Frequency and Distribution of Ophthalmic Surgical Procedures among Patients with Inherited Retinal Diseases.

OBJECTIVE: In this study, we aimed to characterize the frequency and distribution of ocular surgeries in patients with inherited retinal diseases (IRDs) and evaluate associated patient and disease factors. DESIGN: Retrospective cohort. PARTICIPANTS: Subjects aged ≥ 18 years who were followed at the Johns Hopkins Genetic Eye Disease Center. METHODS: We studied a retrospective cohort of patients with an IRD diagnosis to analyze the occurrence of laser and incisional surgeries. Subjects were categorized into 2 groups: central dysfunction (macular/cone/cone-rod dystrophy, "MCCRD group") and panretinal or peripheral dysfunction (retinitis pigmentosa-like, "RP group"). Genetic testing status was recorded. The association of patient and disease factors on the frequency, distribution, and timing of surgeries was analyzed. MAIN OUTCOME MEASURES: Prevalence, prevalence odds ratio (POR), hazard ratio (HR) of ophthalmic procedures by phenotype. RESULTS: A total of 1472 eyes of 736 subjects were evaluated. Among them, 31.3% (n = 230) had undergone ocular surgery, and 78.3% of those (n = 180/230) had a history of more than 1 surgery. A total of 602 surgical procedures were analyzed. Cataract extraction with intraocular lens implantation (CEIOL) was the most common (51.2%), followed by yttrium aluminum garnet capsulotomy, refractive surgery, retinal surgery, and others. Cataract extraction with intraocular lens implantation occurred more frequently in RP than in MCCRD subjects (POR, 2.59; P = 0.002). Retinitis pigmentosa subjects underwent CEIOL at a younger age than patients with MCCRD (HR, 2.11; P < 0.001). CONCLUSIONS: Approximately one-third of patients with IRD had a history of laser or incisional surgery. Cataract extraction with intraocular lens implantation was the most common surgery; its frequency and timing may be associated with the IRD phenotype. This data may inform the design of prospective research. Such efforts may illuminate routine clinical decision-making and contribute to surgical strategy development for cell and gene therapy delivery. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Neurosurgical Outcomes Among Non-English Speakers: A Systematic Review and a Framework for Future Research.

OBJECTIVE: In 2019, 22% of adults in the United States reported speaking a language other than English at home, representing 52% growth since 2000. This diversity in languages - and resulting possible communication barriers - represents a potential challenge to effective care. In this manuscript, we summarize clinical outcomes and healthcare utilization patterns of adult and pediatric neurosurgical patients who are non-English primary language speakers (NEPLS). METHODS: We systematically queried 5 databases from inception through October 2022. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were followed to identify studies for inclusion. The Newcastle-Ottawa Scale was used to assess the quality of studies. Additionally, a retrospective chart review was conducted to assess differences in postoperative communication patterns in a cohort of English and Spanish speaking patients with craniosynostosis at our institution. RESULTS: Our search yielded 442 abstracts; ten were included in the final cohort. Outcomes for 973 unique NEPLS with a neurosurgical condition were included; Spanish was the most represented language. Delivery and timing of surgical treatment was the most frequently reported metric; 75% of studies demonstrated a statistically significant delay in time to surgery or decreased likelihood for NEPLS to receive surgical treatment. Length of stay was reported in 3 studies; all demonstrated that NEPLS had longer length of stay. CONCLUSIONS: There is a paucity of literature reporting outcomes among NEPLS. It is critical to examine NEPLS patients' outcomes and experiences, as language barriers are potentially modifiable demographic factors. We present a framework that demonstrates opportunities for further research to improve quality of care.

Disparities in Preoperative Goals of Care Documentation in Veterans.

Importance: Preoperative goals of care discussion and documentation are important for patients undergoing surgery, a major health care stressor that incurs risk. Objective: To assess the association of race, ethnicity, and other factors, including history of mental health disability, with disparities in preoperative goals of care documentation among veterans. Design, Setting, and Participants: This retrospective cross-sectional study assessed data from the Veterans Healthcare Administration (VHA) of 229 737 veterans who underwent surgical procedures between January 1, 2017, and October 18, 2022. Exposures: Patient-level (ie, race, ethnicity, medical comorbidities, history of mental health comorbidity) and system-level (ie, facility complexity level) factors. Main Outcomes and Measures: Preoperative life-sustaining treatment (LST) note documentation or no LST note documentation within 30 days prior to or on day of surgery. The standardized mean differences were calculated to assess the magnitude of differences between groups. Odds ratios (ORs) and 95% CIs were estimated with logistic regression. Results: In this study, 13 408 patients (5.8%) completed preoperative LST from 229 737 VHA patients (209 123 [91.0%] male; 20 614 [9.0%] female; mean [SD] age, 65.5 [11.9] years) who received surgery. Compared with patients who did complete preoperative LST, patients tended to complete preoperative documentation less often if they were female (19 914 [9.2%] vs 700 [5.2%]), Black individuals (42 571 [19.7%] vs 2416 [18.0%]), Hispanic individuals (11 793 [5.5%] vs 631 [4.7%]), or from rural areas (75 637 [35.0%] vs 4273 [31.9%]); had a history of mental health disability (65 974 [30.5%] vs 4053 [30.2%]); or were seen at lowest-complexity (ie, level 3) facilities (7849 [3.6%] vs 78 [0.6%]). Over time, despite the COVID-19 pandemic, patients undergoing surgical procedures completed preoperative LST increasingly more often. Covariate-adjusted estimates of preoperative LST completion demonstrated that patients of racial or ethnic minority background (Black patients: OR, 0.79; 95% CI, 0.77-0.80; P <.001; patients selecting other race: OR, 0.78; 95% CI, 0.74-0.81; P <.001; Hispanic patients: OR, 0.78; 95% CI, 0.76-0.81; P <.001) and patients from rural regions (OR, 0.91; 95% CI, 0.90-0.93; P <.001) had lower likelihoods of completing LST compared with patients who were White or non-Hispanic and patients from urban areas. Patients with any mental health disability history also had lower likelihood of completing preoperative LST than those without a history (OR, 0.93; 95% CI, 0.92-0.94; P = .001). Conclusions and Relevance: In this cross-sectional study, disparities in documentation rates within a VHA cohort persisted based on race, ethnicity, rurality of patient residence, history of mental health disability, and access to high-volume, high-complexity facilities.

Serum Methamphetamine Positivity in Trauma Patients Undergoing Surgery has No Negative Effect on Postoperative Morbidity and Mortality.

Introduction: The link between methamphetamine (METH) use and mortality or morbidity, particularly perioperative complications, associated with trauma surgery are not well characterized. This study aims to address this by performing a comparison of surgical outcomes between METH-negative (METH-) and METH-positive (METH+) trauma patients. Methods: An Institutional Review Board-approved retrospective chart review was performed on all trauma patients admitted to our Level 1 trauma center who underwent surgical operations between 2015 and 2020. Patients were categorized into METH- and METH+ groups. Patient characteristics such as age, sex, race, Injury Severity Score (ISS), presence of peri-operative complications, and mortality, amongst others, were used to perform univariate comparisons. Additional multi-variate comparisons were performed across both the whole cohort and with age, sex, and ISS-matched groups. Results: Of 571 patients who met the final inclusion criteria, 421 were METH- and 150 METH+. The METH+ group also possessed a lower median ISS (P = 0.0478) and did not possess significantly different mortality or morbidity than their METH- counterparts in univariate analysis. Multivariate analysis in whole-group and matched-group cohorts indicated that METH was not a positive predictor of mortality or morbidity. Instead, ISS predicted mortality (P = 0.048) and morbidity (P < 0.001). Conclusion: Our results suggest that METH use does not exert a positive effect on mortality or morbidity in the acute trauma surgery setting and that ISS may be a more significant contributor, suggesting severity, and etiology of injury are also important considerations for trauma surgery evaluation.

Treatment Course and Outcomes of Intracranial Teratomas in Pediatric Patients: A Retrospective 15-Year Case Series Study.

INTRODUCTION: There is no standard treatment paradigm for intracranial teratomas, a rare subset of primary intracranial non-germinomatous germ cell tumors (NGGCT), which comprise less than 1% of pediatric brain tumors. This case series retrospectively analyzes treatment and outcomes of pediatric intracranial teratomas from a single institution. METHODS: Authors reviewed a comprehensive pathology database at Stanford's Lucile Packard Children's Hospital for intracranial teratomas in pediatric patients treated from 2006 to 2021; their demographics, treatment, and clinical course were analyzed. RESULTS: Among 14 patients, median follow-up time was 4.6 years and mean age at diagnosis was 10.5 years. Ten had elevated tumor markers and underwent chemotherapy as initial treatment for NGGCT. Ultimately, these patients all required surgery for progressive or residual disease. Two patients did not undergo radiation. After biopsy or resection, 8 patients had pure mature teratoma, five had mixed germ cell tumor with teratoma component, and one had immature teratoma. The patient with immature teratoma died during chemotherapy from septic shock. No patients experienced recurrence. Common sequelae were endocrine (42.8%) and eye movement (50.0%) abnormalities. DISCUSSION/CONCLUSION: We highlight the variable treatment course and outcome for pediatric patients with intracranial teratomas. Elevated tumor markers at presentation, along with imaging findings, favor chemotherapy initiation for presumed NGGCT. Resection of residual tumor is recommended even if tumor markers return to normal. Prognosis remains excellent; no patients had recurrence with a median follow-up of 4.6 years.

Strategies to Improve Perioperative Palliative Care Integration for Seriously Ill Veterans.

CONTEXT: Seriously ill patients are at higher risk for adverse surgical outcomes. Palliative care (PC) interventions for seriously ill surgical patients are associated with improved quality of patient care and patient-centered outcomes, yet, they are underutilized perioperatively. OBJECTIVES: To identify strategies for improving perioperative PC integration for seriously ill Veterans from the perspectives of PC providers and surgeons. METHODS: We conducted semistructured, in-depth individual and group interviews with Veteran Health Administration PC team members and surgeons between July 2020 and April 2021. Participants were purposively sampled from high- and low-collaboration sites based on the proportion of received perioperative palliative consults. We performed a team-based thematic analysis with dual coding (inter-rater reliability above 0.8). RESULTS: Interviews with 20 interdisciplinary PC providers and 13 surgeons at geographically distributed Veteran Affairs sites converged on four strategies for improving palliative care integration and goals of care conversations in the perioperative period: 1) develop and maintain collaborative, trusting relationships between palliative care providers and surgeons; 2) establish risk assessment processes to identify patients who may benefit from a PC consult; 3) involve both PC providers and surgeons at the appropriate time in the perioperative workflow; 4) provide sufficient resources to allow for an interdisciplinary sharing of care. CONCLUSION: The study demonstrates that individual, programmatic, and organizational efforts could facilitate interservice collaboration between PC clinicians and surgeons.

Patient Characteristics Associated With Occurrence of Preoperative Goals-of-Care Conversations.

Importance: Communication about patients' goals and planned and potential treatment is central to advance care planning. Undertaking or confirming advance care plans is also essential to preoperative preparation, particularly among patients who are frail or will undergo high-risk surgery. Objective: To evaluate the association between patient risk of hospitalization or death and goals-of-care conversations documented with a completed Life-Sustaining Treatment (LST) Decisions Initiative note among veterans undergoing surgery. Design, Setting, and Participants: This retrospective cross-sectional study included 190 040 veterans who underwent operations between January 1, 2017, and February 28, 2020. Statistical analysis took place from November 1, 2021, to November 17, 2022. Exposure: Patient risk of hospitalization or death, evaluated with a Care Assessment Need (CAN) score (range, 0-99, with a higher score representing a greater risk of hospitalization or death), dichotomized as less than 80 or 80 or more. Main Outcomes and Measures: Preoperative LST note completion (30 days before or on the day of surgery) or no LST note completion within the 30-day preoperative period prior to or on the day of the index operation. Results: Of 190 040 veterans (90.8% men; mean [SD] age, 65.2 [11.9] years), 3.8% completed an LST note before surgery, and 96.2% did not complete an LST note. In the groups with and without LST note completion before surgery, most were aged between 65 and 84 years (62.1% vs 56.7%), male (94.3% vs 90.7%), and White (82.2% vs 78.3%). Compared with patients who completed an LST note before surgery, patients who did not complete an LST note before surgery tended to be female (9.3% vs 5.7%), Black (19.2% vs 15.7%), married (50.2% vs 46.5%), and in better health (Charlson Comorbidity Index score of 0, 25.9% vs 15.2%); to have a lower risk of hospitalization or death (CAN score <80, 98.3% vs 96.9%); or to undergo neurosurgical (9.8% vs 6.2%) or urologic surgical procedures (5.9% vs 2.0%). Over the 3-year interval, unadjusted rates of LST note completion before surgery increased from 0.1% to 9.6%. Covariate-adjusted estimates of LST note completion indicated that veterans at a relatively elevated risk of hospitalization or death (CAN score ≥80) had higher odds of completing an LST note before surgery (odds ratio [OR], 1.29; 95% CI, 1.09-1.53) compared with those with CAN scores less than 80. High-risk surgery was not associated with increased LST note completion before surgery (OR, 0.93; 95% CI, 0.86-1.01). Veterans who underwent cardiothoracic surgery had the highest likelihood of LST note completion before surgery (OR, 1.35; 95% CI, 1.24-1.47). Conclusions and Relevance: Despite increasing LST note implementation, a minority of veterans completed an LST note preoperatively. Although doing so was more common among veterans with an elevated risk compared with those at lower risk, improving proactive communication and documentation of goals, particularly among higher-risk veterans, is needed. Doing so may promote goal-concordant surgical care and outcomes.

"It's Not Us Versus Them": Building Cross-Disciplinary Relationships in the Perioperative Period.

CONTEXT: Palliative care (PC) interventions improve quality outcomes for surgical patients, yet they are underutilized in the perioperative period. Developing cross-disciplinary provider relationships increases PC consults. However, the attributes of collaborative relationships and how they evolve are unclear. OBJECTIVES: To identify perceptions of PC providers and surgeons on how collaborative cross-disciplinary relationships are built and maintained in the perioperative period. METHODS: This cross-sectional multiphase qualitative study included 23 semistructured interviews with 10 PC teams (20 providers) and 13 surgeons at geographically distributed Veteran Health Administration (VHA) sites. An analytic approach relied on team-based thematic analysis with a dual review (Krippendorf α above 0.8). RESULTS: Respondents defined successful collaborative work relationships between PC and surgeons as having the following features: 1) mutual trust; 2) mutual respect; 3) perceived usefulness; 4) shared clinical objectives; 5) effective communication; and 6) organizational enablers. In addition, the analysis elucidated a framework of six strategies for developing collaborative relationships between PC and surgical teams in the perioperative period: 1) being present, available, and responsive; 2) understanding roles; 3) establishing communication; 4) recognizing an intermediary and connecting role of supporting team members; 5) working as a team; and 6) building on previous experiences. CONCLUSION: The study informs future interventions to improve the quality of care for seriously ill patients by better-involving PC in the perioperative period. Future work will extend this approach to incorporate the perspectives of patients on their providers' collaboration and how it impacts patient-related outcomes at the intersection of PC and surgery.

Early palliative care consultation offsets hospitalization duration and costs for elderly patients with traumatic brain injuries: Insights from a Level 1 trauma center.

We identified factors and outcomes associated with inpatient palliative care (PC) consultation, stratified into early and late timing, for patients over age 65 with traumatic brain injuries (TBI). Patients over age 65 presenting to a single institution with TBI and intracranial hemorrhage from January 2013-September 2020 were included. Patient demographics and various outcomes were analyzed. Inpatient PC consultation was uncommon (4 % out of 576 patients). Characteristics associated with likelihood of consultation were severe TBI (OR = 5.030, 95 % CI 1.096-23.082, p =.038) and pre-existing dementia (OR = 6.577, 95 % CI 1.726-25.073, p =.006). Average consultation timing was 8.6 (standard deviation ± 7.0) days. Patients with PC consults had longer overall (p =.0031) and intensive care unit (ICU) length of stays (LOS) (p <.0001), more days intubated (p <.0001) and higher costs (p =.0006), although those with earlier-than-average PC consultation had shorter overall (p =.0062) and ICU (p =.011) LOS as well as fewer ventilator days (p =.030) and lower costs (p =.0003). Older patients with TBI are more likely to receive PC based on pre-existing dementia and severe TBI. Patients with PC consultations had worse LOS and higher costs. However, these effects were mitigated by earlier PC involvement. Our study emphasizes the need for timely PC consultation in a vulnerable patient population.

Updates in intraoperative strategies for enhancing intra-axial brain tumor control.

To ensure excellent postoperative clinical outcomes while preserving critical neurologic function, neurosurgeons who manage patients with intra-axial brain tumors can use intraoperative technologies and tools to achieve maximal safe resection. Neurosurgical oncology revolves around safe and optimal extent of resection, which further dictates subsequent treatment regimens and patient outcomes. Various methods can be adapted for treating both primary and secondary intra-axial brain lesions. We present a review of recent advances and published research centered on different innovative tools and techniques, including fluorescence-guided surgery, new methods of drug delivery, and minimally invasive procedural options.

Trends and outcomes of early and late palliative care consultation for adult patients with glioblastoma: A SEER-Medicare retrospective study.

Background: Glioblastoma (GBM) carries a poor prognosis despite standard of care. Early palliative care (PC) has been shown to enhance survival and quality of life while reducing healthcare costs for other cancers. This study investigates differences in PC timing on outcomes for patients with GBM. Methods: This study used Surveillance, Epidemiology, and End Results (SEER)-Medicare data from 1997 to 2016. Based on ICD codes, three groups were defined: (1) early PC within 10 weeks of diagnosis, (2) late PC, and (3) no PC. Outcomes were compared between the three groups. Results: Out of 10 812 patients with GBM, 1648 (15.24%) patients had PC consultation with an overall positive trend over time. There were no significant differences in patient characteristics. The late PC group had significantly higher number of hospice claims (1.06 ± 0.69) compared to those without PC, in the last month of life. There were significant differences in survival among the three groups (P < .0001), with late PC patients with the longest mean time to death from diagnosis (11.72 ± 13.20 months). Conclusion: We present the first investigation of PC consultation prevalence and outcomes, stratified by early versus late timing, for adult GBM patients. Despite an overall increase in PC consultations, only a minority of GBM patients receive PC. Patients with late PC had the longest survival times and had greater hospice use in the last month of life compared to other subgroups. Prospective studies can provide additional valuable information about this unique population of patients with GBM.

Modifiers of and Disparities in Palliative and Supportive Care Timing and Utilization among Neurosurgical Patients with Malignant Central Nervous System Tumors.

Patients with primary or secondary central nervous system (CNS) malignancies benefit from utilization of palliative care (PC) in addition to other supportive services, such as home health and social work. Guidelines propose early initiation of PC for patients with advanced cancers. We analyzed a cohort of privately insured patients with malignant brain or spinal tumors derived from the Optum Clinformatics Datamart Database to investigate health disparities in access to and utilization of supportive services. We introduce a novel construct, "provider patient racial diversity index" (provider pRDI), which is a measure of the proportion of non-white minority patients a provider encounters to approximate a provider's patient demographics and suggest a provider's cultural sensitivity and exposure to diversity. Our analysis demonstrates low rates of PC, home health, and social work services among racial minority patients. Notably, Hispanic patients had low likelihood of engaging with all three categories of supportive services. However, patients who saw providers categorized into high provider pRDI (categories II and III) were increasingly more likely to interface with supportive care services and at an earlier point in their disease courses. This study suggests that prospective studies that examine potential interventions at the provider level, including diversity training, are needed.

An integrated risk model stratifying seizure risk following brain tumor resection among seizure-naive patients without antiepileptic prophylaxis.

OBJECTIVE: The natural history of seizure risk after brain tumor resection is not well understood. Identifying seizure-naive patients at highest risk for postoperative seizure events remains a clinical need. In this study, the authors sought to develop a predictive modeling strategy for anticipating postcraniotomy seizures after brain tumor resection. METHODS: The IBM Watson Health MarketScan Claims Database was canvassed for antiepileptic drug (AED)- and seizure-naive patients who underwent brain tumor resection (2007-2016). The primary event of interest was short-term seizure risk (within 90 days postdischarge). The secondary event of interest was long-term seizure risk during the follow-up period. To model early-onset and long-term postdischarge seizure risk, a penalized logistic regression classifier and multivariable Cox regression model, respectively, were built, which integrated patient-, tumor-, and hospitalization-specific features. To compare empirical seizure rates, equally sized cohort tertiles were created and labeled as low risk, medium risk, and high risk. RESULTS: Of 5470 patients, 983 (18.0%) had a postdischarge-coded seizure event. The integrated binary classification approach for predicting early-onset seizures outperformed models using feature subsets (area under the curve [AUC] = 0.751, hospitalization features only AUC = 0.667, patient features only AUC = 0.603, and tumor features only AUC = 0.694). Held-out validation patient cases that were predicted by the integrated model to have elevated short-term risk more frequently developed seizures within 90 days of discharge (24.1% high risk vs 3.8% low risk, p < 0.001). Compared with those in the low-risk tertile by the long-term seizure risk model, patients in the medium-risk and high-risk tertiles had 2.13 (95% CI 1.45-3.11) and 6.24 (95% CI 4.40-8.84) times higher long-term risk for postdischarge seizures. Only patients predicted as high risk developed status epilepticus within 90 days of discharge (1.7% high risk vs 0% low risk, p = 0.003). CONCLUSIONS: The authors have presented a risk-stratified model that accurately predicted short- and long-term seizure risk in patients who underwent brain tumor resection, which may be used to stratify future study of postoperative AED prophylaxis in highest-risk patient subpopulations.

Quality of Life and Role of Palliative and Supportive Care for Patients With Brain Metastases and Caregivers: A Review.

Brain metastases (BM) are the most commonly diagnosed secondary brain lesions in adults, influencing these patients' symptoms and treatment courses. With improvements in oncologic treatments, patients with BM are now living longer with their advanced cancers, and issues pertaining to quality of life become more pressing. The American Society of Clinical Oncology has recommended early implementation of palliative care for cancer patients, though incorporation and implementation of palliative and other supportive services in the setting of true multidisciplinary care requires additional attention and research for patients with intracranial metastases. We review the physical, cognitive, and psychosocial challenges patients with BM and their caregivers face during their cancer course as well as the current published research on quality of life metrics relating to this patient population and the diverse roles specialty palliative care, rehabilitation services, and other healthcare providers play in a comprehensive multidisciplinary care model.

The Challenges and Future of Immunotherapy for Gliomas.

ABSTRACT: Gliomas and glioblastoma comprise the majority of brain malignancies and are difficult to treat despite standard of care and advances in immunotherapy. The challenges of controlling glioma growth and recurrence involve the uniquely immunosuppressive tumor microenvironment and systemic blunting of immune responses. In addition to highlighting key features of glioma and glioblastoma composition and immunogenicity, this review presents several future directions for immunotherapy, such as vaccines and synergistic combination treatment regimens, to better combat these tumors.

Palliative Care Service Utilization and Advance Care Planning for Adult Glioblastoma Patients: A Systematic Review.

Glioblastoma (GBM) has a median overall survival of 16-21 months. As patients with GBM suffer concurrently from terminal cancer and a disease with progressive neurocognitive decline, advance care planning (ACP) and palliative care (PC) are critical. We conducted a systematic review exploring published literature on the prevalence of ACP, end-of-life (EOL) services utilization (including PC services), and experiences among adults with GBM. We searched from database inception until 20 December 2020. Preferred reporting items for systematic reviews guidelines were followed. Included studies were assessed for quality using the Newcastle-Ottawa Scale. The 16 articles were all nonrandomized studies conducted in six countries with all but two published in 2014 or later. ACP documentation varied from 4-55%, PC referral was pursued in 39-40% of cases, and hospice referrals were made for 66-76% of patients. Hospitalizations frequently occurred at the EOL with 20-56% of patients spending over 25% of their overall survival time hospitalized. Many GBM patients do not pursue ACP or have access to PC. There is a dearth of focused and high-quality studies on ACP, PC, and hospice use among adults with GBM. Prospective studies that address these and additional aspects related to EOL care, such as healthcare costs and inpatient supportive care needs, are needed.

Risk of secondary neoplasms after external-beam radiation therapy treatment of pediatric low-grade gliomas: a SEER analysis, 1973-2015.

OBJECTIVE: Although past studies have associated external-beam radiation therapy (EBRT) with higher incidences of secondary neoplasms (SNs), its effect on SN development from pediatric low-grade gliomas (LGGs), defined as WHO grade I and II gliomas of astrocytic or oligodendrocytic origin, is not well understood. Utilizing a national cancer registry, the authors sought to characterize the risk of SN development after EBRT treatment of pediatric LGG. METHODS: A total of 1245 pediatric patient (aged 0-17 years) records from 1973 to 2015 were assembled from the Surveillance, Epidemiology, and End Results (SEER) database. Univariable and multivariable subdistribution hazard regression models were used to evaluate the prognostic impact of demographic, tumor, and treatment-related covariates. Propensity score matching was used to balance baseline characteristics. Cumulative incidence analyses measured the time to, and rate of, SN development, stratified by receipt of EBRT and controlled for competing mortality risk. The Fine and Gray semiparametric model was used to estimate future SN risk in EBRT- and non-EBRT-treated pediatric patients. RESULTS: In this study, 366 patients received EBRT and 879 did not. Forty-six patients developed SNs after an LGG diagnosis, and 27 of these patients received EBRT (OR 3.61, 95% CI 1.90-6.95; p < 0.001). For patients alive 30 years from the initial LGG diagnosis, the absolute risk of SN development in the EBRT-treated cohort was 12.61% (95% CI 8.31-13.00) compared with 4.99% (95% CI 4.38-12.23) in the non-EBRT-treated cohort (p = 0.013). Cumulative incidence curves that were adjusted for competing events still demonstrated higher rates of SN development in the EBRT-treated patients with LGGs. After matching across available covariates and again adjusting for the competing risk of mortality, a clear association between EBRT and SN development remained (subhazard ratio 2.26, 95% CI 1.21-4.20; p = 0.010). CONCLUSIONS: Radiation therapy was associated with an increased risk of future SNs for pediatric patients surviving LGGs. These data suggest that the long-term implications of EBRT should be considered when making treatment decisions for this patient population.

Large intramedullary bronchogenic cyst of the cervical spine: illustrative case.

BACKGROUND: Spinal bronchogenic cysts are rare entities arising from errors in embryogenesis and consisting of respiratory epithelial cells. To date, there are three other published accounts of intramedullary cysts, which were partially resected and thereby warrant close follow-up and monitoring. The authors present an illustrative case of a patient presenting with Klippel-Feil anomaly and a large intramedullary bronchogenic cyst in the upper cervical spine. OBSERVATIONS: The authors noted fusion of the C5-6 laminae as they performed the C2-6 laminectomy. After dural opening, an intramedullary lesion with a smooth, fibrous component emerging from the dorsal spinal cord was immediately observed. The dorsal spinal columns were not involved with this cyst wall or the other smaller cysts, which all contained gray fluid. The cyst walls were partially resected and sent for pathological examination. LESSONS: Spinal developmental cysts are associated with other anatomical anomalies, such as Klippel-Feil anomaly, arising from errors in embryogenesis. For intramedullary lesions such as this patient's bronchogenic cyst, partial resection and decompression are the goals of surgery because aggressive debulking may lead to neurological compromise. Close imaging follow-up is warranted.