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Eric M. Thompson, Lissa C. Baird and Nathan R. Selden

Object

Growing concern about potential adverse effects of ionizing radiation exposure during imaging studies is particularly relevant to the pediatric population. To decrease radiation exposure, many institutions use rapid-sequence (or quick-brain) MRI to evaluate cerebral ventricle size. There are obstacles, however, to widespread implementation of this imaging modality. The purpose of this study was to define and quantify these obstacles to positively affect institutional and governmental policy.

Methods

A 9-question survey was emailed to pediatric neurosurgeons who were either members or candidate members of the American Society of Pediatric Neurosurgeons at every one of 101 institutions in the US and Canada having such a neurosurgeon on staff. Responses were compiled and descriptive statistics were performed.

Results

Fifty-six institutions completed the survey. Forty-four (79%) of the 56 institutions currently have a rapid-sequence MRI protocol to evaluate ventricle size, while 36 (64%) use it routinely. Of the 44 institutions with a rapid-sequence MRI protocol, 29 (66%) have had a rapid-sequence MRI protocol for less than 5 years while 39 (89%) have had a rapid-sequence MRI protocol for no more than 10 years. Thirty-six (88%) of 41 rapid-sequence MRI users responding to this question obtain a T2-weighted rapid-sequence MRI while 13 (32%) obtain a T1-weighted rapid-sequence MRI. Twenty-eight (64%) of 44 institutions never use sedation while an additional 12 (27%) rarely use sedation to obtain a rapid-sequence MRI (less than 5% of studies). Of the institutions with an established rapid-sequence MRI protocol, obstacles to routine use include lack of emergency access to MRI facilities in 18 (41%), lack of staffing of MRI facilities in 12 (27%), and the inability to reimburse a rapid-sequence MRI protocol in 6 (14%). In the 12 institutions without rapid-sequence MRI, obstacles to implementation include lack of emergency access to MRI facilities in 8 (67%), lack of staffing of MRI facilities in 7 (58%), the inability to reimburse in 3 (25%), and lack of administrative support in 3 (25%). To evaluate pediatric head trauma, 53 (96%) of 55 institutions responding to this question use noncontrast CT, no institution uses rapid-sequence MRI, and only 2 (4%) use standard MRI.

Conclusions

Many North American institutions have a rapid-sequence MRI protocol to evaluate ventricle size, with most developing this technique within the past 5 years. Most institutions never use sedation, and most obtain T2-weighted sequences. The greatest obstacles to the routine use of rapid-sequence MRI in institutions with and in those without a rapid-sequence MRI protocol are the lack of emergency access and staffing of the MRI facility during nights and weekends.

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Carolyn S. Quinsey, Katie Krause, Lissa C. Baird, Christina M. Sayama and Nathan R. Selden

OBJECTIVE

The relationship between a tethered cord (TC) and neurofibromatosis type 1 (NF1) and NF2 is not known. The purpose of this study was to define the incidence of TC in pediatric neurosurgical patients who present with NF.

METHODS

The authors performed a single-institution (tertiary care pediatric hospital) 10-year retrospective analysis of patients who were diagnosed with or who underwent surgery for a TC and/or NF. Clinical and radiological characteristics were analyzed, as was histopathology.

RESULTS

A total of 424 patients underwent surgery for a TC during the study period, and 67 patients with NF were seen in the pediatric neurosurgery clinic. Of these 67 patients, 9 (13%) were diagnosed with a TC, and filum lysis surgery was recommended. Among the 9 patients with NF recommended for TC-release surgery, 4 (44%) were female, the mean age was 8 years (range 4–14 years), the conus position ranged from L1–2 to L-3, and 3 (33%) had a filum lipoma, defined as high signal intensity on T1-weighted MR images. All 9 of these patients presented with neuromotor, skeletal, voiding, and/or pain-related symptoms. Histopathological examination consistently revealed dense fibroconnective tissue and blood vessels.

CONCLUSIONS

Despite the lack of any known pathophysiological relationship between NF and TC, the incidence of a symptomatic TC in patients with NF1 and NF2 who presented for any reason to this tertiary care pediatric neurosurgery clinic was 13%. Counseling patients and families regarding TC symptomatology might be indicated in this patient population.

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Paul Klimo Jr., Clinton J. Thompson, Lissa C. Baird and Ann Marie Flannery

Object

The objective of this systematic review and meta-analysis was to answer the following question: Are antibiotic-impregnated shunts (AISs) superior to standard shunts (SSs) at reducing the risk of shunt infection in pediatric patients with hydrocephalus?

Methods

Both the US National Library of Medicine PubMed/MEDLINE database and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words relevant to AIS use in children. Abstracts were reviewed, after which studies meeting the inclusion criteria were selected. An evidentiary table was assembled summarizing the studies and the quality of their evidence (Classes I–III). A meta-analysis was conducted using a random-effects model to calculate a cumulative estimate of treatment effect using risk ratio (RR). Heterogeneity was assessed using the chi-square and I2 statistics. Based on the quality of the literature and the result of the meta-analysis, a recommendation was rendered (Level I, II, or III).

Results

Six studies, all Class III, met our inclusion criteria. All but one study focused on a retrospective cohort and all but one were conducted at a single institution. Four of the studies failed to demonstrate a lowered infection rate with the use of an AIS. However, when the data from individual studies were pooled together, the infection rate in the AIS group was 5.5% compared with 8.6% in the SS group. Using a random-effects model, the cumulative RR was 0.51 (95% CI 0.29–0.89, p < 0.001), indicating that a shunt infection was 1.96 times more likely in patients who received an SS.

Conclusions

We recommend AIS tubing because of the associated lower risk of shunt infection compared to the use of conventional silicone hardware (quality of evidence: Class III; strength of recommendation: Level III).

Recommendation: Antibiotic-impregnated shunt (AIS) tubing may be associated with a lower risk of shunt infection compared with conventional silicone hardware and thus is an option for children who require placement of a shunt. Strength of Recommendation: Level III, unclear degree of clinical certainty.

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Lissa C. Baird, Catherine A. Mazzola, Kurtis I. Auguste, Paul Klimo Jr. and Ann Marie Flannery

Object

The objective of this systematic review was to examine the existing literature to compare differing shunt components used to treat hydrocephalus in children, find whether there is a superior shunt design for the treatment of pediatric hydrocephalus, and make evidence-based recommendations for the selection of shunt implants when placing shunts.

Methods

Both the US National Library of Medicine PubMed/MEDLINE database and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words chosen to identify publications comparing the use of shunt implant components. Abstracts of these publications were reviewed, after which studies meeting the inclusion criteria were selected. An evidentiary table was compiled summarizing the selected articles and quality of evidence. These data were then analyzed by the Pediatric Hydrocephalus Systematic Review and Evidence-Based Guidelines Task Force to consider evidence-based treatment recommendations.

Results

Two hundred sixty-nine articles were identified using the search parameters, and 43 articles were recalled for full-text review. Of these, 22 papers met the study criteria for a comparison of shunt components and were included in the evidentiary table. The included studies consisted of 1 Class I study, 11 Class II studies, and 10 Class III studies. The remaining 21 articles were excluded.

Conclusions

An analysis of the evidence did not demonstrate a clear advantage for any specific shunt component, mechanism, or valve design over another.

Recommendation: There is insufficient evidence to demonstrate an advantage for one shunt hardware design over another in the treatment of pediatric hydrocephalus. Current designs described in the evidentiary tables are all treatment options. Strength of Recommendation: Level I, high degree of clinical certainty.

Recommendation: There is insufficient evidence to recommend the use of a programmable valve versus a nonprogrammable valve. Programmable and nonprogrammable valves are both options for the treatment of pediatric hydrocephalus. Strength of Recommendation: Level II, moderate degree of clinical certainty.

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David D. Limbrick Jr., Lissa C. Baird, Paul Klimo Jr., Jay Riva-Cambrin and Ann Marie Flannery

Object

The objective of this systematic review was to examine the existing literature comparing CSF shunts and endoscopic third ventriculostomy (ETV) for the treatment of pediatric hydrocephalus and to make evidence-based recommendations regarding the selection of surgical technique for this condition.

Methods

Both the US National Library of Medicine and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words specifically chosen to identify published articles detailing the use of CSF shunts and ETV for the treatment of pediatric hydrocephalus. Articles meeting specific criteria that had been determined a priori were examined, and data were abstracted and compiled in evidentiary tables. These data were then analyzed by the Pediatric Hydrocephalus Systematic Review and Evidence-Based Guidelines Task Force to consider treatment recommendations based on the evidence.

Results

Of the 122 articles identified using optimized search parameters, 52 were recalled for full-text review. One additional article, originally not retrieved in the search, was also reviewed. Fourteen articles met all study criteria and contained comparative data on CSF shunts and ETV. In total, 6 articles (1 Class II and 5 Class III) were accepted for inclusion in the evidentiary table; 8 articles were excluded for various reasons. The tabulated evidence supported the evaluation of CSF shunts versus ETV.

Conclusions

Cerebrospinal fluid shunts and ETV demonstrated equivalent outcomes in the clinical etiologies studied.

Recommendation: Both CSF shunts and ETV are options in the treatment of pediatric hydrocephalus. Strength of Recommendation: Level II, moderate clinical certainty.

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Pawel P. Jankowski, Lissa C. Baird, Sassan Keshavarzi, Mary E. Goolsby, William R. Taylor and Andrew D. Nguyen

The authors describe here a unique case of contiguous, synchronous meningioma and lymphoma in the spinal column. Both tumors were present at the same vertebral level, one intradural and the other extradural. A patient presented with bilateral leg pain, acute weakness, and sensory loss in the lower extremities. Magnetic resonance imaging revealed an intradural mass at T6–7 with ambiguous boundaries relative to the thecal sac and compressing the spinal cord. The patient underwent resection of the epidural and intradural mass at T6–7. Histopathology revealed the epidural specimen to be a double-hit B-cell lymphoma and the intradural mass to be a transitional meningioma. Postoperatively, the patient did well, with an immediate return of strength and sensation. A postoperative MR image showed complete resection of the intradural mass. The authors suggest that biopsy may be prudent in patients with known systemic lymphoma presenting with a spinal lesion that has unclear boundaries relative to the thecal sac prior to commencing radiation and chemotherapy.

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Paul Klimo Jr., Mark Van Poppel, Clinton J. Thompson, Lissa C. Baird, Ann-Christine Duhaime and Ann Marie Flannery

Object

The objective of this systematic review and meta-analysis was twofold: to answer the question “What is the evidence for the effectiveness of prophylactic intravenous antibiotics for infection prevention in shunt surgery?” and to make treatment recommendations based on the available evidence.

Methods

The US National Library of Medicine PubMed/MEDLINE database and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words relevant to prophylactic antibiotic use in children undergoing a shunt operation. Abstracts were reviewed to identify which studies met the inclusion criteria. An evidentiary table was assembled summarizing the studies and the quality of evidence (Classes I-III). A meta-analysis was conducted using a random-effects model to calculate a cumulative estimate of treatment effect using risk ratio (RR). Heterogeneity was assessed using chi-square and I2 statistics. A sensitivity analysis was also conducted. Based on the quality of the literature and the result of the meta-analysis, a recommendation was rendered (Level I, II, or III).

Results

Nine studies (4 Class I, 3 Class II, and 2 Class III) met our inclusion criteria. Of 7 randomized controlled trials (RCTs), 3 were downgraded from Class I to Class II because of significant quality issues, and all RCTs were potentially underpowered. In only 2 Class in retrospective cohort studies were preoperative antibiotic agents found to be protective against shunt infection. When data from the individual studies were pooled together, the infection rate in the prophylactic antibiotics group was 5.9% compared with 10.7% in the control group. Using a random-effects model, the cumulative RR was 0.55 (95% CI 0.38–0.81), indicating a protective benefit of prophylactic preoperative intravenous antibiotics. A sensitivity analysis of RCTs only (n = 7) also demonstrated a statistical benefit, but an analysis of higher-quality RCTs only (n = 4) did not.

Conclusions

Within the limits of this systematic review and meta-analysis, administration of preoperative antibiotic agents for shunt surgery in children was found to lower the infection risk (quality of evidence: Class II; strength of recommendation, Level II).

Recommendation

The use of preoperative antibiotic agents can be recommended to prevent shunt infection in patients with hydrocephalus. It was only by combining the results of the various underpowered studies (meta-analysis) that the use of preoperative antibiotics for shunt surgery in children was shown to lower the risk of shunt infection. Strength of Recommendation: Level II, moderate degree of clinical certainty.

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Brandon G. Rocque, Bonita S. Agee, Eric M. Thompson, Mark Piedra, Lissa C. Baird, Nathan R. Selden, Stephanie Greene, Christopher P. Deibert, Todd C. Hankinson, Sean M. Lew, Bermans J. Iskandar, Taryn M. Bragg, David Frim, Gerald Grant, Nalin Gupta, Kurtis I. Auguste, Dimitrios C. Nikas, Michael Vassilyadi, Carrie R. Muh, Nicholas M. Wetjen and Sandi K. Lam

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.

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Ann Marie Flannery, Catherine A. Mazzola, Paul Klimo Jr., Ann-Christine Duhaime, Lissa C. Baird, Mandeep S. Tamber, David D. Limbrick Jr., Dimitrios C. Nikas, Joanna Kemp, Alexander F. Post, Kurtis I. Auguste, Asim F. Choudhri, Laura S. Mitchell and Debby Buffa

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Jennifer M. Strahle, Rukayat Taiwo, Christine Averill, James Torner, Chevis N. Shannon, Christopher M. Bonfield, Gerald F. Tuite, Tammy Bethel-Anderson, Jerrel Rutlin, Douglas L. Brockmeyer, John C. Wellons III, Jeffrey R. Leonard, Francesco T. Mangano, James M. Johnston, Manish N. Shah, Bermans J. Iskandar, Elizabeth C. Tyler-Kabara, David J. Daniels, Eric M. Jackson, Gerald A. Grant, Daniel E. Couture, P. David Adelson, Tord D. Alden, Philipp R. Aldana, Richard C. E. Anderson, Nathan R. Selden, Lissa C. Baird, Karin Bierbrauer, Joshua J. Chern, William E. Whitehead, Richard G. Ellenbogen, Herbert E. Fuchs, Daniel J. Guillaume, Todd C. Hankinson, Mark R. Iantosca, W. Jerry Oakes, Robert F. Keating, Nickalus R. Khan, Michael S. Muhlbauer, J. Gordon McComb, Arnold H. Menezes, John Ragheb, Jodi L. Smith, Cormac O. Maher, Stephanie Greene, Michael Kelly, Brent R. O’Neill, Mark D. Krieger, Mandeep Tamber, Susan R. Durham, Greg Olavarria, Scellig S. D. Stone, Bruce A. Kaufman, Gregory G. Heuer, David F. Bauer, Gregory Albert, Jeffrey P. Greenfield, Scott D. Wait, Mark D. Van Poppel, Ramin Eskandari, Timothy Mapstone, Joshua S. Shimony, Ralph G. Dacey Jr., Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.

OBJECTIVE

Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors’ goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.

METHODS

A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).

RESULTS

Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB–C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.

CONCLUSIONS

Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.