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  • Journal of Neurosurgery: Pediatrics x
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Yasuomi Nonaka, Masakazu Miyajima, Ikuko Ogino, Madoka Nakajima and Hajime Arai

Object

Some cases of compensatory hydrocephalus have been reported in which cognitive deficiency progresses despite the absence of progressive ventricular dilation. In this study, the differentially expressed genes in compensated hydrocephalic H-Tx rat cortices were determined. A molecular mechanism that induces neuronal death in the cerebral cortex of compensated hydrocephalus is proposed.

Methods

The cerebral cortices of 8-week-old H-Tx rats with spontaneously arrested hydrocephalus (hH-Tx) and nonhydrocephalic H-Tx (nH-Tx) control rats were subjected to cDNA microarray analysis followed by canonical pathway analysis.

Results

In the hH-Tx rats, many genes in the amyloidal processing pathway showed altered expression, including Akt3 and p38 MAPK. These latter genes are involved in tau protein phosphorylation, and their increased expression in hydrocephalus was confirmed by real-time polymerase chain reaction analysis. Immunohistological and immunoblot analysis revealed elevated phosphorylated tau expression in the cerebral cortex neurons of the hH-Tx rats.

Conclusions

The accumulation of phosphorylated tau protein in the cerebral cortex may be one of the mechanisms by which later cognitive dysfunction develops in patients with compensated hydrocephalus. More work needs to be done to determine if the accumulation of phosphorylated tau in the cortex can help predict which patients may decompensate thus requiring more aggressive treatment for compensated hydrocephalus.

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Bernardo Ratilal, João Costa and Cristina Sampaio

Object

Systemic antibiotics and antibiotic-impregnated shunt (AIS) systems are often used to prevent shunt infection. The authors conducted a systematic review to evaluate its effectiveness of antibiotics in patients who underwent placement of intracranial ventricular shunts.

Methods

The authors searched the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, LILACS, and the meeting proceedings from the American Association of Neurological Surgeons and from the European Association of Neurosurgical Societies until June 2005. Randomized and quasi-randomized trials comparing the use of prophylactic antibiotics (either systemic or AIS systems) in intracranial ventricular shunt procedures with placebo or no antibiotics were included in the review.

Results

Seventeen trials involving 2134 patients were included. Results from the metaanalysis showed that the use of systemic antibiotic prophylaxis for internal shunts was associated with a decrease in shunt infection (odds ratio 0.51; 95% confidence interval 0.36–0.73). The authors found no significant differences between the subgroups evaluated in type of internal shunt (ventriculoperitoneal/ventriculoatrial), age, or duration of the administration of antibiotics.

Conclusions

The authors found a benefit of systemic prophylactic antibiotics in preventing shunt infection, regardless of the patient's age and the type of internal shunt used. The benefit of its use after the first 24 hours postoperatively remains uncertain. Future trials should be conducted to evaluate the effectiveness of different regimens of systemic antibiotics rather than placebo, namely single preoperative dose versus multiple doses. It should also be mandatory to evaluate the effectiveness of systemic prophylactic antibiotics for external ventricular drains. Evidence suggests that antibiotic-impregnated catheters reduce the incidence of shunt infection, although more well-designed clinical trials are needed.

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Paritosh Pandey, Srikantha Umesh, Dhananjaya Bhat, Dwarakanath Srinivas, Rojin Abraham, Shibu Pillai, Anandh Balasubramaniam, Indira Devi, Sommanna Sampath and Bengaluru A. Chandramouli

Object

Cerebellar abscesses are common neurosurgical emergencies in developing countries, and have a distressingly high mortality rate of 10 to 15% even today. There is still no consensus on the standard approach to these lesions, and controversy persists over whether these lesions should be treated with primary excision or aspiration.

Methods

The authors retrospectively analyzed 82 cases of cerebellar pyogenic abscesses in children treated at their institution over a period of 10 years. This represents the largest such series being described in literature. All lesions except 1 were otogenic in origin. The clinical and radiological features are discussed.

Results

Primary excision was undertaken in 66 patients (80%) and aspiration in 16 patients (20%). Five patients in whom the abscesses were initially treated with aspiration subsequently underwent elective excision. Nine (12.6%) of 71 patients in whom the abscesses were excised had residual abscesses on postoperative imaging; in those who had undergone aspiration as the primary treatment, 6 (54.5%) of 11 patients had recurrent abscesses. There were no deaths among the patients who underwent excision of the abscess. Also, excision of posterior fossa abscesses required fewer repeated procedures with lower recurrence rates, and statistically lower rate of complications.

Conclusions

Compared to primary aspiration, the authors found that primary excision is the preferred method for treating cerebellar abscesses.

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Samuel R. Browd, James T. Goodrich and Marion L. Walker

✓Craniopagus twins represent a rare phenomenon of congenital misfortune. Modern neurosurgical techniques have created opportunities for successful separation and the promise of a normal existence for these children, who in the past were often left as historical footnotes or put on display as oddities of nature. The authors document a brief history of conjoined twinning and discuss the modern science of craniopagus epidemiology, classification, and separation. In particular, the strategies used and the rationale for staged surgical separation are highlighted.

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Eelco W. Hoving, Johan M. Kros, Ellis Groninger and Wilfred F. A. den Dunnen

✓Desmoplastic infantile gangliogliomas (DIGs) are rare supratentorial tumors that arise in infancy. Despite the large size of these lesions, the prognosis is generally considered favorable after gross-total resection (GTR); however, in incidental cases tumor progression has been described. The authors report on a child harboring a DIG with a high-grade primitive cell population and glial differentiation. The progressive clinical course was determined by this cell component in spite of GTR and adjuvant chemotherapy. The significance of the presence of a high-grade primitive tumor component in the context of DIG is discussed.

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Jacques van der Meulen, Joris van der Vlugt, Jolanda Okkerse and Bert Hofman

Object

The aim of this study was to analyze the presence of beaten-copper patterns (BCPs) in children with craniosynostosis before 18 months of age and its association with their IQ at a later age.

Methods

The authors conducted a retrospective analysis of 538 cephalograms (obtained at a mean patient age of 1.16 years) from 95 patients. The BCP location and percentage of brain surface area covered were related to patient IQ scores obtained by the same psychologist using the Snijders–Oomen Nonverbal Intelligence Test–Revised, 51/2–17, and the Wechsler Preschool and Primary Scale of Intelligence–Revised.

Results

As much as 71.6% of patients presented with a BCP before 18 months of age (mean surface area of BCP 20.3%, 93% of patients presented with bilateral BCPs). The mean IQ was 95 ± 21.3 (range 50–136) at a mean patient age of 8.4 ± 2.59 years. There was a significant increase in the surface area covered by BCPs in the first 3 years of life (p < 0.001) and a significant difference in IQs between syndromic (30 cases, mean IQ 88.9) and nonsyndromic craniosynostosis cases (54 cases, mean IQ 98.9, p = 0.03). No significant correlation was found between IQ and the appearance of BCPs on presurgery radiographs (Pearson correlation coefficient = 0.143, p = 0.19) or their location (Spearman rank correlation coefficient = 0.091, p = 0.45). The BCPs appeared predominantly in the occipital region (41.1%).

Conclusions

Although the radiographic appearance of a BCP before the age of 18 months is an uncommon finding in healthy children, a craniosynostosis study group showed a preoperative BCP incidence of 71.6% and an increased incidence during the period of rapid brain expansion in the first 3 years of life. Note, however, that the presence of such a pattern had no significant long-term effect on patient intelligence levels.

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Yuo Iizuka, Takanori Kakihara, Mitimasa Suzuki, Shinji Komura and Hiroyuki Azusawa

✓ It is commonly believed that in vein of Galen aneurysmal malformations (VGAMs) venous structures normally constituting the deep or Galenic venous system, such as the internal cerebral vein (ICV) and the basal vein of Rosenthal, are not connected to the vein of Galen. In this report, the authors describe 2 cases of successfully treated VGAM in which drainage of an ICV into the vein of Galen was confirmed by follow-up angiography. Two mural types of VGAM were treated using transarterial glue embolization when 1 child was 5 months and the other was 6 months old. The postoperative outcomes for these babies were complete cures. Follow-up digital subtraction angiography obtained after 12 months (Case 1) and 6 months (Case 2) confirmed that the shrunken median prosencephalic vein connects with the deep venous system. The possibility of normal deep Galenic venous drainage must be considered in endovascular management of VGAM. The goal of endovascular intervention is to close only the ventral component of the dilated median prosencephalic vein.

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Claudia Faria, José Miguéns, João Lobo Antunes, Cândida Barroso, José Pimentel, Maria Do Carmo Martins, Vasco Moura-Nunes and Lúcia Roque

✓Papillary glioneuronal tumors (PGNTs) are rare lesions of the central nervous system, and no information exists on the genetic alterations in these neoplasms. The authors report on such a case in a child. Genetic studies revealed that the tumor was characterized by gains and structural alterations involving only chromosome 7 with breakpoints at 7p22. By using comparative genomic hybridization, the authors observed a high-level amplification region at 7p14~q12. Fluorescence in situ hybridization with a probe for EGFR revealed that this gene was not amplified. Similar to other patients with PGNTs, the patient in the present case fared well. From a genetic point of view the data in the present case are in accordance with previous findings of EGFR amplifications as uncommon in low-grade gliomas and gangliogliomas. Recurrent rearrangements of chromosome 7 have been noted in other mixed glioneuronal tumors. The data in this case suggest that genes located at chromosome 7 can also be involved in the pathogenesis of PGNT. In clinical terms it will be especially important to corroborate, through the analysis of further cases, the involvement of the chromosome 7p22 locus, a region where glial and neuronal linked genes (RAC1 and NXPH1) are known to be located.

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Sherman C. Stein and Wensheng Guo

Object

The goal of this study was to determine whether failure rates of hydrocephalus shunts have fallen over the years as a result of experience or technical improvements.

Methods

A structured search was performed of the English language literature for case series reporting failure rates after shunt insertion. A metaanalytic model was constructed to pool data from multiple studies and to analyze failure rates statistically for temporal trends. Separate models were used for children (< 17 years old) and adults.

Results

In children, the shunt failure rate was 31.3% for the 1st year and 4.5% per year thereafter. There were no significant changes in either rate over time. Although 1st-year failure rates in adults have fallen slightly over time, late failure rates have risen.

Conclusions

Progress in preventing shunt failures has not been made over the last several decades. Any improvements made in shunt materials or insertion techniques have been overshadowed by biological and other factors.

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Matthew J. McGirt, Kaisorn L. Chaichana, April Atiba, Ali Bydon, Timothy F. Witham, Kevin C. Yao and George I. Jallo

Object

Gross-total resection of pediatric intramedullary spinal cord tumor (IMSCT) can be achieved in the majority of cases while preserving long-term neurological function. Nevertheless, postoperative progressive spinal deformity often complicates functional outcome years after surgery. The authors set out to determine whether laminoplasty in comparison with laminectomy has reduced the incidence of subsequent spinal deformity requiring fusion after IMSCT resection at their institution.

Methods

The first 144 consecutive patients undergoing resection of IMSCTs at a single institution underwent laminectomy with preservation of facet joints. The next 20 consecutive patients presenting for resection of IMSCTs underwent osteoplastic laminotomy regardless of patient or tumor characteristics. All patients were followed up with telephone interviews corroborated by medical records for the following outcomes: 1) neurological and functional status (modified McCormick Scale [MMS] score and Karnofsky Performance Scale [KPS] score); and 2) development of progressive spinal deformity requiring fusion. The incidence of progressive spinal deformity and the long-term neurological function were compared between the laminectomy and osteoplastic laminotomy cohorts. The means are expressed ± the standard deviation.

Results

Overall, the patients' mean age was 8.6 ± 5 years, and they presented with median MMS scores of 2 (interquartile range [IQR] 2–4). A > 95% resection was achieved in 125 cases (76%). There were no differences (p > 0.10) between patients treated with osteoplastic laminotomy and those treated with laminectomy in terms of the following characteristics: age; sex; duration of symptoms; location of tumor; incidence of preoperative scoliosis (Cobb angle > 10°: 7 [35%] with laminoplasty compared with 49 [34%] with laminectomy); involvement of the cervicothoracic junction (7 [35%] compared with 57 [40%]); thoracolumbar junction (4 [20%] compared with 36 [25%]); tumor size; extent of resection; radiation therapy; histopathological findings; or mean operative spinal levels (7.5 ± 2 compared with 7.5 ± 3). Nevertheless, patients who underwent osteoplastic laminotomy had better median preoperative MMS scores than those treated with laminectomy (2 [IQR 2–2] compared with 2 [IQR 2–4]; p = 0.04). A median of 3.5 years (IQR 1–7 years) after surgery, only 1 patient (5%) in the osteoplastic laminotomy cohort required fusion for progressive spinal deformity, compared with 43 (30%) in the laminectomy cohort (p = 0.027). Adjusting for the inter-cohort difference in preoperative MMS scores, osteoplastic laminotomy was associated with a 7-fold reduction in the odds of subsequent fusion for progressive spinal deformity (odds ratio 0.13, 95% confidence interval 0.02–1.00; p = 0.05). The median MMS and KPS scores were similar between patients who underwent osteoplastic laminotomy and those in whom laminectomy was performed (MMS Score 2 [IQR 2–3] for laminotomy compared with 2 [IQR 2–4] for laminectomy, p = 0.54; KPS Score 90 [IQR 70–100] for laminotomy compared with 90 [IQR 80–90] for laminectomy, p = 0.545) at a median of 3.5 years after surgery.

Conclusions

In the authors' experience, osteoplastic laminotomy for the resection of IMSCT in children was associated with a decreased incidence of progressive spinal deformity requiring fusion but did not affect long-term functional outcome. Laminoplasty used for pediatric IMSCT resection may decrease the incidence of progressive spinal deformity requiring subsequent spinal stabilization in some patients.