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

BACKGROUND: 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). .

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Management of Patients With Myelomeningocele: Whether Persistent Ventriculomegaly Adversely Impacts Neurocognitive Development.

BACKGROUND: Myelomeningocele (MM) is the most common congenital anomaly to affect the nervous system and affects 1500-2000 newborn infants per year in the United States. It is accompanied by symptomatic hydrocephalus in approximately 70%-80% of patients. Different treatment strategies for hydrocephalus characteristically result in different effects on the size of the ventricles. OBJECTIVE: The objective of this systematic review was to determine whether persistent ventricular enlargement adversely impacts neurocognitive development in patients with MM. METHODS: The PubMed National Library of Medicine Medline database and Embase were queried using MeSH headings and keywords relevant to neurocognitive or intellectual development and ventricular size or morphology. Abstracts were reviewed by the authors to identify which studies met strict inclusion criteria. An evidence table was constructed that summarized the included studies and reflected the quality of evidence (Classes I-III) that each represented. A recommendation was made that is based on the quality of the evidence. RESULTS: An initial abstract review utilizing strict inclusion/exclusion criteria yielded 48 studies, 9 of which underwent full-text review. There is limited and conflicting Class III evidence from 2 studies. CONCLUSION: Currently, there is insufficient data to conclude that ventricular size and morphology impact neurocognitive development.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-5.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Incidence of Tethered Cord Syndrome in Infants With Myelomeningocele With Prenatal Versus Postnatal Repair.

BACKGROUND: The incidence of spina bifida (SB) is higher in the developing world as compared to the United States because of folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible. OBJECTIVE: The objective of this guideline was to determine if there is a difference in the rate of development of tethered cord syndrome (TCS) in infants who had prenatal closure compared to infants who had MM repair after birth. METHODS: The Guidelines Task Force developed search terms and strategies to search PubMed and Embase for the relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used. Full text articles were reviewed and, when appropriate, included as evidence. RESULTS: A total of 261 abstracts were reviewed. Fifty-four full-text articles were selected for further analysis. Three studies met inclusion criteria. CONCLUSION: There was Class II evidence from 1 study and Class III evidence from another 2 studies demonstrating that TCS develops in infants with prenatal MM closure at an equal or higher rate than with postnatal closure. There was an increased risk of development of inclusion cysts in infants who underwent in utero closure. Continued surveillance for TCS and/or the development of inclusion cysts in children with prenatal and postnatal closure of MM is indicated (Level II). Differences between prenatal and postnatal repair with respect to the development of TCS and/or inclusion cysts should be considered alongside other relevant maternal and fetal outcomes when deciding upon a preferred method for MM closure.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-6.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Pediatric Myelomeningocele: Executive Summary.

BACKGROUND: The incidence of spina bifida (SB) in the developing world is higher than in the United States because of malnutrition and folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible. OBJECTIVE: The objective of the guidelines are, (1) To create clinical recommendations for best practices, based on a systematic review and analysis of available literature, (2) to obtain multi-disciplinary endorsement of these guidelines from relevant organizations, and (3) to disseminate the educational content to physicians to improve the care of infants with MM. METHODS: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. RESULTS: Guidelines authors aimed to systematically review the literature and make evidence based recommendations about the timing of closure after birth, hydrocephalus, the impact of prenatal closure, and the effect of prenatal closure on ambulation ability and tethered spinal cord. Evidence concerning persistent ventriculomegaly and cognitive impairment was also evaluated. Hundreds of abstracts were identified and reviewed for each of the 5 topics. A total of 14 studies met stringent inclusion criteria. CONCLUSION: Based on a comprehensive systematic review, a total of 5 clinical practice recommendations were developed, with 1 Level I, 2 Level II and 2 Level III recommendations.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-1.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Incidence of Shunt-Dependent Hydrocephalus in Infants With Myelomeningocele After Prenatal Versus Postnatal Repair.

BACKGROUND: Myelomeningocele (MM) is a condition that is responsible for considerable morbidity in the pediatric population. A significant proportion of the morbidity related to MM is attributable to hydrocephalus and the surgical management thereof. Postnatal repair remains the most common form of treatment; however, increased rates of prenatal diagnosis, advances in fetal surgery, and a hypothesis that neural injury continues in utero until the MM defect is repaired have led to the development and evaluation of prenatal surgery as a means to improve outcomes in afflicted infants. OBJECTIVE: The objective of this guideline is to systematically evaluate the literature to determine whether there is a difference in the proportion of patients who develop shunt-dependent hydrocephalus in infants who underwent prenatal MM repair compared to infants who had postnatal repair. METHODS: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full-text articles were then reviewed, and when appropriate, included as evidence. RESULTS: A total of 87 abstracts were identified and reviewed by 3 independent reviewers. Thirty-nine full-text articles were selected for analysis. Three studies met selection criteria and were included in the evidence table. CONCLUSION: Class I evidence from 1 study and class III evidence from 2 studies suggest that, in comparison to postnatal repair, prenatal surgery for MM reduces the risk of developing shunt-dependent hydrocephalus. Therefore, prenatal repair of MM is recommended for those fetuses who meet specific criteria for prenatal surgery to reduce the risk of developing shunt-dependent hydrocephalus (level I). Differences between prenatal and postnatal repair with respect to the requirement for permanent cerebrospinal fluid diversion should be considered alongside other relevant maternal and fetal factors when deciding upon a preferred method of MM closure. The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-2.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Management of Patients With Myelomeningocele: Whether Prenatal or Postnatal Closure Affects Future Ambulatory Status.

BACKGROUND: Myelomeningocele (MM) is an open neural tube defect treated by pediatric neurosurgeons with prenatal or postnatal closure. OBJECTIVE: The objective of this systematic review was to answer the question: What is the evidence for the effectiveness of prenatal vs postnatal closure of MM regarding short and long-term ambulatory status? Treatment recommendations were provided based on the available evidence. METHODS: The National Library of Medicine PubMed database and Embase were queried using MeSH headings and keywords relevant to ambulatory status after prenatal or postnatal closure of MM. Abstracts were reviewed to identify which studies met the inclusion criteria. An evidence table was assembled summarizing the studies and the quality of evidence (Classes I-III). Based on the quality of the literature, a recommendation was rendered (Level I, II, or III). RESULTS: One randomized controlled trial (Class II) and 3 retrospective cohort studies (Class III) were included as evidence. Initial ambulatory status depended on anatomic level of the neural tube defect. In the short term, prenatal closure may improve ambulatory status compared to postnatal closure. Spinal cord tethering or dermoid inclusion cyst has been associated with neurologic deterioration in infants closed in utero and after birth. Ambulation may cease in both groups over time. No long-term studies evaluated whether there is a difference in the ability to ambulate upon reaching adulthood. CONCLUSION: Prenatal closure of MM may improve ambulatory status in the short term (Level II). Spinal cord tethering in both groups caused deterioration in the ability to walk. Evaluation and treatment of spinal cord tethering may help maintain ambulatory status (Level III). No studies evaluate whether prenatal or postnatal repair provides improved ability to ambulate upon reaching adulthood.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-3.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on Closure of Myelomeningocele Within 48 Hours to Decrease Infection Risk.

BACKGROUND: Appropriate timing for closure of myelomeningocele (MM) varies in the literature. Older studies present 48 h as the timeframe after which infection complication rates rise. OBJECTIVE: The objective of this guideline is to determine if closing the MM within 48 h decreases the risk of wound infection or ventriculitis. METHODS: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full text articles were then reviewed and when appropriate, included in the evidentiary table. The class of evidence was evaluated, discussed, and assigned to each study that met inclusion criteria. RESULTS: A total of 148 abstracts were identified and reviewed. A total of 31 articles were selected for full text analysis. Only 4 of these studies met inclusion criteria. CONCLUSION: There is insufficient evidence that operating within 48 h decreases risk of wound infection or ventriculitis in 1 Class III study. There is 1 Class III study that provides evidence of global increase in postoperative infection after 48 h, but is not specific to wound infection or ventriculitis. There is 1 Class III study that provides evidence if surgery is going to be delayed greater than 48 h, antibiotics should be given.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-4.

Complications following pediatric cranioplasty after decompressive craniectomy: a multicenter retrospective study.

OBJECTIVE In children, the repair of skull defects arising from decompressive craniectomy presents a unique set of challenges. Single-center studies have identified different risk factors for the common complications of cranioplasty resorption and infection. The goal of the present study was to determine the risk factors for bone resorption and infection after pediatric cranioplasty. METHODS The authors conducted a multicenter retrospective case study that included all patients who underwent cranioplasty to correct a skull defect arising from a decompressive craniectomy at 13 centers between 2000 and 2011 and were less than 19 years old at the time of cranioplasty. Prior systematic review of the literature along with expert opinion guided the selection of variables to be collected. These included: indication for craniectomy; history of abusive head trauma; method of bone storage; method of bone fixation; use of drains; size of bone graft; presence of other implants, including ventriculoperitoneal (VP) shunt; presence of fluid collections; age at craniectomy; and time between craniectomy and cranioplasty. RESULTS A total of 359 patients met the inclusion criteria. The patients' mean age was 8.4 years, and 51.5% were female. Thirty-eight cases (10.5%) were complicated by infection. In multivariate analysis, presence of a cranial implant (primarily VP shunt) (OR 2.41, 95% CI 1.17-4.98), presence of gastrostomy (OR 2.44, 95% CI 1.03-5.79), and ventilator dependence (OR 8.45, 95% CI 1.10-65.08) were significant risk factors for cranioplasty infection. No other variable was associated with infection. Of the 240 patients who underwent a cranioplasty with bone graft, 21.7% showed bone resorption significant enough to warrant repeat surgical intervention. The most important predictor of cranioplasty bone resorption was age at the time of cranioplasty. For every month of increased age the risk of bone flap resorption decreased by 1% (OR 0.99, 95% CI 0.98-0.99, p < 0.001). Other risk factors for resorption in multivariate models were the use of external ventricular drains and lumbar shunts. CONCLUSIONS This is the largest study of pediatric cranioplasty outcomes performed to date. Analysis included variables found to be significant in previous retrospective reports. Presence of a cranial implant such as VP shunt is the most significant risk factor for cranioplasty infection, whereas younger age at cranioplasty is the dominant risk factor for bone resorption.

Pica in a Child with Anterior Cingulate Gyrus Oligodendroglioma: Case Report.

The anterior cingulate gyrus (ACG) is a continued focus of research as its exact role in brain function and vast connections with other anatomical locations is not fully understood. A review of the literature illustrates the role the ACG likely plays in cognitive and emotional processing, as well as a modulating role in motor function and goal-oriented behaviors. While lesions of the cingulate gyrus are rare, each new case broadens our understanding of its role in cognitive neuroscience and higher order processing. The authors present the case of an 8-year-old boy with a 1-month history of staring spells, agitated personality, and hyperphagia notable for the consumption of paper, who was found to have a 3-cm tumor in the left ACG. Following surgical resection of the tumor, his aggressive behavior and pica were ameliorated and the patient made an uneventful recovery, with no evidence of recurrence over the last 6 years since surgical resection. Here we discuss a unique behavioral presentation of pica, along with a review of the current literature, to illustrate functions of the ACG relevant to the location of the lesion.

Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 10: Change in ventricle size as a measurement of effective treatment of hydrocephalus.

OBJECT: The objective of this systematic review is to answer the following question: Does ventricle size after treatment have a predictive value in determining the effectiveness of surgical intervention in pediatric hydrocephalus? METHODS: The US National Library of Medicine PubMed/MEDLINE database and the Cochrane Database of Systematic Reviews were searched using MeSH headings and key words relevant to change in ventricle size after surgical intervention for hydrocephalus in children. An evidentiary table was assembled summarizing the studies and the quality of evidence (Classes I-III). RESULTS: Six articles satisfied inclusion criteria for the evidentiary tables for this part of the guidelines. All were Class III retrospective studies. CONCLUSIONS/RECOMMENDATIon: There is insufficient evidence to recommend a specific change in ventricle size as a measurement of the effective treatment of hydrocephalus and as a measurement of the timing and effectiveness of treatments including ventriculoperitoneal shunts and third ventriculostomies. STRENGTH OF RECOMMENDATION: Level III, unclear clinical certainty.

Coregistered intraoperative ultrasonography in resection of malignant glioma.

OBJECT: The authors present their experience with coregistration of preoperative imaging data to intraoperative ultrasonography in the resection of high-grade gliomas, focusing on methodology and clinical observation. METHODS: Images were obtained preoperatively and coregistered to intraoperative hand-held ultrasound images by merging the respective imaging coordinate systems. After patient registration and imaging calibration, the authors computed the location on the magnetic resonance (MR) space of each pixel on an ultrasound image acquired in the operating room. The data were retrospectively reviewed in 11 patients with high-grade gliomas who underwent ultrasonography-assisted resection at our institution between June 2000 and December 2002. Satisfactory coregistration of intraoperative ultrasound and preoperative MR images was accomplished in all cases. Ultrasound and MR image data were closely congruent. Preoperative setup and intraoperative use of the system were unencumbering. CONCLUSIONS: Based on these preliminary results, intraoperative ultrasonography is an attractive neuronavigational alternative, by which a less expensive and constraining imaging technique is used to acquire updated information. Optimal intraoperative guidance can be provided by the integration of this with other imaging studies.