Neurosurgery Blog


Daily bibliographic review of the Neurosurgery Department. La Fe University Hospital. Valencia, Spain

Neurosurgical treatment of gangliogliomas in children and adolescents

Acta Neurochirurgica (2018) 160:1207–1214

The object of this study was to delineate long-term results of the surgical treatment of pediatric tumors classified as ganglioglioma or gangliocytoma.

Methods A cohort of consecutive patients 19 years or younger who had undergone primary resection of CNS tumors during the years 1980–2016 at a single institution were reviewed in this retrospective study of surgical morbidity, mortality, and academic achievement and/or work participation. Grossmotor function and activities of daily living were scored using the Barthel Index (BI).

Results Patient records for 32 consecutive children and adolescents who had undergone resection for a ganglioglioma were included in this study. Of the 32 patients, 13 were in the first decade at the first surgery, whereas 19 were in the second decade. The male/female ratio was 1.0 (16/16). No patient was lost to follow-up. The tumor was localized to the supratentorial compartment in 26 patients, to the posterior fossa in 5 patients, and to the spinal cord in 1 patient. Only two of the tumors were classified as anaplastic. Of the 30 low-grade tumors, 2 were classified as gangliocytomas, 6 were desmoplastic infantile gangliogliomas, and 22 were ordinary gangliogliomas. The aim of primary surgery was gross-total resection (GTR) and was achieved in 23 patients (71.9%). Altogether, 43 tumor resections were performed. Eight patients underwent a second resection from 1 to 10 years after primary surgery and three of these also had a third resection from 2 to 24 years after initial surgery. The reason for further resection was clinical (seizure control failure/recurrence of epilepsy or progressive neurological deficit) and/or residual tumor progression/recurrence. There was no operative mortality in this series and all 32 patients are alive with follow-up periods from 0.5 to 36 years (median 14 years). Observed 14-year survival is thus 100%. One out of two children with primary anaplastic tumor received local radiotherapy (proton) postoperatively. The other 31 patients did not have any kind of non-surgical adjuvant therapy. Twenty-one out of 26 children with supratentorial tumor had epilepsy as one of their presenting symptoms. Nineteen of these became seizure-free after initial surgery (18 of them after GTR), but 3 patients experienced recurrence of seizures within some years. Functional outcome in terms ofADL, schooling, and work participation was gratifying in most patients. Five patients have persistent hydrocephalus (HC), treated with ventriculoperitoneal (VP) shunts.

Conclusion Low-grade gangliogliomas (GGs) can be surgically treated with good long-term results including seizure and tumor control as well as school and working participation.


Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins’ Law

Neurosurgery 82:652–660, 2018

Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined.

OBJECTIVE: To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins’ law for embryonal tumors.

METHODS: Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263).

RESULTS: Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival.

CONCLUSION: Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins’ law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

Outcomes After Endoscopic Endonasal Resection of Craniopharyngiomas in the Pediatric Population

World Neurosurg. (2017) 108:6-14.

Craniopharyngiomas have traditionally been treated via open transcranial approaches. More recently, endoscopic transsphenoidal approaches have been increasingly used; however, few case series exist in the pediatric population.

METHODS: A retrospective review of patients (aged <18 years) undergoing endoscopic transsphenoidal resection of craniopharyngiomas between 1995 and 2016 was performed. Preoperative data included presenting symptoms, tumor size, location, and components. Postoperative outcomes included symptom resolution, visual outcomes, endocrine outcomes, disease recurrence, and major complications.

RESULTS: Sixteen pediatric patients with mean age of 11.0 years (range, 5-15 years) were included. The median follow-up time was 56.2 months. Mean maximal tumor diameter was 3.98 cm. Most of the tumors had suprasellar (93.8%) and intrasellar (68.8%) components. The gross total resection rate was 93.8%. The most common presenting symptoms were vision changes (81.3%) and increased intracranial pressure (56.3%). Most patients (66.7%) had their presenting symptoms resolved by their first postoperative visit. Vision improved or remained normal in 69.2% of patients. Postoperatively, new incidence of panhypopituitarism or diabetes insipidus developed in 63.6% and 46.7% of patients, respectively. New hypothalamic obesity developed in 28.6% of patients. The postoperative cerebrospinal fluid leak rate was 18.8%. One patient died of intraventricular hemorrhage postoperatively. The major complication rate was 12.5%. Disease recurrence occurred in 1 patient with gross total resection (6.3%).

CONCLUSIONS: Endoscopic transsphenoidal resection for craniopharyngiomas can achieve high rates of total resection with low rates of disease recurrence in larger tumors than previously described. However, hypothalamic-pituitary dysfunction and cerebrospinal fluid leak remain significant postoperative morbidities

Guideline on the Role of Cranial Molding Orthosis (Helmet) Therapy for Patients With Positional Plagiocephaly


Neurosurgery 79:E632–E633, 2016

No evidence-based guidelines exist on the role of cranial-molding orthosis (helmet) therapy for patients with positional plagiocephaly.

OBJECTIVE: To address the clinical question: “Does helmet therapy provide effective treatment for positional plagiocephaly?” and to make treatment recommendations based on the available evidence.

METHODS: The US National Library of Medicine Medline database and the Cochrane Library were queried by using MeSH headings and key words relevant to the objective of this systematic review. Abstracts were reviewed, after which studies meeting the inclusion criteria were selected and graded according to their quality of evidence (Classes I-III). Evidentiary tables were constructed that summarized pertinent study results, and, based on the quality of the literature, recommendations were made (Levels I-III).

RESULTS: Fifteen articles met criteria for inclusion into the evidence tables. There was 1 prospective randomized controlled trial (Class II), 5 prospective comparative studies (Class II), and 9 retrospective comparative studies (Class II).

CONCLUSION: There is a fairly substantive body of nonrandomized evidence that demonstrates more significant and faster improvement of cranial shape in infants with positional plagiocephaly treated with a helmet in comparison with conservative therapy, especially if the deformity is severe, provided that helmet therapy is applied during the appropriate period of infancy. Specific criteria regarding the measurement and quantification of deformity and the most appropriate time window in infancy for treatment of positional plagiocephaly with a helmet remains elusive. In general, infants with a more severe presenting deformity and infants who are helmeted early in infancy tend to have more significant correction (and even normalization) of head shape.

Outcomes of Multimodality Therapy in Pediatric Patients With Ruptured and Unruptured Brain Arteriovenous Malformations

Outcomes of Multimodality Therapy in Pediatric Patients With Ruptured and Unruptured Brain Arteriovenous Malformations

Neurosurgery 78:695–707, 2016

Brain arteriovenous malformations (BAVMs) are a frequent cause of pediatric hemorrhagic stroke, which frequently results in significant morbidity and mortality.

OBJECTIVE: To analyze the results of multimodality treatment for a consecutive series of pediatric patients with ruptured and unruptured BAVMs at a single institution.

METHODS: Forty patients ,18 years of age were retrospectively reviewed. Results were divided by hemorrhage status, ie, ruptured or unruptured, and the intended curative treatment modality, ie, surgical resection or stereotactic radiosurgery.

RESULTS: Twenty-seven patients (68%) presented with hemorrhage, and 13 patients (32%) presented without hemorrhage. Among ruptured patients, 19 (70%) underwent surgery and 8 (30%) underwent stereotactic radiosurgery. In surviving patients who presented with hemorrhage, 23 of 26 (88%) had a modified Rankin Scale (mRS) score of 0 to 2 at the last follow-up, and 24 of 26 (92%) obtained radiographic cure. For unruptured BAVMs, all 6 patients with grade I to III BAVM obtained radiographic cure and had an mRS score of 0 to 1 at the last follow-up, whereas 1 of 5 patients (20%) with grade IV and V BAVM had BAVM obliteration and a mean mRS score of 1.8 at the last follow-up. In a total of 93.6 years of follow-up from date of presentation to last clinical follow-up, there was 1 hemorrhage (1.1%/y). Of 30 patients with radiographic obliteration, 2 patients had radiographic recurrence (7% incidence).

CONCLUSION: The majority of ruptured patients had an mRS score of 0 to 2 at the last follow-up and obtained radiographic cure. Unruptured patients with grade I to III BAVMs had superior outcomes compared with those with grade IV and V AVMs. Treatment of grade I to III BAVMs appears safe, and additional study is needed to determine optimal strategies for the management of unruptured grade IV and V BAVMs.

Lumbar Disk Herniation in Children and Adolescents

Lumbar Disk Herniation in Children and Adolescents

Neurosurgery 77:954–959, 2015

Lumbar disk herniation in adults is thought to be caused by repetitive overloading and age-rated degenerative changes. However, these causes are absent in children and adolescent patients. We assume that structural malformations in the lumbar spine could predispose intervertebral disks to early degeneration and hence need to be surgically fused. This issue has never been raised before.

OBJECTIVE: To investigate the assumption that structural malformations in the lumbar spine could predispose intervertebral disks to early degeneration and hence need to be surgically fused.

METHODS: Lumbar spine configurations, including the height of the intercrestal line, the length of L5 transverse processes, and the presence of transitional vertebrae, were recorded from anteroposterior radiographs taken from 63 consecutive pediatric patients with lumbar disk herniation admitted to our hospital over a period of 8 years. Each configuration was compared in relation to the level of disk herniation. Diskectomy alone was performed in 36 cases; arthrodesis was added in the remaining 27 cases. Patients’ back and leg pain visual analog scale scores and frequency and their Oswestry Disability Index scores were recorded before surgery and at follow-up. The results were compared for assessment of outcome.

RESULTS: Patients with high intercrestal lines and long L5 transverse processes had a significantly higher incidence of L4/5 disk herniation, whereas low intercrestal line and lumbarization were associated with L5/S1 disk herniation. Patients’ visual analog scale scores, pain frequency, and Oswestry Disability Index score all improved significantly after surgery, but there was no significant difference with or without arthrodesis.

CONCLUSION: Pediatric lumbar disk herniation is significantly associated with structural malformations of the lumbar spine, but arthrodesis does not improve the clinical outcome.

Strategies for Computed Tomography Radiation Dose Reduction in Pediatric Neuroimaging

Strategies for Computed Tomography Radiation Dose Reduction in Pediatric Neuroimaging

Neurosurgery 77:228–232, 2015

Radiation exposure from diagnostic imaging is a significant concern, particularly in the care of pediatric patients. Computed tomography (CT) scanning is a significant source of radiation.

OBJECTIVE: To demonstrate that diagnostic quality CT images can be obtained while minimizing the effective radiation dose to the patient.

METHODS: In this retrospective cross-sectional study, noncontrast head CT scan data were reviewed, and indications for scans and estimated radiation dose delivered were recorded. The estimated effective radiation dose (EERD) for each CT protocol was reviewed.

RESULTS: We identified 251 head CT scans in a single month. Of these, 96 scans were using a low-dose shunt protocol with a mean EERD of 0.82 mSv. The remaining 155 scans were performed using the standard protocol, and the mean EERD was 1.65 mSv. Overall, the EERD was minimized while maintaining diagnostic scan quality.

CONCLUSION: Although replacing a CT with magnetic resonance imaging is ideal to completely avoid ionizing radiation, this is not always practical or preferred. Therefore, it is important to have CT protocols in place that minimize radiation dose without sacrificing diagnostic quality. The protocols in place at our institution could be replicated at other academic and community hospitals and imaging centers.

The cranial orbital buttress technique for nonsyndromic unicoronal and metopic craniosynostosis

The cranial orbital buttress technique for nonsyndromic unicoronal and metopic craniosynostosis

Neurosurg Focus 38 (5):E4, 2015

Current craniosynostosis procedures can result in complications due to absorbable plates and screws or other specialized expensive hardware. The authors propose the cranial orbital buttress (COB) technique of frontoorbital remodeling for metopic and unicoronal synostoses, wherein no plates or screws are used. They hypothesize that, with this technique, aesthetically acceptable outcomes for unicoronal and metopic synostosis can be achieved. In this article, they present this technique and compare the results with current frontoorbital remodeling practices.

METHODS The authors conducted a retrospective chart review of cases in which patients with nonsyndromic unicoronal or metopic synostosis underwent cranio-orbital surgery at their institution from 1985 through 2009. Operative parameters, surgical variations, and complications were analyzed. The COB technique uses a 1-piece switch, hemiforeheads, or multiple pieces for forehead remodeling. The supraorbital bar is reconstructed in patients with metopic synostosis using a double wedge or greenstick fracture technique, and in patients with unicoronal synostosis a hinge procedure based on a 1.5-orbital osteotomy is used. The supraorbital bar is advanced and supported in place by bone graft(s) inserted at the lateral aspect(s) of the orbit(s) to form a buttress, with fixation done using absorbable sutures.

RESULTS A total of 79 cases met the criteria for inclusion in the study. Twenty-nine patients had metopic synostosis, 3 had combined metopic and sagittal synostoses, and 47 had unicoronal synostosis. The patients’ mean age at surgery was 11.4 ± 10.1 months and the mean operative time was 183.4 ± 41.0 minutes. The mean length of hospital stay was 3.7 ± 1.2 days. The mean blood loss was 150.0 ± 125.6 ml, and 33% of patients required a blood transfusion (mean volume 206.9 ± 102.3 ml). In metopic synostosis, hemiforeheads were used most often (24/29, 83%), and the supraorbital bar was remodeled using a bilateral intracranial orbital osteotomy followed by a double wedge modification (23/29,79%) or a greenstick fracture (4/29 14%) for milder cases. Forehead remodeling for unicoronal synostosis was by a forehead switch (39/47, 83%) and the supraorbital bar was remodeled using a 1.5-orbital intracranial orbital osteotomy (34/47, 72%) such that the bar was advanced on the abnormal side and hinged at the midline of the normal orbit. Perioperative complications occurred in 19% of cases and included dural tears (16%), inconsequential subdural hematoma (1.3%), and nasal greenstick fracture (1.3%). The total reoperation rate was 7.6% (cranioplasties for irregular contours, 6.3%; scar revision, 1.3%).

CONCLUSIONS The COB remodeling technique is simple and efficient, gives acceptable outcomes, and is less resource intensive than previous techniques reported in the literature.

Volumetric CT analysis as a predictor of seizure outcome following temporal lobectomy

Volumetric brain analysis in neurosurgery- Part 3

J Neurosurg Pediatr 15:133–143, 2015

The incidence of temporal lobe epilepsy (TLE) due to mesial temporal sclerosis (MTS) can be high in developing countries. Current diagnosis of MTS relies on structural MRI, which is generally unavailable in developing world settings. Given widespread effects on temporal lobe structure beyond hippocampal atrophy in TLE, the authors propose that CT volumetric analysis can be used in patient selection to help predict outcomes following resection.

METHODS Ten pediatric patients received preoperative CT scans and temporal resections at the CURE Children’s Hospital of Uganda. Engel classification of seizure control was determined 12 months postoperatively. Temporal lobe volumes were measured from CT and from normative MR images using the Cavalieri method. Whole brain and fluid volumes were measured using particle filter segmentation. Linear discrimination analysis (LDA) was used to classify seizure outcome by temporal lobe volumes and normalized brain volume.

RESULTS Epilepsy patients showed normal to small brain volumes and small temporal lobes bilaterally. A multivariate measure of the volume of each temporal lobe separated patients who were seizure free (Engel Class IA) from those with incomplete seizure control (Engel Class IB/IIB) with LDA (p < 0.01). Temporal lobe volumes also separate normal subjects, patients with Engel Class IA outcomes, and patients with Class IB/IIB outcomes (p < 0.01). Additionally, the authors demonstrated that age-normalized whole brain volume, in combination with temporal lobe volumes, may further improve outcome prediction (p < 0.01).

CONCLUSIONS This study shows strong evidence that temporal lobe and brain volume can be predictive of seizure outcome following temporal lobe resection, and that volumetric CT analysis of the temporal lobe may be feasible in lieu of structural MRI when the latter is unavailable. Furthermore, since the authors’ methods are modality independent, these findings suggest that temporal lobe and normative brain volumes may further be useful in the selection of patients for temporal lobe resection when structural MRI is available.

Predicting the “usefulness” of 5-ALA-derived tumor fluorescence for fluorescence-guided resections in pediatric brain tumors

5-ALA in pedidatric brain tumors

Acta Neurochir (2014) 156:2315–2324

Five-aminolevulinic acid (Gliolan, medac, Wedel, Germany, 5-ALA) is approved for fluorescence-guided resections of adult malignant gliomas. Case reports indicate that 5-ALA can be used for children, yet no prospective study has been conducted as of yet. As a basis for a study, we conducted a survey among certified European Gliolan users to collect data on their experiences with children.

Methods Information on patient characteristics, MRI characteristics of tumors, histology, fluorescence qualities, and outcomes were requested. Surgeons were further asked to indicate whether fluorescence was “useful”, i.e., leading to changes in surgical strategy or identification of residual tumor. Recursive partitioning analysis (RPA) was used for defining cohorts with high or low likelihoods for useful fluorescence.

Results Data on 78 patients <18 years of age were submitted by 20 centers. Fluorescence was found useful in 12 of 14 glioblastomas (85 %), four of five anaplastic astrocytomas (60 %), and eight of ten ependymomas grades II and III (80 %). Fluorescence was found inconsistently useful in PNETs (three of seven; 43 %), gangliogliomas (two of five; 40 %), medulloblastomas (two of eight, 25 %) and pilocytic astrocytomas (two of 13; 15 %). RPA of pre-operative factors showed tumors with supratentorial location, strong contrast enhancement and first operation to have a likelihood of useful fluorescence of 64.3 %, as opposed to infratentorial tumors with first surgery (23.1 %).

Conclusions Our survey demonstrates 5-ALA as being used in pediatric brain tumors. 5-ALA may be especially useful for contrast-enhancing supratentorial tumors. These data indicate controlled studies to be necessary and also provide a basis for planning such a study.

Corridor-Based Endonasal Endoscopic Surgery for Pediatric Skull Base Pathology


Operative Neurosurgery 10:273–293, 2014

Pediatric anatomy is more restricted, and the propagation of endonasal endoscopic approaches in the pediatric population has been limited.

OBJECTIVE: To demonstrate the feasibility of the endonasal endoscopic approach in a variety of age groups and to perform measurements of the corridors and spaces available for surgery as a guide for case selection.

METHODS: Only patients ,18 years were included. The choice of operative corridor/ approach is described in relation to pathological entity and location. Preoperative/ postoperative visual fields and endocrine panels, extent of resection, as well as postoperative long-term complications are described. Prospective magnetic resonance image-based anatomic measurements of key distances were performed to determine age-dependent surgical indications and limitations.

RESULTS: Forty purely endoscopic procedures were performed in 33 pediatric patients (5-18 years of age) harboring a variety of skull base lesions, from benign tumors to congenital malformations. For the 20 patients in whom gross total resection was the intended goal of surgery, gross total resection was attained in 15 (75%). There were 2 infections (5%) and no cerebrospinal fluid leaks. Significant improvement was shown in 58.3% of patients with visual deficits. Hormone overproduction resolved in 75% of patients, while preoperative hormone insufficiency only improved in 29.2%. Wider intercarotid distance at the superior clivus (P = .01) and shorter nare-dens working distance (P = .001) predicted improved outcomes and fewer postoperative complications.

CONCLUSION: Endonasal endoscopic skull base approaches are viable in the pediatric population, they are not impeded by sphenoid sinus aeration, and they have minimal risk of cerebrospinal fluid leak and meningitis. Outcomes and complications can be predicted based on specific radio anatomical skull base measurements rather than age.

Antibiotic-impregnated shunts: effects in pediatric population


J Neurosurg Pediatrics 13:101–106, 2014

Infection after both primary and revision shunt surgeries remains a major problem in pediatric neurosurgical practice. Antibiotic-impregnated shunt (AIS) tubing has been proposed to reduce infection rates. The authors report their experience with AIS catheters in their large pediatric neurosurgery department.

Methods. The authors conducted a retrospective case review of consecutive shunt operations performed before (1993–2003) and after (2005–2009) introduction of AIS tubing, with analysis of shunt infection rates and causative organisms identified.

Results. The historical control group consisted of 1592 consecutive shunt operations (657 primary insertions), and the AIS study group consisted of 500 consecutive shunt operations (184 primary insertions). Patients ranged in age from 0–17 years. In the historical group, 135 infections were identified (8.4%). In the AIS study group, 25 infections were identified (5%), representing a significant reduction (p < 0.005). The latency to diagnosis of infection was 23 days in the historical group and 139 days in the AIS study group. The infection rates in infants 0–6 months of age were 12.2% (historical group) and 6.7% (AIS group, p < 0.005), and in infants 7–12 months of age the rates were 7.9% (historical group) and 2.7% (AIS group, p < 0.005). In the historical control group, the frequency rank order of causative organisms was coagulase-negative staphylococcus (51.9%), Staphylococcus aureus (31.6%), streptococcus or enterococcus spp. (8.8%), gram-negative organisms (4.4%), and Propionibacterium acnes (2.2%). Organisms responsible for infections in AIS were S. aureus (40%), followed by streptococcus or enterococcus spp. (20%), P. acnes and coagulase-negative staphylococcus (both 16%), and gram-negative organisms (4%). No unusually antibioticresistant bacteria were identified in either group. The authors further subdivided the AIS group into those undergoing primary AIS insertion (Subgroup 1), those undergoing revision of non-AIS systems using AIS components (Subgroup 2), and those undergoing revision of AIS systems using AIS components (Subgroup 3). Infection rates were 1.6% in Subgroup 1, 2.5% in Subgroup 2, and 11.7% in Subgroup 3. Staphylococcus aureus was the most common organism identified in infections of the Subgroups 2 and 3.

Conclusions. Use of AIS tubing significantly improves shunt infection rates in both general pediatric and infant populations with no evidence of increased antibiotic resistance, which is in agreement with previous studies. However, the increased infection rate in revision surgery in children with AIS catheters in situ raises questions about their long-term application.

Management of subdural hygromas associated with arachnoid cysts

Subdural hygromas associated with cysts

J Neurosurg Pediatrics 12:434–443, 2013

Arachnoid cysts may occasionally be associated with subdural hygromas. The management of these concurrent findings is controversial.

Methods. The authors reviewed their experience with arachnoid cysts and identified 8 patients with intracranial arachnoid cysts and an associated subdural hygroma. The medical records and images for these patients were also examined.

Results. In total, 8 patients presented with concurrent subdural hygroma and arachnoid cyst. Of these 8 patients, 6 presented with headaches and 4 had nausea and vomiting. Six patients had a history of trauma. One patient was treated surgically at the time of initial presentation, and 7 patients were managed without surgery. All patients experienced complete resolution of their presenting signs and symptoms.

Conclusions. Subdural hygroma may lead to symptomatic presentation for otherwise asymptomatic arachnoid cysts. The natural course of cyst-associated subdural hygromas, even when symptomatic, is generally benign, and symptom resolution can be expected in most cases. The authors suggest that symptomatic hygroma is not an absolute indication for surgical treatment and that expectant management can result in good outcomes in many cases.

Reoperation for Refractory Epilepsy in Childhood: A Second Chance for Selected Patients


Neurosurgery 73:695–704, 2013

Reoperations account for 10% in pediatric epilepsy surgery cohorts, and they are especially relevant in young children with catastrophic epilepsy. OBJECTIVE: To determine surgical outcomes and their predictive factors in reoperations for refractory epilepsy in childhood.

METHODS: We retrospectively analyzed presurgical findings, resections, and outcomes of 23 consecutive children who underwent reoperations from 2000 to 2011.

RESULTS: Etiology included cortical dysplasia with/without glioneuronal tumor in 19 patients (83%), sole glioneuronal tumor in 2, and tuberous sclerosis and Rasmussen encephalitis in 1 each. The reasons for the failure of the initial surgery were functional considerations in 8 (35%), incorrect delineation of the epileptogenic zone in 8 (35%), and resection not performed as initially planned in 7 (30%) cases. Final procedures included 8 (35%) intralobar, 8 (35%) multilobar resections, and 7 (30%) hemispherotomies. Following reoperations, 14 (61%) patients were seizure free, 6 (26%) showed significant or worthwhile improvement, and 3 (13%) did not respond to surgery. Six of 8 patients who underwent the first resection before the age of 3 years, 6 of 8 whose first resection was limited by functional considerations, and all 7 with hemispherotomy as the final resection achieved seizure freedom after reoperation.

CONCLUSION: Reoperation is particularly beneficial for selected children with refractory epilepsy associated with cortical dysplasia that did not respond to an initial limited and/or early resection but achieved seizure freedom after extensive procedures. When indicated, reoperation should be performed at the youngest possible age to profit from higher functional plasticity in compensating for neurological deficit.

Orthotic (helmet) therapy in the treatment of plagiocephaly

Type IV plagio

Neurosurg Focus 35 (4):E2, 2013

The goal of this study was to review the current literature on orthotic (helmet) therapy use in the treatment of deformational plagiocephaly. Methods. PubMed was used to search English articles using the medical subject headings “deformational plagiocephaly” and “orthosis,” and “deformational plagiocephaly” and “helmet.”

Results. Forty-two articles were found. There were no Class I studies, 7 Class II studies, 1 Class III study, and 13 Class IV studies. Cranial orthoses have been shown to be effective in treating deformational plagiocephaly. It continues to be debated as to whether the statistical significance of treatment with cranial orthoses compared with conservative therapies is clinically significant. Children older than 12 months of age with deformational plagiocephaly may still benefit from orthotic therapy. The long-term effects of orthotic therapy are controversial.

Conclusions. There is a lack of Class I literature evidence supporting the use of helmet therapy in deformational plagiocephaly. There are controversies surrounding the use of orthotic therapy such as appropriate use, cost, use in older children, and long-term outcomes. Clinical indications for orthotic therapy need to be better defined with further research studies.

Dura Splitting Decompression for Chiari I Malformation in Pediatric Patients


Neurosurgery 72:922–929, 2013

Dural splitting decompression may be an effective and safe treatment for Chiari I malformation.

OBJECTIVE: To compare clinical outcomes, complications, and resource utilization for patients undergoing Chiari I decompression with or without duraplasty.

METHODS: Between 2000 and 2009, the senior author performed 113 Chiari I decompression operations with dural splitting or duraplasty in children less than 18 years of age; 110 were included in a retrospective cohort analysis of safety, efficacy, and treatment cost. Patients without significant syringomyelia underwent dural splitting decompression, and patients with syringomyelia underwent duraplasty.

RESULTS: Sixty-three patients without significant syringomyelia (57%) underwent dural splitting decompression. They were significantly younger than patients undergoing duraplasty (8.3 ± 4.9 years vs 10.4 ± 4.4 years; P < .05). Headaches improved or resolved in most patients in both groups (90.5% vs 93.6%; P = .59). Dysphagia, long tract signs, cranial nerve, and bulbar symptoms also improved similarly in both groups. Three duraplasty patients were treated medically for aseptic meningitis; one underwent reoperation for a symptomatic pseudomeningocele. No patient undergoing dural splitting decompression experienced a cerebrospinal fluid-related complication. Extradural decompression required less operative time than duraplasty (105.5 vs 168.9 minutes, P < .001), a shorter length of stay (2.4 vs 2.8 days, P = .011), and lower total cost for the primary hospitalization ($26 837 vs $29 862, P = .015).

CONCLUSION: In this retrospective cohort study, dural splitting decompression was equally effective, safer, and lower cost for treatment of Chiari I malformation without syringomyelia. A multicenter trial with groups balanced for the presence of syringomyelia is necessary to determine whether these results are generalizable.

Risk Factors for Pediatric Arachnoid Cyst Rupture/Hemorrhage: A Case-Control Study


As the availability of imaging modalities has increased, the finding of arachnoid cysts has become common. Accurate patient counseling regarding physical activity or risk factors for cyst rupture or hemorrhage has been hampered by the lack of definitive association studies.

OBJECTIVE: This case-control study evaluated factors that are associated with arachnoid cyst rupture (intracystic hemorrhage, adjacent subdural hematoma, or adjacent subdural hygroma) in pediatric patients with previously asymptomatic arachnoid cysts.

METHODS: Patients with arachnoid cysts and intracystic hemorrhage, adjacent subdural hygroma, or adjacent subdural hematoma treated at a single institution from 2005 to 2010 were retrospectively identified. Two unruptured/nonhemorrhagic controls were matched to each case based on patient age, sex, anatomical cyst location, and side. Risk factors evaluated included arachnoid cyst size, recent history of head trauma, and altitude at residence.

RESULTS: The proportion of imaged arachnoid cysts that presented either originally or subsequently with a rupture or hemorrhage was 6.0%. Larger cyst size, as defined by maximal cyst diameter, was significantly associated with cyst rupture/hemorrhage (P < .001). When dichotomized with a 5-cm cutoff, 9/13 larger cysts ruptured and/or hemorrhaged, whereas only 5/29 smaller cysts ruptured/hemorrhaged (odds ratio = 16.5 (confidence interval [2.5, N]). A recent history of head trauma was also significantly associated with the outcome (P < .001; odds ratio = 25.1 (confidence interval [4.0, N]). Altitude was not associated with arachnoid cyst rupture or hemorrhage.

CONCLUSION: This case-control study suggests that larger arachnoid cyst size and recent head trauma are risk factors for symptomatic arachnoid cyst rupture/hemorrhage.

Use of the NeuroBalloon catheter for endoscopic third ventriculostomy

endoscopic third ventriculostomy

J Neurosurg Pediatrics 11:302–306, 2013

Endoscopic third ventriculostomy (ETV) has become the procedure of choice for treatment of obstructive hydrocephalus.

While patient selection is the most critical factor in determining the success of an ETV procedure, the technical challenge lies in the proper site of fenestration and the successful creation of a patent stoma.

Positioning of a single balloon catheter at the level or below the floor of the third ventricle to achieve an optimal ventriculostomy can at times be challenging.

Here, the authors describe the use of a double-barrel balloon catheter (NeuroBalloon catheter), which facilitates positioning across, as well as dilation of, the floor of the third ventricle.

The surgical technique and nuances of using the NeuroBalloon catheter and the experience in more than 1000 cases are described. The occurrence of vascular injury was less than 0.1%, and the risk of balloon rupture was less than 2%.

The authors found that the placement and deployment of this balloon catheter facilitate the creation of an adequate ventriculostomy in a few simple steps.

Endoscopic endonasal skull base surgery in the pediatric population


J Neurosurg Pediatrics 11:227–241, 2013

The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies.

Methods. A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed.

Results. A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eightyfive patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up.

Conclusions. Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population

The enigma of bifocal germ cell tumors in the suprasellar and pineal regions: synchronous lesions or metastasis?

The enigma of bifocal germ cell tumors in the suprasellar

J Neurosurg Pediatrics 11:107–114, 2013

Intracranial germ cell tumors (GCTs) frequently present with bifocal lesions in both the suprasellar and pineal areas. The pathogenesis of these bifocal GCTs has been the subject of controversy. Bifocal GCTs may be caused by synchronous tumors or by metastatic spread of tumor cells from one site to the other. The prognosis associated with bifocal GCTs has also been a cause of concern.

Methods. The authors constructed a single-institution patient cohort comprising 181 patients with intracranial GCTs. The clinical characteristics of bifocal GCTs were compared with those of suprasellar and pineal GCTs.

Results. Bifocal GCTs were observed in 23 patients (12.8%). Eighteen patients presented with bifocal GCTs that were diagnosed as germinomas, but 5 patients exhibited mixed GCTs. Analyses of age distributions and comparisons of tumor sizes were compatible with a model of a metastatic origin of bifocal GCTs. Eleven patients (47.8%) presenting with bifocal GCTs exhibited tumor seeding at presentation. Tumor seeding was significantly associated with bifocal lesions (p < 0.001). Patients with bifocal germinomas showed significantly shorter event-free survival and overall survival than did those presenting with germinomas from a single site of origin.

Conclusions. Bifocal GCTs are not restricted to germinomas, as had been previously reported, but do include mixed GCTs. The authors hypothesize that bifocal GCTs may result from the metastatic spread of suprasellar or pineal GCTs. The bifocal presentation of germinomas may be a poor prognostic sign and should alert clinicians to the possibility of a disseminated disease.

Neurosurgery Department. “La Fe” University Hospital. Valencia, Spain


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