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OBJECTIVE: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort. METHODS: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months. RESULTS: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013). CONCLUSIONS: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.
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OBJECTIVE: Cerebral revascularization surgery (CRS) has been used to prevent stroke in children with sickle cell disease (SCD) and cerebral vasculopathy (e.g., moyamoya syndrome). While results suggest that it may be an effective treatment, surgical indications have not been well defined. This study sought to determine indications for offering revascularization surgery in centers with established sickle cell programs in the US. METHODS: Three sequential surveys utilizing the Delphi methodology were administered to neurosurgeons participating in the Stroke in Sickle Cell Revascularization Surgery study. Respondents were presented with clinical scenarios of patients with SCD and varying degrees of ischemic presentation and vasculopathy, and the group's agreement to offer surgical revascularization was measured. Consensus was defined as ≥ 75% similar responses. RESULTS: The response rate to all 3 surveys was 100%. Seventeen neurosurgeons from 16 different centers participated. The presence of moyamoya collaterals (MMCs) and arterial stenosis matching an ischemic distribution yielded the strongest recommendations to offer surgery. There was consensus to offer revascularization in the presence of MMCs and at least 50% arterial stenosis matching an ischemic distribution. In contrast, there was no consensus to offer revascularization with 50%-70% stenosis not matching an ischemic presentation in the absence of MMCs. The presence of the ivy sign in the distribution of the stenotic artery also contributed to the consensus to offer surgery in certain scenarios. CONCLUSIONS: There were several clinical scenarios that attained consensus to offer surgery; the strongest was moderate to severe arterial stenosis that matched the distribution of ischemic presentation in the presence of MMCs. Radiological findings of decreased cerebral flow or perfusion also facilitated attaining consensus to offer surgery. The findings of this study reflect expert opinion about questions that deserve prospective clinical research. Determination of indications for CRS can guide clinical practice and aid the design of prospective studies.
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OBJECTIVE: The aim of this study was to compare clinical and craniometric outcomes of patients treated for hydrocephalus following fetal myelomeningocele repair (fMMR) via a ventriculoperitoneal shunt (VPS) or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC). METHODS: This was a retrospective cohort study of children who were treated for hydrocephalus following fMMR via VPS or ETV with or without CPC (ETV ± CPC) at Vanderbilt between 2012 and 2021. The primary outcomes were treatment failure and time to failure (TTF). Secondary outcomes included changes in hydrocephalus metrics (fronto-occipital horn ratio [FOHR] and head circumference measurements) and healthcare resource utilization (number of hospital admissions, clinic visits, and neuroimaging findings). RESULTS: Among 88 patients who underwent fMMR, 37 (42%) required permanent CSF diversion, of whom 19 received treatment at the authors' institution. Twelve patients underwent ETV ± CPC, and 7 underwent VPS placement at a median corrected age of 23 weeks versus 1 week (p = 0.002). The preoperative median head circumference percentiles and z-scores for patients in the ETV ± CPC cohort were similar to those of the VPS cohort (percentiles: 98.5 vs 94.0, p = 0.064; z-scores: 2.32 vs 1.60, p = 0.111). There was no difference in preoperative median FOHR measurements between the two cohorts (0.57 vs 0.59, p = 0.53). At 6 months postoperatively, the median head circumference percentile and z-score for the ETV ± CPC cohort remained similar between the two cohorts (percentiles: 98.0 vs 67.5, p = 0.315; z-scores: 2.12 vs 0.52, p = 0.307). There was no difference in the change in FOHR (-0.06 vs -0.09, p = 0.37) and change in head circumference percentile (-1.33 vs -28.6, p = 0.058) between the cohorts 6 months after the index CSF diversion procedure. One patient in the ETV ± CPC cohort experienced a seizure and a nonoperative subdural hemorrhage postoperatively; no other complications were observed. Six of the 7 patients in the VPS cohort required shunt revision with a median TTF of 9.8 months while 2 of the 12 ETV ± CPC patients required a repeat ETV at a median of 17.5 months (86% vs 17%, p = 0.013). The median number of hydrocephalus-related hospital readmissions was significantly lower in the ETV ± CPC cohort than in the VPS cohort (0 vs 1, p = 0.006). The ETV ± CPC cohort had fewer CT scans (0 vs 2, p = 0.004) and radiographs (0 vs 2, p < 0.001) than the VPS cohort. CONCLUSIONS: In a single-center cohort, hydrocephalic fMMR patients treated via ETV ± CPC remained shunt free, while a majority of patients receiving an upfront shunt required revision. This is the first study comparing ETV ± CPC with VPS in the fMMR hydrocephalus population. While larger, multicenter studies are needed, these results suggest that ETV/CPC may be a preferred means of CSF diversion following fMMR.
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OBJECTIVE: The Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study 1) to determine if a new, better-performing version of the Endoscopic Third Ventriculostomy Success Score (ETVSS) could be developed, 2) to explore the performance characteristics of the original ETVSS in a modern endoscopic third ventriculostomy (ETV) cohort, and 3) to determine if the addition of radiological variables to the ETVSS improved its predictive abilities. METHODS: From April 2008 to August 2019, children (corrected age ≤ 17.5 years) who underwent a first-time ETV for hydrocephalus were included in a prospective multicenter HCRN study. All children had at least 6 months of clinical follow-up and were followed since the index ETV in the HCRN Core Data Registry. Children who underwent choroid plexus cauterization were excluded. Outcome (ETV success) was defined as the lack of ETV failure within 6 months of the index procedure. Kaplan-Meier curves were constructed to evaluate time-dependent variables. Multivariable binary logistic models were built to evaluate predictors of ETV success. Model performance was evaluated with Hosmer-Lemeshow and Harrell's C statistics. RESULTS: Seven hundred sixty-one children underwent a first-time ETV. The rate of 6-month ETV success was 76%. The Hosmer-Lemeshow and Harrell's C statistics of the logistic model containing more granular age and etiology categorizations did not differ significantly from a model containing the ETVSS categories. In children ≥ 12 months of age with ETVSSs of 50 or 60, the original ETVSS underestimated success, but this analysis was limited by a small sample size. Fronto-occipital horn ratio (p = 0.37), maximum width of the third ventricle (p = 0.39), and downward concavity of the floor of the third ventricle (p = 0.63) did not predict ETV success. A possible association between the degree of prepontine adhesions on preoperative MRI and ETV success was detected, but this did not reach statistical significance. CONCLUSIONS: This modern, multicenter study of ETV success shows that the original ETVSS continues to demonstrate good predictive ability, which was not substantially improved with a new success score. There might be an association between preoperative prepontine adhesions and ETV success, and this needs to be evaluated in a future large prospective study.
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Hidrocefalia , Tercer Ventrículo , Ventriculostomía , Humanos , Ventriculostomía/métodos , Hidrocefalia/cirugía , Hidrocefalia/diagnóstico por imagen , Femenino , Masculino , Tercer Ventrículo/cirugía , Tercer Ventrículo/diagnóstico por imagen , Niño , Preescolar , Estudios Prospectivos , Lactante , Resultado del Tratamiento , Adolescente , Neuroendoscopía/métodos , Estudios de SeguimientoRESUMEN
OBJECTIVE: Knowledge-based tools used to standardize perioperative care, such as the shunt infection prevention protocol of the Hydrocephalus Clinical Research Network (HCRN), have demonstrated their ability to reduce surgeon-based and center-based variations in outcomes and improve patient care. The mere presence of high-quality evidence, however, does not necessarily translate into improved patient outcomes owing to the implementation gap. To advance understanding of how knowledge-based tools are being utilized in the routine clinical care of children with hydrocephalus, the HCRN-Quality (HCRNq) network was started in 2019. With a focus on CSF shunt infection, the authors present baseline data regarding CSF shunt infection rates and current shunt infection prevention practices in use at HCRNq sites. METHODS: Baseline shunt surgery practices, infection rate, and risk factor data were prospectively collected within HCRNq. No standard infection protocol was recommended, but site use of a protocol was implied if at least 3 of 6 common shunt infection prevention practices were used in > 80% of shunt surgical procedures. Univariable and multivariable analyses of shunt infection risk factors were performed. RESULTS: Thirty sites accrued data on 2437 procedures between November 2019 and June 2021. The unadjusted infection rate across all sites was 3.9% (range 0%-13%) and did not differ among shunt insertion, shunt revision, or shunt insertion after infection. Protocol use was implied for only 15/30 centers and 60% of shunt operations. On univariable analysis, iodine/DuraPrep (OR 0.57, 95% CI 0.37-0.88, p = 0.02) and the use of an antibiotic-impregnated catheter in any segment of the shunt (or both) decreased infection risk (OR 0.53, 95% CI 0.34-0.82, p = 0.01). Iodine-based prep solutions (OR 0.56, 95% 0.36-0.86, p = 0.02) and the use of antibiotic-impregnated catheters (OR 0.52, 95% CI 0.34-0.81, p = 0.01) retained significance in the multivariable model, but no relationship between protocol use and infection risk was demonstrated in this baseline analysis. CONCLUSIONS: The authors have demonstrated that children undergoing CSF shunt surgery at HCRNq sites share similar demographic characteristics with other large North American multicenter cohorts, with similar observed baseline infection rates and risk factors. Many centers have implemented standardized shunt infection prevention practices, but considerable practice variation remains. As such, there is an opportunity to decrease shunt infection rates in these centers through continued standardization of care.
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Hidrocefalia , Yodo , Niño , Humanos , Lactante , Antibacterianos/uso terapéutico , Derivaciones del Líquido Cefalorraquídeo/efectos adversos , Hidrocefalia/etiología , Mejoramiento de la Calidad , Estudios ProspectivosRESUMEN
OBJECTIVE: Understanding the impact of the social determinants of health on the utilization of healthcare resources is an important step in eliminating inequalities. The goal of this study was to determine the role of social determinants of health in referral patterns, timing of consultation/intervention, and quality of life in children with Chiari malformation type I (CM-I). METHODS: A retrospective study was conducted of children aged 0 to 18 years who underwent surgical treatment for CM-I at a single pediatric facility from 2015 to 2019. The variables included demographic and socioeconomic characteristics, referral patterns, timing, and quality of life data based on the Chiari Health Index for Pediatrics (CHIP). RESULTS: The cohort consisted of 103 surgically treated CM-I patients. No differences were seen in race, sex, insurance, or household income when evaluating referral source (community, specialist, or emergency department) or when comparing patients with incidental versus symptomatic findings. In the evaluation of timing from initial evaluation to surgery, no statistical differences were seen between racial, sex, insurance status, or income groups. Children from households of lower median family income were significantly more likely to report pain at the time of consultation (pain group median [interquartile range] $46,660 [$41,004-$50,367] vs nonpain group $53,604 [$41,427-$59,828], p = 0.004). Those in the lower-income group also reported lower CHIP scores corresponding to increased symptomatology in the nonpain physical symptoms (p = 0.004) and psychosocial domains (p = 0.018). CONCLUSIONS: There was no evidence of a difference in referral patterns or a delay in time from clinic presentation to surgery based on the traditional social determinants of health categories. Children from households in the lower-income group were associated with increased severity of pain and nonpain symptoms.
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Malformación de Arnold-Chiari , Niño , Humanos , Estudios Retrospectivos , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/complicaciones , Calidad de Vida , Determinantes Sociales de la Salud , Derivación y Consulta , Dolor/complicacionesRESUMEN
This essay describes a care team and family rallying around a young girl facing what seem to be insurmountable odds.
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OBJECTIVE: Abdominal pseudocyst (APC) can cause distal site failure in children with ventriculoperitoneal shunts and is specifically designated as an infection in Hydrocephalus Clinical Research Network (HCRN) protocols. Specific management and outcomes of children with APCs have not been reported in a multicenter study. In this study, the authors investigated the management and outcomes of APC in children with shunted hydrocephalus who were treated at centers in the HCRN. METHODS: The HCRN Registry was queried to identify children < 18 years old with shunts who were diagnosed with an APC (i.e., a loculated abdominal fluid collection containing the peritoneal catheter with abdominal distention and/or displacement of peritoneal contents). The primary outcome was shunt failure after APC treatment. The primary variable was reimplantation of the distal catheter after pseudocyst treatment back into the peritoneum versus implantation in a nonperitoneal site. Other risk factors for shunt failure after APC treatment and variability in APC management were investigated. RESULTS: Among 141 children from 14 centers who underwent first-time management of an APC over a 14-year period, the median time from previous shunt surgery to APC diagnosis was 3.8 months. Overall, 17.7% of children had a positive culture: APC cultures were positive in 14.2% and CSF cultures in 15.6%. Six other children underwent shunt revision without removal; all underwent reoperation within 1 month. There was no difference in shunt survival (log-rank test, p = 0.42) or number of subsequent revisions within 6, 12, or 24 months for shunts reimplanted in the abdomen versus those implanted in a nonperitoneal location. Nonperitoneal implantation was associated with more noninfectious revisions (42.3% vs 22.9%, p = 0.019), whereas infection was more common after reimplantation in the abdomen (25.7% vs 7.0%, p = 0.003). Univariable analysis demonstrated that younger age at APC diagnosis (8.3 vs 12.2 years, p = 0.006) and prior shunt procedure within 12 weeks of APC diagnosis (59.5% vs 40.5%, p = 0.012) were associated with shunt failure after APC treatment. Multivariable modeling confirmed that prior shunt surgery within 12 weeks of APC diagnosis was independently associated with failure (HR 1.79 [95% CI 1.04-3.07], p = 0.035). CONCLUSIONS: In the HCRN, APCs in the setting of CSF shunts are usually managed with externalization. Shunt surgery within 12 weeks of APC diagnosis was associated with risk of failure after APC treatment. Although no differences were found in overall shunt failure rate, noninfectious shunt revisions were more common in the nonperitoneal distal catheter sites, and infection was a more common reason for failure after reimplantation of the shunt in the abdomen.
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Quistes , Hidrocefalia , Humanos , Niño , Lactante , Adolescente , Abdomen/cirugía , Derivación Ventriculoperitoneal/efectos adversos , Procedimientos Neuroquirúrgicos/efectos adversos , Hidrocefalia/cirugía , Hidrocefalia/complicaciones , Quistes/etiología , Reoperación , Derivaciones del Líquido Cefalorraquídeo/efectos adversosRESUMEN
OBJECTIVE: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT. METHODS: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF). RESULTS: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort. CONCLUSIONS: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion.
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Hidrocefalia , Neoplasias Infratentoriales , Neuroendoscopía , Niño , Humanos , Ventriculostomía/efectos adversos , Neuroendoscopía/efectos adversos , Derivación Ventriculoperitoneal/efectos adversos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Hidrocefalia/epidemiología , Resultado del Tratamiento , Neoplasias Infratentoriales/complicaciones , Neoplasias Infratentoriales/cirugía , Estudios RetrospectivosRESUMEN
OBJECTIVE: The focus of this modified Delphi study was to investigate and build consensus regarding the medical management of children with moderate and severe acute spinal cord injury (SCI) during their initial inpatient hospitalization. This impetus for the study was based on the AANS/CNS guidelines for pediatric SCI published in 2013, which indicated that there was no consensus provided in the literature describing the medical management of pediatric patients with SCIs. METHODS: An international, multidisciplinary group of 19 physicians, including pediatric neurosurgeons, orthopedic surgeons, and intensivists, were asked to participate. The authors chose to include both complete and incomplete injuries with traumatic as well as iatrogenic etiologies (e.g., spinal deformity surgery, spinal traction, intradural spinal surgery, etc.) due to the overall low incidence of pediatric SCI, potentially similar pathophysiology, and scarce literature exploring whether different etiologies of SCI should be managed differently. An initial survey of current practices was administered, and based on the responses, a follow-up survey of potential consensus statements was distributed. Consensus was defined as ≥ 80% of participants reaching agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A final meeting was held virtually to generate final consensus statements. RESULTS: Following the final Delphi round, 35 statements reached consensus after modification and consolidation of previous statements. Statements were categorized into the following eight sections: inpatient care unit, spinal immobilization, pharmacological management, cardiopulmonary management, venous thromboembolism prophylaxis, genitourinary management, gastrointestinal/nutritional management, and pressure ulcer prophylaxis. All participants stated that they would be willing or somewhat willing to change their practices based on consensus guidelines. CONCLUSIONS: General management strategies were similar for both iatrogenic (e.g., spinal deformity, traction, etc.) and traumatic SCIs. Steroids were recommended only for injury after intradural surgery, not after acute traumatic or iatrogenic extradural surgery. Consensus was reached that mean arterial pressure ranges are preferred for blood pressure targets following SCI, with goals between 80 and 90 mm Hg for children at least 6 years of age. Further multicenter study of steroid use following acute neuromonitoring changes was recommended.
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OBJECTIVES: The acute cerebral physiologic effects of ketamine in children have been incompletely described. We assessed the acute effects of ketamine on intracranial pressure (ICP) and cerebral perfusion pressure (CPP) in children with severe traumatic brain injury (TBI). DESIGN: In this retrospective observational study, patients received bolus doses of ketamine for sedation or as a treatment for ICP crisis (ICP > 20 mm Hg for > 5 min). Administration times were synchronized with ICP and CPP recordings at 1-minute intervals logged in an automated database within the electronic health record. ICP and CPP were each averaged in epochs following drug administration and compared with baseline values. Age-based CPP thresholds were subtracted from CPP recordings and compared with baseline values. Trends in ICP and CPP over time were assessed using generalized least squares regression. SETTING: A 30-bed tertiary care children's hospital PICU. PATIENTS: Children with severe TBI who underwent ICP monitoring. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We analyzed data from 33 patients, ages 1 month to 16 years, 22 of whom received bolus doses of ketamine, with 127 doses analyzed. Demographics, patient, and injury characteristics were similar between patients who did versus did not receive ketamine boluses. In analysis of the subset of ketamine doses used only for sedation, there was no significant difference in ICP or CPP from baseline. Eighteen ketamine doses were given during ICP crises in 11 patients. ICP decreased following these doses and threshold-subtracted CPP rose. CONCLUSIONS: In this retrospective, exploratory study, ICP did not increase following ketamine administration. In the setting of a guidelines-based protocol, ketamine was associated with a reduction in ICP during ICP crises. If these findings are reproduced in a larger study, ketamine may warrant consideration as a treatment for intracranial hypertension in children with severe TBI.
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Lesiones Traumáticas del Encéfalo , Hipertensión Intracraneal , Ketamina , Humanos , Niño , Ketamina/farmacología , Ketamina/uso terapéutico , Estudios Retrospectivos , Presión Intracraneal/fisiología , Circulación Cerebrovascular , Lesiones Traumáticas del Encéfalo/complicaciones , Lesiones Traumáticas del Encéfalo/tratamiento farmacológico , Hipertensión Intracraneal/tratamiento farmacológico , Hipertensión Intracraneal/etiologíaRESUMEN
The current nomenclature of Chiari malformations includes the standard designations, Chiari 1-4, which were described by Hans Chiari in the late nineteenth century, and more recent additions, Chiari 0, 0.5, and 1.5, which emerged when the standard nomenclature failed to include important anatomical variations. The authors describe these entities and propose that to best optimize clinical care and research, it would be wise to place less focus on the eponyms and more effort on developing a descriptive or pathophysiological nomenclature.
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Malformación de Arnold-Chiari , HumanosRESUMEN
OBJECTIVE: The objective of this study was to determine the effects of in utero bipedicle flaps on maternal-fetal morbidity/mortality, the need for CSF diversion, and long-term functional outcomes. METHODS: Eighty-six patients who underwent fetal myelomeningocele repair from 2011 to 2021 at a single institution were reviewed. Primary outcomes included intrauterine fetal demise, postnatal death, postnatal myelomeningocele repair dehiscence, and CSF diversion by final follow-up. RESULTS: The cohorts were no different with regard to race, ethnicity, maternal age at fetal surgery, body mass index, gravidity, parity, gestational age at fetal surgery, estimated fetal weight at fetal surgery, or fetal lesion level. Of the 86 patients, 64 underwent primary linear repair and 22 underwent bipedicle flap repair. There were no significant differences in rates of intrauterine fetal demise, postnatal mortality, midline repair site dehiscence, or the need for CSF diversion by final follow-up. Operative times were longer (32.5 vs 18.7 minutes, p < 0.001) and gestational age at delivery was lower (232 vs 241 days, p = 0.01) in the bipedicle flap cohort, but long-term functional outcomes were not different. CONCLUSIONS: Analysis of the total cohort affirms the long-term benefits of fetal myelomeningocele repair. In utero bipedicle flaps are safe and can be used for high-tension lesions without increasing perioperative risks to the mother or fetus. In utero flaps preserve the long-term benefits seen with primary linear repair and may expand inclusion criteria for fetal repair, providing life-changing care for more patients.
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Meningomielocele , Embarazo , Femenino , Humanos , Meningomielocele/cirugía , Estudios de Cohortes , Estudios de Seguimiento , Feto/cirugía , Muerte FetalRESUMEN
INTRODUCTION: Intrauterine myelomeningocele repair (IUMR) and postnatal myelomeningocele repair (PNMR) differ in terms of both setting and surgical technique. A simplified technique in IUMR, in which a dural onlay is used followed by skin closure, has been adopted at our institution. The goal of this study was to compare the rates of clinical tethering in IUMR and PNMR patients, as well as to evaluate the appearance on MRI. METHODS: We conducted a retrospective review of 36 patients with MMC repaired at our institution, with 2:1 PNMR to IUMR matching based on lesion level. A pediatric neuroradiologist blinded to the clinical details reviewed the patients' lumbar spine MRIs for the distance from neural tissue to skin and the presence or absence of a syrinx. An EMR review was then done to evaluate for detethering procedures and need for CSF diversion. RESULTS: Mean age at MRI was 4.0 years and mean age at last follow-up was 6.1 years, with no significant difference between the PNMR and IUMR groups. There was no significant difference between groups in the distance from neural tissue to skin (PNMR 13.5 mm vs IUMR 17.6 mm; p = 0.5). There was no difference in need for detethering operations between groups (PNMR 12.5% vs IUMR 16.7%; RR 0.75; CI 0.1-5.1). CONCLUSIONS: There was no significant difference between postnatal- and intrauterine-repaired myelomeningocele on MRI or in need for detethering operations. These results imply that a more straightforward and time-efficient IUMR closure technique does not lead to an increased rate of tethering when compared to the multilayered PNMR.
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Meningomielocele , Siringomielia , Humanos , Niño , Preescolar , Meningomielocele/diagnóstico por imagen , Meningomielocele/cirugía , Estudios de Cohortes , Estudios Retrospectivos , Imagen por Resonancia MagnéticaRESUMEN
OBJECTIVE: Long-term follow-up is often recommended for patients with hydrocephalus, but the frequency of clinical follow-up, timing and modality of imaging, and duration of surveillance have not been clearly defined. Here, the authors used the modified Delphi method to identify areas of consensus regarding the modality, frequency, and duration of hydrocephalus surveillance following surgical treatment. METHODS: Pediatric neurosurgeons serving as institutional liaisons to the Hydrocephalus Clinical Research Network (HCRN), or its implementation/quality improvement arm (HCRNq), were invited to participate in this modified Delphi study. Thirty-seven consensus statements were generated and distributed via an anonymous electronic survey, with responses structured as a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A subsequent, virtual meeting offered the opportunity for open discussion and modification of the statements in an effort to reach consensus (defined as ≥ 80% agreement or disagreement). RESULTS: Nineteen pediatric neurosurgeons participated in the first round, after which 15 statements reached consensus. During the second round, 14 participants met virtually for review and discussion. Some statements were modified and 2 statements were combined, resulting in a total of 36 statements. At the conclusion of the session, consensus was achieved for 17 statements regarding the following: 1) the role of standardization; 2) preferred imaging modalities; 3) postoperative follow-up after shunt surgery (subdivided into immediate postoperative imaging, delayed postoperative imaging, routine clinical surveillance, and routine radiological surveillance); and 4) postoperative follow-up after an endoscopic third ventriculostomy. Consensus could not be achieved for 19 statements. CONCLUSIONS: Using the modified Delphi method, 17 consensus statements were developed with respect to both clinical and radiological follow-up after a shunt or endoscopic third ventriculostomy. The frequency, modality, and duration of surveillance were addressed, highlighting areas in which no clear data exist to guide clinical practice. Although further studies are needed to evaluate the clinical utility and cost-effectiveness of hydrocephalus surveillance, the current study provides a framework to guide future efforts to develop standardized clinical protocols for the postoperative surveillance of patients with hydrocephalus. Ultimately, the standardization of hydrocephalus surveillance has the potential to improve patient care as well as optimize the use of healthcare resources.
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OBJECTIVE: Endoscopic third ventriculostomy (ETV) is an option for treatment of hydrocephalus, including for patients who have a history of previous treatment with CSF shunt insertion. The purpose of this study was to report the success of postshunt ETV by using data from a multicenter prospective registry. METHODS: Prospectively collected data in the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (i.e., HCRN Registry) were reviewed. Children who underwent ETV between 2008 and 2019 and had a history of previous treatment with a CSF shunt were included. A Kaplan-Meier survival curve was created for the primary outcome: time from postshunt ETV to subsequent CSF shunt placement or revision. Univariable Cox proportional hazards models were created to evaluate for an association between clinical and demographic variables and subsequent shunt surgery. Postshunt ETV complications were also identified and categorized. RESULTS: A total of 203 children were included: 57% male and 43% female; 74% White, 23% Black, and 4% other race. The most common hydrocephalus etiologies were postintraventricular hemorrhage secondary to prematurity (56, 28%) and aqueductal stenosis (42, 21%). The ETV Success Score ranged from 10 to 80. The median patient age was 4.1 years. The overall success of postshunt ETV at 6 months was 41%. Only the surgeon's report of a clear view of the basilar artery was associated with a lower likelihood of postshunt ETV failure (HR 0.43, 95% CI 0.23-0.82, p = 0.009). None of the following variables were associated with postshunt ETV success: age at the time of postshunt ETV, etiology of hydrocephalus, sex, race, ventricle size, number of previous shunt operations, ETV performed at time of shunt infection, and use of external ventricular drainage. Overall, complications were reported in 22% of patients, with CSF leak (8.6%) being the most common complication. CONCLUSIONS: Postshunt ETV was successful in treating hydrocephalus, without subsequent need for a CSF shunt, in 41% of patients, with a clear view of the basilar artery being the only variable significantly associated with success. Complications occurred in 22% of patients. ETV is an option for treatment of hydrocephalus in children who have previously undergone shunt placement, but with a lower than expected likelihood of success.
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OBJECTIVE: Primary treatment of hydrocephalus with endoscopic third ventriculostomy (ETV) and choroid plexus cauterization (CPC) is well described in the neurosurgical literature, with wide reported ranges of success and complication rates. The purpose of this study was to describe the safety and efficacy of ETV revision after initial ETV+CPC failure. METHODS: Prospectively collected data in the Hydrocephalus Clinical Research Network Core Data Project registry were reviewed. Children who underwent ETV+CPC as the initial treatment for hydrocephalus between 2013 and 2019 and in whom the initial ETV+CPC was completed (i.e., not abandoned) were included. Log-rank survival analysis (the primary analysis) was used to compare time to failure (defined as any other surgical treatment for hydrocephalus or death related to hydrocephalus) of initial ETV+CPC versus that of ETV revision by using random-effects modeling to account for the inclusion of patients in both the initial and revision groups. Secondary analysis compared ETV revision to shunt placement after failure of initial ETV+CPC by using the log-rank test, as well as shunt failure after ETV+CPC to that after ETV revision. Cox regression analysis was used to identify predictors of failure among children treated with ETV revision. RESULTS: The authors identified 521 ETV+CPC procedures that met their inclusion criteria. Ninety-one children underwent ETV revision after ETV+CPC failure. ETV revision had a lower 1-year success rate than initial ETV+CPC (29.5% vs 45%, p < 0.001). ETV revision after initial ETV+CPC failure had a lower success rate than shunting (29.5% vs 77.8%, p < 0.001). Shunt survival after initial ETV+CPC failure was not significantly different from shunt survival after ETV revision failure (p = 0.963). Complication rates were similar for all examined surgical procedures (initial ETV+CPC, ETV revision, ventriculoperitoneal shunt [VPS] placement after ETV+CPC, and VPS placement after ETV revision). Only young age was predictive of ETV revision failure (p = 0.02). CONCLUSIONS: ETV revision had a significantly lower 1-year success rate than initial ETV+CPC and VPS placement after ETV+CPC. Complication rates were similar for all studied procedures. Younger age, but not time since initial ETV+CPC, was a risk factor for ETV revision failure.
RESUMEN
OBJECTIVE: Two previous Hydrocephalus Clinical Research Network (HCRN) studies have demonstrated that compliance with a standardized CSF shunt infection protocol reduces shunt infections. In this third iteration, a simplified protocol consisting of 5 steps was implemented. This analysis provides an updated evaluation of protocol compliance and evaluates modifiable shunt infection risk factors. METHODS: The new simplified protocol was implemented at HCRN centers on November 1, 2016, for all shunt procedures, excluding external ventricular drains, ventricular reservoirs, and subgaleal shunts. Procedures performed through December 31, 2019, were included (38 months). Compliance with the protocol, use of antibiotic-impregnated catheters (AICs), and other variables of interest were collected at the index operation. Outcome events for a minimum of 6 months postoperatively were recorded. The definition of infection was unchanged from the authors' previous report. RESULTS: A total of 4913 procedures were performed at 13 HCRN centers. The overall infection rate was 5.1%. Surgeons were compliant with all 5 steps of the protocol in 79.4% of procedures. The infection rate for the protocol alone was 8.1% and dropped to 4.9% when AICs were added. Multivariate analysis identified having ≥ 2 complex chronic conditions (odds ratio [OR] 1.76, 95% confidence interval [CI] 1.26-2.44, p = 0.01) and a history of prior shunt surgery within 12 weeks (OR 1.84, 95% CI 1.37-2.47, p < 0.01) as independent risk factors for shunt infection. The use of AICs (OR 0.70, 95% CI 0.50-0.97, p = 0.05) and vancomycin irrigation (OR 0.36, 95% CI 0.21-0.62, p < 0.01) were identified as independent factors protective against shunt infection. CONCLUSIONS: The authors report the third iteration of their quality improvement protocol to reduce the risk of shunt infection. Compliance with the protocol was high. These updated data suggest that the incorporation of AICs is an important, modifiable infection prevention measure. Vancomycin irrigation was also identified as a protective factor but requires further study to better understand its role in preventing shunt infection.
Asunto(s)
Antibacterianos , Hidrocefalia , Humanos , Lactante , Antibacterianos/uso terapéutico , Vancomicina , Mejoramiento de la Calidad , Derivaciones del Líquido Cefalorraquídeo/efectos adversos , Catéteres/efectos adversos , Infección de la Herida Quirúrgica/etiología , Hidrocefalia/etiologíaRESUMEN
Children with acute neurologic illness are at high risk of mortality and long-term neurologic disability. Severe traumatic brain injury, cardiac arrest, stroke, and central nervous system infection are often complicated by cerebral hypoxia, hypoperfusion, and edema, leading to secondary neurologic injury and worse outcome. Owing to the paucity of targeted neuroprotective therapies for these conditions, management emphasizes close physiologic monitoring and supportive care. In this review, we will discuss advanced neurologic monitoring strategies in pediatric acute neurologic illness, emphasizing the physiologic concepts underlying each tool. We will also highlight recent innovations including novel monitoring modalities, and the application of neurologic monitoring in critically ill patients at risk of developing neurologic sequelae.