Low-grade fibromyxoid tumor of the dura: A new entity?

Background: Low-grade fibromyxoid tumors are uncommon in children. Their differentiation from high-grade fibromyxoid tumors, as seen in adults, is imperative to diagnosis. Awareness of the entity and its subsequent behavior may guide management and predict outcomes. Case Description: We describe the case of a previously unreported low-grade fibromyxoid tumor of the cerebellum in an 8-year-old male. Extensive immunohistochemical, next-generation sequencing, and attempted DNA methylation profiling are reported. There has been no recurrence during the 6-year follow-up. Screening excluded multiple myxoid tumors, including low-grade fibromyxoid sarcoma. The findings suggest that, with gross total resection, the lesions may not recur. Conclusion: The case of fibromyxoid tumor with 6-year follow-up and the limited literature of similar tumors are reviewed.

Perioperative Complications and Radiographic Outcomes Following Cervical Laminoplasty: A Review of 105 Cases from a Single Academic Center.

OBJECTIVE: To assess complications and to compare preoperative and postoperative radiographic measures in patients who underwent cervical laminoplasty. METHODS: We evaluated patients undergoing cervical laminoplasty between 2011 and 2022 at an academic medical center. Patient demographics, intraoperative and postoperative complications, and preoperative and postoperative radiographic parameters were recorded. RESULTS: Of 150 patients undergoing cervical laminoplasty during 2011-2022, 105 met the inclusion criteria. We identified 22 (21.0%) adverse events in 20 patients: 4 intraoperative (3.8%), 10 perioperative (9.5%), and 8 delayed postoperative (7.6%). Among adverse events, 13 (12.4%) were judged to be minor and 9 (8.6%) were judged to be major complications. Revision surgery was required in 6 (5.7%) patients. Both preoperative and postoperative cervical spine radiographic imaging was available in 47 (44.8%) of the 105 patients. Mean preoperative and postoperative C2-7 Cobb angles were 14.7° and 12.3°, respectively (P = 0.04). Mean preoperative and postoperative sagittal vertical axis measurements were 29.2 mm and 33.3 mm, respectively (P = 0.07). Mean preoperative and postoperative T1 slopes were 32.3° and 30.2°, respectively (P = 0.07). CONCLUSIONS: Cervical laminoplasty carries a significant risk of adverse events, the majority of which are minor, and has a low rate of reoperation (5.7%). The procedure does not result in significant changes in sagittal vertical axis or T1 slope. Cervical laminoplasty remains a valuable surgical option for selected patients with cervical myelopathy.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Surgical Interventions.

BACKGROUND: Chiari malformation type I (CIM) diagnoses have increased in recent years. Controversy regarding the best operative management prompted a review of the literature to offer guidance on surgical interventions. OBJECTIVE: To assess the literature to determine (1) whether posterior fossa decompression or posterior fossa decompression with duraplasty is more effective in preoperative symptom resolution; (2) whether there is benefit from cerebellar tonsillar resection/reduction; (3) the role of intraoperative neuromonitoring; (4) in patients with a syrinx, how long should a syrinx be observed for improvement before additional surgery is performed; and 5) what is the optimal duration of follow-up care after preoperative symptom resolution. METHODS: A systematic review was performed using the National Library of Medicine/PubMed and Embase databases for studies on CIM in children and adults. The most appropriate surgical interventions, the use of neuromonitoring, and clinical improvement during follow-up were reviewed for studies published between 1946 and January 23, 2021. RESULTS: A total of 80 studies met inclusion criteria. Posterior fossa decompression with or without duraplasty or cerebellar tonsil reduction all appeared to show some benefit for symptom relief and syrinx reduction. There was insufficient evidence to determine whether duraplasty or cerebellar tonsil reduction was needed for specific patient groups. There was no strong correlation between symptom relief and syringomyelia resolution. Many surgeons follow patients for 6-12 months before considering reoperation for persistent syringomyelia. No benefit or harm was seen with the use of neuromonitoring. CONCLUSION: This evidence-based clinical guidelines for the treatment of CIM provide 1 Class II and 4 Class III recommendations. In patients with CIM with or without syringomyelia, treatment options include bone decompression with or without duraplasty or cerebellar tonsil reduction. Improved syrinx resolution may potentially be seen with dural patch grafting. Symptom resolution and syrinx resolution did not correlate directly. Reoperation for a persistent syrinx was potentially beneficial if the syrinx had not improved 6 to 12 months after the initial operation. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/3-surgical-interventions .

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Diagnosis.

BACKGROUND: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians. OBJECTIVE: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM. METHODS: PubMed and Embase were queried between 1946 and January 23, 2021, using the search strategies provided in Appendix I of the full guidelines. RESULTS: The literature search yielded 567 abstracts, of which 151 were selected for full-text review, 109 were then rejected for not meeting the inclusion criteria or for being off-topic, and 42 were included in this systematic review. CONCLUSION: Three Grade C recommendations were made based on Level III evidence. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/1-imaging .

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Symptoms.

BACKGROUND: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians. OBJECTIVE: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM. METHODS: PubMed and Embase were queried between 1946 and January 23, 2021, using the search strategies provided in Appendix I of the full guidelines. RESULTS: The literature search yielded 430 abstracts, of which 79 were selected for full-text review, 44 were then rejected for not meeting the inclusion criteria or for being off-topic, and 35 were included in this systematic review. CONCLUSION: Four Grade C recommendations were made based on Class III evidence, and 1 question had insufficient evidence. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/2-symptoms .

Treatment of hydrocephalus in an ovine model with an intraparenchymal stent.

OBJECTIVE: While CSF diversion is highly effective at treating hydrocephalus, shunting is unfortunately associated with a very high revision rate. Studies have demonstrated that proximal catheter obstruction is a major cause of failure. A novel proximal access device was developed, and pilot testing was performed in a sheep model of hydrocephalus. METHODS: Hydrocephalus was induced in 8 sheep using a cisternal injection of 4 ml of 25% kaolin, and the sheep were randomized to either a standard ventricular catheter or a novel intraparenchymal stent (IPS). Both groups received identical valves and distal catheters. The novel device included a 3D-printed stainless steel port and a 6 × 40-mm covered peripheral vascular stent. Animals were euthanized for signs of hydrocephalus or at a time point of 2 months. MRI was performed to determine ventricular size. Time to failure and Evans indices were compared using the Wilcoxon rank-sum test. RESULTS: All 4 experimental devices were placed without difficulty into the right lateral ventricle. There was a trend toward longer survival in the experimental group (40 vs 26 days, p = 0.24). Within the IPS group, 3 of the 4 sheep did not experience clinical symptoms of shunt failure and had an average of 37% decrease in Evans index. While 3 of 4 traditional proximal catheters demonstrated debris within the inlet holes, there was no obstructive material found in the IPSs. CONCLUSIONS: An IPS was successfully used to treat hydrocephalus in a sheep model. While statistical significance was not achieved, there were clear benefits to using a stent, including a decreased clog rate and the ability to perform a percutaneous revision. Further testing is needed to ensure efficacy and safety prior to human application.

Rare Radiographic Changes in a Cervicomedullary Spinal Ependymoma After Biopsy and Drainage of an Intratumoral Cyst: A Staged Approach.

Spinal ependymomas are the most common intramedullary spinal tumor, with a large proportion containing a small intratumoral cyst. Although the signal intensity varies, spinal ependymomas are generally well-demarcated, are not associated with a pre-syrinx, and do not extend above the foramen magnum. Our case demonstrates unique radiographic findings of a cervical ependymoma with a staged approach to diagnosis and resection. The patient is a 19-year-old female who presented with a three-year history of neck pain, progressive arm and leg weakness, falls, and functional decline. MRI revealed an expansile dorsal and centrally located T2 hypointense cervical lesion with a large intratumoral cyst extending from the foramen magnum to the C7 pedicle. Contrasted T1 scans showed an irregular enhancement pattern along the superior tumoral border down to the C3 pedicle. She underwent a C1 laminectomy for open biopsy and cysto-subarachnoid shunt. Postoperative MRI revealed a well-demarcated enhancing mass extending from the foramen magnum to C2. Pathology revealed Grade II ependymoma. She underwent an occipital to C3 laminectomy with gross total resection. Postoperatively she experienced weakness and orthostatic hypotension that improved remarkably upon discharge. Initial imaging was concerning for a higher-grade tumor, with holocervical cord involvement and cervical kyphosis. Given concern for grade and possible extensive C1-7 laminectomy and fusion for resection, a smaller surgery involving drainage of the cyst and biopsy was performed. Postoperative MRI revealed regression of the pre-syrinx, improved tumoral definition, and improvement of cervical kyphosis. This staged approach spared the patient unnecessary surgical intervention such as extensive laminectomy and fusion. We conclude that in cases of a large intratumoral cyst in an extensive intramedullary spinal cord lesion, open biopsy and drainage followed by resection in a staged fashion should be considered. Radiographic changes from the first procedure may affect the surgical approach for ultimate resection.

Pediatric Bilateral Lumbosacral Dislocation Without Fracture: An Exemplary Case and Literature Review.

Lumbosacral facet dislocations are rare lesions typically seen in high-energy trauma. This type of injury is a severe flexion-distraction injury and is extremely rare, with only six other documented reports. A recent case series proposed a classification for lumbosacral injuries which would classify the present case as a 1C, meaning a bilateral dislocation with anterior slippage of the L5 vertebra without fractures of the articulating processes (AP), pars interarticularis (PI), or vertebral bodies (VB). In this case report, we discuss the third case of a class 1C injury in a pediatric patient, review the associated literature and discuss the presentation, diagnosis, management, and prognosis of these rare dislocations.

Spontaneous intracranial hemorrhage presenting in a patient with vitamin K deficiency and COVID-19: illustrative case.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is known to cause more severe symptoms in the adult population, but pediatric patients may experience severe neurological symptoms, including encephalopathy, seizures, and meningeal signs. COVID-19 has also been implicated in both ischemic and hemorrhagic cerebrovascular events. This virus inhibits angiotensin-converting enzyme 2, decreasing angiotensin (1-7), decreasing vagal tone, disrupting blood pressure autoregulation, and contributing to a systemic vascular inflammatory response, all of which may further increase the risk of intracranial hemorrhage. However, there has only been one reported case of intracranial hemorrhage developing in a pediatric patient with COVID-19. OBSERVATIONS: The authors discuss the first case of a pediatric patient with COVID-19 presenting with intracranial hemorrhage. This patient presented with lethargy and a bulging fontanelle and was found to have extensive intracranial hemorrhage with hydrocephalus. Laboratory tests were consistent with hyponatremia and vitamin K deficiency. Despite emergency ventriculostomy placement, the patient died of his disease. LESSONS: This case demonstrates an association between COVID-19 and intracranial hemorrhage, and the authors have described several different mechanisms by which the virus may potentiate this process. This role of COVID-19 may be particularly important in patients who are already at a higher risk of intracranial hemorrhage, such as those with vitamin K deficiency.

Coil and Onyx embolization of a torcular herophili dural arteriovenous fistula in a full-term neonate with advanced heart failure using a transumbilical approach.

Neonatal dural arteriovenous fistulas (DAVFs) are rare, but if left untreated will advance to life-threatening neurological and cardiovascular compromise. Endovascular treatment is the preferred treatment modality for DAVFs. The goal of endovascular therapy is to obliterate feeding vasculature and prevent secondary complications. Endovascular access can be difficult to obtain in a neonate. The authors present the case of a full-term, normal birth weight neonate with severe congestive heart failure secondary to a congenital DAVF of the torcular herophili that was successfully treated with transumbilical arterial coil embolization and a liquid embolic agent.

Rupture of Giant Anterior Sacral Meningocele in a Patient with Marfan Syndrome: Diagnosis and Management.

BACKGROUND: Marfan syndrome is a genetic disorder that results in the weakening of connective tissues. Dural ectasia has been defined as a feature of Marfan syndrome and is present in up to 92% of patients. Rarely, dural ectasia can erode through the sacrum expanding into an anterior sacral meningocele. CASE DESCRIPTION: Information for this case report was gathered from patient notes and imaging from the patient chart. This is a case of a 46-year-old woman who presented with urinary incontinence, early satiety, and back pain in the setting of a known anterior sacral meningocele. Before operative management, the anterior sacral meningocele ruptured with the patient presenting signs and symptoms of intracranial hypotension. Conservative management did not alleviate the pain. She was ultimately managed with posterior sacroplasty followed by anterior sacral meningocele resection and placement of a lumboperitoneal shunt. The patient did not have reaccumulation of the meningocele or recurrent symptoms at the latest follow-up. CONCLUSIONS: The progression of dural ectasia in Marfan syndrome to an anterior sacral meningocele is uncommon. It is important to identify the characteristics associated with an expanding dural ectasia as this patient's symptoms progressed over time and the meningocele grew large. Given its rarity, there are no guidelines in place regarding size at which repair of an anterior sacral meningocele should occur prophylactically. It is important to review these cases in order continue to learn about progression, management, and outcomes of patients with an anterior sacral meningocele.

Imaging and surgical approach to a pediatric penetrating intradural wooden splinter: case report.

Penetrating spinal injuries by wood are infrequently reported. They are particularly rare in children. Only 6 cases of wooden fragments causing penetrating intradural spinal injury have been reported. The authors report a case of a 3-year-old girl who suffered a penetrating wound on her lower back after sliding on a wood floor. A portion of the extraspinal part of the wooden splinter was removed prior to presentation; however, a high suspicion for retained foreign body was maintained. Findings on CT were equivocal, but the diagnosis was confirmed on MRI. An incomplete cauda equina syndrome was noted on examination. She was taken to the operating room for removal of the wooden foreign body, repair of a durotomy, and repair of a CSF leak. At 8 months after surgery, the patient had fully recovered without sequelae. The roles of imaging modalities, prophylactic antibiotics, and surgery are discussed.

Risk factors for wound complications following spine surgery.

BACKGROUND: Wound complications, including surgical site infections (SSIs) and wound dehiscence, are among the most common complications following spine surgery often leading to readmission. The authors sought to identify preoperative characteristics predictive of wound complications after spine surgery. METHODS: The American College of Surgeons National Surgical Quality Improvement Program database for years 2012-2014 was reviewed for patients undergoing spine surgery, defined by the Current Procedural Terminology codes. Forty-four preoperative and surgical characteristics were analyzed for associations with wound complications. RESULTS: Of the 99,152 patients included in this study, 2.2% experienced at least one wound complication (superficial SSI: 0.9%, deep SSI: 0.8%, organ space SSI: 0.4%, and dehiscence: 0.3%). Multivariate binary logistic regression testing found 10 preoperative characteristics associated with wound complications: body mass index ≥30, smoker, female, chronic steroid use, hematocrit <38%, infected wound, inpatient status, emergency case, and operation time >3 hours. A risk score for each patient was created from the number of characteristics present. Receiver operating characteristic curves of the unweighted and weighted risk scores generated areas under the curve of 0.701 (95% CI: 0.690-0.713) and 0.715 (95% CI: 0.704-0.726), respectively. Patients with unweighted risk scores >7 were 25-fold more likely to develop a wound complication compared to patients with scores of 0. In addition, mortality rate, reoperation rate, and total length of stay each increased nearly 10-fold with increasing risk score. CONCLUSION: This study introduces a novel risk score for the development of wound dehiscence and SSIs in patients undergoing spine surgery, using new risk factors identified here.

Novel Technique of Percutaneous Fat Graft for Repair of Persistent Large Pseudomeningocele.

BACKGROUND: Pseudomeningocele is an uncommon but problematic complication in lumbar spine surgery. Initial conservative measures frequently are successful, but persistence requires additional management. The current surgical approach can involve a range of techniques, including blood patches, hydrogel/fibrin sealants, drains, open surgical repair of the dura, or a combination of the all techniques if symptoms persist. This report demonstrates a novel technique for repair via a percutaneous approach to deliver an autologous fat graft into the pseudomeningocele. CASE DESCRIPTION: A 64-year-old woman with a history of multiple complex lumbar spinal surgeries with previous durotomy had persistent positional headaches from an unresolved pseudomeningocele from L3 to L5. The defect and symptoms remained after all available surgical options were exhausted, including blood patches, hydrogel sealants (Duraseal), and open surgical repair with muscle and Gelfoam grafts. After autologous fat harvest, the patient underwent percutaneous repair of the pseudomeningocele with simultaneous aspiration of cerebrospinal fluid and injection of fat into the defect space. CONCLUSIONS: At 3 months postoperatively, the patient's symptoms were resolved with no clinical or radiographic findings of nerve root or thecal sac impingement and complete obliteration of the pseudomeningocele. This less-invasive approach offers the option of open surgical repair for persistent pseudomeningocele with the use of autologous graft material.

mTOR activation is increased in pilocytic astrocytomas from older adults compared with children.

BACKGROUND: Recent studies suggest that the behavior and biology of WHO grade I pilocytic astrocytomas (PAs) in adults is different than that associated with grade I PAs in children. METHODS: We evaluated Ki-67 labeling, BRAF abnormalities, isocitrate dehydrogenase R132 immunoreactivity phosphorylation (activation) of p44/42 mitogen activated protein kinase (MAPK), and mammalian target of rapamycin (mTOR) in formalin-fixed tissue from 21 adult (18 years or older, mean age 37 years) and 10 children (mean age 9.4 years) WHO grade I PAs. RESULTS: The mean Ki-67 labeling was 4.8% in adults and 3.8% in children. There was no significant difference between Ki-67 labeling in children and adults or either subgroups of adults. No differences were found in phospho p44/42MAPK in adult subgroups (18-33 years and 34 and older) compared to children. Activation/phosphorylation of mTOR was biphasic in adults being significantly lower than children in young adults but significantly higher than children in older adults (age 34 and older). CONCLUSIONS: Identifying mTOR phosphorylation/activation may represent a difference in biology and a new marker to guide chemotherapy with recently approved mTOR inhibitors.

Risk factors associated with venous thromboembolism in patients undergoing spine surgery.

OBJECTIVE Patients undergoing spinal surgery are at risk for developing venous thromboembolism (VTE). The authors sought to identify risk factors for VTE in these patients. METHODS The American College of Surgeons National Surgical Quality Improvement Project database for the years 2006-2010 was reviewed for patients who had undergone spinal surgery according to their primary Current Procedural Terminology code(s). Clinical factors were analyzed to identify associations with VTE. RESULTS Patients who underwent spinal surgery (n = 22,434) were identified. The rate of VTE in the cohort was 1.1% (pulmonary embolism 0.4%; deep vein thrombosis 0.8%). Multivariate binary logistic regression analysis revealed 13 factors associated with VTE. Preoperative factors included dependent functional status, paraplegia, quadriplegia, disseminated cancer, inpatient status, hypertension, history of transient ischemic attack, sepsis, and African American race. Operative factors included surgery duration > 4 hours, emergency presentation, and American Society of Anesthesiologists Class III-V, whereas postoperative sepsis was the only significant postoperative factor. A risk score was developed based on the number of factors present in each patient. Patients with a score of ≥ 7 had a 100-fold increased risk of developing VTE over patients with a score of 0. The receiver-operating-characteristic curve of the risk score generated an area under the curve of 0.756 (95% CI 0.726-0.787). CONCLUSIONS A risk score based on race, preoperative comorbidities, and operative characteristics of patients undergoing spinal surgery predicts the postoperative VTE rate. Many of these risks can be identified before surgery. Future protocols should focus on VTE prevention in patients who are predisposed to it.

Acute Presentation of Chiari I Malformation with Hemiparesis in a Pediatric Patient.

BACKGROUND: Chiari I malformation (CM-I) is defined by cerebellar tonsillar herniation through the foramen magnum. Patients typically present with chronic complaints, including headache, dizziness, and numbness, although there are few reports in the literature of pediatric patients presenting acutely with neurological deficit caused by CM-I. We report a child who presented acutely with hemiparesis and magnetic resonance imaging findings consistent with CM-I and spinal cord edema. CASE DESCRIPTION: A 3-year old boy with normal development presented with difficulty walking and increased drooling. His mother stated he was running into objects and had balance issues for several days. Neurological examination showed ataxia with falling to the right after a few steps and weakness of the right arm and leg. His medical history was remarkable only for mild asthma, although he had recently been treated for an upper respiratory viral infection. Computed tomography of the head demonstrated no brainstem mass. Magnetic resonance imaging of the head and cervical spine showed tonsillar ectopia approximately 2 cm below the craniocervical junction with increased T2 signal in the spinal cord from C1 to C3 consistent with syringomyelia and cord edema. The patient underwent suboccipital craniectomy with removal of the posterior arch of C1 and dural patch graft. His postoperative course was unremarkable, with complete resolution of his symptoms at his 1-month follow-up visit. CONCLUSIONS: This case highlights an unusual presentation of CM-I with neurological deficit related to spinal cord edema, possibly precipitated by the "water-hammer" effect of this patient's coughing fits. Providers should be aware of the acute presentations of CM-I.

Risk Modeling Predicts Complication Rates for Spinal Surgery.

STUDY DESIGN: Retrospective review of clinical data registry. OBJECTIVE: In the current era of quality reporting and pay for performance, neurosurgeons must develop models to identify patients at high risk of complications. We sought to identify risk factors for complications in spine surgery and to develop a score predictive of complications. SUMMARY OF BACKGROUND DATA: We examined spinal surgeries from the American College of Surgeons National Surgical Quality Improvement Project (ACS-NSQIP) database. 22,430 cases were identified based on common procedural terminology. METHODS: Univariate analysis followed by multivariate regression was used to identify significant factors. RESULTS: The overall complication rate for the cohort was 9.9%. The most common complications were postoperative bleeding requiring transfusion (4.1%), nonwound infections (3.1%), and wound-related infections (2.2%). Multivariate regression analysis identified 20 factors associated with complications. Assigning 1 point for the presence of each factor a risk model was developed. The range of scores for the cohort was 0 to 13 with a median score of 4. Complication rates for a risk score of 0 to 4 was 3.7% and for scores 5 to 13 was 18.5%. The risk model robustly predicted complication rates, with complication rate of 1.2% for score of 0 (n = 412, 1.8% of total) and 63.6% and 100% for scores of 12 and 13 (n = 22 patients, 0.1% of total cohort) respectively (P < 0.001). The risk score also correlated strongly with total length of stay, mortality, and total work relative value units for the case. CONCLUSION: Patient-specific risk factors including comorbidities are strongly associated with surgical complications, length of stay, cost of care, and mortality in spine surgery and can be used to develop risk models that are highly predictive of complications. LEVEL OF EVIDENCE: 3.