bluuebunny/crossref_metadata_2025 huggingface dataset:
Bilateral extradural hematoma formation following excision of a thoracic intradural lesion
Authors: Surash Surash , Bhargava Deepti , Tyagi Atul
Published: September 2009

The authors report an unusual case of a 15-year-old girl who previously underwent resection of a posterior fossa medulloblastoma, and later underwent resection of a thoracic extramedullary metastatic lesion. Her consciousness deteriorated hours after removal of her spinal metastasis. A CT head scan identified bilateral extradural cranial hematomas, which were evacuated. To the authors' knowledge, this is the first report of a patient developing bilateral acute cranial extradural hematomas following excision of a thoracic intradural lesion.
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.
Combination of preoperative embolization and surgery in the treatment of a giant congenital neuroid nevus of the forehead and scalp in a child
Authors: Margulis Alexander , Koplewitz Benjamin Z. , Maly Alexander , Cohen José , Yanko-Arzi Ravit , Gomori John M. , Neuman Rami
Published: September 2009

Congenital melanocytic nevi of neuroectodermal origin are composed primarily of melanocytes and occasional neural elements. A subset of large/giant congenital melanocytic nevi is characterized by neuroidal differentiation resembling the histological pattern of neurofibroma (neuroid congenital melanocytic nevi). The authors report the case of a male infant born with a neuroid congenital melanocytic nevus presenting as a large mass covering extensive portions of his scalp and forehead. The atypical feeding arteries to the nevus were larger in diameter then the patient's internal carotid arteries and formed a rich network of large blood vessels in the nevus. Selective preoperative embolization using calibrated particles was followed by a complete excision and staged reconstruction with tissue expanders. The authors believe that this multidisciplinary approach allowed for a safe surgical procedure with minimal blood loss for this most unusual pediatric head and neck tumor.
Hydrocephalus and its treatment according to Rhazes
Authors: Aciduman Ahmet , Belen Deniz
Published: September 2009

The renowned medieval Persian physician Rhazes was an early proponent of experimental medicine. Rhazes made fundamental and enduring contributions to medicine and to other scientific fields. He wrote over 200 scientific books, more than half of which concerned medicine. He was well versed in Persian, Greek, and Indian medical knowledge, and made numerous contributions to the medical field through his own observations and discoveries. He was also a pioneer in the field of neurosurgery and, as he was predominantly a pediatrician, he dealt with the subject of hydrocephalus. A large part of his medical tome, al-Hawi, deals with head-related disorders including the hydrocephalus. Although he did not introduce novel concepts of hydrocephalus and its management, by combining the different approaches of experienced scholars he endeavored to improve treatment and knowledge of this problematic disease.
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.
Cerebrovascular moyamoya disease in a 5-year-old girl who underwent interruption of the right common carotid artery in the neonatal period
Authors: Maeda Yoshihiko , Nomura Sadahiro , Kurokawa Tetsu , Ishihara Hideyuki , Harada Kei , Yoneda Hiroshi , Kato Shoichi , Fujisawa Hirosuke , Suzuki Michiyasu
Published: September 2009

The authors report on the case of a girl with cerebrovascular moyamoya disease born with severe respiratory failure caused by a congenital diaphragmatic hernia. Cardiopulmonary management included extracorporeal membrane oxygenation until the diaphragm defect was repaired. The right common carotid artery (CA) was interrupted and cannulated for extracorporeal membrane oxygenation. When she was 5 years of age, the patient experienced ischemic symptoms in her right extremities. Angiography revealed stenosis of the terminal portion of the internal CA (ICA) with the development of moyamoya vessels on the left side of the brain; the right ICA was supplied by extracranial anastomotic arteries. Indirect extracranial–intracranial bypass surgery was performed in the left hemisphere, and the hypoperfusion improved. The same change in the intracranial ICA with the development of moyamoya vessels occurred on her right side when she was 7 years old. Decreased cerebral blood flow occurred twice, and the moyamoya vessels developed to compensate for the cerebral ischemia. However, the occlusion of the extracranial common CA in infancy induced extracranial anastomosis rather than moyamoya vessel proliferation, and collateral circulation was formed at the lesion site. This finding indicates that neoangiogenesis requires both cerebral ischemia and growth factors derived from the lesion.
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.
Intratentorial osteochondrolipoma in a 9-year-old boy
Authors: Ahmadi Sebastian A. , van Landeghem Frank K. H. , Blechschmidt Cristiane , Lieber Kerstin , Haberl Ernst-Johannes , Thomale Ulrich-Wilhelm
Published: September 2009

Intracranial osteolipomas and chondromas are rare benign tumors. Forty-five chondromas, mostly supratentorial, have been reported in the literature since 1981, with origins most commonly in the sellar regions. Twenty-one osteolipomas have been described to date, usually located near the tuber cinereum or the corpus callosum. The authors present a case of an osteochondrolipoma arising from the tentorium diagnosed in a pediatric patient at the age of 9 years. The case and treatment are discussed, and a review of the literature is provided.
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.
Isolated amygdala neurocysticercosis in a patient presenting with déjà vu and olfactory auras
Authors: Lee Darrin J. , Owen Christopher M. , Khanifar Elham , Kim Ronald C. , Binder Devin K.
Published: September 2009

Neurocysticercosis is the most common parasitic infection in the CNS and a leading cause of epilepsy. Since it is a circumscribed lesional cause of epilepsy, specific locations of neurocysticercal lesions may lead to specific clinical presentations. The authors describe a 17-year-old Hispanic boy who had a single enhancing bilobar mass in the right amygdala. Initially, the patient presented with secondarily generalized tonic-clonic seizures, which resolved with antiepilepsy drug therapy. On further investigation, he was found to have persistent olfactory and déjà vu auras. A right amygdalectomy without hippocampectomy was performed, and both the seizures and auras immediately resolved. Pathological analysis revealed neurocysticercosis. To the authors' knowledge, this case is the first reported instance of 2 distinct mesial temporal aura semiologies associated with localized neurocysticercosis in the amygdala and successfully treated with resection. Uniquely, the case demonstrates that both olfactory and déjà vu auras can emanate from the amygdala.
High expression of stathmin protein predicts a fulminant course in medulloblastoma
Authors: Kuo Meng-Fai , Wang Huei-Shyong , Kuo Quang-Ting , Shun Chia-Tung , Hsu Hey-Chi , Yang Shih-Hong , Yuan Ray-Hwang
Published: September 2009

Object

Stathmin, an important cytosolic phosphoprotein, is involved in cell proliferation and motility. This study was performed to elucidate the role of stathmin in the progression of medulloblastoma.

Methods

The expression of stathmin protein was examined by immunohistochemical staining of tumor sections obtained in 17 consecutive patients with medulloblastoma who underwent resection between 1995 and 2005. Four patients were excluded because they were either lost to follow-up or underwent biopsy sampling only, leaving a total of 13 patients in the study. The stathmin expression was scored according to the immunoreactive fraction of tumor cells, and the level was correlated with various clinicopathological factors.

Results

The expression level of stathmin protein was ≤ 10% in 9 patients, 11–50% in 1, and > 50% in 3. No staining was seen in the tissues adjacent to the tumors. For comparison, the authors grouped the expression level of stathmin into high (> 50%) and low (≤ 50%). It was found that patients with high expression of stathmin had more frequent tumor dissemination at the time of resection or soon after total excision of the tumor (p = 0.0035), and hence experienced a fulminant course with lower patient survival (p < 0.0001), with an average survival period of 6.7 months (range 2–10 months). The expression level of stathmin did not correlate with patient age, sex, CSF cytological findings, use of adjuvant therapies, Ki 67 index, or risk classification of the tumors according to previously described categories in the literature.

Conclusions

High stathmin expression correlates with tumor dissemination, is an important prognostic factor of medulloblastoma, and may serve as a useful marker for more intensive adjuvant therapies.
Cerebrospinal fluid shunt operations without cranial bandaging
Authors: Winston Ken R. , Trinidad Elizabeth , Wilkinson C. Corbett , McBride Lori A.
Published: September 2009

Object

Cranial bandages are commonly applied over scalp incisions immediately after cerebrospinal fluid (CSF) shunt surgery, putatively to prevent complications, particularly infection. These bandages require resources, consume the time of healthcare workers, and incur non-negligible expenses. It is therefore both reasonable and important to examine the efficacy of cranial bandaging.

Methods

The combined experience of 3 neurosurgeons over 6.75 years with using no cranial bandaging after operations for implantation or revision of CSF shunts is the basis of this report. These data were prospectively accrued and retrospectively analyzed.

Results

The infection rate was 4.2% (95% CI 3.1–5.6%) for 1064 operations performed without postoperative cranial bandaging after either shunt insertion or revision surgery through clean or clean-contaminated wounds. The age distribution extended from premature infants through adults 77 years of age.

Conclusions

The results of this investigation support the position that bandaging scalp wounds after CSF shunt implantation or revision surgery adds no benefit beyond the easier, simpler, faster, and cheaper practice of using antibiotic ointment as a dressing without bandaging.
Successful ventricle to direct heart shunt placement as a salvage cerebrospinal fluid diversion technique
Authors: Thompson Eric M. , Giles Stephen G. , Song Howard K. , Litvack Zachary N. , Golshani Kiarash J. , Nemecek Andrew N.
Published: September 2009

The authors report a complex case in a 35-year-old woman who underwent shunt placement at birth for myelomeningocele. She had previously undergone more than 30 shunt revisions, with placement of the distal catheter in the peritoneum multiple times, and also in the pleura, the gall bladder, and the upper venous system. All shunts had failed and the possible placement sites were now anatomically hostile. A median sternotomy was performed as the next option. The catheter was placed directly into the appendage of the right atrium and secured with a pursestring suture. One month postoperatively, the patient presented with a large pericardial effusion after the distal catheter migrated out of the atrium and into the pericardial space. A repeat sternotomy was performed to drain the pericardial CSF collection. The catheter was reinserted into the atrial appendage, and a tunnel was created in the atrial wall to fix the device more securely. At 1 year postoperatively, the patient had no further symptoms of shunt obstruction or cardiac tamponade, and imaging studies suggested that the shunt system was functional. The authors report the first successful ventricle to direct heart shunt in an adult.
Risk factors associated with occipital encephalocele: a case-control study
Authors: Dadmehr Majid , Nejat Farideh , Khashab Mostafa El , Ansari Saeed , Baradaran Nima , Ertiaei Abolhasan , Bateni Farzad
Published: September 2009

Object

An encephalocele is characterized by congenital herniation of the brain tissue and/or meninges through a skull defect. The underlying cause is complex and not fully understood, but environmental agents are suspected. The authors aimed to determine the known risk factors for encephaloceles.

Methods

Potential risk factors were studied in 31 children with encephaloceles who had been referred to the outpatient clinic. At the same time, 31 children with non-CNS anomalies were randomly selected from the same hospital as a control group. Both cohorts were assessed in person through interviews with the mothers.

Results

Most children with encephaloceles were female. There was no significant risk factor in the case group as compared with controls, although the families of patients with encephaloceles had a better economic status (p = 0.03) and the fathers had a higher mean age.

Conclusions

Although the authors of this study could not identify any significant risk factors for encephaloceles, environmental factors can still be mentioned as probable etiological elements. Additional studies with larger sample sizes and more comprehensive evaluations are required to confirm the role of environmental or genetic factors to prevent the occurrence of encephaloceles.
Transient selective downward gaze paralysis complicating posterior fossa tumor resection in children
Authors: Chern Joshua J. , Relyea Katherine , Edmond Jane C. , Whitehead William E. , Curry Daniel J. , Luerssen Thomas G. , Jea Andrew
Published: September 2009

Selective downward gaze paralysis has not previously been described as a complication after posterior fossa operations in children. The authors found downgaze palsy to be a transient complication after resection of large pediatric posterior fossa midline tumors reaching the aqueduct of Sylvius. They reviewed the cases of 2 children with large posterior fossa midline tumors who underwent resection via an inferior transventricular approach. They developed a hypothetical scheme to account for downward gaze paralysis based on anatomy and insight gained from experimental studies.

The authors describe potential risk factors for developing transient selective downward gaze paralysis with the hope of making more pediatric neurosurgeons aware of this complication following removal of lesions around the mesencephalic periaqueductal gray matter. Recognition and understanding of downward gaze palsy after posterior fossa surgery should improve preoperative counseling and promote postoperative family coping.
Shunt complications in children with myelomeningocele: effect of timing of shunt placement
Authors: Radmanesh Farid , Nejat Farideh , Khashab Mostafa El , Ghodsi Syed Mohammad , Ardebili Hasan Eftekhar
Published: September 2009

Object

There has been controversy over whether CSF shunt insertion simultaneously with repair of myelomeningocele (MMC) might increase shunt-related complications. The purpose of this study was to evaluate shunt complication rates in patients who underwent concurrent MMC surgery and shunt placement and compare them to the rates in patients treated with shunt placement in a separate procedure.

Methods

The authors retrospectively reviewed the outcome of shunt placement in 127 patients with MMC who were followed up for ≥ 1 year after shunt surgery. In 65 patients shunt surgery was performed in a second operation after MMC repair and in 46 the 2 procedures were performed concurrently. In 7 patients shunt placement was the initial surgery, and in 9 it was the only procedure performed. The patients were evaluated for shunt complications.

Results

There was no statistically significant between-groups difference in age at which patients underwent shunt placement. The overall rates of shunt infection and shunt malfunction were 16.5 and 39.4%, respectively. There was a high rate of shunt infection and mortality in those patients treated with CSF shunting only. There was no statistically significant difference between complication rates in patients in whom the 2 procedures were performed concurrently and those who underwent separate operations.

Conclusions

The order in which myelomeningocele repair and shunt placement were carried out did not have a significant effect on the rate of shunt complications. Thus, when indicated these procedures can be performed concurrently with a level of risk comparable to that associated with delayed shunt placement.
Primary pediatric intraspinal sarcomas
Authors: Klimo Paul , Codd Patrick J. , Grier Holcombe , Goumnerova Liliana C.
Published: September 2009

Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery.

Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children—2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve—and discuss their management and outcome with a review of the current literature.
Neurosurgical management of intractable rolandic epilepsy in children: role of resection in eloquent cortex
Authors: Benifla Mony , Sala Francesco , Jane John , Otsubo Hiroshi , Ochi Ayako , Drake James , Weiss Shelly , Donner Elizabeth , Fujimoto Ayataka , Holowka Stephanie , Widjaja Elysa , Snead O. Carter , Smith Mary Lou , Tamber Mandeep S. , Rutka James T.
Published: September 2009

Object

The authors undertook this study to review their experience with cortical resections in the rolandic region in children with intractable epilepsy.

Methods

The authors retrospectively reviewed the medical records obtained in 22 children with intractable epilepsy arising from the rolandic region. All patients underwent preoperative electroencephalography (EEG), MR imaging, prolonged video-EEG recordings, functional MR imaging, magnetoencephalography, and in some instances PET/SPECT studies. In 21 patients invasive subdural grid and depth electrode monitoring was performed. Resection of the epileptogenic zones in the rolandic region was undertaken in all cases. Seizure outcome was graded according to the Engel classification. Functional outcome was determined using validated outcome scores.

Results

There were 10 girls and 12 boys, whose mean age at seizure onset was 3.2 years. The mean age at surgery was 10 years. Seizure duration prior to surgery was a mean of 7.4 years. Nine patients had preoperative hemiparesis. Neuropsychological testing revealed impairment in some domains in 19 patients in whom evaluation was possible. Magnetic resonance imaging abnormalities were identified in 19 patients. Magnetoencephalography was performed in all patients and showed perirolandic spike clusters on the affected side in 20 patients. The mean duration of invasive monitoring was 4.2 days. The mean number of seizures during the period of invasive monitoring was 17. All patients underwent resection that involved primary motor and/or sensory cortex. The most common pathological entity encountered was cortical dysplasia, in 13 children. Immediately postoperatively, 20 patients had differing degrees of hemiparesis, from mild to severe. The hemiparesis improved in all affected patients by 3–6 months postoperatively. With a mean follow-up of 4.1 years (minimum 2 years), seizure outcome in 14 children (64%) was Engel Class I and seizure outcome in 4 (18%) was Engel Class II. In this series, seizure outcome following perirolandic resection was intimately related to the child's age at the time of surgery. By univariate logistic regression analysis, age at surgery was a statistically significant factor predicting seizure outcome (p < 0.024).

Conclusions

Resection of rolandic cortex for intractable epilepsy is possible with expected morbidity. Accurate mapping of regions of functional cortex and epileptogenic zones may lead to improved seizure outcome in children with intractable rolandic epilepsy. It is important to counsel patients and families preoperatively to prepare them for possible worsened functional outcome involving motor, sensory and/or language pathways.
Surgical treatment of ectopic recurrence of craniopharyngioma
Authors: Elliott Robert E. , Moshel Yaron A. , Wisoff Jeffrey H.
Published: September 2009

Local recurrence following radical resection is one of the most common complications of pediatric craniopharyngioma. Only 28 cases of ectopic recurrence of craniopharyngioma have been reported in the literature, and only 13 cases occurred in patients originally treated as children. In this consecutive series of 86 children who underwent radical resection of primary and recurrent craniopharyngiomas, 4 patients (4.7%) experienced ectopic tumor recurrence, accounting for 27% of all recurrences after gross-total resection. The authors report on the successful surgical treatment of these 4 patients and the impact of ectopic craniopharyngioma recurrence on survival.
How long should cerebrospinal fluid cultures be held to detect shunt infections?
Authors: Desai Atman , Lollis Stuart Scott , Missios Symeon , Radwan Tarek , Zuaro Deborah E. , Schwarzman Joseph D. , Duhaime Ann-Christine
Published: September 2009

Object

Infections of CSF hardware may be indolent, and some patients have received antibiotic treatment for various reasons before CSF is obtained to check for a shunt infection. At present, there are few data in the literature to guide the decision as to how long to hold CSF specimens when attempting to diagnose hardware infections, and institutions vary in the duration at which cultures are considered “final.”

Methods

The authors reviewed the microbiology data from CSF specimens obtained from shunts, ventriculostomies, reservoirs, and lumbar drains at their institution over a 36-month period to discover how long after collection cultures became positive. The authors also sought to discover whether this time was affected by prior treatment with antibiotics.

Results

Of 158 positive CSF specimens obtained from hardware, the time to recovery ranged between 1–10 days, with a mean of 3.02 days (SD 2.37 days, 95% CI 2.66–3.38 days). One hundred and twenty-seven positive specimens were associated with clinical infections, and ~ 25% of these grew organisms after > 3 days, with some as long as 10 days after specimens were obtained. The most common organisms grown from individual patients were coagulase-negative Staphylococcus spp (34 cultures), Propionibacterium spp (21), Bacillus spp (6), Pseudomonas aeruginosa (4), and Staphylococcus aureus (4 cultures). Mean and maximum days to recovery were different across species, with S. aureus showing the shortest and Propionibacterium spp showing the longest incubation times. There appeared to be no significant difference in the time to recovery between specimens obtained in patients who had received prior antibiotic treatment versus those who had not.

Conclusions

A substantial number of positive CSF specimens obtained in patients with clinical infections grew bacteria after > 3 days, with some requiring as long as 10 days. Thus, a routine 10-day observation period for CSF specimens can be justified.
Hypothalamic lipoma associated with severe obesity
Authors: Puget Stéphanie , Garnett Matthew R. , Leclercq Delphine , Pinto-Primard Graziella , Samara-Boustani Dinane , Sainte-Rose Christian , Genevieve David , Polak Michel , De Lonlay Pascale , Brunelle Francis , Benabid Alim Louis , Boddaert Nathalie
Published: September 2009

The association between hypothalamic dysfunction and obesity is well documented in both clinical and experimental models. The authors describe 2 children who developed obesity that could not be explained by endocrinological, genetic, or eating disorders. In both cases, cranial MR imaging revealed the typical appearance of a lipoma in the paramedian hypothalamus. In the absence of other etiologies, the authors hypothesized that in these 2 children obesity was caused by their hypothalamic lipomas. To the authors' knowledge, these are the first cases of hypothalamic lipomas likely to be causing obesity that have been described in children. These cases highlight the importance of performing cranial MR imaging in children with otherwise unexplained obesity.