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Lekhaj C. Daggubati, Justin R. Davanzo, and Elias B. Rizk

Neuromas are a difficult-to-treat peripheral nerve pathology that can cause crippling pain. Optimal treatment is widely debated as pharmacological intervention frequently is not sufficient and surgical interventions are plagued with recurrence. The majority of amputees report severe and chronic stump pain. Avoiding complex surgery at the stump site would prevent infection or wound dehiscence. Recent advances in neuromodulation with external pulse emitters allow for pain relief with localized nerve stimulation. The authors describe the novel placement of a sciatic nerve stimulator in a 77-year-old man for painful stump neuromas of the common peroneal and tibial nerves.

The video can be found here:https://youtu.be/96kKs3qjtqc

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Bao Y. Sciscent, David R. Hallan, and Elias B. Rizk

OBJECTIVE

Although medical advances have allowed most patients with spina bifida (SB) to survive into adulthood, these patients may have physical impairments, urological complications, infections, and neurocognitive deficits. These factors can cause psychological distress and impact the transition from pediatric to adult care. There remains limited research on mental health disorders (MHDs) and substance use disorders (SUDs) in SB patients during this vulnerable transition period. This study aimed to investigate the 10-year incidence of MHDs and SUDs in 18- to 25-year-old patients with SB.

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TriNetX, a federated de-identified database, was retrospectively queried to identify 18- to 25-year-old patients with SB. The presence of MHDs and SUDs based on ICD-10 codes in SB patients (cohort 1) was analyzed and compared with those of patients without SB (cohort 2). Subgroup analysis was performed on SB patients with hydrocephalus and neurogenic bladder (NB). SB patients were further compared to patients with a spinal cord injury (SCI).

RESULTS

After propensity score matching, the authors identified 1494 patients in each cohort. SB patients were more likely to have depression (OR 1.949, 95% CI 1.64–2.317), anxiety (OR 1.603, 95% CI 1.359–1.891), somatoform disorders (OR 2.102, 95% CI 1.052–4.199), and suicidal ideations or attempts and self-harm (OR 1.424, 95% CI 1.014–1.999). The prevalence of attention-deficit/hyperactivity disorder (ADHD) and eating disorders were comparable between cohorts. SB patients also had increased rates of nicotine dependence (OR 1.546, 95% CI 1.22–1.959) but not of alcohol or opioid disorders. In SB patients, the presence of hydrocephalus and NB was not associated with significantly increased rates of any measured MHDs or SUDs. When compared with SCI patients, SB patients were more likely to have anxiety (OR 1.377, 95% CI 1.028–1.845) and ADHD (OR 1.875, 95% CI 1.084–3.242). However, SB patients had lower rates of nicotine dependence (OR 0.682, 95% CI 0.482–0.963) and opioid-related disorders (OR 0.434, 95% CI 0.223–0.845). SB and SCI patients shared similar rates of depression, suicidal ideations or attempts and self-harm, and alcohol-related disorders.

CONCLUSIONS

Young adults with SB have higher prevalence rates of MHDs and SUDs compared with the general population. Therefore, incorporation of mental health and substance use management is critical to facilitate transition to adulthood.

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Justin R. Davanzo, J. Christopher Zacko, Charles S. Specht, and Elias B. Rizk

This is the first reported case of an adult presenting with tethering symptoms, limb discrepancy on physical examination, a low-lying spinal cord, and duplicate filum terminale discovered intraoperatively. Intraoperative imaging and pathological analysis of a specimen confirmed the diagnosis of duplicate filum. This is the first reported adult case with duplication of the filum terminale. Release of both fila was necessary in this case to relieve the tethering symptoms.

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Russell Payne, Zeinab Nasralah, Emily Sieg, Elias B. Rizk, Michael Glantz, and Kimberly Harbaugh

OBJECTIVE

A thorough understanding of anatomy is critical for successful carpal tunnel release. Several texts depict the median nerve (MN) as taking a course parallel to the long axis of the forearm (LAF). The authors report on their attempt to formally assess the course of the MN as it travels to the carpal tunnel in the distal wrist and discuss its potential clinical significance.

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The width of the wrist, the distance from the radial wrist to the MN, and the distance from the distal volar wrist crease to the point where the MN emerges between the flexor carpi radialis (FCR) tendon and the flexor digitorum superficialis (FDS) tendons were recorded during cadaveric dissection of 76 wrist specimens. The presence or absence of palmaris longus was documented. Finally, the angles between the MN and FCR tendon and between the MN and the LAF were measured using ImageJ.

RESULTS

The relative position of the MN at the distal wrist crease, as determined by the ratio of the distance from the MN to the radial wrist divided by wrist width, revealed a mean value of 0.48, indicating that the nerve was usually located just radial to midline. The mean distance between the distal wrist crease and the MN's emergence was 34.6 mm. The mean angle between the MN and the FCR tendon was 14.1°. The angle between the MN and the LAF had a mean value of 8.8° (range 0.0°–32.2°). The nerve was parallel to the LAF in only 10.7% of the studied wrists. Palmaris longus was absent in 14 (18.4%) of the 76 wrists.

CONCLUSIONS

The MN takes an angular approach to the carpal tunnel in the distal wrist in the vast majority of cases. This newly described finding will be useful to both clinicians and anatomists.

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Christina Huang, Elias Rizk, Mark Iantosca, Andrea L. Zaenglein, Klaus F. Helm, Arabinda K. Choudhary, and Mark S. Dias

An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.

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Russell Payne, Oliver D. Mrowczynski, Becky Slagle-Webb, Alexandre Bourcier, Christine Mau, Dawit Aregawi, Achuthamangalam B. Madhankumar, Sang Y. Lee, Kimberly Harbaugh, James Connor, and Elias B. Rizk

OBJECTIVE

Malignant peripheral nerve sheath tumors (MPNSTs) are soft-tissue sarcomas arising from peripheral nerves. MPNSTs have increased expression of the oncogene aurora kinase A, leading to enhanced cellular proliferation. This makes them extremely aggressive with high potential for metastasis and a devastating prognosis; 5-year survival estimates range from a dismal 15% to 60%. MPNSTs are currently treated with resection (sometimes requiring limb amputation) in combination with chemoradiation, both of which demonstrate limited effectiveness. The authors present the results of immunohistochemical, in vitro, and in vivo analyses of MLN8237 for the treatment of MPNSTs in an orthoxenograft murine model.

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Immunohistochemistry was performed on tumor sections to confirm the increased expression of aurora kinase A. Cytotoxicity analysis was then performed on an MPNST cell line (STS26T) to assess the efficacy of MLN8237 in vitro. A murine orthoxenograft MPNST model transfected to express luciferase was then developed to assess the efficacy of aurora kinase A inhibition in the treatment of MPNSTs in vivo. Mice with confirmed tumor on in vivo imaging were divided into 3 groups: 1) controls, 2) mice treated with MLN8237, and 3) mice treated with doxorubicin/ifosfamide. Treatment was carried out for 32 days, with imaging performed at weekly intervals until postinjection day 42. Average bioluminescence among groups was compared at weekly intervals using 1-way ANOVA. A survival analysis was performed using Kaplan-Meier curves.

RESULTS

Immunohistochemical analysis showed robust expression of aurora kinase A in tumor cells. Cytotoxicity analysis revealed STS26T susceptibility to MLN8237 in vitro. The group receiving treatment with MLN8237 showed a statistically significant difference in tumor size compared with the control group starting at postinjection day 21 and persisting until the end of the study. The MLN8237 group also showed decreased tumor size compared with the doxorubicin/ifosfamide group at the conclusion of the study (p = 0.036). Survival analysis revealed a significantly increased median survival in the MLN8237 group (83 days) compared with both the control (64 days) and doxorubicin/ifosfamide (67 days) groups. A hazard ratio comparing the 2 treatment groups showed a decreased hazard rate in the MLN8237 group compared with the doxorubicin/ifosfamide group (HR 2.945; p = 0.0134).

CONCLUSIONS

The results of this study demonstrate that MLN8237 is superior to combination treatment with doxorubicin/ifosfamide in a preclinical orthoxenograft murine model. These data have major implications for the future of MPNST research by providing a robust murine model as well as providing evidence that MLN8237 may be an effective treatment for MPNSTs.

Free access

Michael J. Gigliotti, Christine Mau, Charles S. Specht, Cynthia Lawson, Sarah McNutt, Shreela Natarajan, Elias B. Rizk, and Mark Iantosca

OBJECTIVE

TheWHO Classification of Tumours of the Central Nervous System(2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population.

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The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with “posterior fossa” or “spindle cell tumors” or “Ewing sarcoma” or “high-grade” or “spindle cell sarcoma” or “leptomeningeal melanocytoma” as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome.

RESULTS

The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease.

CONCLUSIONS

回顾这3例和现有的文学ure support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.

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保Debarati Bhanja, y Sciscent, Lekhaj C。Daggubati, Casey A. Ryan, Natalie K. Pahapill, Sprague W. Hazard, and Elias B. Rizk

OBJECTIVE

Awake craniotomy (AC) is employed to maximize tumor resection while preserving neurological function in eloquent brain tissue. This technique is used frequently in adults but remains poorly established in children. Its use has been limited due to concern for children’s neuropsychological differences compared with adults and how these differences may interfere with the safety and feasibility of the procedure. Among studies that have reported pediatric ACs, complication rates and anesthetic management vary. This systematic review was performed to comprehensively analyze outcomes and synthesize anesthetic protocols of pediatric ACs.

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作者遵循棱镜来提取studies that reported AC in children with intracranial pathologies. The Medline/PubMed, Ovid, and Embase databases were searched from database inception to 2021, using the terms ("awake") AND ("Pediatric*" OR "child*") AND (("brain" AND "surgery") OR "craniotomy"). Data extracted included patient age, pathology, and anesthetic protocol. Primary outcomes assessed were premature conversion to general anesthesia, intraoperative seizures, completion of monitoring tasks, and postoperative complications.

RESULTS

Thirty eligible studies published from 1997 to 2020 were included that described a total of 130 children ranging in age from 7 to 17 years who had undergone AC. Of all patients reported, 59% were male and 70% had left-sided lesions. Procedure indications included the following etiologies: tumors (77.6%), epilepsy (20%), and vascular disorders (2.4%). Four (4.1%) of 98 patients required conversion to general anesthesia due to complications or discomfort during AC. In addition, 8 (7.8%) of 103 patients experienced intraoperative seizures. Furthermore, 19 (20.6%) of 92 patients had difficulty completing monitoring tasks. Postoperative complications occurred in 19 (19.4%) of 98 patients and included aphasia (n = 4), hemiparesis (n = 2), sensory deficit (n = 3), motor deficit (n = 4), or others (n = 6). The most commonly reported anesthetic techniques were asleep-awake-asleep protocols using propofol, remifentanil or fentanyl, a local scalp nerve block, and with or without dexmedetomidine.

CONCLUSIONS

The findings of this systematic review suggest the tolerability and safety of ACs in the pediatric population. Although pediatric intracranial pathologies pose etiologies that certainly may benefit from AC, there is a need for surgeons and anesthesiologists to perform individualized risk-benefit analyses due to the risks associated with awake procedures in children. Age-specific, standardized guidelines for preoperative planning, intraoperative mapping, monitoring tasks, and anesthesia protocols will help to continue minimizing complications, while improving tolerability, and streamlining workflow in the treatment of this patient population.

Free access

Christoph J. Griessenauer, Elias Rizk, Joseph H. Miller, Philipp Hendrix, R. Shane Tubbs, Mark S. Dias, Kelsie Riemenschneider, and Joshua J. Chern

Object

Tectal plate gliomas are generally low-grade astrocytomas with favorable prognosis, and observation of the lesion and management of hydrocephalus remain the mainstay of treatment.

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A cohort of patients with tectal plate gliomas at 2 academic institutions was retrospectively reviewed.

Results

Forty-four patients with a mean age of 10.2 years who harbored tectal plate gliomas were included in the study. The mean clinical and radiological follow-up was 7.6 ± 3.3 years (median 7.9 years, range 1.5–14.7 years) and 6.5 ± 3.1 years (median 6.5 years, range 1.1–14.7 years), respectively. The most frequent intervention was CSF diversion (81.8% of patients) followed by biopsy (11.4%), radiotherapy (4.5%), chemotherapy (4.5%), and resection (2.3%). On MR imaging tectal plate gliomas most commonly showed T1-weighted isointensity (71.4%), T2-weighted hyperintensity (88.1%), and rarely enhanced (19%). The initial mean volume was 1.6 ± 2.2 cm3and it increased to 2.0 ± 4.4 cm3(p = 0.628) at the last follow-up. Frontal and occipital horn ratio (FOHR) and third ventricular width statistically decreased over time (p < 0.001 and p < 0.05, respectively).

Conclusions

The authors' results support existing evidence that tectal plate gliomas frequently follow a benign clinical and radiographic course and rarely require any intervention beyond management of associated hydrocephalus.

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Mark S. Dias, Ming Wang, Menglu Liang, Elias B. Rizk, Robin Bowman, Michael D. Partington, Jeffrey P. Blount, Brandon G. Rocque, Betsy Hopson, Amy Lee, and William O. Walker

OBJECTIVE

The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates.

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Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression.

RESULTS

Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16–0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20–0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50–9.88, p = 0.005).

CONCLUSIONS

Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors’ prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.

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