bluuebunny/crossref_metadata_2025 huggingface dataset:
Death in Shunted Hydrocephalic Children in the 1990s
Authors: Iskandar Bermans J. , Tubbs Shane , Mapstone Timothy B. , Grabb Paul A. , Bartolucci Alfred A. , Oakes W. Jerry
Published: April 2003

Using a combined search of the Children’s Hospital (Birmingham, Ala., USA) medical records and the Jefferson County Health Department death records, we reviewed all shunt-related deaths that occurred between January 1990 and July 1996. Of these, we excluded patients who died of nonhydrocephalus-related reasons, such as bronchopulmonary dysplasia, as well as patients who had other serious neurological illnesses such as brain tumor and hydranencephaly. Twenty-eight patients died of shunt-related causes in the 6.5-year period. A survival analysis showed that 96% survived 32 months after first shunting. Of 28 patients, 23 were beyond help prior to medical evaluation. However, at least 10 of these patients had symptoms suggestive of shunt failure at least 24 h and as long as 2 weeks prior to their demise. We conclude that hydrocephalic children still die of shunt failure despite the modern technology of the 1990s. Some of these causes may be avoidable through early detection of symptoms. Guidelines to patients, families, and primary caregivers should be emphasized.
Abnormalities of Water Metabolism after Surgery for Optic/Chiasmatic Astrocytomas in Children
Authors: Daaboul Jorge , Steinbok Paul
Published: April 2003

A major concern during the early postoperative period after surgical resection of optic chiasmatic gliomas is the derangement of sodium and water metabolism which may add to the morbidity of the procedure. The purpose of this study was to characterize the abnormalities of water and sodium metabolism in children with optic chiasmatic gliomas treated surgically at British Columbia’s Children’s Hospital and to identify therapeutic modalities which might prevent or ameliorate the development of these complications. A retrospective chart review of children with optic/chiasmatic gliomas undergoing operations on the tumor was performed and the pre- and postoperative radiographs reviewed by an independent neuroradiologist. There were 11 patients who underwent 13 operations for either resection (n = 9) or biopsy (n = 4) of their optic chiasm tumor. The extent of resection in patients undergoing more than simple biopsy ranged from 83 to 99%, and all patients with resection had exophytic tumor extending into the hypothalamus. Postoperative syndrome of inappropriate antidiuretic hormone secretion (SIADH) and/or diabetes insipidus occurred after 8 of the 9 tumor resections and was associated with significant morbidity. No disturbance of water metabolism occurred after biopsy only. In patients with SIADH, the urinary sodium level rose markedly 6–12 h prior to the development of hyponatremia, and it was concluded that this was a valuable predictor of impending hyponatremia. Replacement of urine output was noted to be the most reliable way to avoid rapid fluctuations in serum sodium and to avoid the morbidity of diabetes insipidus or SIADH and is recommended in the postsurgical patients who cannot regulate fluid intake.
Unusual Association of Intractable Temporal Lobe Seizures and Intracranial Aneurysms in an Adolescent: Is It a Coincidence?
Authors: Baeesa Saleh S. , Dang Tommy , Keene Daniel L. , Ventureyra Enrique C.G.
Published: April 2003

Intracranial aneurysms in the pediatric age group are rare occurrences. They usually present with subarachnoid hemorrhage or mass effect. Their association with epilepsy has rarely been reported; such concurrence may not be a coincidence. We present a 16-year-old girl with a 5-year history of medically intractable complex partial seizures. Preoperative electrophysiological and neuroimaging studies demonstrated an epileptogenic focus and atrophy in the right mesial temporal lobe, and ipsilateral incidental aneurysm at the carotid artery bifurcation. The patient underwent a complete right anterior temporal lobectomy, followed by clipping of the aneurysm. We concluded that the epilepsy management in association with cerebral aneurysms is controversial, but when surgery is indicated, clipping of the aneurysm and resection of the epileptogenic focus may provide the optimal outcome. The relevant literature is reviewed and the possible mechanisms of production of epilepsy by intracranial aneurysms are discussed.
Myelitis: A Rare Presentation of Mumps
Authors: Bansal R. , Kalita J. , Misra U.K. , Kishore J.
Published: April 2003

We report a rare case of mumps myelitis in which parotid swelling appeared 3 days after the symptoms of myelitis. A 10-year-old boy presented with acute paraplegia of grade I–II on MRC (Medical Research Council) scale and retention of urine with normal sensations. Central motor conduction to tibialis anterior (CMCT-TA) on the right side was 32 ms and 24 ms on the left side. Spinal MRI revealed hyperintense signal changes extending from C3 to T12. After 5 days of methylprednisolone therapy, there was marked improvement in weakness, micturition normalized and CMCT-TA also returned to normal. MRI repeated after 1 month was also normal. This response seems to be due to antiedema and to the antiinflammatory effect of methylprednisolone, because of a temporal relationship between MPS and clinical improvement. However, the possibility of natural recovery cannot be ruled out.
Dural Tenting Sutures in Pediatric Neurosurgery
Authors: Winston Ken R.
Published: April 2003

Dural tenting sutures were used on a selective basis in 329 consecutive cranial operations in children; 16 (4.8%) required dural tenting sutures for the control of epidural bleeding and 313 (95.2%) required no dural sutures. Dural tenting sutures were never placed for prophylaxis or to satisfy a routine. Reoperation for postoperative epidural hematoma was required in 1 child (0.3%) and that child was subsequently discovered to have hemophilia. The author concludes that there is no compelling evidence for the prophylactic use of dural tenting sutures in children.
Intramedullary Lipoma of Dorsocervicothoracic Spinal Cord with Intracranial Extension and Hydrocephalus
Authors: Kogler Andrej , Orsolic Kresimir , Kogler Visnja
Published: April 2003

An intradural, intramedullary lipoma originating within the cervicothoracic cord with significant extension into the posterior fossa is reported. The lipoma was found incidentally by CT scan at the infant’s age of 2 days. Follow-up studies by CT and MRI were performed on several occasions. Because of hydrocephalus, VP shunting was performed. After shunting and shunt revisions, surgery of lipoma was performed at the age of 2 years. Posterior fossa craniectomy and laminectomy from C1 to T4 revealed a lipoma, which was maximally reduced. Six months after surgery, the child was well and showed good recovery.
Bithalamic Involvement Predicts Poor Outcome among Children with Thalamic Glial Tumors
Authors: Reardon David A. , Gajjar Amar , Sanford Robert A. , Heideman Richard L. , Walter Andrew W. , Thompson Stephen J. , Merchant Thomas E. , Li Hao , Jenkins Jesse J. , Langston James , Boyett James M. , Kun Larry E.
Published: April 2003

Clinical features and treatment of 36 consecutive pediatric patients with thalamic glial tumors confirmed by histology and characterized by neuroimaging were reviewed to identify prognostic factors. The median age at diagnosis was 10 years (range 1–18 years). Twenty-four patients had low-grade tumors (juvenile pilocytic astrocytoma n = 9, fibrillary astrocytoma n = 6, astrocytomas not otherwise specified n = 6, ganglioglioma n = 2 and oligodendroglioma n = 1) and 12 patients had high-grade tumors (glioblastoma multiforme n = 7, anaplastic astrocytoma n = 4 and unclassified malignant tumor n = 1). With a median follow-up of 4.3 years among survivors, estimates of 4-year progression-free survival (PFS) and overall survival (OS) for the entire group are 28 ± 10 and 37 ± 10%, respectively. Low-grade tumors were associated with a significantly better 4-year PFS (36 ± 12 vs. 0% for the high-grade group; p = 0.03) and OS (52 ± 12 vs. 0%; p < 0.001). This review identified that bithalamic involvement, characterized by neuroimaging, exerted an independent and significant negative impact on PFS and OS for patients with low-grade tumors. Estimates of 4-year PFS and OS among patients with low-grade bithalamic versus monothalamic tumors were 58 ± 15 vs. 0% and 85 ± 11 vs. 0% (p < 0.00001), respectively. The presence of bithalamic involvement did not affect outcome among patients with high-grade tumors. Additionally, age at diagnosis, enhancement with neuroimaging contrast, extension beyond the thalamus and extent of surgical resection did not correlate with overall outcome. Because treatment approaches varied during the study period, the impact of radiation therapy or chemotherapy could not be assessed. This contemporary, single-institution series of pediatric thalamic glial tumors demonstrates, for the first time, the statistical significance of bithalamic involvement as a marker of poor prognosis among patients with low-grade glial lesions.
Focal Plaque of Demyelination Mimicking Cerebral Tumor in a Pediatric Patient
Authors: Kumar Krishna , Toth Cory , Jay Venita
Published: April 2003

Focal, demyelinating lesions of the cerebrum mimicking brain tumors are a rare phenomenon, and even rarer in a pediatric population. We document the case of a 14-year-old female whose clinical, radiological and operative findings were strongly suggestive of glioma. However, histopathology revealed a demyelinating process. This case is significant as the lesion could not be distinguished from a glioma at any time in the presentation. At 41 months follow-up, the patient remains stable without further evidence of demyelination in other areas of the brain. Such a case suggests a cautious note for the pediatrician when presented with a similar patient and illustrates the importance of consideration of a demyelinating lesion in the differential diagnosis of a mass lesion in a pediatric population.
Cortical Microdysplasia and Surgical Outcome in Refractory Epilepsy of Childhood
Authors: Keene Daniel L. , Jimenez Cita-Carmen , Ventureyra Enrique
Published: April 2003

This paper examines the relationship between the degree and type of cortical microdysplasia as reported on pathological examination and the degree of seizure control after cortical resection in 70 children with medically refractory epilepsy. Cases selected for this review had to have had medically refractory epilepsy; had their first cortical resection before the age of 18 years, and have sufficient pathological material available for review. The pathological review was done blinded as to results of seizure control outcome postsurgery. Pathological material was classified to type and degree of microdysplasia, according to the classification scheme of Brannstrom. 11% of patients had no evidence of dysplasia; 77% had microdysplasia type 2, and 12% had focal dysplasia (type 3). No significant correlation between degree of seizure control postoperatively and type of microdysplasia was seen in this series.
Presentation, Management and Follow-Up of Schilder’s Disease
Authors: Garell P. Charles , Menezes Arnold H. , Baumbach Gary , Moore Steven A. , Nelson Gina , Mathews Katherine , Afifi Adel K.
Published: April 2003

Schilder’s diffuse myelinoclastic sclerosis is a rare demyelinating disease which often mimics intracranial neoplasm or abscess. We have treated 3 patients with this disorder in the past 5 years and followed their postoperative course. Certain distinct features of this disease will allow neurosurgeons to preoperatively entertain this diagnosis. We discuss postoperative treatment and propose a new hypothesis regarding the variable prognoses of this disorder. Schilder’s disease constitutes an important diagnosis for any neurosurgeon to be aware of (especially those treating the pediatric age group) which has not received adequate coverage in the neurosurgical literature.
Stereotactically GuidedE ndoscopy for the Treatment of Arachnoid Cysts
Authors: Rampini Paolo , Egidi Marcello , Zavanone Mario , Orsi Massimo , Alimehmeti Ridvan
Published: April 2003

Arachnoid cysts are well known to the neurosurgeon, and the evolution of their surgical treatment has followed technological advances in neurosurgical procedures and techniques. With the recent mastering of neuroendoscopy by the neurosurgeons, it is becoming the modality of choice for the treatment of arachnoid cysts. A neonate harboring a middle parasagittal arachnoid cyst benefited from stereotactically guided endoscopy. We report this case because of its peculiarities and introduce technical details about the procedure which are not found in the literature.
Pediatric Neurosurgery Work Force: A Pilot Study
Authors: Flannery Ann Marie
Published: April 2003

The physician work force of tomorrow is influenced by the number of physicians and specialists trained today. Little information exists about the need for pediatric neurosurgeons in the next 10 years or about the activity of pediatric neurosurgeons currently in practice. The objective of this study was to obtain preliminary data about pediatric neurosurgeons and current work loads. A questionnaire was distributed to physicians attending the Pediatric Section Meeting of the American Association of Neurological Surgeons, 1997, 93 questionnaires were returned. A majority of the respondents were between 35 and 55 years of age. The largest group geographically was from the northeast. Fifty-five respondents described their practice as academic and of that group, 48% reported that their practice was either exclusively or at least 95% pediatric neurosurgery. Self-described work load varied with geographic location. In the northeast, only 26% of practitioners felt they were not busy enough, whereas in the southwest, 60% reported not being busy enough. Academic practitioners were more likely than other respondents to describe their practice as too busy or appropriately busy. Thirty-six respondents indicated plans to either expand their practice or retire within the next 10 years. This pilot study demonstrates the potential for future growth in pediatric neurosurgery as well as providing information about the current self-reported work load and its distribution over practice types and geographic locations. This study provides guidance for development of future studies to improve the sensitivity and usefulness of acquired data.
Psychopharmacologic Treatment of Acquired Attention Disorders in Children with Brain Injury
Authors: Mahalick David M. , Carmel Peter W. , Greenberg John P. , Molofsky Walter , Brown Jeffrey A. , Heary Robert F. , Marks David , Zampella Edward , Hodosh Richard , von der Schmidt III Edward
Published: April 2003

This investigation examined the efficacy of psychostimulant therapy in alleviating neurobehavioral dysfunction attendant to pediatric brain injury. The most commonly reported neurobehavioral sequelae associated with head injury in the pediatric population involve deficits along the attentional matrix. This is also the most common objectively documented neurobehavioral finding among children as well as adults. There are several investigations in the adult literature which have employed the use of psychostimulants in treating both psychiatric and neuropsychological residua associated with head injury. Overall, the results of these studies are equivocal, but suggest a beneficial impact on general functioning. The present prospective investigation utilized a double-blind, placebo-controlled, cross-over experimental design to examine the efficacy of methylphenidate in treating children with acquired attentional disorders secondary to brain injury. A cohort of 14 children with varying degrees of head injury were recruited for participation. As expected, differences between drug and placebo conditions uniformly achieved statistical significance. Additionally, there were no differences in performance between baseline and placebo conditions on neurobehavioral tasks of attention and concentration. Current findings suggest that methylphenidate (and probably other psychostimulants such as Cylert, Adderal, Wellbutrin and dextroamphetamine sulfate) is an extremely effective agent in treating attentional disorders secondary to brain injury in children.
Pancraniosynostosis after Surgery for Single Sutural Craniosynostosis
Authors: Greene Jr. Clarence S.
Published: April 2003

Three children who developed pancraniosynostosis after surgery to correct nonsyndromic single suture synostosis (2 sagittal, 1 unilateral coronal) were noted on prolonged follow-up to have a decreased rate of head growth, and beaten copper findings on plain radiographs of the skull. All had elevated intracranial pressure as recorded by lumbar puncture. The children were reoperated, and then cranial growth resumed, and the radiographic abnormalities resolved. Because of the risk of pancraniosynostosis after surgery for single suture craniosynostosis, extended neurosurgical follow-up for patients with craniosynostosis is warranted.
Gastroesophageal Reflux in Infants with Hydrocephalus before and after Ventriculo-Peritoneal Shunt Operation
Authors: Shteyer E. , Rothman E. , Constantini S. , Granot E.
Published: April 2003

Gastroesophageal reflux (GER) is common in neurologically impaired children, especially those with central nervous system disorders. The cause of GER in these children has not yet been defined, but in animal studies, acute elevation of intracranial pressure (ICP) has been shown to result in a decrease in lower esophageal sphincter pressure. Ten infants with hydrocephalus underwent esophageal pH monitoring prior to and after a ventriculoperitoneal (V-P) shunt operation. A significant degree of reflux was present in 5 patients with hydrocephalus prior to shunt operation and reverted to normal in 2. In the other 3 infants, the degree of reflux decreased as evidenced by fewer abnormal parameters and lower scoring in each of the parameters measured. Our study supports the contention that increased ICP in infants is indeed associated with GER. As 4 of the 5 infants with significant reflux suffered from an Arnold-Chiari malformation, a causal relation between increased ICP due to defects involving the fourth ventricle floor and GER is suggested.
Primitive Cerebral Neuroectodermal Tumors Excluding Medulloblastomas: A Retrospective Study of 30 Cases
Authors: Mikaeloff Yann , Raquin Marie-Anne , Lellouch-Tubiana Arielle , Terrier-Lacombe Marie-José , Zerah Michel , Bulteau Christine , Habrand Jean-Louis , Kalifa Chantal
Published: April 2003

We present a retrospective study of 30 cases of primitive cerebral neuroectodermal tumors (PNET), excluding medulloblastomas, referred to us postoperatively for additional therapy to evaluate prognostic factors and treatment efficiency. The histologic types were: pinealoblastomas (n = 7); ependymoblastomas (n = 2); medulloepitheliomas (n = 4), and other PNET (n = 17). The tumor was located in the supratentorial area in 24 patients and in the posterior fossa in 6 patients. Among the supratentorial tumors, 8 were metastatic. Maximal surgical resection was performed. Sixteen of 30 patients had no measurable disease after surgery and were considered as standard-risk (SR) cases, and 14 with a local residue or metastasis as high-risk (HR) cases. The objective of postsurgical treatment was to avoid radiotherapy in children below 4 years of age. It consisted of radiotherapy alone in 6 patients, chemotherapy alone in 17, and radiotherapy with chemotherapy in 7. Furthermore, high-dose chemotherapy (busulfan, thiotepa) and autologous bone marrow transplantation, performed in 6 patients, yielded a response rate of 3/6. Event-free survival (EFS) of SR patients was 37% at 3 years (95% confidence interval (CI) 14–60%) and overall survival 44% (95% CI 26–62%). Only 1 of the HR patients achieved a complete remission and all of them died early. The critical prognostic factors appear to be the completeness of initial surgical resection and absence of metastasis. These tumors have a poor prognosis. Novel strategies (high-dose chemotherapy) are needed to improve their outcome because the children concerned are very young and the effects of radiotherapy are particularly deleterious when tumors are situated in the supratentorial area.
Intramedullary Spinal Cord Ependymomas in Children: Treatment, Results and Follow-Up
Authors: Lonjon M. , Goh K.Y.C. , Epstein F.J.
Published: April 2003

A retrospective review of 20 pediatric patients with intramedullary spinal cord ependymomas, all of whom underwent operative resection between 1985 and 1996, was undertaken to determine surgical results, long-term follow-up and tumor recurrence. Twelve children operated on in the same period with filum or cauda equina ependymomas were not included in this study. Nine children had had previous treatment before referral. Gross total resection was achieved in 14 patients and subtotal in 6. None of these had a post-operative radiation therapy. The median follow-up period was 67 months (range 25–177 months). All children were clinically evaluated before and after operation and at the last follow-up. The clinical grade at the last follow-up showed improvement in 8 patients (40%), was unchanged in 10 (50%) and deteriorated in 2 (10%). Three patients had a recurrence, 2 at the primary site (2 and 3 years after our surgery) and 1 at a distant site (3 years after). The actuarial 5- and 10-year survival rates were both 90%; 5- and 10-year progression-free survival rates were 93 and 70%, respectively. We conclude that a complete removal can be achieved in almost all cases of intramedullary spinal cord ependymomas in children, and that the long survival rates justify avoiding post-operative radiation therapy.
Supratentorial Dorsal Cistern Epidermoid Cyst in Childhood
Authors: Caruso Gerardo , Germanó Antonino , Caffo Mariella , Belvedere Massimo , La Torre Domenico , Tomasello Francesco
Published: April 2003

Epidermoid cysts (ECs) are rare developmental lesions occurring anywhere along the central nervous system. Usually affecting adult patients, the occurrence of supratentorial dorsal cistern ECs has been reported in only 1 certain case of pediatric age. An additional pediatric case is presented. An 11-year-old boy had an 8-year history of petit mal seizures. Neurologic examination on admission was negative. T1-weighted magnetic resonance images demonstrated homogenous, hypointense, intradural, extracerebral, right frontal, precentral, parasagittal mass with relatively high signal intensity on T2-weighted images. The lesion was completely removed using a microneurosurgical technique with special care taken with regard to the adjacent nervous and vascular structures. Histologically, an EC was diagnosed. The patient had no recurrence for 2 years and seizures were significantly reduced. This case suggests the need to include ECs in the differential diagnosis of intradural supratentorial extracerebral lesions in childhood.
Application of Signal Transduction Inhibition as a Therapeutic Strategy for Central Nervous System Tumors
Authors: Pollack Ian F. , Bredel Markus , Erff Melanie
Published: April 2003

During the last decade, rapid progress has been made in understanding the molecular pathways underlying the proliferation of both normal and neoplastic cells. The processes by which messages to initiate protein synthesis, cell cycle progression, and even cell death are transmitted from the cell surface or the cytoplasm to the nucleus are broadly referred to as ‘signal transduction’. These multistep pathways involve a host of proteins that interact with other proteins in overlapping cascades that flow downstream in a stepwise fashion from the cell membrane to the nucleus. Inappropriate overactivation or underactivation of various components of such signaling pathways can contribute to pathological processes, such as neoplasia. Conversely, molecular and pharmacological interventions that target and attempt to reverse the aberrant state of activation can potentially be of therapeutic benefit. The present article provides a background for understanding the contribution of signal transduction pathways to the proliferation of normal and neoplastic cells and describes ways in which targetted inhibition of selected signaling pathway components has been exploited to inhibit tumor growth in vitro and in vivo. Because most studies to date involving central nervous system (CNS) tumors have focused on gliomas, in view of their frequency in both the pediatric and adult age groups, the discussion of therapeutic applications for CNS neoplasia will deal primarily with these lesions. However, the basic concepts presented are generalizable to most tumor types and have been successfully applied in vitro in medulloblastomas as well. Ultimately, the translation of such strategies to the treatment of patients with malignant brain tumors may provide novel approaches for improving the poor outlook associated with these neoplasms.
Frontal and Occipital Horn Ratio: A Linear Estimate of Ventricular Size for Multiple Imaging Modalities in Pediatric Hydrocephalus
Authors: O’Hayon B.B. , Drake J.M. , Ossip M.G. , Tuli S. , Clarke M.
Published: April 2003

Objective: Measurement of ventricular size is important in pediatric patients with hydrocephalus, especially those who are being followed with cerebrospinal fluid (CSF) shunts. While volumetric techniques are a more accurate estimate of true ventricular volume, they are often impracticable when multiple modalities including ultrasound are used. Volumetric area and linear measurements were compared to find the most reasonable measurement method. Methods: Sixty-four computed tomography (CT), magnetic reasonance imaging (MRI), and ultrasound (US) scans from 25 children aged 0–17 years with hydrocephalus, before and after treatment, were measured. Measurements included ventricular volume, a ventricular/brain ratio, and four standard linear measures (Evans’ ratio, Huckman’s measurement, minimal lateral ventricular width, and lateral ventricular span at the body). We also included a new ratio, which accounts for often disproportionate occipital horn expansion in pediatric patients, called the frontal and occipital horn ratio. Volume and linear measurements were compared using the Spearman’s correlation coefficients and correlations were further differentiated using a Z test statistic. The frontal and occipital horn ratio was also measured on CT, MRI, and US scans from 44 normal children aged 0–17 years to identify normal values. The effect of age was determined by linear regression. Results: The best linear correlation with ventricular size was the frontal + occipital horn ratio (r = 0.852) and was equivalent to the ventricular/brain ratio (r = 0.891), previously shown to have the highest correlation with ventricular volume. Evans’ ratio correlates less well (r = 0.423). The normal frontal and occipital horn ratio is 0.37 and is independent of age. Conclusions: The frontal and occipital horn ratio is a simple method of evaluating ventricular size in pediatric hydrocephalus patients with CSF shunts.
The Embryogenesis of Congenital Vertebral Dislocation: Early Embryonic Buckling?
Authors: Dias Mark S. , Li Veetai , Landi Michael , Schwend Richard , Grabb Paul
Published: April 2003

Congenital vertebral dislocation (CVD) is a rare congenital spinal malformation characterized by a translatory or rotatory vertebral displacement, or both, at a single level, that results in an abrupt angulation of the neural canal. The more caudal vertebra is dysplastic and appears at first glance to be posteriorly dislocated into the vertebral canal as a posterior hemivertebra, but is actually well aligned with the more caudal vertebral column. Unfortunately, the present classification of complex congenital vertebral anomalies is confusing, and CVD has been grouped together with other congenital vertebral malformations under the terms ‘segmental spinal dysgenesis’, ‘medial spinal aplasia’, and others. Moreover, a putative embryonic mechanism has never been proposed for CVD. Based upon our experience with 6 children and a critical review of the literature, we identify CVD as a distinct entity having characteristic anatomical features. We propose a novel putative embryonic mechanism – early embryonic ‘buckling’ – which likely occurs by the 6th embryonic week, prior to the period of axonal outgrowth and the beginning of vertebral chondrification.
Bilateral Epidural Hematoma in a Pyknodysostotic Child
Authors: Çırak Bayram , Mut Melike , Akalan Nejat
Published: April 2003

Pyknodysostosis is a rarely encountered disease. It is characterized by abnormal facial development, fragility of the bones with relatively mild trauma, cranial abnormalities including widely opened sutures and fontanels frontal bossing, wrinkled skin, finger and nail abnormalities. In the literature, pyknodysostosis was reported only as case reports. Here, we describe a pyknodysostotic child with a bilateral parietal epidural hematoma, caused by a relatively mild head trauma. He was operated on, and the hematoma was evacuated through two different parietal craniotomies. We reported this case, since pyknodysostosis is a rare disease, and our case is the first one who had a surgical intervention as well as being the first with an epidural hematoma in pyknodysostosis.
Large Chronic Cephalohematoma without Calcification
Authors: Firlik KatrinaS. , Adelson P.David
Published: April 2003

Cephalohematomas following birth normally resorb within the 1st month of life. In cases of prolonged resorption, over greater than 1 month, cephalohematomas typically begin to calcify. We report the case of a 3-month-old child with a persistent, large cephalohematoma that did not calcify. After observation alone failed to demonstrate a decrease in the size of the hematoma, 30 cm3 of old blood was aspirated, and the patient's head was wrapped. Unlike calcified cephalohematomas, this noncalcified lesion did not require open surgical intervention. A treatment protocol for cephalohematomas is presented.
Spontaneous Resolution of a Cervicothoracic Syrinx in a Child
Authors: Avellino AnthonyM. , Britz GavinW. , McDowell JamesR. , Shaw DennisW. , Ellenbogen RichardG. , Roberts TheodoreS.
Published: April 2003

A child with near complete spontaneous resolution of a cervicothoracic syrinx and improvement in a Chiari type I malformation without surgical intervention is presented. The child was followed clinically with serial magnetic resonance (MR) imaging and has remained neurologically stable over an 11-year period. To our knowledge, only 3 pediatric cases of spontaneous resolution of a spinal cord syrinx as documented by MR imaging without surgical intervention have been reported. This case contributes to the literature on the natural history of syringes.
An Assessment of Observer Bias in the Shunt Design Trial
Authors: Kestle J. , Milner R. , Drake D.
Published: April 2003

Introduction: An international multicenter randomized trial comparing standard pressure differential valves, Orbis Sigma valves and PS Medical Delta valves, for children with newly diagnosed hydrocephalus failed to show a difference in the time to first shunt failure (power 80%). In order to avoid observer bias, the primary endpoint, shunt failure, was defined by detailed clinical and radiological criteria. Surgeons notified the data center when their patient reached endpoint. Their decision was blindly reviewed by a centralized adjudication process. The current analysis asks: (1) Did the surgeons and the blinded adjudication process agree on the presence of shunt failure? (2) Do the shunt survival curves change when the surgeons’ assessment of outcome is used? Methods: The two methods of outcome assessment (surgeons’ decision and adjudication process) were compared with a kappa statistic for the presence of shunt failure and a log rank statistic for time to shunt failure. Results: The surgeon and the adjudication process agreed on the presence of the primary outcome in 313/344 patients in the trial (kappa = 0.81). The 31 cases of disagreement were evenly distributed among the three shunts. The survival curves generated from the surgeons’ decision were not significantly different from those generated by the adjudication process (log rank = 0.70, p = 0.37) and did not alter the study conclusions. Discussion: Observer bias in the assessment of outcome is always a concern in an unblinded clinical trial such as this one. We did not detect an observer bias of sufficient magnitude to alter the study conclusions.
Pediatric Atlantoaxial Instability: Management with Screw Fixation
Authors: Wang Jin , Vokshoor Amir , Kim Stanley , Elton Scott , Kosnik Edward , Bartkowski Henry
Published: April 2003

Sixteen pediatric patients (age range 3–15 years; mean 9.4 years) with atlantoaxial instability underwent screw fixation at Columbus Children’s Hospital between 1992 and 1998. Three patients with type II odontoid fractures underwent odontoid screw fixation. The remaining group of 13 patients had posterior C1-2 transarticular screw fixation and Sonntag C1-2 fusion. The group included 3 patients with rotatory C1-2 fixation, 4 patients with os odontoideum, 4 patients with congenital atlantoaxial instability and 2 patients with traumatic C1-2 instability. Postoperatively, all patients were placed in a Miami-J collar only. At 3 months follow-up, all patients achieved fusion. Bony fusion across the fracture line was clearly evident in patients with odontoid screws. The only complications in this series were a transient swallowing difficulty that resolved spontaneously in 2 weeks, and another patient’s C1-2 fusion had extended to C2-3 at 9 months follow-up. This study demonstrates that children at 3 years of age and older, who sustain a type II odontoid fracture with an intact transverse ligament, can be safely managed with odontoid screws if the fracture is less than 4 weeks old. Posterior C1-2 transarticular screw fixation can be done safely and results in a high fusion rate in children older than 4 years of age. The technical difficulties of screw fixation in children are discussed.
Brain Mapping in Sedated Infants and Young Children with Passive-Functional Magnetic Resonance Imaging
Authors: Souweidane M.M. , Kim K.H.S. , McDowall R. , Ruge M.I. , Lis E. , Krol G. , Hirsch J.
Published: April 2003

Functional magnetic resonance imaging (fMRI) in pediatric patients presents a unique set of problems due to the need for patient compliance, the frequent need for sedation and an early developmental status. A new method for using fMRI in sedated infants and young children is presented using passive stimuli focused on visual, sensorimotor and language functions. All of these stimuli are presented such that no patient interaction is required. Eight sedated children undergoing diagnostic MRI scans of the brain participated in these passive fMRI procedures. Cortical regions were identified using standard techniques applied to the blood-oxygen-level-dependent signal which is the basis for fMRI. The results support the feasibility of brain mapping in sedated children with passive fMRI techniques.
Cutaneomeningospinal Angiomatosis (Cobb Syndrome) with Tethered Cord
Authors: Brant Adam J. , James Hector E. , Tung Howard
Published: April 2003

A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similiar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb’s syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.
Malignant Glial Tumor Arising from the Site of a Previous Hamartoma/Ganglioglioma: Coincidence or Malignant Transformation?
Authors: Mittler Mark A. , Walters Beverly C. , Fried Arno H. , Sotomayor Edgar A. , Stopa Edward G.
Published: April 2003

Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar ‘glial-neuronal hamartoma’ at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.
Endoscopic-Guided Proximal Catheter Placement in Treatment of Posterior Fossa Cysts
Authors: Sandberg David I. , Souweidane Mark M.
Published: April 2003

Purpose: Treatment of posterior fossa cysts by cystoperitoneal shunting may be complicated by a malpositioned proximal catheter located within the brainstem or cerebellum causing acute shunt malfunction or neurological deficits. We propose that proximal catheter placement from a posterior fossa approach aided by a malleable endoscope may prevent malposition and its complications. Methods: We present 4 procedures we performed on 3 patients with posterior fossa cysts using a posterior fossa approach. In each case, the proximal catheter was molded along with a malleable endoscope to place the catheter parallel to the long axis of the fourth ventricle. Direct visualization during catheter placement insured an intracavitary position. Results: Ultimately, the procedure was successful in all 3 patients as judged by intracavitary catheter position and decrease in cyst size on postoperative imaging. In 1 patient, revision using the same technique was required based upon suboptimal catheter position within one of numerous cystic compartments within the posterior fossa. There were no complications related to direct or indirect brainstem injury. Conclusions: Many posterior fossa cysts can be treated effectively and safely via a posterior fossa approach with the aid of a malleable endoscope. Direct visualization facilitates intracavitary catheter placement and orientation of the catheter in the long axis of the cyst, thereby decreasing the risk of injury to surrounding structures.
MR Artifact Mimicking a Temporal Lobe Lesion in an Epilepsy Patient
Authors: Mummaneni P.V. , Simon E.M. , Peacock W.J. , Barkovich A.J.
Published: April 2003

A ten-year-old healthy child presented with a right upper extremity focal seizure which secondarily generalized. Magnetic resonance imaging (MR) revealed a 1-cm area of abnormal signal intensity in the left posterior temporal lobe at the gray-white junction. This did not appear on all imaging sequences, raising the suspicion of an artifact. Repeat MR revealed no intracranial or extracranial pathology. This case illustrates MR ‘wrap around’ artifact that mimicked a temporal lobe abnormality in an epilepsy patient. The physics of MR are reviewed as they pertain to this artifact.
Does Age or other Factors Influence the Incidence of Ventriculoperitoneal Shunt Infections?
Authors: Davis Steven E. , Levy Michael L. , McComb J. Gordon , Masri-Lavine Lena
Published: April 2003

Some studies indicate that infants, especially those less than 1 month of age have a higher incidence of ventriculoperitoneal shunt infections. To look at age as well as other variables that might relate to the rate of shunt infection, we reviewed the records of all patients undergoing a ventriculoperitoneal shunt insertion or revision at our institution from January 1, 1985, to December 31, 1994. There were a total of 2,325 ventriculoperitoneal shunting procedures performed on 1,193 patients with a male:female ratio of 678:515. The overall infection rate was 3.2% (74 infections). Analyzed by age, the infection rates were as follows: <1 month 9/223 (4.0%), 1–6 months 16/449 (3.6%), 6–12 months 13/297 (4.4%), 12–18 months 3/122 (2.5%), 18–24 months 7/116 (6.0%) and 24+ months 26/1,118 (2.3%). There was no statistically significant difference between age groups (p > 0.05). Upon selectively examining premature neonates who developed hydrocephalus secondary to intraventricular hemorrhage from the figures given above, one finds that 2/44 (4.5%) of neonates became infected, which was also not significant. The infection rate was the same irrespective of whether the procedure was to insert or revise the shunt, or whether another operative procedure was done under the same anesthesia. The etiology of the hydrocephalus was not a factor, nor was the presence of an open neural tube defect. The presence of fluid accumulation along the shunt tract or at another neurological operative site was associated with a significant increase in incidence of infection 15/168 (8.9%) when compared to those with no fluid accumulation (p < 0.001). The type of infecting organism was divided roughly in thirds, with relatively equal representation from Staphyloccocus epidermidis/coagulase negative and Staphylococcus aureus. The remaining third was comprised of a wide variety of organisms.
Coexistence of a Ganglioglioma and Rasmussen’s Encephalitis
Authors: Firlik Katrina S. , Adelson P. David , Hamilton Ronald L.
Published: April 2003

A small number of recent reports have documented coexisting cerebral pathologic entities in patients with Rasmussen’s encephalitis. We report the case of a 4-year-old boy who presented with refractory seizures and was found to have both a ganglioglioma and Rasmussen’s encephalitis of the same hemisphere. The patient ultimately underwent a functional hemispherectomy with excellent seizure control. We also review the clinical and pathologic hallmarks of both gangliogliomas and Rasmussen’s encephalitis. We hope that the addition of this patient to the small but growing literature on dual pathology in Rasmussen’s encephalitis may help shed some light on the etiology of this mysterious disease.
Surgical Treatment of Hydrocephalus: A Historical Perspective
Authors: Rachel Rivka A.
Published: April 2003

Surgical treatment for hydrocephalus has a long and eventful history. This review emphasizes the significant advances made in this century, describing the personalities involved, technical approaches attempted, and materials tested. It concludes by suggesting that better methods of treatment can and should continue to be developed.
Genetic Alterations in Pediatric Medulloblastomas Detected by Comparative Genomic Hybridization
Authors: Nishizaki Takafumi , Harada Katsumi , Kubota Hisashi , Harada Kei , Ozaki Satoshi , Ito Haruhide , Sasaki Kohsuke
Published: April 2003

Children with medulloblastomas show diverse clinical courses even when receiving similar treatments. In this study, comparative genomic hybridization, which allows the detection of losses and gains in DNA copy number along the entire genome, was used to investigate the genetic alterations in 6 cases of medulloblastoma with adequate follow-up periods and similar treatments, and in a medulloblastoma cell line. In the cell line, the number of aberrations was the highest of all samples examined. In 6 clinical samples, frequently altered regions (more than 3 cases) observed in all medulloblastomas were gains of 7q and 17q (4 cases each), and of 2p, 2q and 7p (3 cases each). High-grade amplification was observed at the loci 8q, 17q and 21q, each in a single case. The case with the most favorable outcome 9 years after surgery had the smallest number of chromosomal changes among the cases examined. Our results may indicate that further acquisition of genetic alterations detected by comparative genomic hybridization are associated with unfavorable prognosis in patients with medulloblastomas.
Extrarenal Nephroblastic Proliferation in Spinal Dysraphism
Authors: Abrahams John M. , Pawel Bruce R. , Duhaime Ann-Christine , Sutton Leslie N. , Schut Luis
Published: April 2003

Four cases of extrarenal nephrogenic proliferation in the sacrococcygeal region with spinal dysraphism are presented. In two of the cases, features of Wilm's or incipient Wilm's tumor were present. The previous literature on sacrococcygeal nephrogenic tissue is reviewed, and the impact of these findings on the histogenesis of extrarenal sacrococcygeal Wilm's tumor is discussed.
Successful Surgical Treatment of a Thalamic Hydatid Cyst with Contralateral Transcallosal Approach
Authors: Kurtsoy Ali , Öktem I. Suat , Koç R. Kemal , Akdemir Hidayet , Menkü Ahmet , Tucer Bülent
Published: April 2003

An extremely rare case of a thalamic hydatid cyst is presented and the literature is reviewed. A right thalamic hydatid cyst without rim enhancement or perifocal edema was detected by computed tomography and magnetic resonance. This lesion was extirpated successfully with intact contents via contralateral transcallosal approach. To our knowledge, this is the second hydatid cyst of the thalamus, an unusual location, and the first hydatid cyst to be removed completely with intact contents reported in the literature.
Posterior Fossa Syndrome: Identifiable Risk Factors and Irreversible Complications
Authors: Doxey Deborah , Bruce Derek , Sklar Frederick , Swift Dale , Shapiro Ken
Published: April 2003

Cerebellar mutism was first described by Rekate et al. in 1985 as a transient condition which occurs after posterior fossa operations in children. Posterior fossa syndrome (PFS) and cerebellar mutism are often used interchangeably in the literature. In our experience, we found cerebellar mutism to be a reversible component of a persistent neurologic syndrome. The cause and identifiable risk factors have not been clearly elucidated in the literature. To further characterize PFS, we reviewed 253 children with posterior fossa tumors who underwent surgical resection. We documented 20 cases of PFS (8%), 12 males and 8 females. Age ranged from 1.5 to 13 years (mean = 6.5). Of the 20, 16 were medulloblastoma, 3 ependymoma and 1 astrocytoma. There was a 21% incidence (16/76) of PFS in medulloblastoma of the posterior fossa. The incidence for ependymoma was 13% (3/24) and 1% (1/102) for astrocytoma. All 20 cases (100%) had brainstem involvement by the tumor. The most frequent postoperative findings included mutism, ataxia, 6th and 7th nerve palsies and hemiparesis. Mutism had a latency range of 1–7 days (mean = 1.7) and a duration of 6–365 days (mean = 69.2, median = 35). Although mutism resolved in all cases, the remaining neurologic complications which characterized our findings of PFS were rarely reversible. We describe potential risk factors for developing PFS after surgery with hopes of making neurosurgeons more aware of potential problems following the removal of lesions in this area. Early recognition of PFS would further promote patient and family understanding and coping with this syndrome.