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.

Refining the role of cervical spine immobilization and stabilization in pediatric non-accidental trauma, a retrospective series of 1008 patients.

INTRODUCTION: Prompt detection of traumatic cervical spine injury is important as delayed or missed diagnosis can have disastrous consequences. Given the understood mechanism of non-accidental trauma (NAT), it is reasonable to suspect that cervical spine injury can occur. Current management of young children being evaluated for NAT includes placement of a rigid collar until clinical clearance or an MRI can be obtained. Currently, there exists a lack of robust data to guide cervical bracing. Anecdotally, our group has not observed a single patient with a diagnosis of NAT who required operative stabilization for cervical spine instability. This study will be the largest series to date and aims to systematically investigate this observation to determine the likelihood that children with a diagnosis of NAT harbor cervical spine instability related to their injuries. METHODS: Patient data from the Children's Hospital Colorado Trauma Registry diagnosed with non-burn-only NAT were reviewed retrospectively. Children less than 4 years of age pulled from the registry from January 1, 2005, to March 31, 2021, were included. Demographic, admission/discharge, imaging, and clinic management data were collected for each patient and analyzed. RESULTS: There were 1008 patients included in the cohort. The age at presentation ranged from 5 days to 4 years (mean 10.4 months). No patient had X-ray or CT findings concerning for cervical instability. Three patients had MRI findings concerning for cervical instability. Two of these underwent external bracing, and the third died from unrelated injuries during their hospitalization. Only four patients were discharged in a cervical collar, and all were ultimately cleared from bracing. No patient underwent a spinal stabilization procedure. CONCLUSIONS: While the mechanism of injury in many NAT cases would seem to make significant cervical spine injury possible, this single-center retrospective review of a large experience indicates that such injury is exceedingly rare. Further study is merited to understand the underlying pathophysiology. However, it is reasonable to consider cervical collar clearance in the setting of normal radiographs and a reassuring neurological exam. Furthermore, if concerns exist regarding cervical spine instability on MRI, an initial trial of conservative management is warranted.

Risk factors for unchanged ventricles during pediatric shunt malfunction.

OBJECTIVE: Children whose ventricles do not change during shunt malfunction present a diagnostic dilemma. This study was performed to identify risk factors for unchanged ventricular size at shunt malfunction. METHODS: This retrospective 1:1 age-matched case-control study identified children with shunted hydrocephalus who underwent shunt revision with intraoperative evidence of malfunction at one of the three participating institutions from 1997 to 2019. Cases were defined as patients with a change of < 0.05 in the frontal-occipital horn ratio (FOR) between malfunction and baseline, and controls included patients with FOR changes ≥ 0.05. The presence of infection, abdominal pseudocyst, pseudomeningocele, or wound drainage and lack of baseline cranial imaging at the time of malfunction warranted exclusion. RESULTS: Of 450 included patients, 60% were male, 73% were Caucasian, and 67% had an occipital shunt. The median age was 4.3 (IQR 0.97-9.21) years at malfunction. On univariable analysis, unchanged ventricles at malfunction were associated with a frontal shunt (41% vs 28%, p < 0.001), programmable valve (17% vs 9%, p = 0.011), nonsiphoning shunt (85% vs 66%, p < 0.001), larger baseline FOR (0.44 ± 0.12 vs 0.38 ± 0.11, p < 0.001), no prior shunt infection (87% vs 76%, p = 0.003), and no prior shunt revisions (68% vs 52%, p < 0.001). On multivariable analysis with collinear variables removed, patients with a frontal shunt (OR 1.67, 95% CI 1.08-2.70, p = 0.037), programmable valve (OR 2.63, 95% CI 1.32-5.26, p = 0.007), nonsiphoning shunt at malfunction (OR 2.76, 95% CI 1.63-4.67, p < 0.001), larger baseline FOR (OR 3.13, 95% CI 2.21-4.43, p < 0.001), and no prior shunt infection (OR 2.34, 95% CI 1.27-4.30, p = 0.007) were more likely to have unchanged ventricles at malfunction. CONCLUSIONS: In a multicenter cohort of children with shunt malfunction, those with a frontal shunt, programmable valve, nonsiphoning shunt, baseline large ventricles, and no prior shunt infection were more likely than others to have unchanged ventricles at shunt failure.

Robust deep learning classification of adamantinomatous craniopharyngioma from limited preoperative radiographic images.

Deep learning (DL) is a widely applied mathematical modeling technique. Classically, DL models utilize large volumes of training data, which are not available in many healthcare contexts. For patients with brain tumors, non-invasive diagnosis would represent a substantial clinical advance, potentially sparing patients from the risks associated with surgical intervention on the brain. Such an approach will depend upon highly accurate models built using the limited datasets that are available. Herein, we present a novel genetic algorithm (GA) that identifies optimal architecture parameters using feature embeddings from state-of-the-art image classification networks to identify the pediatric brain tumor, adamantinomatous craniopharyngioma (ACP). We optimized classification models for preoperative Computed Tomography (CT), Magnetic Resonance Imaging (MRI), and combined CT and MRI datasets with demonstrated test accuracies of 85.3%, 83.3%, and 87.8%, respectively. Notably, our GA improved baseline model performance by up to 38%. This work advances DL and its applications within healthcare by identifying optimized networks in small-scale data contexts. The proposed system is easily implementable and scalable for non-invasive computer-aided diagnosis, even for uncommon diseases.

Transcriptional analyses of adult and pediatric adamantinomatous craniopharyngioma reveals similar expression signatures regarding potential therapeutic targets.

Adamantinomatous craniopharyngioma (ACP) is a biologically benign but clinically aggressive lesion that has a significant impact on quality of life. The incidence of the disease has a bimodal distribution, with peaks occurring in children and older adults. Our group previously published the results of a transcriptome analysis of pediatric ACPs that identified several genes that were consistently overexpressed relative to other pediatric brain tumors and normal tissue. We now present the results of a transcriptome analysis comparing pediatric to adult ACP to identify biological differences between these groups that may provide novel therapeutic insights or support the assertion that potential therapies identified through the study of pediatric ACP may also have a role in adult ACP. Using our compiled transcriptome dataset of 27 pediatric and 9 adult ACPs, obtained through the Advancing Treatment for Pediatric Craniopharyngioma Consortium, we interrogated potential age-related transcriptional differences using several rigorous mathematical analyses. These included: canonical differential expression analysis; divisive, agglomerative, and probabilistic based hierarchical clustering; information theory based characterizations; and the deep learning approach, HD Spot. Our work indicates that there is no therapeutically relevant difference in ACP gene expression based on age. As such, potential therapeutic targets identified in pediatric ACP are also likely to have relvance for adult patients.

Converting Pediatric Patients and Young Adults From a Shunt to a Third Ventriculostomy: A Multicenter Evaluation.

BACKGROUND: Endoscopic third ventriculostomy (ETV) is an effective primary treatment for certain forms of hydrocephalus. However, its use in children with an existing shunt is less well known. OBJECTIVE: To report a multicenter experience in attempting to convert patients from shunt dependence to a third ventriculostomy and to determine predictors of success. METHODS: Three participating centers provided retrospectively collected information on patients with an attempted conversion from a shunt to an ETV between December 1, 2008, and April 1, 2018. Demographic, clinical, and radiological data were recorded. Success was defined as shunt independence at the last follow-up. RESULTS: Eighty patients with an existing ventricular shunt underwent an ETV. The median age at the time of the index ETV was 9.9 yr, and 44 (55%) patients were male. The overall success rate was 64% (51/80), with a median duration of follow-up of 2.0 yr (range, 0.1-9.4 yr). Four patients required a successful repeat ETV at a median of 1.7 yr (range, 0.1-5.7 yr) following the index ETV. Only age was predictive of ETV failure on multivariate analysis (odds ratio 0.86 [95% CI 0.78-0.94], P = .005). No patient less than 6 mo of age underwent an ETV, and of the 5 patients between 6 and 12 mo of age, 4 failed. CONCLUSION: Although not every shunted patient will be a candidate for an ETV, nor will they be successfully converted, an ETV should at least be considered in every child who presents with a shunt malfunction or who has an externalized shunt.

Interrater reliability of a method to assess hypothalamic involvement in pediatric adamantinomatous craniopharyngioma.

OBJECTIVE: Pediatric adamantinomatous craniopharyngiomas (ACPs) are histologically benign brain tumors that confer significant neuroendocrine morbidity. Previous studies have demonstrated that injury to the hypothalamus is associated with worsened quality of life and a shorter lifespan. This insight helps many surgeons define the goals of surgery for patients with ACP. Puget and colleagues proposed a 3-tiered preoperative and postoperative grading system based on the degree of hypothalamic involvement identified on MRI. In a prospective cohort from their institution, the authors found that use of the system to guide operative goals was associated with decreased morbidity. To date, however, the Puget system has not been externally validated. Here, the authors present an interrater reliability study that assesses the generalizability of this system for surgeons planning initial operative intervention for children with craniopharyngiomas. METHODS: A panel of 6 experts, consisting of pediatric neurosurgeons and pediatric neuroradiologists, graded 30 preoperative and postoperative MRI scans according to the Puget system. Interrater reliability was calculated using Fleiss' κ and Krippendorff's α statistics. RESULTS: Interrater reliability in the preoperative context demonstrated moderate agreement (κ = 0.50, α = 0.51). Interrater reliability in the postoperative context was 0.27 for both methods of statistical evaluation. CONCLUSIONS: Interrater reliability for the system as defined is moderate. Slight refinements of the Puget MRI grading system, such as collapsing the 3 grades into 2, may improve its reliability, making the system more generalizable.

Evolution of minimally invasive techniques within an academic surgical practice at a single institution.

AIM: We aimed to understand how changes in surgical techniques are transferred into surgical practice. We hypothesized that the integration of minimally invasive surgery (MIS) techniques into a pediatric surgical practice is dependent on the hiring of junior partners who can transfer their MIS knowledge to senior surgeons. MATERIALS AND METHODS: We reviewed the operative approach to six different general pediatric surgical procedures from 2000 to 2012, including appendectomy, fundoplication, gastrostomy tube placement, pyloromyotomy, colectomy, and lobectomy (lung). The percentage of MIS cases was calculated for each procedure annually. Each year six to nine fellowship-trained pediatric surgeons operated at our institution. Three surgeons completed training in the era of MIS (hired in 2001, 2007, and 2009) and remained on staff for at least 3 years. RESULTS: In 2000, 49% of procedures were performed with MIS. This increased to 98% in 2013 (P<.0001). Appendectomy, pyloromyotomy, and fundoplication demonstrated early adoption and uniform use of laparoscopy (>85%) by 2007. Gastrostomy tube placement reached 90% laparoscopy utilization in 2009. Lobectomy (lung) and colectomy also reached >80% MIS use in 2009 and 2010, respectively. The same trends were seen when looking only at senior surgeons who completed training prior to 2000. CONCLUSIONS: The hiring of junior surgeons with MIS training was associated with an increase in adoption of MIS techniques by the entire group. Procedures that were introduced early in the MIS era demonstrated a gradual rise toward uniform adoption of MIS techniques. More advanced and recently adopted MIS techniques demonstrated a rapid rise to uniform adoption.

Contributing factors for cancellations of outpatient pediatric urology procedures: single center experience.

PURPOSE: Surgery cancellations in the pediatric population are often due to preventable causes and can lead to decreased operating room efficiency. We hypothesized that clinical and demographic patient factors are associated with preventable cancellations of scheduled outpatient pediatric urology procedures at our institution. MATERIALS AND METHODS: A retrospective review of cancelled outpatient pediatric urology procedures from January 1 to July 31, 2010 was performed. Data regarding demographics, procedure type, time to surgery from preoperative visit, reason for cancellation, potential operating room and surgeon lost revenue, and ultimate patient outcome were extracted from the electronic medical record. RESULTS: We identified 114 cancellations during the study period, comprising 13.3% of scheduled outpatient procedures. Preventable cancellations included insurance/financial related (11.4%), preoperative fasting violation (8.8%) and condition improved the day of surgery (4.4%). Nonpreventable cancellations included patient illness (40.3%), weather/traffic related (1.7%) and other nonspecified reasons (29%). Compared to nonpreventable cancellations, preventable cancellations were more likely associated with circumcision (OR 2.39, CI 1.04-5.46). Preventable cancellations were also associated with a shorter distance to the hospital (p = 0.03). There was no significant association between preventable cancellations and age, race/ethnicity, caregiver type or time to surgery. Potential associated lost revenue averaged $4,802 per cancellation. CONCLUSIONS: While the most common cause of surgical cancellation is patient illness, a significant number of cancellations are preventable. These findings suggest that future targeted hospital interventions, including improved evaluation of insurance status and preoperative parental education regarding preoperative requirements, may improve operating room use.