bluuebunny/crossref_metadata_2025_split huggingface dataset:
Coexistent intraventricular abnormalities in periventricular giant arachnoid cysts
Authors: Rangel-Castilla Leonardo , Torres-Corzo Jaime , Vecchia Roberto Rodriguez Della , Mohanty Aaron , Nauta Haring J. W. 
Published: September 2009

Object
Arachnoid cysts are congenital lesions that arise during development by splitting of the arachnoid membrane. Large cysts can be adjacent to CSF pathways causing a marked midline shift and hydrocephalus. The association between a large arachnoid cyst and hydrocephalus has been commonly described as being due to a mass effect, but these previous reports have not focused closely on any associated intraventricular abnormalities.

Methods
Seven patients who were previously treated with a cystoperitoneal shunt presented with shunt failure, hydrocephalus, and/or cyst expansion. All of these patients had giant arachnoid cysts extending to the periventricular region from the original site, which was the sylvian fissure in 4 patients, and the suprasellar cistern, quadrigeminal cistern, and interhemispheric fissure in 1 patient each. Endoscopic exploration of the ventricular system and cyst fenestration was then performed in all patients.

Results
The endoscopic findings were obstruction of the cerebral aqueduct by a membrane not related to the cyst in 5 patients, occlusion of the foramen of Monro in 6, septum pellucidum hypoplasia in 2, and occlusion of the cerebral aqueduct by a quadrigeminal arachnoid cyst in 1. Endoscopic procedures performed were septum pellucidum fenestration and/or foraminoplasty in 5 patients, aqueductoplasty in 2, endoscopic third ventriculostomy in 5, fenestration of the lamina terminalis in 1, and direct cystocisternostomy in 1. After the endoscopic procedure, signs and symptoms of increased intracranial pressure and hydrocephalus improved in all patients, with a reduction in size of the cyst and the ventricle.

Conclusions
Ventricular abnormalities contributing to hydrocephalus may be associated with arachnoid cysts. These abnormalities may more likely reflect a common origin than a casual relation. Foramen of Monro stenosis and cerebral aqueduct occlusion associated with an arachnoid cyst can be more frequent than has been previously believed. In cases of periventricular giant arachnoid cysts, endoscopic exploration is a good alternative for examining the ventricular system and identifying and treating CSF obstructions caused by and/or related to arachnoid cysts.
Seckel syndrome and moyamoya
Authors: Codd Patrick J. , Scott R. Michael , Smith Edward R. 
Published: September 2009

Seckel syndrome is an autosomal recessive disorder characterized by intrauterine and postnatal growth delay, microcephaly with mental retardation, and facial dysmorphisms including micrognathia, a recessed forehead, and a large beaked nose. Occurring in 1 in 10,000 children without sex preference, it is the most common primordial microcephalic osteodysplastic dwarfism and has been associated with a variety of congenital brain malformations and intracranial aneurysms. Moyamoya syndrome is an idiopathic, chronic, progressive cerebrovascular disorder marked by stenosis of the intracranial internal carotid arteries and concurrent development of hypertrophied collateral vessels. These tortuous arterial collaterals appear radiographically as “puffs of smoke,” giving the syndrome its name. In this report, the authors describe the case of a 16-year-old girl with coincident Seckel and moyamoya syndromes. To their knowledge, this is the first reported case of such an association being treated with surgical revascularization.
The patient presented with persistent headaches and a 2-year history of progressive hand, arm, and face numbness. Imaging studies revealed multiple completed cerebral infarcts, global ischemic changes, and vascular anatomy consistent with moyamoya syndrome. Bilateral pial synangioses successfully revascularized each hemisphere with resolution of the patient's symptoms. The patient died 1 year later of complications related to treatment of a rapidly progressing intracranial aneurysm.
This report documents the first case associating moyamoya and Seckel syndromes. In addition, the report reveals the rapid development of an intracranial aneurysm in a patient with this syndrome. When coupled with previous reports of other types of cerebrovascular disease in patients with Seckel syndrome or other primordial dwarfisms, the authors' findings are important because they suggest that physicians treating patients with dwarfism should consider the diagnosis of moyamoya syndrome when symptoms suggestive of cerebral ischemia are present. Prompt diagnosis and treatment of moyamoya syndrome, including the use of proven surgical revascularization procedures such as pial synangiosis, may significantly improve the long-term outcomes of these patients.
Tethered cord release: a long-term study in 114 patients
Authors: Bowman Robin M. , Mohan Avinash , Ito Joy , Seibly Jason M. , McLone David G. 
Published: September 2009

Object
All children born with a myelomeningocele at the authors' institution undergo aggressive treatment to maintain or improve functional outcome. Consequently, when any neurological, orthopedic, and/or urological changes are noted, a search for the cause is initiated. The most common cause of decline in a child born with a myelomeningocele is shunt malfunction. The second most common cause is tethering of the distal spinal cord at the site of the original back closure. In this report, the authors review the indicators of symptomatic spinal cord tethering and discuss the surgical interventions and outcomes in the children with myelomeningocele who underwent treatment at Children's Memorial Hospital from 1975 to 2008.

Methods
Among the 502 children who underwent original closure at Children's Memorial Hospital, a symptomatic tethered spinal cord developed in 114 (23%). Eighty-one patients (71%) have undergone 1 untethering procedure, and 33 patients (29%) have undergone multiple untetherings, for a total of 163 total surgeries. The indicators of symptomatic spinal cord tethering include scoliosis, decline in lower-extremity (LE) motor strength, LE contractures, LE spasticity, gait change, urinary changes, and pain.

Results
Pain has shown the best response to surgical untethering, with 100% of children experiencing postoperative improvement. The results of long-term follow-up (average 12 years, range 1 month–23.3 years) in this cohort demonstrated scoliosis progression after surgical untethering in 52% of patients, with 28% requiring spinal fusion. On the 3-month postoperative manual muscle test, 70% of patients showed improved LE muscle strength compared to preoperatively. Gait was also similarly improved after untethering as evaluated by an orthopedic surgeon. Spasticity improved in two-thirds of the cohort, and as expected, LE contractures were stable (78%) postoperatively, as assessed by orthopedic and rehabilitation medicine specialists. Urologically, 64% of patients showed improvements on postoperative bladder evaluation.

Conclusions
Although this is a clinical outcome study with no control group, the authors' experience has been that tethered cord release is beneficial in maintaining neurological, urological, and orthopedic functioning in children born with a myelomeningocele.
Embryopathogenetic surgicoanatomical classification of dysraphism and surgical outcome of spinal lipoma: a nationwide multicenter cooperative study in Japan
Authors: Oi Shizuo , Nomura Sadahiro , Nagasaka Masato , Arai Hajime , Shirane Reizo , Yamanouchi Yasuo , Nishimoto Hiroshi , Date Hiroaki 
Published: September 2009

Object
The natural history of asymptomatic spinal lipoma in infancy remains unclear, and the indication for the prophylactic untethering operation is still debatable. To address this question, a multicenter cooperative study for the treatment of spinal lipoma was performed by the 7 most active institutions in neurosurgical care for spina bifida in Japan between 2001 and 2005.

Methods
Patients were classified using the embryopathogenetic surgicoanatomical classification. Their neurosurgical postoperative course was analyzed using the Spina Bifida Neurological Scale. Among 261 patients, 159 were asymptomatic and 102 were symptomatic.

Results
Of the 136 patients for whom prophylactic surgeries were performed, 135 remained asymptomatic and only 1 (0.4%) of the 261 patients presented with mild sensory disturbance. Mild foot deformity was identified in 1 (4.3%) of 23 conservatively observed patients. Of 100 symptomatic patients, deterioration after surgery was seen in 6%, and improvement in 44%. Complete resolution of symptoms was seen in only 14.2%. Filar types for patients > 3 years old improved in Spina Bifida Neurological Scale scores from 12.3 to 14.0. The mean age of symptomatic patients with lipomyelomeningocele was the youngest of all (1.3 years), which indicates lipomyelomeningocele may deteriorate in early infancy. Improvements from surgery were seen for all types of lipoma except the caudal type, presenting at an older mean age (15 years).

Conclusions
A low rate of postsurgical worsening indicates that surgeries for asymptomatic and symptomatic lipomas are safe. Surgeries done after the onset of symptoms seldom cure the patients. These two results support early untethering for any kind of lipoma; however, further study of the natural history is required.
Predicting postresection hydrocephalus in pediatric patients with posterior fossa tumors
Authors: Riva-Cambrin Jay , Detsky Allan S. , Lamberti-Pasculli Maria , Sargent Michael A. , Armstrong Derek , Moineddin Rahim , Cochrane D. Douglas , Drake James M. 
Published: September 2009

Object
Approximately 30% of children with posterior fossa tumors exhibit hydrocephalus after tumor resection. Recent literature has suggested that prophylactic endoscopic third ventriculostomy diminishes the risk of this event. Because the majority of patients will not have postoperative hydrocephalus, a preoperative clinical prediction rule that identifies patients at high or low risk for postresection hydrocephalus would be helpful to optimize the care of these children.

Methods
The authors evaluated a derivation cohort of 343 consecutive children with posterior fossa tumors who underwent treatment between 1989 and 2003. Multivariate methods were used on these data to generate the Canadian Preoperative Prediction Rule for Hydrocephalus. The rule's estimated risk of postresection hydrocephalus was compared with risk observed in 111 independent patients in the validation cohort.

Results
Variables identified as significant in predicting postresection hydrocephalus were age < 2 years (score of 3), papilledema (score of 1), moderate to severe hydrocephalus (score of 2), cerebral metastases (score of 3), and specific estimated tumor pathologies (score of 1). Patients with scores ≥ 5 were deemed as high risk. Predicted probabilities for the high- and low-risk groups were 0.73 and 0.25, respectively, from the derivation cohort, and 0.59 and 0.14 after prevalence adjustment compared with the observed values of 0.42 and 0.17 in the validation cohort.

Conclusions
A patient's score on the Preoperative Prediction Rule for Hydrocephalus will allow improved patient counseling and surgical planning by identifying patients at high risk of developing postresection hydrocephalus. These patients might selectively be exposed to the risks of preresection CSF diversion to improve outcome.
Effectiveness of ketamine in decreasing intracranial pressure in children with intracranial hypertension
Authors: Bar-Joseph Gad , Guilburd Yoav , Tamir Ada , Guilburd Joseph N. 
Published: September 2009

Object
Deepening sedation is often needed in patients with intracranial hypertension. All widely used sedative and anesthetic agents (opioids, benzodiazepines, propofol, and barbiturates) decrease blood pressure and may therefore decrease cerebral perfusion pressure (CPP). Ketamine is a potent, safe, rapid-onset anesthetic agent that does not decrease blood pressure. However, ketamine's use in patients with traumatic brain injury and intracranial hypertension is precluded because it is widely stated that it increases intracranial pressure (ICP). Based on anecdotal clinical experience, the authors hypothesized that ketamine does not increase—but may rather decrease—ICP.

Methods
The authors conducted a prospective, controlled, clinical trial of data obtained in a pediatric intensive care unit of a regional trauma center. All patients were sedated and mechanically ventilated prior to inclusion in the study. Children with sustained, elevated ICP (> 18 mm Hg) resistant to first-tier therapies received a single ketamine dose (1–1.5 mg/kg) either to prevent further ICP increase during a potentially distressing intervention (Group 1) or as an additional measure to lower ICP (Group 2). Hemodynamic, ICP, and CPP values were recorded before ketamine administration, and repeated-measures analysis of variance was used to compare these values with those recorded every minute for 10 minutes following ketamine administration.

Results
The results of 82 ketamine administrations in 30 patients were analyzed. Overall, following ketamine administration, ICP decreased by 30% (from 25.8 ± 8.4 to 18.0 ± 8.5 mm Hg) (p < 0.001) and CPP increased from 54.4 ± 11.7 to 58.3 ± 13.4 mm Hg (p < 0.005). In Group 1, ICP decreased significantly following ketamine administration and increased by > 2 mm Hg during the distressing intervention in only 1 of 17 events. In Group 2, when ketamine was administered to lower persistent intracranial hypertension, ICP decreased by 33% (from 26.0 ± 9.1 to 17.5 ± 9.1 mm Hg) (p < 0.0001) following ketamine administration.

Conclusions
In ventilation-treated patients with intracranial hypertension, ketamine effectively decreased ICP and prevented untoward ICP elevations during potentially distressing interventions, without lowering blood pressure and CPP. These results refute the notion that ketamine increases ICP. Ketamine is a safe and effective drug for patients with traumatic brain injury and intracranial hypertension, and it can possibly be used safely in trauma emergency situations.
Correlation between ventriculomegaly on prenatal magnetic resonance imaging and the need for postnatal ventricular shunt placement
Authors: Hankinson Todd C. , Vanaman Monique , Kan Peter , Laifer-Narin Sherelle , DeLaPaz Robert , Feldstein Neil , Anderson Richard C. E. 
Published: September 2009

Object
Pediatric neurosurgeons are increasingly called on to provide prognostic data regarding the antenatal diagnosis of ventriculomegaly. This study was designed to determine if there is a correlation between prenatal MR imaging results and the need for ventricular shunt placement during the neonatal period.

Methods
The authors retrospectively reviewed the prenatal MR imaging data of 38 consecutive patients who had been referred for neurosurgical consultation following the diagnosis of ventriculomegaly. The outcome measure was placement of a ventricular shunt. Assessed parameters included prenatal atrial diameter (AD), gestational age at MR imaging, time between imaging studies, presence of concomitant CNS anomalies, laterality of ventriculomegaly, fetal sex, and temporal evolution of ventriculomegaly. Logistic regression analysis was completed with the calculation of appropriate ORs and 95% CIs.

Results
Six patients (16%) required shunt placement, all with an AD ≥ 20 mm (mean 23.8 mm) at the time of imaging. Eight patients had presented with an AD ≥ 20 mm. Atrial diameter was the only presenting feature that correlated with shunt placement (OR 1.58, 95% CI 1.10–2.25, p = 0.01). Logistic regression analysis revealed no statistical correlation between the need for ventricular shunting and gestational age at MR imaging, time between imaging studies, fetal sex, presence of additional CNS anomalies, and laterality of the ventriculomegaly.

Conclusions
When assessed using MR imaging, an AD ≥ 20 mm at any gestational age is highly associated with the need for postnatal shunting. Patients with concomitant CNS anomalies did not require shunts at a greater rate than those with isolated ventriculomegaly. Further studies are required to assess the long-term outcome of this patient population.
Diagnosis and management of deformational plagiocephaly
Authors: Robinson Shenandoah , Proctor Mark 
Published: September 2009

Object
The increase in the prevalence of nonsynostotic occipital deformational plagiocephaly in infants, which resulted from the American Academy of Pediatrics' 1992 recommendation to have healthy infants sleep supine, has been accompanied by significant controversy in diagnosis and management. The controversy was exacerbated by the 1998 FDA classification of cranial orthotic devices as Class II devices requiring premarket notification, and the subsequent increase in treatment-associated costs.

Methods
Two independent reviews of the literature were conducted to clarify the objective evidence available within the context of pediatric craniofacial knowledge.

Results
. Although deformational plagiocephaly is not a life-threatening problem, it is a source of disfigurement for children that may be detrimental to their well-being. Current methods for quantifying the degree of disfigurement have limited interrater reliability, and no prospective randomized controlled trials comparing the efficacy of cranial orthoses to repositioning and physical therapy protocols have been published. Despite this lack of Class I evidence, cranial orthoses are routinely and effectively used to treat persistent severe deformational plagiocephaly. The need for the current FDA regulations has not been supported by clinical experience and reported complications.

Conclusions
This review resulted in the following recommendations: 1) more parental education is needed to minimize the development and progression of deformational plagiocephaly; 2) mild deformity can be treated with repositioning and physical therapy protocols; and 3) severe deformity is likely to be corrected more quickly and effectively with cranial orthosis (when used during the appropriate period of infancy) than with repositioning and physical therapy. The available data do not support the need for FDA classification for cranial orthoses as Class II devices requiring premarket notification. Removal of the regulations, which centralized production of the orthoses to larger companies and markedly increased charges, will probably eliminate much of the controversy and parental anxiety generated by marketing strategies.
Minimal residual calcification and recurrence after gross-total resection of craniopharyngioma in children
Authors: Elliott Robert E. , Moshel Yaron A. , Wisoff Jeffrey H. 
Published: September 2009

Object
The purpose of this study was to assess the impact of minimal residual calcification without enhancing tumor on the rate of recurrence after gross-total resection (GTR) of craniopharyngioma in children.

Methods
Data were retrospectively collected in 86 patients younger than 21 years of age in whom 103 craniopharyngioma resections were performed by the senior author between 1986 and 2008. Forty-nine patients (27 boys and 22 girls, with a mean age of 8.6 years) fulfilled the criteria for inclusion in this study by having tumor calcification on the preoperative CT scan, undergoing GTR, and having complete postoperative CT and MR imaging and clinical follow-up.

Results
Thirteen patients (27%) had residual calcification (≤ 2 mm in 12 patients; 3.5 mm in 1 patient) on their postoperative CT scan. At a mean follow-up of 9.4 years (median 10 years), 2 (15%) of 13 patients with and 10 (28%) of 36 patients without residual calcification experienced tumor recurrence. There were no significant differences between these groups in terms of the duration of follow-up, time to recurrence, rate of recurrence, or recurrence-free survival.

Conclusions
The absence or presence of minimal residual calcification does not have an impact on the risk of recurrence after GTR in pediatric craniopharyngiomas. The authors recommend withholding irradiation or other adjuvant therapy in the setting of minimal residual calcification without enhancing tumor. Close follow-up with frequent serial imaging in all patients after GTR is imperative to identify and treat early recurrence.
Successful surgical treatment of craniopharyngioma in very young children
Authors: Elliott Robert E. , Wisoff Jeffrey H. 
Published: September 2009

Object
Given the potential morbidity of cranial irradiation in young children, the risk-benefit analysis of limited surgery plus irradiation versus radical resection may favor the latter strategy. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes of patients 5 years of age and younger who underwent radical resection of craniopharyngiomas.

Methods
Between 1991 and 2008, 19 children age ≤ 5 years were diagnosed with a craniopharyngioma and underwent radical resection by the senior author (J.H.W.). Data were retrospectively collected on these 19 patients (11 males, 8 females; mean age 3.2 years) to assess the efficacy and impact of surgical treatment.

Results
Eighteen (95%) of 19 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. There was no operative death and 18 of (95%) 19 patients were alive at a mean follow-up of 9.4 years (median 8.3 years). Six patients (31%) had a total of 7 tumor recurrences treated by repeat GTR in 5 patients and Gamma knife surgery in 1 patient. No patient required conventional, fractionated radiation therapy. Disease control was achieved surgically in 17 (89.5%) patients and with surgery and Gamma knife surgery in 1 patient, yielding an overall rate of disease control of 95% without the use of conventional radiotherapy. New-onset diabetes insipidus occurred in 50% of patients. Vision worsened in 1 patient, and there was no long-term neurological morbidity.

Conclusions
In this retrospective series, children aged ≤ 5 years with craniopharyngiomas can have excellent outcomes with minimal morbidity after radical resection by an experienced surgeon. Disease control in this population can be successfully achieved with GTR alone in the majority of cases, avoiding the detrimental effects of radiotherapy in this vulnerable population.
Preoperative Onyx embolization of a giant cavernous malformation involving the dural sinuses
Authors: Dashti Shervin R. , Fiorella David , Spetzler Robert F. , Beres Elisa , McDougall Cameron G. , Albuquerque Felipe C. 
Published: September 2009

Cavernous malformations (CMs) or hemangiomas arising from within the dural sinuses are rare entities that differ from their parenchymal counterparts in that they are highly vascular lesions. While parenchymal CMs are typically angiographically occult, intrasinus malformations may have large, dural-based arterial feeding vessels that are amenable to preoperative embolization. The novel liquid embolic Onyx (ev3, Inc.) is an ideal agent for the embolization of these lesions. The authors present the first known case of a giant intrasinus CM embolized with Onyx before gross-total resection.
The authors report the case of a 9-year-old boy with brief apneic episodes in whom MR imaging revealed a giant CM arising from within the right transverse and sigmoid sinuses and infiltrating the right tentorium cerebelli. At another institution, the patient had undergone 1 prior embolization and 2 unsuccessful attempts at resection. Both surgeries had been complicated by massive blood loss and were aborted.
Under the authors' care, the patient underwent preoperative transarterial embolization with Onyx during which a substantial volume of the mass lesion was filled with embolisate. Subsequently, complete circumferential excision of the mass from the tentorium was accomplished with minimal intraoperative blood loss.