Genetically Modified Porcine Kidneys Have Sufficient Tissue Integrity for Use in Pig-to-Human Xenotransplantation.

OBJECTIVE: We sought to determine if genetically modified porcine kidneys used for xenotransplantation had sufficient tissue integrity to support long term function in a human recipient. BACKGROUND: Kidney transplantation remains the best available treatment for patients with end-stage kidney disease. However, a shortage of available donor human kidneys prevents many patients from achieving the benefits of transplantation. Xenotransplantation is a potential solution to this shortage. Recent pre-clinical human studies have demonstrated kidneys from genetically modified pig donors can be transplanted without hyperacute rejection and capable of providing creatinine and other solute clearance. It is unknown whether the porcine kidneys would tolerate the relatively higher resting blood pressure in an adult human recipient compared to the pig donor or non-human primate (NHP) recipients used in translational studies. Furthermore, previous experience in NHPs raised concerns about the tissue integrity of the porcine ureter and post-xenotransplant growth of the porcine kidney. METHODS: Kidneys recovered from porcine donors with 10 gene edits were transplanted into decedent brain dead recipients who were not eligible for organ donation. Decedents underwent bilateral native nephrectomy prior to transplant and were followed for 3-7 days. Standard induction and maintenance immunosuppression was used as previously reported. Vital signs including blood pressure were recorded frequently. Kidney xenografts were assessed daily, serially biopsied, and were measured at implantation and study completion. RESULTS: Three decedents underwent successful xenotransplantation. Subcapsular hematomas developed requiring incision of the xenograft capsules to prevent Page kidney. Blood pressures were maintained in a physiologic range for adult humans (median arterial pressures (MAP) 108.5mmHg (Interquartile Range (IQR): 97-114mmHg), 74mmHg (IQR: 71-78mmHg), and 95mmHg (IQR: 88-99mmHg. respectively) and no bleeding complications or aneurysm formation was observed. Serial biopsies were taken from the xenografts without apparent loss of tissue integrity, despite the lack of a capsule. Ureteroneocystotomies remained intact without evidence of urine leak. Xenograft growth was observed, but plateaued, in 1 decedent with increased volume of the left and right xenografts by 25% and 26%, respectively, and in the context of human growth hormone levels consistently less <0.1 ng/ml and insulin-like growth factor 1 levels ranging from 34-50 ng/ml. CONCLUSIONS: The findings of this study suggest kidneys from 10-gene edited porcine donors have sufficient tissue integrity to tolerate xenotransplantation into a living human recipient. There was no evidence of anastomotic complications and the xenografts tolerated needle biopsy without issue. Xenograft growth occurred but plateaued by study end; further observation and investigation will be required to confirm this finding and elucidate underlying mechanisms.

The Transcription Factor Mohawk Facilitates Skeletal Muscle Repair via Modulation of the Inflammatory Environment.

Efficient repair of skeletal muscle relies upon the precise coordination of cells between the satellite cell niche and innate immune cells that are recruited to the site of injury. The expression of pro-inflammatory cytokines and chemokines such as TNFα, IFNγ, CXCL1, and CCL2, by muscle and tissue resident immune cells recruits neutrophils and M1 macrophages to the injury and activates satellite cells. These signal cascades lead to highly integrated temporal and spatial control of muscle repair. Despite the therapeutic potential of these factors for improving tissue regeneration after traumatic and chronic injuries, their transcriptional regulation is not well understood. The transcription factor Mohawk (Mkx) functions as a repressor of myogenic differentiation and regulates fiber type specification. Embryonically, Mkx is expressed in all progenitor cells of the musculoskeletal system and is expressed in human and mouse myeloid lineage cells. An analysis of mice deficient for Mkx revealed a delay in postnatal muscle repair characterized by impaired clearance of necrotic fibers and smaller newly regenerated fibers. Further, there was a delay in the expression of inflammatory signals such as Ccl2, Ifnγ, and Tgfß. This was coupled with impaired recruitment of pro-inflammatory macrophages to the site of muscle damage. These studies demonstrate that Mkx plays a critical role in adult skeletal muscle repair that is mediated through the initial activation of the inflammatory response.

Age-stratified comorbid and pharmacologic analysis of patients with glioblastoma.

Background: Increased age is a strong and unfavorable prognostic factor for patients with glioblastoma (GBM). However, the relationships between stratified patient age, comorbidities, and medications have yet to be explored in GBM patient survival analyses. Objective: To evaluate co-morbid conditions, tumor-related symptoms, medication prescriptions, and subject age for patients with GBM and to establish potential targets for prospective studies. Methods: Electronic health records for 565 patients with IDHwt GBM were evaluated at a single center between January 1, 2000 and August 9, 2021 were retrospectively assessed. Data were stratified by MGMT promoter methylation status when available and were used to construct multivariable time-dependent cox models and intra-cohort hazards. Results: Younger (<65 years of age) but not older (≥65 years) GBM patients demonstrated a worse prognosis with movement related disabilities (P < 0.0001), gait/balance difficulty (P = 0.04) and weakness (P = 0.007), as well as psychiatric conditions, mental health disorders (P = 0.002) and anxiety (P = 0.001). In contrast, older but not younger GBM patients demonstrated a worse prognosis with epilepsy (P = 0.039). Both groups had worse survival with confusion/altered mental status (P = 0.023 vs < 0.000) and an improved survival with a Temozolomide prescription. Older but not younger GBM patients experienced an improved hazard with a prescription of ace-inhibitor medications (P = 0.048). Conclusion: Age-dependent novel associations between clinical symptoms and medications prescribed for co-morbid conditions were demonstrated in patients with GBM. The results of the current work support future mechanistic studies that investigate the negative relationship(s) between increased age, comorbidities, and drug therapies for differential clinical decision-making across the lifespan of patients with GBM.

Long-term quality of life outcomes in patients undergoing microsurgical resection of vestibular schwannoma.

Background: While previous studies have assessed patient reported quality of life (QOL) of various vestibular schwannoma (VS) treatment modalities, few studies have assessed QOL as related to the amount of residual tumor and need for retreatment in a large series of patients. Objective: To assess patient reported QOL outcomes following VS resection with a focus on extent of resection and retreatment. Methods: A retrospective chart review was performed using single-center institutional data of adult patients who underwent VS resection by the senior authors between 1989-2018 at Loyola University Medical Center. The Penn Acoustic Neuroma Quality of Life (PANQOL) survey was sent to all patients via postal mail. Results: Fifty-five percent of 367 total patients were female with a mean age of 61.6 years (SD 12.63). The mean period between surgery and PANQOL response was 11.4 years (IQR: 4.74-7.37). The median tumor size was 2 cm (IQR: 1.5-2.8). The mean total PANQOL score was 70 (SD 19). Patients who required retreatment reported lower overall scores (µdiff = -10.11, 95% CI: -19.48 to -0.74; p = 0.03) and face domain scores (µdiff = -20.34, 95% CI: -29.78 to -10.91; p < .001). There was no association between extent of resection and PANQOL scores in any domain. Conclusion: In an analysis of 367 patients who underwent microsurgical resection of VS, extent of resection did not affect PANQOL scores in contrast to previous reports in the literature, while the need for retreatment and facial function had a significant impact on patient-reported outcomes.

Bilateral thalamic high-grade astrocytomas in an early-adolescent child: A case report.

An early-adolescent girl presented with incoordination, headache, vomiting and dysphonia. MRI brain demonstrated diffuse increased T2 and FLAIR signal in bilateral thalami, consistent with anaplastic astrocytomas. A stereotactic burr-hole biopsy provided frozen tissues sections demonstrating an IDH-1 wildtype astrocytoma (anaplastic grade III according to prior WHO classification 2016-21). Chemoradiotherapy was commenced. Bilateral thalamic high-grade astrocytomas are very rare in the paediatric population and require timely diagnosis and interdisciplinary management. CT and MR imaging help point towards this diagnosis in the correct clinical context.

Is There a Plateau to the Learning Curve for Acoustic Neuroma Resection?-Experience and Outcomes from a Single Interdisciplinary Team Over Thirty Years.

Objective The evolution of acoustic neuroma (AN) care continues to shift focus on balancing optimized tumor resection and control with preservation of neurological function. Prior learning curve analyses of AN resection have demonstrated a plateau between 20 and 100 surgeries. In this study of 860 consecutive AN surgeries, we investigate the presence of an extended learning curve tail for AN resection. Methods A retrospective cohort study of AN resections by a single interdisciplinary team between 1988 and 2018 was performed. Proportional odds models and restricted cubic splines were used to determine the association between the timing of surgery and odds of improved postoperative outcomes. Results The likelihood of improved postoperative House-Brackmann (HB) scores increased in the first 400 procedures, with HB 1 at 36% in 1988 compared with 79% in 2004. While the probability of a better HB score increased over time, there was a temporary decrease in slope of the cubic spline between 2005 and 2009. The last 400 cases continued to see improvement in optimal HB outcomes: adjusted odds of HB 1 score were twofold higher in both 2005 to 2009 (adjusted odds ratio [aOR]: 2.11, 95% confidence interval [CI]: 1.38-3.22, p < 0.001) and 2010 to 2018 (aOR: 2.18, 95% CI: 1.49-3.19, p < 0.001). Conclusion In contrast to prior studies, our study demonstrates the steepest growth for learning, as measured by rates of preservation of facial function outcomes (HB 1), occurs in the first 400 AN resections. Additionally, improvements in patient outcomes continued even 30 years into practice, underlining the importance of lifelong learning.

Risk factors and treatment interventions associated with incomplete thrombus resolution and pulmonary hypertension after pulmonary embolism.

BACKGROUND: Residual pulmonary vascular occlusion (RPVO) affects one half of patients after a pulmonary embolism (PE). The relationship between the risk factors and therapeutic interventions for the development of RPVO and chronic thromboembolic pulmonary hypertension is unknown. METHODS: This retrospective review included PE patients within a 26-month period who had baseline and follow-up imaging studies (ie, computed tomography [CT], ventilation/perfusion scans, transthoracic echocardiography) available. We collected the incidence of RPVO, percentage of pulmonary artery occlusion (%PAO), baseline CT %PAO, most recent CT %PAO, and difference between the baseline and most recent %PAO on CT (Δ%PAO). RESULTS: A total of 354 patients had imaging reports available; 197 with CT and 315 with transthoracic echocardiography. The median follow-up time was 144 days (interquartile range [IQR], 102-186 days). RPVO was present in 38.9% of the 354 patients. The median Δ%PAO was -10.0% (IQR, -32% to -1.2%). Fewer patients with a provoked PE developed RPVO (P ≤ .01), and the initial troponin level was lower in patients who developed RPVO (P = .03). The initial thrombus was larger in the patients who received advanced intervention vs anticoagulation (baseline CT %PAO: median, 61.2%; [IQR, 27.5%-75.0%] vs median, 12.5% [IQR, 2.5%-40.0%]; P ≤ .0001). Catheter-directed thrombolysis (CDT; median Δ%PAO, -47.5%; IQR, -63.7% to -8.7%) and surgical pulmonary embolectomy (SPE; median Δ%PAO, -42.5; IQR, -68.1% to -18.7%) had the largest thrombus reduction compared with anticoagulation (P = .01). Of the 354 patients, 76 developed pulmonary hypertension; however, only 14 received pulmonary hypertension medications and 12 underwent pulmonary thromboendarterectomy. Cancer (odds ratio [OR], 1.7) and planned prolonged anticoagulation (>1 year; OR, 2.20) increased the risk of RPVO. In contrast, the risk was lower for men (OR, 0.61), patients with recent surgery (OR, 0.33), and patients treated with SPE (OR, 0.42). A larger Δ%PAO was found in men (coefficient, -8.94), patients with a lower body mass index (coefficient, -0.66), patients treated with CDT (coefficient, -18.12), and patients treated with SPE (coefficient, -21.69). A lower Δ%PAO was found in African-American patients (coefficient, 7.31). CONCLUSIONS: The use of CDT and SPE showed long-term benefit in thrombus reduction.

Regional Social Vulnerability is Associated With Geographic Disparity in Waitlist Outcomes for Patients With Non-Hepatocellular Carcinoma Model for End-stage Liver Disease Exceptions in the United States.

OBJECTIVE: This study was undertaken to evaluate the role of regional social vulnerability in geographic disparity for patients listed for liver transplantation with non-hepatocellular carcinoma (HCC) model for end-stage liver disease (MELD) exceptions. SUMMARY AND BACKGROUND: Prior work has demonstrated regional variability in the appropriateness of MELD exceptions for diagnoses other than HCC. METHODS: Adults listed at a single center for first-time liver-only transplantation without HCC after June 18, 2013 in the Scientific Registry of Transplant Recipients database as of March 2021 were examined. Candidates were mapped to hospital referral regions (HRRs). Adjusted likelihood of mortality and liver transplantation were modeled. Advantaged HRRs were defined as those where exception patients were more likely to be transplanted, yet no more likely to die in adjusted analysis. The Centers for Disease Control's Social Vulnerability Index (SVI) was used as the measure for community health. Higher SVIs indicate poorer community health. RESULTS: There were 49,494 candidates in the cohort, of whom 4337 (8.8%) had MELD exceptions. Among continental US HRRs, 27.3% (n = 78) were identified as advantaged. The mean SVI of advantaged HRRs was 0.42 versus 0.53 in nonadvantaged HRRs ( P = 0.002), indicating better community health in these areas. Only 25.3% of advantaged HRRs were in spatial clusters of high SVI versus 40.7% of nonadvantaged HRRs, whereas 44.6% of advantaged HRRs were in spatial clusters of low SVI versus 38.0% of nonadvantaged HRRs ( P = 0.037). CONCLUSIONS: An advantage for non-HCC MELD exception patients is associated with lower social vulnerability on a population level. These findings suggest assigning similar waitlist priority to all non-HCC exception candidates without considering geographic differences in social determinants of health may actually exacerbate rather than ameliorate disparity.

Normal Graft Function After Pig-to-Human Kidney Xenotransplant.

This case series examines the ability of a pig-to-human xenograft to provide life-sustaining kidney function.

Internal Auditory Canal Glioneural Hamartoma: A Rare Mass Masquerading as a Vestibular Schwannoma.

Glioneural hamartomas are exceedingly rare lesions. When localized to the internal auditory canal (IAC), they can cause symptoms referrable to seventh and eighth cranial nerve compression. Here, the authors present a rare case of an IAC glioneural hamartoma. A 57-year-old male presented for evaluation of presumed intracanalicular vestibular schwannomas found on work-up of dizziness and progressive right-sided hearing loss. Surgical intervention pursued progressive symptoms and new onset headaches. The patient underwent uncomplicated retrosigmoid craniectomy for gross total resection. Histopathological evaluation revealed a glioneural hamartoma. A MEDLINE database search used the terms' cerebellopontine angle' OR 'internal auditory canal' AND 'hamartoma' OR 'heterotopia'. Clinicopathological characteristics and outcomes of the present case were compared to those in the literature. The literature review yielded nine articles describing 11 cases (eight females, three males; median age 40 years, range 11-71) of intracanalicular glioneural hamartomas. Patients most commonly presented with hearing loss and were presumed to have a diagnosis of vestibular schwannoma before histologic diagnosis. Glioneural hamartomas are rare lesions that may be found in the IAC. Although benign, they may be safely resected for cranial nerve function preservation goals with a low risk of recurrence.

Assessing Surgical Approaches for Acoustic Neuroma Resection: Do Patients Perceive a Difference in Quality-of-Life Outcomes?

OBJECTIVE: The objective of this study is to further patient-physician discussion regarding postoperative quality of life expectations after surgical acoustic neuroma resection. STUDY DESIGN: This study is retrospective prospective. Qualifying patients were identified and administered Penn Acoustic Neuroma Quality-of-Life (PANQOL) Scale. SETTING: The setting was Loyola University Chicago Health System. PATIENTS: Three hundred twenty-six patients at our center with surgically resected acoustic neuroma between January 1990 and July 2021 completed the PANQOL. INTERVENTIONS: During postresection follow-up visits, patients were administered the PANQOL survey. MAIN OUTCOME MEASURES: The total PANQOL is comprised of questions addressing quality of life in seven domains of hearing, balance, face, energy, pain, health, and anxiety. Univariate and multivariable analyses were performed to test for associations between surgical approach and/or patient characteristics. RESULTS: Patients who were treated with retrosigmoid approach reported slightly higher PANQOL pain scores when compared with translabyrinthine approach. No association was found between responses on hearing PANQOL and surgical approach. No association was found between approach and total PANQOL score. However, on average female patients reported lower total PANQOL compared with male patients. CONCLUSION: The lack of association between patient response on hearing PANQOL and surgical approach illustrates the impact of preoperative patient counseling in appropriately setting patient expectations. The difference in pain PANQOL response may be due to a higher rate of occipital neuralgia due to incision placement and soft tissue retraction in the retrosigmoid patient group. Surgeons may consider alternative surgical incisions and soft tissue dissection to improve patient's quality of life with respect to postoperative pain.

Perpetuating Disparity: Failure of the Kidney Transplant System to Provide the Most Kidney Transplants to Communities With the Greatest Need.

BACKGROUND: The burden of end-stage kidney disease (ESKD) and kidney transplant rates vary significantly across the United States. This study aims to examine the mismatch between ESKD burden and kidney transplant rates from a perspective of spatial epidemiology. METHODS: US Renal Data System data from 2015 to 2017 on incident ESKD and kidney transplants per 1000 incident ESKD cases was analyzed. Clustering of ESKD burden and kidney transplant rates at the county level was determined using local Moran's I and correlated to county health scores. Higher percentile county health scores indicated worse overall community health. RESULTS: Significant clusters of high-ESKD burden tended to coincide with clusters of low kidney transplant rates, and vice versa. The most common cluster type had high incident ESKD with low transplant rates (377 counties). Counties in these clusters had the lowest overall mean transplant rate (61.1), highest overall mean ESKD incidence (61.3), and highest mean county health scores percentile (80.9%, P <0.001 vs all other cluster types). By comparison, counties in clusters with low ESKD incidence and high transplant rates (n=359) had the highest mean transplant rate (110.6), the lowest mean ESKD incidence (28.9), and the lowest county health scores (20.2%). All comparisons to high-ESKD/low-transplant clusters were significant at P value <0.001. CONCLUSION: There was a significant mismatch between kidney transplant rates and ESKD burden, where areas with the greatest need had the lowest transplant rates. This pattern exacerbates pre-existing disparities, as disadvantaged high-ESKD regions already suffer from worse access to care and overall community health, as evidenced by the highest county health scores in the study.

Greater community vulnerability is associated with poor living donor navigator program fidelity.

BACKGROUND: Community-level factors contribute to living donor kidney transplantation disparities but may also influence the interventions aimed to mitigate these disparities. The Living Donor Navigator Program was designed to separate the advocacy role from the patient in need of transplantation-friends/family are encouraged to participate as the patients' advocates to identify living donors, though some of the patients participate alone as self-advocates. Self-advocates have a lower living donor kidney transplantation likelihood compared to the patients with an advocate. We sought to evaluate the relationship between the patients' community-level vulnerability and living donor navigator self-advocacy as a surrogate for program fidelity. METHODS: This single-center, retrospective study included 110 Living Donor Navigator participants (April 2017-June 2019). Program fidelity was assessed using the participants' advocacy status. Measures of community vulnerability were obtained from the Centers for Disease Control and Prevention Social Vulnerability Index. Modified Poisson regression was used to evaluate the association between community-level vulnerability and living donor navigator self-advocacy. RESULTS: Of the 110 participants, 19% (n = 21) were self-advocates. For every 10% increase in community-level vulnerability, patients had 17% higher risk of self-advocacy (adjusted relative risk 1.17, 95% confidence interval: 1.03-1.32, P = .01). Living in areas with greater unemployment (adjusted relative risk: 1.18, 95% confidence interval: 1.04-1.33, P = .01), single-parent households (adjusted relative risk: 1.23, 95% confidence interval: 1.06-1.42, P = .006), minority population (adjusted relative risk: 1.30, 95% confidence interval: 1.04-1.55, P = .02), or no-vehicle households (adjusted relative risk: 1.17, 95% confidence interval: 1.02-1.35, P = .02) were associated with increased risk of self-advocacy. CONCLUSION: Having a greater community-level vulnerability was associated with poor Living Donor Navigator Program fidelity. The potential barriers identified using the Social Vulnerability Index may direct resource allocation and program refinement to optimize program fidelity and efficacy for all participants.

Occipital Neuralgia following Acoustic Neuroma Resection.

Background While postoperative outcomes of acoustic neuroma (AN) resection commonly consider hearing preservation and facial function, headache is a critical quality of life factor. Postoperative headache is described in the literature; however, there is limited discussion specific to occipital neuralgia (ON) following AN resection. Objective The aim of this study is to investigate the effectiveness of conservative management and surgery. Methods We conducted a retrospective review of 872 AN patients who underwent resection at our institution between 1988 and 2017 and identified 15 patients (1.9%) that met International Classification of Headache Disorders criteria for ON. Results Of the 15 ON patients, surgical approaches included 13 (87%) retrosigmoid (RS), one (7%) translabyrinthine (TL), and one (7%) combined RS + TL. Mean clinical follow-up was 119 months (11-263). Six (40%) patients obtained pain relief through conservative management, while the remaining nine (60%) underwent surgery or ablative procedure. Three (38%) patients received an external neurolysis, four (50%) received a neurectomy, one (13%) had both procedures, and one (13%) received two C2 to 3 radio frequency ablations. Of the nine patients who underwent procedural ON treatment, seven (78%) patients achieved pain relief, one patient (11%) continued to have pain, and one patient (11%) was lost to follow-up. Of the six patients whose pain was controlled with conservative management and nerve blocks, five (83%) found relief by using neuropathic pain medication and one (17%) found relief on nonsteroidal anti-inflammatory drug. Conclusion Our series demonstrates success with conservative management in some, but overall a minority (40%) of patients, reserving decompression only for refractory cases.

Abnormal time-zero histology is predictive of kidney transplant outcomes.

INTRODUCTION: Time-zero biopsies can detect donor-derived lesions at the time of kidney transplantation, but their utility in predicting long-term outcomes is unclear under the updated Kidney Allocation System. METHODS: We conducted a single-center retrospective cohort study of 272 consecutive post-reperfusion time-zero biopsies. We tested the hypothesis that abnormal time-zero histology is a strong indicator of donor quality that increases the precision of the kidney donor profile index (KDPI) score to predict long-term outcomes. RESULTS: We detected abnormal biopsies in 42% of the cohort, which were independently associated with a 1.2-fold increased hazard for a composite of acute rejection, allograft failure, and death after adjusting for clinical characteristics including KDPI. By Kaplan-Meier analysis, the relationship between abnormal time-zero histology and the composite endpoint was only significant in the subgroup of deceased donor kidney transplants with KDPI scores >35. Abnormal time-zero histology, particularly vascular intimal fibrosis and arteriolar hyalinosis scores, was independently associated with lower 12-month estimated GFR. CONCLUSION: In conclusion, abnormal time-zero histology is relatively common and identifies a group of kidney recipients at increased risk for worse long-term outcomes. Further studies are needed to determine the optimal patient population in which to deploy time-zero biopsies as an additional surveillance tool.

Survival benefit of concomitant chemoradiation in adult supratentorial primary glioblastoma. A propensity score weighted population-based analysis.

BACKGROUND: This population study aims to assess the impact of the implementation of the original Stupp protocol on overall survival in patients with new-diagnosed supratentorial primary GBM. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to study the survival of histologically confirmed adult supratentorial GBM patients diagnosed between 1998 and 2016. Kaplan-Meier, and a univariate and propensity-score weighted multivariate Cox proportional hazard model adjusted for age at diagnosis, sex, race, marital status and extent of resection was used to assess the survival of patients prior to implementation of the Stupp protocol in 2005 (Pre-Stupp) and following implementation of the Stupp Protocol until 2016 (Post Stupp). RESULTS: Overall, 6390 patients satisfied inclusion exclusion criteria. Median survival times were 13 months for the Pre-Stupp and 15 months for Post-Stupp groups (P<0.001). The 1-, 2-, 5- and 10-year survival rates for the Pre-Stupp group were 51%, 18%, 5% and 2% respectively compared to 59%, 27%, 8% and 4% on the Post-Stupp group. Propensity-score weighted analysis showed a lower mortality risk for patients who underwent concomitant chemoradiation during the Post-Stupp era (HR=0.77, 95% CI 0.62-0.94). There was a 42% relative reduction in the risk of death for patients treated during the Post-Stupp era. CONCLUSIONS: This population-based propensity-score study with long-term follow-up suggests that the implementation of the Stupp protocol in 2005 had a positive impact on the survival of patients with supratentorial GBM. This "real-world" analysis validates the results of the original randomized control trial on which this protocol is based.

Surgical correction of pediatric spinal deformities with coexisting intraspinal pathology: A case report and literature review.

BACKGROUND: Surgical correction of spinal deformities with coexisting intraspinal pathology (SDCIP) requires special consideration to minimize risks of further injury to an already abnormal spinal cord. However, there is a paucity of literature on this topic. Here, the authors present a pediatric patient with a residual pilocytic astrocytoma and syringomyelia who underwent surgical correction of progressive postlaminectomy kyphoscoliosis. Techniques employed are compared to those in the literature to compile a set of guidelines for surgical correction of SDCIP. METHODS: A systematic MEDLINE search was conducted using the following keywords; "pediatric," "spinal tumor resection," "deformity correction," "postlaminectomy," "scoliosis correction," "intraspinal pathology," "tethered cord," "syringomyelia," or "diastematomyelia." Recommendations for surgical technique for pediatric SDCIP correction were reviewed. RESULTS: The presented case demonstrates recommendations that primarily compressive forces on the convexity of the coronal curve should be used when performing in situ correction of SDCIP. Undercorrection is favored to minimize risks of traction on the abnormal spinal cord. The literature yielded 13 articles describing various intraoperative techniques. Notably, seven articles described use of compressive forces on the convex side of the deformity as the primary mode of correction, while only five articles provided recommendations on how to safely and effectively surgically correct SDCIP. CONCLUSION: The authors demonstrated with their case analysis and literature review that there are no clear current guidelines regarding the safe and effective techniques for in situ correction and fusion for the management of pediatric SDCIP.

Anaplastic Hemangiopericytoma of the Jugular Foramen: Case Report and Systematic Review.

BACKGROUND: Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the jugular foramen (JF). The authors also conduct a systematic review of the literature to examine the current fund of knowledge on JF HPC/SFTs. METHODS: A systematic MEDLINE search was conducted using key words "hemangiopericytoma" OR "solitary fibrous tumor" AND "jugular foramen" OR "extracranial" OR "skull base." Clinicopathologic characteristics and outcomes of the present case were reviewed and compared with those in the literature. RESULTS: A 41-year-old male, who had undergone stereotactic radiation therapy 6 years ago for a presumed glomus jugulare tumor, presented to our institution with worsening dysphagia, hoarseness, persistent tongue weakness, and radiographic evidence of tumor progression. The patient underwent uncomplicated gross total resection with sacrifice of the infiltrated hypoglossal nerve. Histopathologic evaluation revealed anaplastic HPC/SFT (World Health Organization grade III). Review of the literature yielded 9 additional cases of JF HPC/SFT in 5 males (56%) and 4 females (44%), with a mean age of 49.6 years old. Patients commonly presented with pain (37.5%) and lower cranial nerve deficits (100%). Preoperative diagnoses included glomus jugulare (n = 2) or JF schwannomas (n = 3). All patients underwent microsurgical resection of the lesion, except for 1 who refused all treatment after diagnostic biopsy. CONCLUSION: The authors present the only reported case of anaplastic HPC of the JF. The illustrative case and those found on systematic review of the literature highlight the importance of tissue diagnosis and appropriate management.