Neurosurgery Blog

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.

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.

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

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 71:985–993, 2012

It is unclear whether long-term seizure outcomes in children are similar to those in adult epilepsy surgery patients.

OBJECTIVE: To determine 5-year outcomes and antiepilepsy drug (AED) use in pediatric epilepsy surgery patients from a single institution.

METHODS: The cohort consisted of children younger than 18 years of age whose 5-year outcome data would have been available by 2010. Comparisons were made between patients with and without 5-year data (n = 338), patients with 5-year data for seizure outcome (n = 257), and seizure-free patients on and off AEDs (n = 137).

RESULTS: Five-year data were available from 76% of patients. More seizure-free patients with focal resections for hippocampal sclerosis and tumors lacked 5-year data compared with other cases. Of those with 5-year data, 53% were continuously seizure free, 18% had late seizure recurrence, 3% became seizure free after initial failure, and 25% were never seizure free. Patients were more likely to be continuously seizure free if their surgery was performed during the period 2001 to 2005 (68%) compared with surgery performed from 1996 to 2000 (61%), 1991 to 1995 (36%), and 1986 to 1990 (46%). More patients had 1 or fewer seizures per month in the late seizure recurrence (47%) compared with the not seizure-free group (20%). Four late deaths occurred in the not seizure-free group compared with 1 in the seizure-free group. Of patients who were continuously seizure free, 55% were not taking AEDs, and more cortical dysplasia patients (74%) had stopped taking AEDs compared with hemimegalencephaly patients (18%).

CONCLUSION: In children, 5-year outcomes improved over 20 years of clinical experience. Our results are similar to those of adult epilepsy surgery patients despite mostly extratemporal and hemispheric operations for diverse developmental etiologies.

 Neurosurgery 71:909–915, 2012

Craniopharyngiomas are the most common benign histological tumors to involve the hypothalamopituitary region in childhood. When the tumor location is unfavorable, a gross total or partial resection followed by radiotherapy is the main treatment option in adults. However, it presents the risk of morbidity, especially for children. Intracystic bleomycin has been used to potentially delay the use of radiotherapy or radical resection to decrease morbidity.

OBJECTIVE: To determine the benefit and harm of intracystic bleomycin vs other treatments for cystic craniopharyngiomas in children. METHODS: We searched the electronic databases of CENTRAL, MEDLINE/PubMed, and EMBASE/Ovid with prespecified terms. In addition, we searched reference lists of relevant articles and reviews, conference proceedings, and ongoing trial databases.

RESULTS: We could not identify any studies in which the only difference between the treatment groups was the use of intracystic bleomycin. We did identify a randomized, controlled trial comparing intracystic bleomycin with intracystic 32P (n = 7 children). The trial had a high risk of bias. Survival could not be evaluated. There was no evidence of a significant difference in cyst reduction, neurological status, third nerve paralysis, fever, or total adverse effects between the treatment groups. There was a significant difference in favor of the 32P group for the occurrence of headache and vomiting.

CONCLUSION: Based on the currently available evidence, we are not able to give recommendations for the use of intracystic bleomycin in the treatment of cystic craniopharyngiomas in children. High-quality randomized, controlled trials are needed.

J Neurosurg 117:141–149, 2012

The aim of this study was to evaluate and compare CSF flow after endoscopic third ventriculostomy (ETV) and endoscopic aqueductoplasty (EAP) in patients presenting with obstructive hydrocephalus caused by aqueductal stenosis.

Methods. In patients harboring aqueductal stenosis who underwent EAP (n = 8), ETV (n = 8), and both ETV and EAP (n = 6), CSF flow through the restored aqueduct and through the ventriculostomy was investigated using cine cardiac-gated phase-contrast MRI. For qualitative evaluation of CSF flow, an in-plane phase-contrast sequence in the midsagittal plane was used. The MR images were displayed in a closed-loop cine format. Quantitative through-plane measurements were performed in the axial plane perpendicular to the aqueduct and/or floor of the third ventricle.

Results. Evaluation revealed significantly higher CSF flow through the ventriculostomies compared with flow through the aqueducts. This was true both when comparing the ETV group with the EAP group and when comparing the flow of the ventriculostomy and aqueduct within the ETV and EAP group. There was no difference in aqueductal CSF flow between patients who underwent EAP alone and patients who underwent ETV and EAP. There was also no difference in ventriculostomy CSF flow between patients who underwent ETV alone and patients who underwent ETV and EAP. Fifty percent of the restored aqueducts became occluded at a mean of 46 months after surgery (range 18–126 months). In contrast, all ETVs remained patent in the mean follow-up period of 110 months after surgery, although 1 patient required shunt placement after 66 months.

Conclusions. Cerebrospinal fluid flow through ventriculostomies is significantly higher than aqueductal CSF flow after EAP. This could be one factor to explain why the reclosure rate of aqueducts after EAP is higher than the reclosure rate of the ventriculostoma after ETV.

J Neurosurg Pediatrics 9:670–675, 2012. (http://thejns.org/doi/abs/10.3171/2012.2.PEDS11538) 

A growing skull fracture (GSF) is a rare but significant late complication of skull fractures, usually occurring during infancy and early childhood. Delayed diagnosis and improper treatment could exacerbate this disease. The aim of this study was to introduce a new hypothesis about, describe the stages of, and discuss the treatment strategy for GSF.

Methods. The authors performed a retrospective review of 27 patients with GSF, who were grouped according to 3 different GSF stages.

Results. Over a period of 20 years, 27 patients with GSF (16 males and 11 females) were treated in the authors’ department. The mean follow-up period was 26.5 months. Six patients were in the prephase of GSF (Stage 1), 10 patients in the early phase (Stage 2), and 11 in the late phase (Stage 3). All patients underwent duraplasty. All 6 patients at Stage 1 and 5 patients at Stage 2 underwent craniotomy without cranioplasty. Five patients at Stage 2 and all of the patients at Stage 3 underwent cranioplasty with autologous bone and alloplastic materials, respectively. Among all patients, 5 underwent ventriculoperitoneal shunt placement. Symptoms in all patients at Stages 1 and 2 were alleviated or disappeared, and the cranial bones developed without deformity during follow-up. Among patients with Stage 3 GSF, no obvious improvement in neurological deficits was observed. Three patients underwent additional operations because of cranial deformation or infection.

Conclusions. The authors identify the stages of GSF according to a new hypothesis. They conclude that accurately diagnosing and treating GSF during Stages 1 and 2 leads to a better prognosis.

Neurosurgery 70:847–859, 2012 DOI: 10.1227/NEU.0b013e318236717f

Treatment of an isolated fourth ventricle should be considered when clinical symptoms or a significant mass effect occur.

OBJECTIVE: To report clinical and radiographic outcomes after endoscopic transaqueductal or transcisternal stent placement into the fourth ventricle. METHODS: In 19 patients (age, 34th week of gestation-20 years; median age, 17.5 months), 22 endoscopic procedures were performed. Either an aqueductoplasty or, in cases with a supratentorially extended fourth ventricular component, an interventricular fenestration was performed. In all patients, a stent connected to the cerebrospinal fluid–diverting shunt was placed through the fenestration. Surgical complications and radiological and clinical outcomes are reported.

RESULTS: All 19 patients had a mean follow-up of 26.9 ± 18.2 months. No persisting neurological complications were observed; 27.3% of patients experienced complete resolution of presenting symptoms, whereas 68.3% demonstrated partial resolution. Symptoms with short duration (< 4 weeks) resolved completely, whereas long-standing symptoms partially improved. Short-term shunt complications (n = 2; insufficient catheter placement and subdural hygroma) and a need for long-term stent revisions (n = 3; stent retraction and shunt revision for other causes) were observed. The mean fourth ventricular volume was reduced after surgery (44.2 ± 25.8 to 23.1 ± 21.9 mL; P < .01). Pontine diameter increased from 0.9 ± 0.3 to 1.2 ± 0.3 cm (P < .01) after surgery. Both effects were still demonstrated on later radiological follow-up of 24.4 ± 14.2 months (fourth ventricular size, 24.7 ± 28.1 mL; P < .01; pontine diameter, 1.3 ± 0.3 cm; P < .01).

CONCLUSION: The clinical and radiological outcomes after endoscopic aqueductoplasty and interventriculostomy in children with an isolated fourth ventricle indicate that this procedure is feasible, effective, and safe.

Neurosurgery 70:40–48, 2012 DOI: 10.1227/NEU.0b013e31822ff0ed

The long-term prognosis of cerebellar astrocytomas needs to be reviewed.

OBJECTIVE: To elucidate the factors influencing tumor recurrence or progression and to determine how long these patient with cerebellar astrocytomas require surveillance with neuroimaging.

METHODS: A retrospective review of 101 children surgically treated for a cerebellar astrocytoma and followed up for >10 years was performed.

RESULTS: Mean follow-up was 18.4 years. Total resection confirmed by postoperative imaging was performed in 51 patients (50.5%; group A). Twenty-three patients (22.8%) had surgical total resection; however, equivocal residual tumor was found on postoperative imaging (group B). Subtotal resection leaving a portion of brainstem or cerebellar peduncle was performed in 27 patients (26.7; group C). Of these 50 residual tumors, 16 (32%) showed spontaneous regression and 8 (16%) showed arrested growth. Radiographic recurrence or progression was noted in 29 patients (28.7%). Only 3 of 51 patients (5.9%) of group A with total resection had recurrence, whereas 26 of 50 residual tumors (52%; groups B and C) progressed. The only factor affecting recurrence or progression by multivariate analysis was the extent of surgical resection. All tumor recurrence or progression except for 1 (96.6%) occurred within 8 years from the original surgery (range, 2-132 months).

CONCLUSIONS: Overall prognosis of cerebellar astrocytomas is good; the 10-year survival rate was 100% and recurrence- or progression-free rate was 71.3% in our cohort. Almost half of residual tumors showed spontaneous regression or arrested growth in the long term. Eight to 10 years is considered to be a reasonable follow-up period by neuroimaging.

Neurosurg Focus 31 (5):E5, 2011. DOI: 10.3171/2011.8.FOCUS11177

Traumatic brain injury (TBI) is the current leading cause of death in children over 1 year of age. Adequate management and care of pediatric patients is critical to ensure the best functional outcome in this population.

In their controversial trial, Cooper et al. concluded that decompressive craniectomy following TBI did not improve clinical outcome of the analyzed adult population. While the study did not target pediatric populations, the results do raise important and timely clinical questions regarding the effectiveness of decompressive surgery in pediatric patients. There is still a paucity of evidence regarding the effectiveness of this therapy in a pediatric population, and there is an especially noticeable knowledge gap surrounding age-stratified interventions in pediatric trauma.

The purposes of this review are to first explore the anatomical variations between pediatric and adult populations in the setting of TBI. Second, the authors assess how these differences between adult and pediatric populations could translate into differences in the impact of decompressive surgery following TBI.

Neurosurgery 69:630–643, 2011 DOI: 10.1227/NEU.0b013e31821a872d

Controversy persists regarding the optimal treatment of pediatric craniopharyngiomas.

OBJECTIVE: We performed a meta-analysis of reported series of transcranial (TC) and transsphenoidal (TS) surgery for pediatric craniopharyngiomas to determine whether comparisons between the outcomes in TS and TC approaches are valid.

METHODS: Online databases were searched for English-language articles reporting quantifiable outcome data published between 1990 and 2010 pertaining to the surgical treatment of pediatric craniopharyngiomas. Forty-eight studies describing 2955 patients having TC surgery and 13 studies describing 373 patients having TS surgery met inclusion criteria.

RESULTS: Before surgery, patients who had TC surgery had less visual loss, more frequent hydrocephalus and increased intracranial pressure, larger tumors, and more suprasellar disease. After surgery, patients in the TC group had lower rates of gross total resection (GTR), more frequent recurrence after GTR, higher neurological morbidity, more frequent diabetes insipidus, less improvement, and greater deterioration in vision. There was no difference in operative mortality, obesity/hyperphagia, or overall survival percentages.

CONCLUSION: Directly comparing outcomes after TC and TS surgery for pediatric craniopharyngiomas does not appear to be valid. Baseline differences in patients who underwent each approach create selection bias that may explain the improved rates of disease control and lower morbidity of TS resection. Although TS approaches are becoming increasingly used for smaller tumors and those primarily intrasellar, tumors more amenable to TC surgery include large tumors with significant lateral extension, those that engulf vascular structures, and those with significant peripheral calcification.

Neurosurg Focus 31 (2):E3, 2011,DOI: 10.3171/2011.5.FOCUS1196

Sagittal craniosynostosis is the most common form of craniosynostosis and is commonly treated within the first year of life. Optimal treatment of patients older than 1 year of age is not well characterized. The authors reviewed cases of sagittal craniosynostosis involving patients who were treated surgically at their institution when they were older than 1 year in order to determine the rate of intracranial hypertension (ICH), potential to develop nonhealing cranial defects, and the need for various surgical procedures to treat the more mature phenotype.

Methods. A retrospective chart review was conducted of all cases in the Children’s Hospital of Pittsburgh Neurosurgery Database involving patients who underwent cranial vault remodeling for scaphocephaly after 1 year of age between October 2000 and December 2010.

Results. Ten patients were identified who met the inclusion criteria. Five patients underwent anterior two-thirds cranial vault remodeling procedures, 3 patients underwent posterior vault remodeling, and 2 patients underwent 2-staged total vault remodeling. All patients had improved head shapes, and mean cephalic indices improved from 65.4 to 69.1 (p = 0.05). Six patients exhibited signs of ICH. No patients with more than 3 months of follow-up exhibited palpable calvarial defects.

Conclusions. Patients with sagittal synostosis treated after 1 year of age demonstrate increased rates of ICH, warranting diligent evaluations and surveillance to detect it; rarely develop clinically significant cranial defects if appropriate bone grafting is performed at the time of surgery; and achieve acceptable improvements in head shape.

J Neurosurg Pediatrics 8:171-176, 2011.DOI: 10.3171/2011.5.PEDS1139

Nonaccidental head trauma (NAHT) is a major cause of death in infants. During the current economic recession, the authors noticed an anecdotal increase in infants with NAHT without an increase in the overall number of infants admitted with traumatic injuries. An analysis was performed to determine whether there was an association between economic recession and NAHT.

Methods. With Institutional Review Board approval, the trauma database was searched for NAHT in infants 0–2 years old during nonrecession (December 2001 to November 2007) and recession (December 2007 to June 2010) periods. Incidence is reported as infants with NAHT per month summarized over time periods. Continuous variables were compared using Mann-Whitney U-tests, and proportions were compared using the Fisher exact test.

Results. Six hundred thirty-nine infant traumas were observed during the study time period. From the nonrecession to the recession period, there was an 8.2% reduction in all traumas (458 in 72 months [6.4 /month] vs 181 in 31 months [5.8/month]) and a 3.5% reduction in accidental head traumas (142 in 72 months [2.0/month] vs 59 in 31 months [1.9/month]). Nonaccidental head trauma accounted for 14.6% of all traumas (93/639). The median patient age was 4.0 months and 52% were boys. There were no significant differences in the representative counties of referral or demographics between nonrecession and recession populations (all p > 0.05). The monthly incidence rates of NAHT doubled from nonrecession to recession periods (50 in 72 months [0.7/month] vs 43 in 31 months [1.4/month]; p = 0.01). During this recession, at least 1 NAHT was reported in 68% of the months compared with 44% of the months during the nonrecession period (p = 0.03). The severity of NAHTs also increased, with a greater proportion of deaths (11.6% vs 4%, respectively; p = 0.16) and severe brain injury (Glasgow Coma Scale score ≤ 8: 19.5% vs 4%, respectively; p = 0.06) during the recession.

Conclusions. In the context of an overall reduction in head trauma, the significant increase in the incidence of NAHT appears coincident with economic recession. Although the cause is likely multifactorial, a full analysis of the basis of this increase is beyond the scope of this study. This study highlights the need to protect vulnerable infants during challenging economic times.

J Neurosurg Pediatrics 8:177–183, 2011. DOI: 10.3171/2011.5.PEDS10362

Posterior fossa decompression with duraplasty for Chiari malformation Type I (CM-I) is a common pediatric neurosurgery procedure. Published series report a complication rate ranging from 3% to 40% for this procedure. Historically, many dural substitutes have been used, including bovine grafts, human cadaveric pericardium, synthetic dura, and autologous pericranium. The authors hypothesized that a recently observed increase in complications was dependent on the graft used.

Methods. Between January 2004 and January 2008, 114 consecutive patients ≤ 18 years old underwent primary CM-I decompression using duraplasty. Records were retrospectively reviewed for short- and intermediate-term complications and operative technique, focusing on the choice of duraplasty graft with or without application of a tissue sealant.

Results. The average age of the patients was 8.6 years. The dural graft used was variable: 15 were treated with cadaveric pericardium, 12 with Durepair, and 87 with EnDura. Tisseel was used in 75 patients, DuraSeal in 12, and no tissue sealant was used in 27 patients. The overall complication rate was 21.1%. The most common complications included aseptic meningitis, symptomatic pseudomeningocele, or a CSF leak requiring reoperation. The overall complication rates were as follows: cadaveric pericardium 26.7%, Durepair 41.7%, and EnDura 17.2%; reoperation rates were 13%, 25%, and 8.1%, respectively. Prior to adopting a different graft product, the overall complication rate was 18.1%; following the change the rate increased to 35%. Complication rates for tissue sealants were 14.8% for no sealant, 18.7% for Tisseel, and 50% for DuraSeal. Nine patients were treated with the combination of Durepair and DuraSeal and this subgroup had a 56% complication rate.

Conclusions. Complication rates after CM-I decompression may be dependent on the dural graft with or without the addition of tissue sealant. The complication rate at the authors’ institution approximately doubled following the adoption of a different graft product. Tissue sealants used in combination with a dural substitute to augment a duraplasty may increase the risk of aseptic meningitis and/or CSF leak. The mechanism of the apparent increased inflammation with this combination remains under investigation.

Neurosurgery 69:215–224, 2011 DOI: 10.1227/NEU.0b013e3182134340

The diagnostic accuracy and reproducibility for glioma histological diagnosis are suboptimal.

OBJECTIVE: To characterize radiological and histological features in pediatric malignant gliomas and to determine whether they had an impact on survival.

METHODS: We retrospectively reviewed a series of 96 pediatric malignant gliomas. All histological samples were blindly and independently reviewed and classified according to World Health Organization 2007 and Sainte-Anne classifications. Radiological features were reviewed independently. Statistical analyses were performed to investigate the relationship between clinical, radiological, and histological features and survival.

RESULTS: Cohort median age was 7.8 years; median follow-up was 4.8 years. Tumors involved cerebral hemispheres or basal ganglia in 82% of cases and brainstem in the remaining 18%. After histopathological review, low-grade gliomas and nonglial tumors were excluded (n = 27). The World Health Organization classification was not able to demonstrate differences between groups and patients survival. The Sainte-Anne classification identified a 3-year survival rate difference between the histological subgroups (oligodendroglioma A, oligodendroglioma B, malignant glioneuronal tumors, and glioblastomas; P = .02). The malignant glioneuronal tumor was the only glioma subtype with specific radiological features. Tumor location was significantly associated with 3-year survival rate (P = .005). Meningeal attachment was the only radiological criteria associated with longer survival (P = .02).

CONCLUSION: The Sainte-Anne classification was better able to distinguish pediatric malignant gliomas in terms of survival compared with the World Health Organization classification. In this series, neither of these 2 histological classifications provided a prognostic stratification of the patients.

J Neurosurg 114:1350–1359, 2011.DOI: 10.3171/2010.11.JNS10670

Craniopharyngioma accounts for 2%–5% of all primary intracranial neoplasms. The optimal management of craniopharyngioma remains controversial. The authors evaluated the early results of surgery and the longterm risk of tumor recurrence in a large series of patients undergoing resection of craniopharyngiomas.

Methods. Between 1990 and 2008, 112 consecutive patients (57 male and 55 female patients with a mean [± SEM] age of 33.3 ± 1.8 years) underwent resection of craniopharyngiomas at the authors’ hospital. Recurrence or growth of residual tumor tissue during follow-up was assessed using MR imaging.

Results. There were 3 perioperative deaths (2.7%). Severe adverse events were more frequent in patients who underwent operations via the transcranial route (37%) than the transsphenoidal approach (5.6%; p < 0.001). Magnetic resonance imaging showed radical resection of the tumor in 78 (71.6%) of the remaining 109 patients. Previous surgery and maximum tumor diameter were associated with persistence of disease after surgery. Craniopharyngioma recurred in 26 (24.5%) of 106 patients. Presence of residual tumor on the first postoperative MR imaging, male sex, and no postoperative radiation therapy were associated with a risk of tumor recurrence. Quality-of-life data were assessed in the 91 patients who attended the authors’ institution for follow-up visits. Among them, 8.8% patients were partially or completely dependent on others for daily living activities before surgery. This percentage increased to 14.3% at the last follow-up visit. The 5- and 10-year overall survival rates were 94.4% (95% CI 90.0%–98.8%) and 90.3% (95% CI 83.4%–97.3%), respectively.

Conclusions. Complete surgical removal of craniopharyngioma can be achieved with reasonable safety in more than 70% of patients. Recurrence of craniopharyngioma may occur even after apparent radical excision. Prompt management of residual or recurring disease by radiotherapy, repeat surgery, or a combination of both is usually successful in controlling further tumor growth.

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2011.2.PEDS10278

Pituitary adenomas are uncommon in childhood. Although medical treatment can be effective in treating prolactinomas and some growth hormone (GH)–secreting tumors, resection is indicated when visual function is affected or the side effects of medical therapy are intolerable. The authors of this report describe their 10-year experience in managing pituitary adenomas via the microscopic endonasal transsphenoidal approach in a pediatric population.

Methods. They performed a retrospective review of a surgical case series based at a single institution and consisting of 34 consecutive pediatric patients with endocrine-active (32 patients) and endocrine-inactive (2 patients) adenomas. These patients were surgically treated via an endonasal transsphenoidal approach between 1999 and 2008. Patient charts were reviewed, and clinical data were compiled and analyzed using the chi-square and Kaplan-Meier tests.

Results. The patient cohort consisted of 20 girls and 14 boys, with ages ranging from 9 to 18 years and a median age of 16 years. Thirty-two patients (94%) underwent surgery for endocrine-active tumors, including 10 (29%) with Cushing disease, 21 (62%) with prolactinomas, and 1 (3%) with GH-secreting tumors. Two patients with nonsecreting adenomas underwent surgery for apoplexy. The mean tumor volume was 5.4 cm3, and 13 patients (38%) had suprasellar extension and 7 (21%) had cavernous sinus invasion. Gross-total resection was achieved in 26 patients (76%), although it was significantly less likely to be achieved in the setting of cavernous sinus invasion (p < 0.001) but was unaffected by suprasellar extension. Residual tumor was treated with radiation therapy in 6 patients (18%). The average duration of hospital stay was 1.6 days. The median follow-up time was 18 months. After surgery, 19 patients (56%) had normal hormone function without adjuvant therapy, 8 (24%) had normal function with adjuvant therapy, and 5 (15%) had persistently elevated hormone levels. Patients with a macroprolactinoma were significantly more likely to require postoperative adjuvant therapy than were those with a microprolactinoma (p < 0.03).

Conclusions. Endonasal transsphenoidal resection is a safe, well-tolerated, and potentially curative treatment option for pituitary adenomas in children. Despite the technical challenges associated with this approach in the pediatric population, these tumors can be effectively managed with minimal morbidity. Endocrine function is usually preserved, and the majority of patients will not require lifelong medical therapy.

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2011.1.PEDS10341

The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention.

Methods. The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up.

Results. The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5–22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up.

Conclusions. The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2010.12.PEDS10359

The authors present their experience in the surgical treatment of metopic synostosis by orbital bandeau remodeling and frontal bone rotation. The pitfalls and advantages of the surgical technique are discussed, along with the long-term clinical results in 48 consecutive cases.

Methods. Forty-eight consecutive patients in whom trigonocephaly was diagnosed between 1990 and 2009 were treated with frontal bone rotation and frontoorbital bandeau remodeling. Of these patients, 38 (79%) were boys and 10 (21%) were girls. The age at the time of surgical treatment ranged between 4 and 42 months (mean ± SD 11.4 ± 8.7 months). The average follow-up period was 5.5 ± 4.2 years (range 5 months–19 years). The preoperative and latest postoperative photographs of the patients were evaluated for the following features: 1) shape of the forehead; 2) hypotelorism; and 3) temporal depression. Scores of 0, 1, or 2 were assigned for each item: 0 was normal, 1 meant moderate deformity, and 2 denoted severe deformity.

Results. In the early postoperative period, no complications were documented. The average hospitalization period was 4 days. Follow-up radiographs or 3D CT scans were obtained at regular intervals. The mean preoperative scores for the evaluated items were 1.38 ± 0.49 for the shape of the forehead, 1.33 ± 0.48 for hypotelorism, and 1.7 ± 0.46 for the temporal depression. The mean postoperative scores were 0.06 ± 0.24 for the shape of the forehead, 0.21 ± 0.4 for hypotelorism, and 0.67 ± 0.48 for the temporal depression. Overall, the total preoperative score dropped from 4.4 to 0.93 postoperatively (p < 0.05). All the patients were contented with the cosmetic results.

Conclusions. Early detection and treatment of metopic suture synostosis has a significant, favorable influence on the outcomes. Good understanding of the structural abnormality and the pathophysiological mechanisms of the possible complications is very important for performing proper surgical reconstruction.