Aortic Valve Thrombus, Stroke, and Endovascular Thrombectomy in a Child With APML and Trisomy 21.

APML, a subtype of acute myeloid leukemia, is highly curable, with cure rates over 90%. Despite its therapeutic success, APML poses elevated bleeding risks due to frequent prior disseminated intravascular coagulation. Less commonly recognized but critical is the thrombotic risk. We document a unique pediatric case: a 13-year-old with trisomy 21 diagnosed with APML had an asymptomatic aortic valve thrombus leading to thromboembolic arterial ischemic stroke. Through endovascular thrombectomy, cerebral circulation was re-established, extracting a fibrin thrombus with APML cells. Neurological recovery was swift. This report underscores the importance of vigilance for thrombotic complications in APML, highlighting the potential severity of overlooked risks.

Poor postoperative pain control is associated with poor long-term patient-reported outcomes after elective spine surgery: an observational cohort study.

BACKGROUND CONTEXT: A significant proportion of patients experience poorly controlled surgical pain and fail to achieve satisfactory clinical improvement after spine surgery. However, a direct association between these variables has not been previously demonstrated. PURPOSE: To investigate the association between poor postoperative pain control and patient-reported outcomes after spine surgery. STUDY DESIGN: Ambispective cohort study. PATIENT SAMPLE: Consecutive adult patients (≥18-years old) undergoing inpatient elective cervical or thoracolumbar spine surgery. OUTCOME MEASURE: Poor surgical outcome was defined as failure to achieve a minimal clinically important difference (MCID) of 30% improvement on the Oswestry Disability Index or Neck Disability Index at follow-up (3-months, 1-year, and 2-years). METHODS: Poor pain control was defined as a mean numeric rating scale score of >4 during the first 24-hours after surgery. Multivariable mixed-effects regression was used to investigate the relationship between poor pain control and changes in surgical outcomes while adjusting for known confounders. Secondarily, the Calgary Postoperative Pain After Spine Surgery (CAPPS) Score was investigated for its ability to predict poor surgical outcome. RESULTS: Of 1294 patients, 47.8%, 37.3%, and 39.8% failed to achieve the MCID at 3-months, 1-year, and 2-years, respectively. The incidence of poor pain control was 56.9%. Multivariable analyses showed poor pain control after spine surgery was independently associated with failure to achieve the MCID (OR 2.35 [95% CI=1.59-3.46], p<.001) after adjusting for age (p=.18), female sex (p=.57), any nicotine products (p=.041), ASA physical status >2 (p<.001), ≥3 motion segment surgery (p=.008), revision surgery (p=.001), follow-up time (p<.001), and thoracolumbar surgery compared to cervical surgery (p=.004). The CAPPS score was also found to be independently predictive of poor surgical outcome. CONCLUSION: Poor pain control in the first 24-hours after elective spine surgery was an independent risk factor for poor surgical outcome. Perioperative treatment strategies to improve postoperative pain control may lead to improved patient-reported surgical outcomes.

Impact of preoperative insomnia on poor postoperative pain control after elective spine surgery and the modified Calgary postoperative pain after spine surgery (MCAPPS) score.

Background: Approximately 30% to 64% of patients experience inadequate pain control following spine surgery. The Calgary postoperative pain after spine surgery (CAPPS) score was developed to identify this subset of patients. The impact of preoperative insomnia on postoperative pain control is unknown. This study aimed to investigate the relationship between preoperative insomnia and poor pain control after spine surgery, as well as improve the predictive accuracy of the CAPPS score. Methods: A prospective cohort study was conducted in patients undergoing elective spine surgery. Poor pain control was defined as a mean numeric rating scale pain score >4 at rest within the first 24-hours after surgery. Patients were evaluated using the CAPPS score, which included 7 prognostic factors. A multivariable logistic regression model was used to examine the association between preoperative insomnia severity index (ISI) and poor pain control, adjusting for the CAPPS score. The Modified CAPPS score was derived from this model. Results: Of 219 patients, 49.7% experienced poorly controlled pain. Prevalence of clinical insomnia (ISI≥15) was 26.9%. Preoperative ISI was independently associated with poor pain control (odds ratio [OR] 1.09, [95%CI=1.03-1.16], p=.004), after adjusting for the CAPPS score (OR 1.61, [95%CI=1.38-1.89], p<.001). The model exhibited good discrimination (c-statistics 0.80, [95%CI=0.74-0.86]) and calibration (Hosmer-Lemeshow chi-square=8.95, p=.35). The Modified CAPPS score also demonstrated good discrimination (c-statistic 0.78, [95%CI=0.72-0.84]) and calibration (Hosmer-Lemeshow chi-square=2.92, p=.57). Low-, high-, and extreme-risk groups stratified by the Modified CAPPS score had 17.3%, 49.1%, and 80.7% predicted probability of experiencing inadequate pain control compared to 32.0%, 64.0%, and 85.1% in the CAPPS score. Conclusions: Preoperative insomnia is prevalent and is a modifiable risk factor for poor pain control following spine surgery. Early identification and management of preoperative insomnia may lead to improved postoperative pain outcomes. Future external validation is needed to confirm the accuracy of the Modified CAPPS score.

Comparison of outcomes in the management of abdominal pseudocyst in children with shunted hydrocephalus: a Hydrocephalus Clinical Research Network study.

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.

Treatment of hydrocephalus following posterior fossa tumor resection: a multicenter collaboration from the Hydrocephalus Clinical Research Network.

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.

Neurosurgeons' Perspectives on Vascular Entrustable Professional Activities.

This article discusses subspecialty Canadian neurosurgeons' perceptions of entrustable professional activities (EPAs) assessments and variabilities prior to the implementation of the Competence by Design (CBD) system in Canada. Vascular neurosurgeons were asked to reflect on how they would evaluate and give feedback to neurosurgery residents concerning the EPA "Performing surgery for patients with an intracranial aneurysm." Interviews were transcribed and analyzed using a deductive approach. Themes were derived from these interviews and reflected on the subjectivity and biases present in the EPA assessment forms. Indeed, faculty may require more training in the transitioning to a CBD evaluation system.

Validation of the Calgary Postoperative Pain after Spine Surgery Score for Poor Postoperative Pain Control after Spine Surgery.

OBJECTIVE: The Calgary Postoperative Pain after Spine Surgery (CAPPS) score was developed to identify patients at risk of experiencing poorly controlled pain after spine surgery. The goal of this study was to independently validate the CAPPS score on a prospectively collected patient sample. METHODS: Poor postoperative pain control was defined as a mean numeric rating scale (NRS) for pain >4 at rest in the first 24 hours after surgery. Baseline characteristics in this study (validation cohort) were compared to those of the development cohort used to create the CAPPS score. Predictive performance of the CAPPS score was assessed by the area under the curve (AUC) and percentage misclassification for discrimination. A graphical comparison between predicted probability vs. observed incidence of poorly controlled pain was performed for calibration. RESULTS: Fifty-two percent of 201 patients experienced poorly controlled pain. The validation cohort exhibited lower depression scores and a higher proportion using daily opioid medications compared to the development cohort. The AUC was 0.74 [95%CI = 0.68-0.81] in the validation cohort compared to 0.73 [95%CI = 0.69-0.76] in the development cohort for the eight-tier CAPPS score. When stratified between the low- vs. extreme-risk and low- vs. high-risk groups, the percentage misclassification was 21.2% and 30.7% in the validation cohort, compared to 29.9% and 38.0% in the development cohort, respectively. The predicted probability closely mirrored the observed incidence of poor pain control across all scores. CONCLUSIONS: The CAPPS score, based on seven easily obtained and reliable prognostic variables, was validated using a prospectively collected, independent sample of patients.

Ultrasound Elastography in Neurosurgery: Current Applications and Future Perspectives.

BACKGROUND: Similar to clinical palpation, Ultrasound elastography (USE) helps distinguish between tissues by providing information on their elasticity. While it has been widely explored and has been applied to many body organs, USE has not been studied as extensively for application in neurosurgery. The current systematic review was performed to identify articles related to the use of interoperative USE in neurosurgery. METHODS: Search included MEDLINE(R) database. Only original peer-reviewed full-text articles were included. No language or publication year restrictions were imposed. Two independent reviewers assessed the search results for relevance. The identified articles were screened by title, abstract, and full-text review. RESULTS: Seventeen articles were included in the qualitative analysis and 13 articles were related to oncology, epilepsy (n = 3), and spine (n = 1). In oncology, USE was found useful in defining tumor stiffness, aiding surgical planning, detecting residual tumors, discriminating between tumor and brain tissue, and differentiating between different tumors. In epilepsy, USE could improve the detection of epileptogenic foci, thereby enhancing the prospect of complete and safe resection. The application in spinal surgery was limited to demonstrating that a compressed spinal cord is stiffer than the decompressed one. CONCLUSIONS: USE was found to be a safe, quick, portable, and economic tool that was a useful intraoperative adjunct to provide information corresponding to a variety of neurosurgical diseases, at different stages of surgery. This review describes the current intraoperative neurosurgical applications of USE, the concept of elasticity, and different USE modalities as well as the technical challenges, limitations, and possible future implications.

Hydrocephalus surveillance following CSF diversion: a modified Delphi study.

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.

Endoscopic third ventriculostomy in previously shunt-treated patients.

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.

The Hydrocephalus Clinical Research Network quality improvement initiative: the role of antibiotic-impregnated catheters and vancomycin wound irrigation.

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.

Impact of ventricle size on neuropsychological outcomes in treated pediatric hydrocephalus: an HCRN prospective cohort study.

OBJECTIVE: In pediatric hydrocephalus, shunts tend to result in smaller postoperative ventricles compared with those following an endoscopic third ventriculostomy (ETV). The impact of the final treated ventricle size on neuropsychological and quality-of-life outcomes is currently undetermined. Therefore, the authors sought to ascertain whether treated ventricle size is associated with neurocognitive and academic outcomes postoperatively. METHODS: This prospective cohort study included children aged 5 years and older at the first diagnosis of hydrocephalus at 8 Hydrocephalus Clinical Research Network sites from 2011 to 2015. The treated ventricle size, as measured by the frontal and occipital horn ratio (FOR), was compared with 25 neuropsychological tests 6 months postoperatively after adjusting for age, hydrocephalus etiology, and treatment type (ETV vs shunt). Pre- and posttreatment grade point average (GPA), quality-of-life measures (Hydrocephalus Outcome Questionnaire [HOQ]), and a truncated preoperative neuropsychological battery were also compared with the FOR. RESULTS: Overall, 60 children were included with a mean age of 10.8 years; 17% had ≥ 1 comorbidity. Etiologies for hydrocephalus were midbrain lesions (37%), aqueductal stenosis (22%), posterior fossa tumors (13%), and supratentorial tumors (12%). ETV (78%) was more commonly used than shunting (22%). Of the 25 neuropsychological tests, including full-scale IQ (q = 0.77), 23 tests showed no univariable association with postoperative ventricle size. Verbal learning delayed recall (p = 0.006, q = 0.118) and visual spatial judgment (p = 0.006, q = 0.118) were negatively associated with larger ventricles and remained significant after multivariate adjustment for age, etiology, and procedure type. However, neither delayed verbal learning (p = 0.40) nor visual spatial judgment (p = 0.22) was associated with ventricle size change with surgery. No associations were found between postoperative ventricle size and either GPA or the HOQ. CONCLUSIONS: Minimal associations were found between the treated ventricle size and neuropsychological, academic, or quality-of-life outcomes for pediatric patients in this comprehensive, multicenter study that encompassed heterogeneous hydrocephalus etiologies.

Anterior versus posterior entry site for ventriculoperitoneal shunt insertion: a randomized controlled trial by the Hydrocephalus Clinical Research Network.

OBJECTIVE: The primary objective of this trial was to determine if shunt entry site affects the risk of shunt failure. METHODS: The authors performed a parallel-design randomized controlled trial with an equal allocation of patients who received shunt placement via the anterior entry site and patients who received shunt placement via the posterior entry site. All patients were children with symptoms or signs of hydrocephalus and ventriculomegaly. Patients were ineligible if they had a prior history of shunt insertion. Patients received a ventriculoperitoneal shunt after randomization; randomization was stratified by surgeon. The primary outcome was shunt failure. The planned minimum follow-up was 18 months. The trial was designed to achieve high power to detect a 10% or greater absolute difference in the shunt failure rate at 1 year. An independent, blinded adjudication committee determined eligibility and the primary outcome. The study was conducted by the Hydrocephalus Clinical Research Network. RESULTS: The study randomized 467 pediatric patients at 14 tertiary care pediatric hospitals in North America from April 2015 to January 2019. The adjudication committee, blinded to intervention, excluded 7 patients in each group for not meeting the study inclusion criteria. For the primary analysis, there were 229 patients in the posterior group and 224 patients in the anterior group. The median patient age was 1.3 months, and the most common etiologies of hydrocephalus were postintraventricular hemorrhage secondary to prematurity (32.7%), myelomeningocele (16.8%), and aqueductal stenosis (10.8%). There was no significant difference in the time to shunt failure between the entry sites (log-rank test, stratified by age < 6 months and ≥ 6 months; p = 0.061). The hazard ratio (HR) of a posterior shunt relative to an anterior shunt was calculated using a univariable Cox regression model and was nonsignificant (HR 1.35, 95% CI, 0.98-1.85; p = 0.062). No significant difference was found between entry sites for the surgery duration, number of ventricular catheter passes, ventricular catheter location, and hospital length of stay. There were no significant differences between entry sites for intraoperative complications, postoperative CSF leaks, pseudomeningoceles, shunt infections, skull fractures, postoperative seizures, new-onset epilepsy, or intracranial hemorrhages. CONCLUSIONS: This randomized controlled trial comparing the anterior and posterior shunt entry sites has demonstrated no significant difference in the time to shunt failure. Anterior and posterior entry site surgeries were found to have similar outcomes and similar complication rates.

Quality of life and satisfaction in surgical versus conservative treatment of nonsyndromic children with craniosynostosis.

OBJECTIVE: Craniosynostosis represents the second most common reason for referral to pediatric neurosurgery. However, the quality of life and neurodevelopmental impact of leaving this physical disorder uncorrected is poorly understood. METHODS: This multicenter cross-sectional study identified previously managed nonsyndromic infants (< 24 months of age) with single-suture craniosynostosis at both pediatric neurosurgical centers in Alberta, Canada. The primary variable of interest was the allocated treatment (surgical vs conservative). The primary outcome was the Pediatric Quality of Life Inventory (PedsQL), a validated measure of quality of life examining physical, emotional, social, and school functioning. Treatment decision satisfaction and the reasons for selected management were quantified and collected directly from caregivers. RESULTS: One hundred fourteen children met the inclusion criteria: 78% underwent surgery and 22% had conservative treatment. The most common suture affected was sagittal (54%), followed by metopic (33%), coronal (10%), and lambdoid (3%). Caregivers most commonly opted for surgery because of severe appearance (80%). Seventy-six percent and 72% of the caregivers of children with conservative management did so due to concerns of surgical risks and mild appearance, respectively. There was a statistically significant relationship between both the parents' (p < 0.001) and the surgeon's (p = 0.001) impression of a severe head shape and surgical management. Parental satisfaction with their child's appearance as well as satisfaction with their treatment decision did not differ between management types. Regarding quality of life, on univariate analysis, the conservative group had a statistically higher physical summary score (p = 0.01), psychosocial summary score (p = 0.004), and mean total scale score (p = 0.003) compared to the surgical group. However, after adjusting for severity and age at consult, no significant independent associations between management type and any of the PedsQL summary scores were found. CONCLUSIONS: Alberta families have a high number of children with craniosynostosis treated with conservative management. Conservatively managed infants were largely minimally affected patients, particularly those with metopic synostosis. The study found no independent association between management type (surgery vs conservative) and quality of life when adjusted for important patient factors.

Implementation of competence by design in Canadian neurosurgery residency programs.

OBJECTIVE: The Royal College of Physicians and Surgeons of Canada (RCPSC) recently redesigned the Canadian neurosurgery residency training curriculum by implementing a competency-based model of training known as Competence by Design (CBD) centered around the assessment of Entrustable Professional Activities (EPAs). This sequential explanatory mixed-methods study evaluated potential benefits and pitfalls of CBD in Canadian neurosurgery residency education. METHODS: Two four-month interval surveys were distributed to all Canadian neurosurgery residents participating in CBD. The surveys assessed important educational components: CBD knowledge of key stakeholders, potential system barriers, and educational/psychological impacts on residents. Paired t-tests were done to assess changes over time. Based on longitudinal survey responses, semi-structured interviews were conducted to investigate in-depth residents' experience with CBD in neurosurgery. The qualitative analysis followed an explanatory approach, and a thematic analysis was performed. RESULTS: Surveys had 82% average response rate (n = 25). Over time, most residents self-reported that they retrospectively understood concepts around CBD intentions (p = 0.02). Perceived benefits included faculty evaluations with more feedback that was clearer and more objective (53% and 51%). Pitfalls included the amount of time needed to navigate through EPAs (90%) and residents forgetting to initiate EPA forms (71%). There was no significant change over time. During interviews, five key themes were found. Potential solutions identified by residents to enhance their experience included learning analytics data availability, mobile app refinement, and dedicated time to integrate EPAs in the workflow. CONCLUSION: This study was the first to assess resident-perceived benefits and pitfalls of the neurosurgery CBD training program in an educational framework context. In general, residents believed that theoretical principles behind CBD were valuable, but that technological ability and having enough time to request EPA assessments were significant barriers to success. Long-term studies are required to determine the definitive outcomes of CBD on residents' performance and ultimately, on patient care.

Longitudinal CSF Iron Pathway Proteins in Posthemorrhagic Hydrocephalus: Associations with Ventricle Size and Neurodevelopmental Outcomes.

OBJECTIVE: Iron has been implicated in the pathogenesis of brain injury and hydrocephalus after preterm germinal matrix hemorrhage-intraventricular hemorrhage, however, it is unknown how external or endogenous intraventricular clearance of iron pathway proteins affect the outcome in this group. METHODS: This prospective multicenter cohort included patients with posthemorrhagic hydrocephalus (PHH) who underwent (1) temporary and permanent cerebrospinal fluid (CSF) diversion and (2) Bayley Scales of Infant Development-III testing around 2 years of age. CSF proteins in the iron handling pathway were analyzed longitudinally and compared to ventricle size and neurodevelopmental outcomes. RESULTS: Thirty-seven patients met inclusion criteria with a median estimated gestational age at birth of 25 weeks; 65% were boys. Ventricular CSF levels of hemoglobin, iron, total bilirubin, and ferritin decreased between temporary and permanent CSF diversion with no change in CSF levels of ceruloplasmin, transferrin, haptoglobin, and hepcidin. There was an increase in CSF hemopexin during this interval. Larger ventricle size at permanent CSF diversion was associated with elevated CSF ferritin (p = 0.015) and decreased CSF hemopexin (p = 0.007). CSF levels of proteins at temporary CSF diversion were not associated with outcome, however, higher CSF transferrin at permanent CSF diversion was associated with improved cognitive outcome (p = 0.015). Importantly, longitudinal change in CSF iron pathway proteins, ferritin (decrease), and transferrin (increase) were associated with improved cognitive (p = 0.04) and motor (p = 0.03) scores and improved cognitive (p = 0.04), language (p = 0.035), and motor (p = 0.008) scores, respectively. INTERPRETATION: Longitudinal changes in CSF transferrin (increase) and ferritin (decrease) are associated with improved neurodevelopmental outcomes in neonatal PHH, with implications for understanding the pathogenesis of poor outcomes in PHH. ANN NEUROL 2021;90:217-226.

Treatment strategies for hydrocephalus related to Dandy-Walker syndrome: evaluating procedure selection and success within the Hydrocephalus Clinical Research Network.

OBJECTIVE: Treating Dandy-Walker syndrome-related hydrocephalus (DWSH) involves either a CSF shunt-based or endoscopic third ventriculostomy (ETV)-based procedure. However, comparative investigations are lacking. This study aimed to compare shunt-based and ETV-based treatment strategies utilizing archival data from the Hydrocephalus Clinical Research Network (HCRN) registry. METHODS: A retrospective review of prospectively collected and maintained data on children with DWSH, available from the HCRN registry (14 sites, 2008-2018), was performed. The primary outcome was revision-free survival of the initial surgical intervention. The primary exposure was either shunt-based (i.e., cystoperitoneal shunt [CPS], ventriculoperitoneal shunt [VPS], and/or dual-compartment) or ETV-based (i.e., ETV alone or with choroid plexus cauterization [CPC]) initial surgical treatment. Primary analysis included multivariable Cox proportional hazards models. RESULTS: Of 8400 HCRN patients, 151 (1.8%) had DWSH. Among these, the 102 patients who underwent shunt placement (79 VPSs, 16 CPSs, 3 other, and 4 multiple proximal catheter) were younger (6.6 vs 18.8 months, p < 0.001) and more frequently had 1 or more comorbidities (37.3% vs 14.3%, p = 0.005) than the 49 ETV-treated children (28 ETV-CPC). Fifty percent of the shunt-based and 51% of the ETV-based treatments failed. Notably, 100% (4/4) of the dual-compartment shunts failed. Adjusting for age, baseline ventricular size, and comorbidities, ETV-based treatment was not significantly associated with earlier failure compared with shunt-based treatment (HR for failure 1.32, 95% CI 0.77-2.26; p = 0.321). Complication rates were low: 4.9% and 6.1% (p = 0.715) for shunt- and ETV-based procedures, respectively. There was no difference in survival between ETV-CPC- and ETV-based treatment when adjusting for age (HR for failure 0.86, 95% CI 0.29-2.55, p = 0.783). CONCLUSIONS: In this North American, multicenter, prospective database review, shunt-based and ETV-based primary treatment strategies of DWSH appear similarly durable. Pediatric neurosurgeons can reasonably consider ETV-based initial treatment given the similar durability and the low complication rate. However, given the observational nature of this study, the treating surgeon might need to consider subgroups that were too small for a separate analysis. Very young children with comorbidities were more commonly treated with shunts, and older children with fewer comorbidities were offered ETV-based treatment. Future studies may determine preoperative characteristics associated with ETV treatment success in this population.

Hydrocephalus treatment in patients with craniosynostosis: an analysis from the Hydrocephalus Clinical Research Network prospective registry.

OBJECTIVE: Hydrocephalus may be seen in patients with multisuture craniosynostosis and, less commonly, single-suture craniosynostosis. The optimal treatment for hydrocephalus in this population is unknown. In this study, the authors aimed to evaluate the success rate of ventriculoperitoneal shunt (VPS) treatment and endoscopic third ventriculostomy (ETV) both with and without choroid plexus cauterization (CPC) in patients with craniosynostosis. METHODS: Utilizing the Hydrocephalus Clinical Research Network (HCRN) Core Data Project (Registry), the authors identified all patients who underwent treatment for hydrocephalus associated with craniosynostosis. Descriptive statistics, demographics, and surgical outcomes were evaluated. RESULTS: In total, 42 patients underwent treatment for hydrocephalus associated with craniosynostosis. The median gestational age at birth was 39.0 weeks (IQR 38.0, 40.0); 55% were female and 60% were White. The median age at first craniosynostosis surgery was 0.6 years (IQR 0.3, 1.7), and at the first permanent hydrocephalus surgery it was 1.2 years (IQR 0.5, 2.5). Thirty-three patients (79%) had multiple different sutures fused, and 9 had a single suture: 3 unicoronal (7%), 3 sagittal (7%), 2 lambdoidal (5%), and 1 unknown (2%). Syndromes were identified in 38 patients (90%), with Crouzon syndrome being the most common (n = 16, 42%). Ten patients (28%) received permanent hydrocephalus surgery before the first craniosynostosis surgery. Twenty-eight patients (67%) underwent VPS treatment, with the remaining 14 (33%) undergoing ETV with or without CPC (ETV ± CPC). Within 12 months after initial hydrocephalus intervention, 14 patients (34%) required revision (8 VPS and 6 ETV ± CPC). At the most recent follow-up, 21 patients (50%) required a revision. The revision rate decreased as age increased. The overall infection rate was 5% (VPS 7%, 0% ETV ± CPC). CONCLUSIONS: This is the largest prospective study reported on children with craniosynostosis and hydrocephalus. Hydrocephalus in children with craniosynostosis most commonly occurs in syndromic patients and multisuture fusion. It is treated at varying ages; however, most patients undergo surgery for craniosynostosis prior to hydrocephalus treatment. While VPS treatment is performed more frequently, VPS and ETV are both reasonable options, with decreasing revision rates with increasing age, for the treatment of hydrocephalus associated with craniosynostosis.

Cerebrospinal fluid NCAM-1 concentration is associated with neurodevelopmental outcome in post-hemorrhagic hydrocephalus of prematurity.

OBJECTIVE: Efforts directed at mitigating neurological disability in preterm infants with intraventricular hemorrhage (IVH) and post hemorrhagic hydrocephalus (PHH) are limited by a dearth of quantifiable metrics capable of predicting long-term outcome. The objective of this study was to examine the relationships between candidate cerebrospinal fluid (CSF) biomarkers of PHH and neurodevelopmental outcomes in infants undergoing neurosurgical treatment for PHH. STUDY DESIGN: Preterm infants with PHH were enrolled across the Hydrocephalus Clinical Research Network. CSF samples were collected at the time of temporizing neurosurgical procedure (n = 98). Amyloid precursor protein (APP), L1CAM, NCAM-1, and total protein (TP) were compared in PHH versus control CSF. Fifty-four of these PHH subjects underwent Bayley Scales of Infant Development-III (Bayley-III) testing at 15-30 months corrected age. Controlling for false discovery rate (FDR) and adjusting for post-menstrual age (PMA) and IVH grade, Pearson's partial correlation coefficients were used to examine relationships between CSF proteins and Bayley-III composite cognitive, language, and motor scores. RESULTS: CSF APP, L1CAM, NCAM-1, and TP were elevated in PHH over control at temporizing surgery. CSF NCAM-1 was associated with Bayley-III motor score (R = -0.422, p = 0.007, FDR Q = 0.089), with modest relationships noted with cognition (R = -0.335, p = 0.030, FDR Q = 0.182) and language (R = -0.314, p = 0.048, FDR Q = 0.194) scores. No relationships were observed between CSF APP, L1CAM, or TP and Bayley-III scores. FOHR at the time of temporization did not correlate with Bayley-III scores, though trends were observed with Bayley-III motor (p = 0.0647 and R = -0.2912) and cognitive scores (p = 0.0506 and R = -0.2966). CONCLUSION: CSF NCAM-1 was associated with neurodevelopment in this multi-institutional PHH cohort. This is the first report relating a specific CSF protein, NCAM-1, to neurodevelopment in PHH. Future work will further investigate a possible role for NCAM-1 as a biomarker of PHH-associated neurological disability.