome occurring at the midcervical levels. The diagnosis and management of this entity are presented. Clinical Presentation: Two infants with respiratory distress were evaluated at the Children’s Hospital of Buffalo. During evaluation, cervical spine x-rays showed midcervical translational instability. Neither patient had a neurological deficit nor a history of trauma or bone disease. Intervention: One of the patients was immmobilized with an external orthosis. Conclusion: Each patient remained neurologically intact, and the cervical spine returned to normal alignment after several months. We discuss a possible mechanism for this unusual association and recommend external immobilization.
Avoiding Complicated Shunt Systems by Open Fenestration of Symptomatic Fourth Ventricular Cysts Associated with Hydrocephalus
Authors: Villavicencio Alan T. , Wellons III John C. , George Timothy M.
Published: April 2003

Optimal treatment of the hydrocephalic patient with symptomatic Dandy-Walker malformation or trapped fourth ventricle remains controversial. We describe 6 patients with symptomatic Dandy-Walker malformation or trapped fourth ventricle and hydrocephalus that were treated with an aggressive cyst fenestration. Four of the 6 patients had previously undergone five or more failed shunt procedures. There were no complications associated with surgery, and 5 of the 6 patients (83%) have remained asymptomatic with respect to their posterior fossa cysts. One patient has required subsequent fourth ventricular shunt placement. Median follow-up was 26 months (range 12–66 months). We suggest that suboccipital craniectomy and open fenestration is a valid treatment option in hydrocephalic patients with symptomatic Dandy-Walker malformation or trapped fourth ventricle. Although the associated lateral ventriculomegaly will probably still require a ventriculoperitoneal shunt, the absence of a cystoperitoneal shunt system seems to minimize the incidence of complications and reoperation.
Chemical Meningitis from Bile Reflux in a Lumbar-Gallbladder Shunt
Authors: Barami Kaveh , Sood Sandeep , Ham Steven , Canady Alexa
Published: April 2003

Objective and Importance: The gallbladder is used to divert cerebrospinal fluid (CSF) in patients with hydrocephalus when all other sites have been exhausted. This is seen in hydrocephalic patients who have reached teenage years but have undergone multiple shunt revisions, abdominal operations and repeated neck vein cannulations during childhood. One complication of the lumbar-gallbladder shunt is discussed as well as its pathophysiologic theory and management. Clinical Presentation: A case of a patient with a lumbar-gallbladder shunt who developed chemical meningitis from reflux of bile into the CSF space is presented. The patient presented with generalized seizures. Intervention: Included: ventilatory support, externalization of the shunt, correction of the metabolic abnormalities and administration of anticonvulsants and steroids. Conclusion: This case illustrates an unusual occurrence of reflux of bile into the CNS through a lumbar-gallbladder shunt in a patient with long-term complex communicating hydrocephalus. It also demonstrates its mode of presentation and successful management. To our knowledge, this is the first case report of its kind.
Intraoperative Angiography in the Management of Pediatric Vascular Disorders
Authors: Ghosh Sanjay , Levy MichaelL. , Stanley Phillip , Nelson Marvin , Giannotta SteveL. , McComb J.Gordon
Published: April 2003

We reviewed our experience with intraoperative angiography in the management of 7 cerebral aneurysms and 15 arteriovenous malformations (AVMs) in children over the past 5 years. The patients ranged from 4 months to 18 years of age with a mean age of 9 years. In 4 of the 22 cases, angiography provided information that led to changes during surgery. In 3 cases, the intraoperative angiogram revealed residual AVMs that were then completely resected. In 1 patient with an anterior circulation aneurysm, intraoperative studies revealed findings which resulted in clip repositioning. Intaoperative angiography added a mean of 43 (+/-19) min to the operative time, with a mode of 40 minutes. There was 1 complication, a groin hematoma that readily resolved. Intraoperative angiography prolongs to operative course; however, it is a useful adjunct to the management of vascular disorders in children. We now routinely use and recommend intraoperative angiography to assist with the surgical management of vascular malformations in children.
Predictors of Posttraumatic Convulsions in Head-Injured Children
Authors: Ratan Simmi K. , Kulshreshtha Rajiv , Pandey Ravindra M.
Published: April 2003

The purpose of this study was to determine predictors of posttraumatic convulsions in children. Study subjects included children under 12 years of age, who were admitted to the Safdarjang Hospital, New Delhi, during the year 1997 (January to December) after suffering head trauma. The occurrence of first convulsion after head injury was taken as the outcome variable in the study. Medical records were reviewed for data about clinical, radiological and epidemiological features of such children. The study revealed that children younger than 2 years of age (odds ratio, OR 2.96; 95% confidence interval, CI 1.42–6.21), those suffering severe head injuries, i.e. with low Glasgow Coma Score (OR 3.07; 95% CI 1.40–6.77), and those with longer period of unconsciousness after head trauma, especially longer than 12 h (OR 1.71; 95% CI 0.69–4.19) have higher likelihood of suffering convulsions after head injury. However, none of the radiological findings were found to be significantly associated with posttraumatic convulsions.
Intra-Fourth-Ventricle Teratoma
Authors: Srinivasan U.S. , Joseph Thomas
Published: April 2003

The computed tomographic (CT) scanning characteristics of a case of intra-fourth-ventricle mature teratoma in a 15-month-old child are described and correlated with intraoperative findings.
A Simple and Safe Technique for Endoscopic Third Ventriculocisternostomy
Authors: Wellons III John C. , Bagley Carlos A. , George Timothy M.
Published: April 2003

A simple and safe technique for performing an endoscopic third ventriculocisternostomy is described using a small-diameter semirigid neuroendoscope in conjunction with a perforated ventricular catheter to bluntly fenestrate the floor of the third ventricle. All previous descriptions involve the initial use of an introducer sheath that in our experience lends to loss of cerebrospinal fluid, consequently distorting the anatomic landmarks of the third ventricle as well as compromising the crucial concave shape of its floor. Our technique limits the loss of cerebrospinal fluid volume, therefore, reducing the chance of basilar artery complex perforation as a consequence of distortion of third ventricular landmarks and loss of third ventricular floor concavity.
Preclinical Evaluation of Benzoporphyrin Derivative Combined with a Light-Emitting Diode Array for Photodynamic Therapy of Brain Tumors
Authors: Schmidt Meic H. , Reichert II Kenneth W. , Ozker Kutlan , Meyer Glenn A. , Donohoe Debra L. , Bajic Dawn M. , Whelan Noel T. , Whelan Harry T.
Published: April 2003

Objective: The aim of this study was to investigate the second-generation photosensitizer benzoporphyrin derivative (BPD) and a novel light source applicator based on light-emitting diode (LED) technology for photodynamic therapy (PDT) of brain tumors. Methods: We used a canine model to investigate normal brain stem toxicity. Twenty-one canines underwent posterior fossa craniectomies followed by PDT with BPD. These animals were compared to light only and BPD control. In addition, we investigated the ability of BPD and LED to cause inhibition of cell growth in canine glioma and human glioma cell lines, in vitro. The biodistribution of BPD labeled with 111In-BPD in mice with subcutaneous and intracerebral gliomas and canines with brain tumors was studied. Results: The in vivo canine study resulted in a maximal tolerated dose of 0.75 mg/kg of BPD and 100 J/cm2 of LED light for normal brain tissue. The in vitro study demonstrated 50% growth inhibition for canine and human glioma cell lines of 10 and 4 ng/ml, respectively. The mucine study using 111In-BPD showed a tumor to normal tissue ratio of 12:1 for intracerebral tumors and 3.3:1 for subcutaneous tumors. Nuclear scans of canines with brain tumors showed uptake into tumors to be maximal from 3 to 5 h. Conclusion: Our study supports that BPD and LED light sources when used at appropriate drug and light doses limit normal brain tissue toxicity at doses that can cause significant glioma cell toxicity in vitro. In addition, there is higher BPD uptake in brain tumors as compared to normal brain in a mouse glioma model. These findings make BPD a potential new-generation photosensitizer for the treatment of childhood posterior fossa tumors as well as other malignant cerebral pathology.
Tandem Spinal Cord Injurieswithout Radiographic Abnormalities in a Young Child
Authors: Pollina John , Li Veetai
Published: April 2003

Anatomic features unique to the pediatric spine render this population susceptible to spinal cord injuries without radiographic abnormalities (SCIWORA). To date, published descriptions of SCIWORA have been limited to a single region of the spinal column. We describe a case of a 3-year-old boy in whom, after a motor vehicle accident, tandem SCIWORA lesions involving the lower cervical spinal cord and thoracolumbar junction resulted in severe quadraparesis. The child was initially treated with 24 h of methylprednisolone followed by 3 months of external orthoses of both the cervical and thoracic spine. We include in this article a brief review of the literature and treatment guidelines for SCIWORA and postulate the mechanism of these tandem spinal cord injuries.
The Results of a Practice Survey on the Management of Patients with Shunted Hydrocephalus
Authors: Li Veetai , Dias Mark S.
Published: April 2003

The care of patients with shunted hydrocephalus is one of the most common clinical problems facing a pediatric neurosurgeon. Despite the prevalence of this clinical entity, it is our impression that the management strategies for these patients vary significantly amongst pediatric neurosurgeons. In an effort to better understand how varied these practices are, we surveyed 261 neurosurgeons with an expressed interest in pediatric neurosurgery, of whom 149 (57%) responded. The survey contained questions pertaining to preoperative and intraoperative management, choice of shunt valve, treatment philosophies with respect to shunt revision, evaluation process for potential shunt malfunction, treatment strategies for shunt infections and routine follow-up care. The answers to the questions confirmed our expectations that there are a variety of ways to manage these patients.
Subdural Empyema Complicating Cerebrospinal Fluid Shunt Infection
Authors: Dickerman R.D. , Piatt J.H. , Hsu F. , Frank E.H.
Published: April 2003

Subdural empyema has not been reported previously as a complication of cerebrospinal fluid (CSF) shunt surgery. An infant submitted to CSF shunt insertion for congenital hydrocephalus developed subdural empyema after a failed attempt to treat a superficial scalp wound infection with oral antibiotics. Enterobacter cloacae was isolated from the empyema. Temporizing management of the preceding superficial wound infection with oral antibiotics probably was the cause of this exotic pathogen. The treatment of infected scalp wounds contiguous with shunt hardware must be surgical.
Clinical and Therapeutic Properties of Cushing’s Disease in Childhood
Authors: Cirak Bayram , Palaoglu Selcuk , Akalan Nejat
Published: April 2003

Cushing's disease (CD) in adolescence and childhood is a rare pathology and has some different clinical and therapeutic characteristics than in adults. We report 3 cases treated surgically, with the diagnosis of CD. Their common complaints were short stature and obesity. Endocrinological investigations and radiological evaluation pointed out the diagnosis of CD for all children. They were operated on by the transsphenoidal route, and adenoma resection was performed. Histopathologic and immunohistochemical examination of the tissue specimens confirmed the diagnosis of CD. Long-term follow-up demonstrated weight loss and increase in height.
Brain Tumors in Children Under 3 Years of Age
Authors: Sala Francesco , Colarusso Enzo , Mazza Carlo , Talacchi Andrea , Bricolo Albino
Published: April 2003

Brain tumors in children under 3 years of age differ in clinical presentation and pathological behavior from those in older patients. In this study, we reviewed data from 39 children (24 males and 15 females) under 3 years of age who were treated for intracranial brain tumors since the introduction of magnetic resonance imaging. The purpose was to assess correlations between clinicoradiological and treatment-related factors, and their impact on prognosis. The following factors were analyzed: sex, age, duration of symptoms, intracranial hypertension on admission, tumor location, surgical removal and histology. Associations between these factors and correlations with prognosis were determined using bivariate analyses (2 test) and Kaplan-Meier survival curves. Collins' concept of a period of risk recurrence was tested. Mean follow-up was 41 months (range 0-136). In March 1998, 20 children were still alive (51.2%) with a mean survival time of 65 months (range 2-136). The incidence of supratentorial tumors was significantly higher in children less than 1 year old (p = 0.027). Lateral tumors were 9/10 (90%) supratentorial versus only 7/26 (27%) midline tumors (p = 0.001). Outcome (dead or alive) was significantly better (p = 0.037) for low-grade astrocytomas (9/12 = 75% survival) when compared to ependymomas (2/6 = 33%) and primitive neuroectodermal tumors (3/12 = 25%). Total tumor removal was achieved in 20 cases and was associated with a better outcome (65 vs. 33% survival; p = 0.049). Survival analysis confirmed a worse prognosis for children with ependymomas and primitive neuroectodermal tumors (p = 0.011) and revealed a worse survival for children with intracranial hypertension on admission (p = 0.047). Total tumor removal was associated with a longer survival, although not significantly (p = 0.077). Finally, we found no exceptions to Collins' law.
Primary Antiphospholipid Syndrome with Moyamoya-Like Vascular Changes
Authors: Booth Frances , Yanofsky Rochelle , Ross Ian B. , Lawrence Patrick , Oen Kiem
Published: April 2003

We describe a young girl with antiphospholipid syndrome (APS) and moyamoya-like cerebrovascular changes which reversed after anticoagulation. Although there was a risk of hemorrhage from collateral vessels, we speculate that this treatment may have prevented progression of the vascular abnormalities, while resolution of the thrombus resulted in improved cerebrovascular circulation.
Posterior Fossa Craniotomy: An Alternative to Craniectomy
Authors: Kurpad Shekar N. , Cohen Alan R.
Published: April 2003

We describe a simple midline approach to posterior fossa lesions in children that involves removal and replacement of a bone flap. This approach offers several advantages over conventional suboccipital craniectomy. Elevation of the bone flap is facilitated by the relatively small size of the midline bony keel in children. This method is simple, safe and expeditious, and restores normal anatomical planes and improved protection to the contents of the posterior fossa. While this approach has been described previously for individual cases, the authors employ craniotomy as the standard method of access to the posterior fossa in pediatric patients, and suggest a straightforward technique for removing and replacing the bone flap.
Stereotactic Radiation in Primary Brain Tumors in Children and Adolescents
Authors: Benk Véronique , Clark Brenda G. , Souhami Luis , Algan Ozer , Bahary Jean-Paul , Podgorsak Ervin B. , Freeman Carolyn R.
Published: April 2003

To evaluate treatment outcome and morbidity of stereotactic external-beam irradiation (SEBI) in pediatric patients, we reviewed 14 children treated with SEBI, using a 10-MV isocentric linear accelerator at McGill University between 1988 and 1994. The median follow-up was 46 months (range 6–82 months). The median age was 14 years. There were 8 low-grade astrocytomas, 3 neuromas and 4 other histologies. Twelve patients received fractionated treatments. The median collimator diameter was 2.5 cm (range 1–5 cm). The median biological effective dose delivered to the entire tumor volume was 57 Gy for astrocytomas and 43 Gy for the other histologies. The overall actuarial survival rate and disease-free survival rate at 5 years were 83 and 62%, respectively. For the patients with low-grade astrocytomas, the 5-year survival and disease-free survival rates were 100 and 60%, respectively. Four children had recurrence at a median of 37 months. Four patients developed treatment-related complications: 1 had edema alone, 2 had necrosis and 1 had edema associated with necrosis. Neither the physical nor radiobiological parameters were predictive of the treatment outcome or the treatment complications. Stereotactic irradiation is a valid option for progressive nonresectable tumors in children.
Syndrome of Overdrainage Associated with Disconnection of a Ventriculoperitoneal Shunt
Authors: Rosenthal Guy , Pomeranz Shlomo , Spektor Sergei , Yacoub Mufid , Israel Zvi H.
Published: April 2003

We present the case of a child who developed the syndrome of cerebrospinal fluid (CSF) overdrainage with slit-like ventricles on CT in the setting of a disconnected distal shunt valve. Upgrading the shunt alleviated his symptoms. It is suggested that the presence of a patent fibrous tract allowed the overdrainage of CSF.
Malignant Evolution of Choroid Plexus Papilloma
Authors: Chow Edward , Jenkins Jesse J. , Burger Peter C. , Reardon David A. , Langston James W. , Sanford Robert A. , Heideman Richard L. , Kun Larry E. , Merchant Thomas E.
Published: April 2003

Choroid plexus tumors are rare CNS neoplasms. The distinction between choroid plexus papilloma (CPP) and choroid plexus carcinoma (CPC) is made on the basis of clinical and histological criteria. Malignant evolution of CPP may occur, and the presence of mitotic figures in CPP may predict the likelihood of recurrence or malignant evolution. Close surveillance is mandated for these patients. We report on two such cases of CPP that transformed to CPC at the time of recurrence.
Management of Chiari I Malformations with Holocord Syringohydromyelia
Authors: Feldstein Neil A. , Choudhri Tanvir F.
Published: April 2003

The management of patients with Type I Chiari malformations (CM 1) with or without syringohydromyelia (SHM) has remained quite controversial, and many different surgical procedures have been advocated. Over the past several years, the authors have treated 7 children presenting with CM 1 and holocord syringohydromyelia with suboccipital decompression and duraplasty alone without intradural procedures. All children received MRI imaging at 2–4 months and 1 year postoperatively. On the early postoperative MRI examination, marked reduction in the syringohydromyelia was seen in 6 children, with minimal change in syrinx size in 1 child who was clinically improving after the operation. At 1 year, all children with early collapse remained collapsed, and the child with minimal early collapse demonstrated an approximately 50% reduction in syrinx size. Clinical follow-up (mean 30 months, range 21–50 months) showed good results in all patients: none of the children have required further neurosurgical intervention, and all have shown improvement in their preoperative function. One child with a 46° scoliosis had a complete collapse of her SHM, but ultimately required spinal fusion. The presenting clinical findings, operative technique, MRI imaging and clinical outcomes will be discussed. The results from these 7 patients with CM 1 and holocord syringomyelia suggest that posterior fossa decompression alone (without intradural procedures) can provide excellent radiographic and clinical outcome.
Optic Glioma with Characteristic Bilateral Optic Atrophy in a 3-Year-Old Girl
Authors: Nomura Sadahiro , Suzuki Ryo , Sugiyama Shuichi , Nogami Ken-ichiro , Ito Haruhide
Published: April 2003

We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. Neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right fronto-temporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. Intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP.
Late Shunt Infections
Authors: Baird Clinton , O’Connor Dennis , Pittman Thomas
Published: April 2003

We reviewed the records of the 957 shunt-related operations performed at Cardinal Glennon Children’s Hospital over a 10-year period. During that time, 94 shunt infections were recognized. Eight of the infections occurred more than 9 months postoperatively. These differed from early infections in two ways: (1) Staphylococcus aureus was not found to be a pathogen in any late infection. (2) Abdominal pseudocysts were much more frequently found in patients with late-developing infections. In addition, the pathogens involved and the temporal distribution of cases suggest most infections that occur more than 9 months postoperatively are more likely caused by secondary bacterial seeding than by bacterial inoculation at the time of operation.
Primary Ewing’s Sarcoma of the Skull in Children
Authors: Carlotti Jr. Carlos G. , Drake James M. , Hladky Jean-Pierre , Teshima Ikuko , Becker Laurence E. , Rutka James T.
Published: April 2003

Ewing’s sarcoma (ES) of the skull is rare. Herein, we present 2 cases of ES that involved the cranium in young children. In one case, the lesion originated in the petrous temporal bone; in the other, the frontal bone. Both children were acutely compromised neurologically by signs and symptoms of raised intracranial pressure. In both cases, radiographs revealed massive tumors affecting the skull. Neurosurgical resection of the tumor was undertaken in both instances, and the diagnosis of ES was confirmed by immunohistochemistry, cytogenetic analysis (translocation 11;22), spectral karyotyping and RT-PCR (demonstration of a EWS/FLI1 fusion transcript). Following aggressive surgical resection, both children received intensive chemotherapy. No child has received radiation therapy. One child is alive and well 8 years after diagnosis without any evidence of residual disease. The other is currently undergoing chemotherapy for her tumor. The principles involved in the management of children with cranial-based ES are discussed. These 2 cases serve to illustrate the fact that even children with massive ES tumors of the cranium may be salvaged with aggressive combination therapy.
Pediatric Neurosurgery: Pride and Prejudice
Authors: Winston Ken R.
Published: April 2003

Pediatric neurosurgery now exists as a member of the family of neurosurgery with its own training programs, process of accreditation, national and international conferences and scientific journals. The relentless expansion of science relevant to the practice of neurosurgery and the changing patterns of neurosurgical practice have driven and continue to drive the juggernaut of evolutionary process which sometimes necessitates the birth of new specialties of practice. The history and the development of neurosurgery as they relate to children are presented. There is no more reason to think that the established specialty of pediatric neurosurgery or the patients under the care of pediatric neurosurgeons would benefit from the collapsing of pediatric neurosurgery back into the general neurosurgical fold than to think that all of neurosurgery, and hence all patients cared for by neurosurgeons, would benefit from the return of organized neurosurgery to its general surgical parent. Just as mankind benefits from the steady advancement of all aspects of neurosurgery, children benefit from the existence and steady advancement of pediatric neurosurgery.
Pump-Regulated Cerebrospinal Fluid Drainage
Authors: Vender John R. , Houle Paul , Flannery Anne Marie , Fryburg Karsten , Lee Mark R.
Published: April 2003

The drainage of cerebrospinal fluid (CSF) from the lumbar subarachnoid space is an effective technique for the treatment of CSF fistula and control of intracranial pressure in children and adults. The use of the lumbar drain poses unique challenges, however, in the pediatric population. We present a safe and effective method of pump-controlled lumbar subarachnoid drainage. This technique allows accurate titration of CSF removal while providing a closed system which is not sensitive to position changes or patient activity. Four case histories are reviewed.
Early Vasculopathy following Radiation in a Child with Medulloblastoma
Authors: Maher Cormac O. , Raffel Corey
Published: April 2003

A child with severe radiation vasculopathy 15 months following radiation therapy for medulloblastoma is reported. The patient underwent surgical resection of a posterior fossa medulloblastoma, followed by chemotherapy and radiation therapy. He was treated with 55 Gy to the craniospinal axis. Fifteen months later, the patient presented with a subacute neurologic deterioration from multiple ischemic events that resulted from severe radiation vascular injury. We compare and contrast this case to similar case reports in the literature.
Giant Intramedullary Teratoma in an Infant
Authors: Chidambaram Balasubramaniam B. , Balasubramaniam V. , Shankar S.K. , Santosh Vani
Published: April 2003

Teratomas are uncommon tumors of the nervous system, the commonest site being the pineal region. Spinal teratomas are very rare apart from the sacrococcygeal variety. The authors report the occurrence of an intramedullary teratoma in an infant. The clinical features, imaging studies and the pathology are discussed. In addition the literature is also reviewed.
Molecular Events Implicated in Brain Tumor Angiogenesis and Invasion
Authors: Zagzag David , Friedlander David R. , Margolis Ben , Grumet Martin , Semenza Gregg L. , Zhong Hua , Simons Jonathan W. , Holash Jocelyn , Wiegand Stanley J. , Yancopoulos George D.
Published: April 2003

We have conducted studies designed to help elucidate the molecular mechanisms involved in brain tumor invasion and angiogenesis, which are critical in the growth of malignant tumors of the central nervous system. A variety of molecular factors have been implicated in these processes. Here we focus on three that are of particular importance in the progression of brain tumors. Angiopoietins are involved in the regulation of vascular development. Hypoxia inducible factor-1 is a transcription factor that up-regulates genes, including genes encoding vascular endothelial growth factor under hypoxic conditions. Focal adhesion kinase is associated with infiltration of tumor cells and angiogenesis.
Cranial Vault Expansion: A Comparison of Magnetic Coupled Distraction to Traditional Surgical Repositioning
Authors: Baird Clinton , Fewings Paul , Manepalli Anantha , Pittman Thomas , Rinehart Gregory
Published: April 2003

We compared the effectiveness of transcutaneously activated magnetic distraction of an osteotomized cranial bone flap to surgical repositioning of the flap with immediate, rigid internal fixation. Thirty immature rabbits were studied. All 30 rabbits underwent complete circumferential osteotomies of both parietal bones and postoperatively all were fitted with head frames. The rabbits were divided into 4 groups. The experimental magnetic distraction group consisted of 10 animals (group 1). In these rabbits magnets were secured to both parietal bones and magnets of opposite polarity were placed in the headframes. Each morning, the magnets in the head-frames were moved 0.25 mm farther away from the skull; this continued for 20 days for a total setback of 5 mm. Five animals served as the magnetic distraction controls; in these animals magnets were placed on the parietal bones but none were attached to the headframes (group 2). The experimental surgical repositioning group consisted of 10 rabbits (group 3). In each the parietal bones were elevated and fixed 5 mm above the rest of the skull using vitallium mesh and screws. Five rabbits made up the surgical respositioning control group (group 4). In these animals, parietal osteotomies were performed but the bones were secured to the skull in their original positions using hardware identical to that used in the experimental group. Six weeks later all of the animals were sacrificed. Two significant differences were identified between the experimental groups: (1) the cranial contours of the animals in the magnetic distraction group were rounded while those of the surgically repositioned group were acutely angled; (2) the osteotomies in rabbits in the magnetic distraction group were essentially completely ossified while in rabbits in the surgically repositioned group there were obvious gaps at the osteotomy sites that were filled with fibrous tissue. These conclusions support the potential utility of magnetically activated distraction as an alternative to current methods of cranial vault remodeling.
Ventriculoperitoneal Shunt Flow Dependency on the Number of Patent Holes in a Ventricular Catheter
Authors: Ginsberg Howard J. , Sum Alfred , Drake James M. , Cobbold Richard S.C.
Published: April 2003

Minimally invasive recanalization of obstructed ventricular catheters is a new treatment modality currently under investigation. It has been shown that blocked ventricular catheters can be at least partly reopened with ultrasonic cavitation, fiberoptic delivery of laser energy and electrocautery. It is not known, however, how many of the holes must be reopened in order to establish acceptable pressure-flow characteristics. In this study, the pressure-flow relationship of partially obstructed ventricular catheters was determined using experimental and electrical circuit/computer models. It has been demonstrated in all cases that an additional pressure of no more than 0.5 cm of water is generated when only a single 500-μm-diameter hole in the ventricular catheter permits flow. Partial recanalization of a ventricular catheter provides acceptable CSF hydrodynamics.
Age-Related Differences in Acute Physiologic Response to Focal Traumatic Brain Injury in Piglets
Authors: Durham Susan R. , Raghupathi Ramesh , Helfaer Mark A. , Marwaha Sunil , Duhaime Ann-Christine
Published: April 2003

Introduction: The goal of the present study was to determine whether age-related differences in the acute physiologic response to scaled cortical impact injury contribute to differences in vulnerability to traumatic brain injury (TBI). Methods: Heart rate (HR), mean arterial pressure (MAP), brain temperature (BrT) and cerebral blood flow (CBF) were measured in 22 piglets (7 of age 5 days, 8 of age 1 month, 7 of age 4 months) at baseline and for 3 h following scaled cortical impact injury. Results: There were no age-dependent variations from baseline in HR, MAP or BrT following injury. CBF increased in the 5-day-old animals following injury while CBF in the 1- and 4-month-old animals decreased following injury (p = 0.0049). Conclusion: CBF was shown to have a significant age-dependent response to TBI with the youngest animals exhibiting increased CBF following injury.
A Case of Auto-Erotic Asphyxia in a Long-Term Psychiatric Setting
Authors: Quinn, John , Twomey, Pauline
Published: April 2003

The syndrome of auto-erotic asphyxia (AEA) involves the deliberate induction of cerebral hypoxia to produce or enhance sexual excitement. This sexual practice is normally only discovered when death results from the act because these individuals rarely seek professional advice. The practice of AEA in the living case presented here was initially misinterpreted as parasuicidal behaviour. This type of sexual behaviour should always be considered a differential for parasuicidal activity involving asphyxia. Aetiology, treatment and the need for health professionals to be aware of this syndrome are discussed.
Relations between Neuropsychological Vulnerability Markers and Negative Symptoms in Schizophrenia
Authors: Suslow, Thomas , Junghanns, Klaus , Weitzsch, Christine , Arolt Volker
Published: April 2003

In neuropsychological vulnerability research the visual backward masking task, the Span of Apprehension, the degraded stimulus Continuous Performance Test (dsCPT), and the Wisconsin Card Sorting Test have been described as putative indicators for the predisposition to develop negative (schizophrenic) symptoms. The present study assesses the stability of the association between neuropsychological tests and negative symptoms by examining clinically improved patients. The interdependence between the four cognitive measures and clinical symptomatology was examined in 31 patients with DSM III-R and ICD-10 schizophrenia suffering predominantly from negative symptoms. Backward masking performance was related to affective flattening and anxiety-depression. False alarm rate on dsCPT was associated positively with affective flattening and hallucinations, and negatively with avolition. Card sorting perseverative errors correlated negatively with anhedonia, non-perseverative errors correlated positively with avolition. Correlations notwithstanding, the data provide evidence in support of the relative independence of neuropsychological functions and negative symptoms in clinically improved schizophrenics.
Anhedonia, Alexithymia and Locus of Control in Unipolar Major Depressive Disorders
Authors: Loas G. , Dhee-Perot , , Chaperot , , Fremaux , , Gayant , , Boyer ,
Published: April 2003

The relationship between anhedonia, alexithymia and locus of control was studied in a group of healthy subjects (n = 56) and in a group of unipolar major depressives (n = 59). These dimensions were rated using the Physical Anhedonia Scale (PAS), the Toronto Alexithymia Scale (TAS) and the Internal Powerful others and Chance Scale. The unipolar major depressives were divided into two subgroups using the endogenomorphic subscale of the Hamilton Depression Rating Scale. There are respectively 25 and 17 depressives in the nonendogenomorphic (NENDO) and endogenomorphic (ENDO) subgroups. The unipolar major depressives were significantly more anhedonic, alexithymic and externalized than the controls. In the ENDO group the PAS and TAS Feelings scores were positively correlated suggesting that a third variable of emotional deficit could characterize endogenomorphic depression. In the NENDO group the PAS, TAS Daydreaming, Powerful others and Chance scores were positively correlated. In nonendogenomorphic depression anhedonia could be related to a poverty of imagery and could be interpreted as a consequence of external causes.
Beck Depression Inventory in the Detection of Depression among Neurological Inpatients
Authors: Lykouras Lefteris , Oulis Panayotis , Adrachta Dora , Daskalopoulou Eugenia , Kalfakis Nicos , Triantaphyllou Nicos , Papageorgiou Konstantinos , N.Christodoulou George
Published: April 2003

The validity of the Beck Depression Inventory (BDI) was assessed in a group of 150 neurological inpatients using Receiver Operating Characteristic analysis and DSM- III-R as external criterion. As regards depressive disorders as a whole, it was found that the best trade-off between sensitivity and specificity was the cutoff score of 20. The discriminating ability of the BDI for major depressive disorder was quite satisfactory at the cutoff score of 29 contrary to the dysthymic disorder in which the discriminating power of the BDI was not acceptable. In conclusion, the use of the BDI in neurological settings is useful with cutoff scores depending on the research purposes.
First Hospitalized versus Rehospitalized Patients with an Affective Disorder or Schizophrenia: Differences in Long-Term Course and Outcome
Authors: Vetter Peter H. , Köller Olaf
Published: April 2003

A comparison of course and outcome in schizophrenics and patients with unipolar affective disorders revealed significant differences not only between the two groups but also between first hospitalized and rehospitalized patients within each group. While schizophrenics fared worse in almost all parameters at the end of the 14-year follow-up period, within each group overall course and outcome were also poorer for rehospitalized versus first hospitalized patients. The poorest course was shown by rehospitalized schizophrenics. Future studies on course and outcome should differentiate between first and rehospitalized patients.
Changing Patterns of Attempted Suicide in Greece: Clinicoepidemiological and Psychodynamic Data
Authors: Ierodiakonou, C.S. , Iacovides, A. , Ierodiakonou-Benou I.
Published: April 2003

Greece is known to have one of the lowest rates of both completed and attempted suicides. Yet during the last three decades the pattern of suicide attempts has shown some differentiations, which point to possible changes in family structure and social behavior, bringing the pattern closer to that of western countries. While in 1971–72 the ratio of male to female attempters was 1:4.6, in 1994–96 it was 1:2.3 – the mean age rose from early to middle adulthood – more married people attempted suicide than before – more attempters took alcohol before the act etc. There are, nevertheless, many steady features, e.g. the low rates of attempters in old age (above 65 years: 7.9% of the total), the methods used, the type of preceding stressful life events (more financial than emotional in males), no drug abuse etc. Since the low rates of suicide attempts in Greece have been attributed to cultural parameters and the close bonds in the family, psychodynamic studies were also carried out, which showed that adolescent attempters preserve the ability for outward aggression and their self-destructive act is due to other than intrapsychic factors (usually social and relational problems).