Category: Pediatrics

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Is external hydrocephalus a possible differential diagnosis when child abuse is suspected?

Acta Neurochirurgica (2022) 164:1161–1172

Criteria for diagnosing abusive head trauma (AHT) or “shaken baby syndrome” are not well defined; consequently, these conditions might be diagnosed on failing premises.

Methods The authors have collected a total of 28 infants, from the US (20) and Norway (8), suspected of having been violently shaken, and their caregivers had been suspected, investigated, prosecuted or convicted of having performed this action. Among 26 symptomatic infants, there were 18 boys (69%) and 8 girls (31%)—mean age 5.1 month, without age difference between genders.

Results Twenty-one of 26 symptomatic children (81%) had a head circumference at or above the 90 percentile, and 18 had a head circumference at or above the 97 percentile. After macrocephaly, seizure was the most frequent initial symptom in 13 (50%) of the symptomatic infants. Seventeen (65%) of the symptomatic infants had bilateral retinal haemorrhages, and two had unilateral retinal haemorrhages. All infants had neuroimaging compatible with chronic subdural haematomas/hygromas as well as radiological characteristics compatible with benign external hydrocephalus (BEH).

Conclusions BEH with subdural haematomas/hygromas in infants may sometimes be misdiagnosed as abusive head trauma. Based on the authors’ experience and findings of the study, the following measures are suggested to avoid this diagnostic pitfall: medical experts in infant abuse cases should be trained in recognising clinical and radiological BEH features, clinicians with neuro-paediatric experience should always be included in the expert teams and reliable information about the head circumference development from birth should always be available.

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The Role of Prophylactic Intraventricular Antibiotics in Reducing the Incidence of Infection and Revision Surgery in Pediatric Patients Undergoing Shunt Placement

Neurosurgery 88(2)2021: 301–305

Ventriculoperitoneal shunt placement remains the primary treatment modality for children with hydrocephalus. However, morbidity and revision surgery secondary to infection remains high, even while using antibiotic-impregnated shunts.

OBJECTIVE: To determine whether intraoperative injection of antibiotics is independently associated with reduced rates of infection and revision surgery in children undergoing shunt placement.

METHODS: This is an analysis of a prospectively collected, multicenter, shunt-specific neurosurgical registry consisting of data from over 100 hospitals collected between 2016 and 2017. All patients under 18 yr of age undergoing first-time shunt placement for the definitive treatment of hydrocephaluswere included. The primary exposure of interest was injection of intraventricular antibiotics into the shunt catheter following shunt placement and prior to closure. The use of additional surgical adjuncts, such as antibiotic-impregnated shunts, stereotactic guidance, and endoscopy was collected. The primary outcome metric was the need for additional intervention because of an infection.

RESULTS: A total of 2007 pediatric patients undergoing shunt placement for hydrocephalus were identified. Postoperatively, 97 (4.8%) patients had additional intervention secondary to infection. In a multivariable regression model controlling for patient characteristics, etiology of hydrocephalus, prior temporizing measures, and placement of an antibiotic-impregnated shunt, injection of intraventricular antibiotics was associated with a significant reduction in postoperative infections (odds ratio = 0.29, 95% CI: 0.04-0.89, P=.038). Of those receiving intraventricular antibiotics, only 2 (0.38%)went on to undergo re-intervention due to infection.

CONCLUSION: These data suggest that for this select group of patients, use of intraventricular antibiotics was associated with decreased rates of re-intervention secondary to infection.

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Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on the Treatment of Pediatric Hydrocephalus: Update of the 2014 Guidelines

Neurosurgery 87:1071–1075, 2020

The Congress of Neurological Surgeons reviews its guidelines according to the Institute of Medicine’s recommended best practice of reviewing guidelines every 5 yrs. The authors performed a planned 5-yr review of the medical literature used to develop the “Pediatric hydrocephalus: systematic literature review and evidence-based guidelines” and determined the need for an update to the original guideline based on new available evidence.

OBJECTIVE: To perform an update to include the current medical literature for the “Pediatric hydrocephalus: systematic literature review and evidence-based guidelines”, originally published in 2014.

METHODS: The Guidelines Task Force used the search terms and strategies consistent with the original guidelines to search PubMed and Cochrane Central for relevant literature published between March 2012 and November 2019. The same inclusion/exclusion criteria were also used to screen abstracts and to perform the full-text review. Full text articles were then reviewed and when appropriate, included as evidence and recommendations were added or changed accordingly.

RESULTS: A total of 41 studies yielded by the updated search met inclusion criteria and were included in this update.

CONCLUSION: New literature resulting from the update yielded a new recommendation in Part 2, which states that neuro-endoscopic lavage is a feasible and safe option for the removal of intraventricular clots and may lower the rate of shunt placement (Level III). Additionally a recommendation in part 7 of the guideline now states that antibiotic- impregnated shunt tubing reduces the risk of shunt infection compared with conventional silicone hardware and should be used for children who require placement of a shunt (Level I). <https://www.cns.org/guidelines/browse-guidelines-detail/pediatric- hydrocephalus-guideline>

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Open Resection versus Laser Interstitial Thermal Therapy for the Treatment of Pediatric Insular Epilepsy

Various studies suggest that the insular cortex may play an underappreciated role in pediatric frontotemporal/parietal epilepsy. Here, we report on the postsurgical outcomes in 26 pediatric patients with confirmed insular involvement by depth electrode monitoring.

OBJECTIVE: To describe one of the largest series of pediatric patients with medically refractory epilepsy undergoing laser interstitial thermal therapy (LITT) or surgical resection of at least some portion of the insular cortex.

METHODS: Pediatric patients in whom invasive insular sampling confirmed insular involvement and who subsequently underwent a second stage surgery (LITT or open resection) were included. Complications and Engel Class outcomes at least 1 yr postsurgery were compiled as well as pathology results in the open surgical cases.

RESULTS: The average age in our cohort was 10.3 yr, 58% were male, and the average length of follow-up was 2.43 ± 0.20 (SEM) yr. A total of 14 patients underwent LITT, whereas 12 patients underwent open resection. Complications in patients undergoing either LITT or open resection were mostly minimal and generally transient. Forty-three percent of patients who underwent LITT were Engel Class I, compared to 50% of patients who underwent open insular resection.

CONCLUSION: Both surgical resection and LITT are valid management options in the treatment of medically refractory insular/opercular epilepsy in children. Although LITTmay be a less invasive alternative to craniotomy, further studies are needed to determine its noninferiority in terms of complication rates and seizure freedom, especially in cases of cortical dysplasia that may involve extensive regions of the brain.

 

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Papillary Tumor of the Pineal Region: Systematic Review and Analysis of Prognostic Factors

Neurosurgery, Volume 85, Issue 3, September 2019: E420–E429

Clinical outcomes and biological behavior of papillary tumors of the pineal region (PTPR) are still under investigation. The best therapeutic strategy has not been defined.

OBJECTIVE: To perform a comprehensive patient-level analysis of all PTPR cases and identify their clinical features, treatment options, and prognostic factors.

METHODS: A search of the medical databases for case series and reports on PTPRs from January 2003 to June 2017 was performed. Data addressing PTPR’s clinical presentation, imaging, treatment, and histological features were. Variables associated with the primary outcome of 36-mo survival were identified through Cox regression models.

RESULTS: The initial search yielded 1164 studies, of which 71 were included (60 case reports and 11 case series), containing 177 patients (mean age 33.0 ± 15.3 yr and 53.2% male). Intracranial hypertension and hydrocephalus prevailed as the clinical picture. Surgery was performed on 82.0% and gross total resection (GTR) was achieved on 71.4%. A total of 56.8% recurred after a median 29-mo (quartiles 10.5-45.5). The 36-mo survival rate was 83.5% (95% confidence interval [CI] 76.2-89.2%). Good functional outcomes (Glasgow Outcome Scale 4/5) were observed in 60.0%. The variables of interest were inconsistently reported and the multivariable analysis final sample was 133 patients. After adjustment for age, tumor size (each additional centimeter, hazard ratio [HR] 1.99, 95% CI 1.12-3.53, P = .019) and surgical treatment (HR 0.16, 95% CI 0.05-0.45, P = .001) were associated with 36-mo survival.

CONCLUSION: Tumor size and surgery are associated with improvement in 36-mo survival. We did not observe any significant benefits from GTR or adjuvant treatments.

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Pediatric Supratentorial Ependymoma: Surgical, Clinical, and Molecular Analysis

Neurosurgery 85:41–49, 2019

Pediatric supratentorial ependymomas (SEs) have distinct molecular and behavioral differences from their infratentorial counterparts.

OBJECTIVE: To present our experience with pediatric SEs over a 24-yr period.

METHODS: Clinical, operative, and radiographic information was abstracted retrospectively. Our primary outcomes were progression-free survival (PFS) and overall survival (OS). Detection of C11or f95-RELA rearrangement was performed using interphase fluorescence in situ hybridization (iFISH).

RESULTS: Seventy-three patients were identified (41 female, 32 male); median age was 6.7 yrs (range, 1 mo-18.8 yr); median follow-up was 8.3 yrs (range, 2.0-26.3). Fifty-eight (79.5%) of 73 patients underwent gross total resection (GTR); no patient with subtotal resection had greater than 1 cm3 of residual tumor; 42 patients (57.5%) experienced subsequent disease progression with 17 patients ultimately dying of their disease. Median PFS was 3.7 yrs. Molecular analysis was available for 51 patients (70%). On bivariate analysis, PFS and OS were not statistically affected by age, tumor grade, or extent of resection, although there was a clinically significant trend for the latter in favor of aggressive resection on PFS (P = .061). Children with RELA fusion had significantly higher PFS (P = .013) than those without, although there was no difference in OS when compared with those with no C11orf95-RELA fusion or C11orf95 gene rearrangement alone.

CONCLUSION: In our series, GTR may be associated with better PFS, but did not impact OS. Surprisingly, RELA fusion was not found to be a negative prognostic factor, raising the possibility that the deleterious effects may be overcome by aggressive resection.

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5-ALA fluorescence–guided surgery in pediatric brain tumors—a systematic review

Acta Neurochirurgica (2019) 161:1099–1108

5-Aminolevulinic acid (5-ALA)-guided resection of gliomas in adults enables better differentiation between tumor and normal brain tissue, allowing a higher degree of resection, and improves patient outcomes. In recent years, several reports have emerged regarding the use of 5-ALA in other brain tumor entities, including pediatric brains tumors. Since gross total resection (GTR) of many brain tumors in children is crucial and the role of 5-ALA-guided resection of these tumors is not clear, we sought to perform a comprehensive literature review on this topic.

Methods A systematic literature review of EMBASE and MEDLINE/PubMed databases revealed 19 eligible publications encompassing 175 5-ALA-guided operations on pediatric brain tumors. To prevent bias, publications were revised independently by two authors.

Results We found that 5-ALA-guided resection enabled the surgeons to identify the tumor more easily and was considered helpful mainly in cases of glioblastoma (GBM, 21/27, 78%), anaplastic ependymoma WHO grade III (10/14, 71%), and anaplastic astrocytoma (4/6, 67%). In contrast, cases of pilocytic astrocytomas (PAs) and medulloblastomas 5-ALA-guided surgery did not show consistent fluorescent signals and 5-ALA was considered helpful only in 12% and 22% of cases, respectively. Accumulation of fluorescent porphyrins seems to depend on WHO tumor grading. One important finding is that when 5- ALA-guided resections were considered helpful, the degree of resection was higher than is cases where it was not helpful. The rate of adverse events related to 5-ALA was negligible, especially new postoperative sequelae.

Conclusion 5-ALA could play a role in resection of pediatric brain tumors. However, further prospective clinical trials are needed.

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Robot-assisted stereoelectroencephalography in children

J Neurosurg Pediatr 23:288–296, 2019

The goal in the study was to describe the clinical outcomes associated with robot-assisted stereoelectroencephalography (SEEG) in children.

METHODS The authors performed a retrospective, single-center study in consecutive children with medically refractory epilepsy who were undergoing robot-assisted SEEG. Kaplan-Meier survival analysis was used to calculate the probability of seizure freedom. Both univariate and multivariate methods were used to analyze the preoperative and operative factors associated with seizure freedom.

RESULTS Fifty-seven children underwent a total of 64 robot-assisted procedures. The patients’ mean age was 12 years, an average of 6.4 antiepileptic drugs (AEDs) per patient had failed prior to implantation, and in 56% of the patients the disease was considered nonlesional. On average, children had 12.4 electrodes placed per implantation, with an implantation time of 9.6 minutes per electrode and a 10-day postoperative stay. SEEG analysis yielded a definable epileptogenic zone in 51 (89%) patients; 42 (74%) patients underwent surgery, half of whom were seizure free at last follow-up, 19.6 months from resection. In a multivariate generalized linear model, resective surgery, older age, and shorter SEEGrelated hospital length of stay were associated with seizure freedom. In a Cox proportional hazards model including only the children who underwent resective surgery, older age was the only significant factor associated with seizure freedom. Complications related to bleeding were the major contributors to morbidity. One patient (1.5%) had a symptomatic hemorrhage resulting in a permanent neurological deficit.

CONCLUSIONS The authors report one of the largest pediatric-specific SEEG series demonstrating that the modern surgical management of medically refractory epilepsy in children can lead to seizure freedom in many patients, while also highlighting the challenges posed by this difficult patient population.

 

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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.

Keywords

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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.

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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

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

helmet

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.

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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.

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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.

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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.

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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.

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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.

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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.

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Corridor-Based Endonasal Endoscopic Surgery for Pediatric Skull Base Pathology

CORRIDOR-BASED EEA IN PEDIATRIC PATIENTS

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.

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Antibiotic-impregnated shunts: effects in pediatric population

bactiseal2

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.

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