The effects of socioeconomic status and race on pediatric neurosurgical shunting.

PURPOSE: It is established in the literature that disparities exist in the quality of healthcare for patients from disadvantaged backgrounds and lower socioeconomic status. There may be roadblocks within the field of neurosurgery preventing equal access and quality of care. Our goal was to study the similarities between pediatric patients with shunted hydrocephalus of different insurance types and race. METHODS: A retrospective chart review was performed on all pediatric patients who underwent ventriculoperitoneal shunting from 1990-2010 at our institution. Race and insurance type were recorded and assessed against specific outcome measures to statistically compare complication rates. RESULTS: A complete record was found for 373 patients who received 849 shunting procedures at our institution. No differences were found between racial groups and insurance type for overall shunt survival, total revision number, or average time to failure. However, nonwhite patients spent an average of 3 days longer in the hospital at initial shunting (p = 0.04), and those with public insurance stayed for 5 days longer (p = 0.002). Patients with public insurance were more likely to present with shunt failure from outside hospitals (p = 0.005) and be born prematurely (p < 0.001). Private patients were more likely to have a neoplasm present at time of initial shunt placement (p = 0.003). CONCLUSION: While overall revision rate was not affected by race or insurance status, there were significant delays in discharge for patients with public insurance. Moreover, potential disparities in outpatient access to primary physicians and specialists may be affecting care.

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.

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.

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.

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 .

Incidental brain lesions in children: to treat or not to treat?

Central nervous system (CNS) lesions that are discovered incidentally when imaging children for problems that were unrelated to the detected lesion pose a dilemma to physicians. Because there are few data on the outcome of such cases, we retrospectively reviewed the clinical course of a group of children followed at our institution with brain lesions found incidentally on neuro-imaging. A database of all children with brain lesions followed at the University of Rochester medical center from 2000 to 2010 was reviewed. Data were obtained regarding presentation, magnetic resonance imaging (MRI) features, treatment, progression-free survival, and overall survival of children with brain lesions found incidentally. Of the 244 children with brain lesions seen over this time period, 21 (8.6%) were found to have incidentally discovered brain lesions. Of these 21 children, 12 (57%) underwent surgical resection of their brain lesions. Ten patients (48%) had symptoms considered to be unassociated with the detected lesion. Lesions were found in the cerebellum (n = 7, 33%), midline (n = 5, 24%), and cerebrum (n = 9, 43%). All lesions were ≤5 cm in diameter. Eight patients (38%) had surgery at presentation, one because of imaging features suspicious for a posterior fossae ependymoma, and the seven others because of location in the posterior fossae or brain stem. Of the remaining 13 patients, five had progression of disease on serial MRI scans: four underwent surgery and the fifth was monitored and remained stable after the initial progression stabilized. Nine of the ten patients (90%) with posterior fossae lesions underwent surgery, while only three of 11 with supratentorial lesions underwent surgery (27%) (P = 0.006). The progression free survival was 94% at 12 months (95% CI 65-99%) and 71% at 24 months (95% CI 39-88%). At a median follow-up of 32 months, the overall survival was 100%. Incidentally detected CNS lesions are usually small. The outcome for children with such lesions is excellent. Close monitoring of these patients with serial MRIs may be a safe alternative to immediate biopsy and/or resection for select patients.

Spurious progression in pediatric brain tumors.

In this study, we sought to characterize post-therapy MRI changes mimicking progression, which we refer to as "spurious progression" (SP) in children with brain tumors. We analyzed whether SP is associated with particular tumor types or therapeutic modalities. Between 2000 and 2009, we identified 181 consecutive children <21 years of age at our center who were treated for brain tumors and had at least three MRI scans within a year after completing therapy. SP was defined as MRI abnormalities characterized by increase in size, enhancement, edema, or cystic changes within 12 months following therapy, and stabilization or improvement on subsequent imaging. One-hundred forty-one patients with brain tumors were evaluable. Fifty-six (40%) had imaging abnormalities initially suggestive of disease progression; of these, 34 (24%) had true disease progression (TP). The remaining 22 (16%) had SP based on either stability, decrease in enhancement, edema, size, or disappearance of these cystic or non-cystic abnormalities. SP occurred in patients with low grade (n = 20) and high grade lesions (n = 2). Median time to SP was 2.4 months (range, 0.7-8.3 months), with time to stability, decrease, or disappearance at a median of 4 months (range 1.4-7.7 months). Five patients were clinically symptomatic from SP and were treated with steroids, cyst drainage, and/or surgery. Therefore, SP occurs more commonly in children with low grade tumors, but can also occur with high grade brain tumors, regardless of therapeutic approach.

Indications for pediatric external ventricular drain placement and risk factors for conversion to a ventriculoperitoneal shunt.

BACKGROUND/AIMS: The external ventricular drain (EVD) is commonly used for the treatment of hydrocephalus and intracranial pressure (ICP) monitoring. In this study, we retrospectively reviewed indications for EVD placement over the last 20 years in pediatric patients at our institution. Additionally, we evaluated the rate that an EVD needed to be converted to a ventriculoperitoneal shunt (VPS) and ascertained how hydrocephalus etiology impacted long-term drainage requirements. METHODS: Patients that received an EVD between 1990 and 2010 at our institution were identified using billing codes. Demographics, etiology of hydrocephalus, length of stay (LOS) and EVD duration were recorded. Only patients receiving their first EVD were included; those with any prior ventricular catheter (including prior shunts) were excluded from this study. RESULTS: A total of 180 patients underwent first-time placement of an EVD over the 20-year period. Of these, 78.9% were placed on the right side and 81.7% were performed through a frontal burr hole site. The average age of all patients at their initial procedure was 9.3 years (median 9.8; range 0-18), hospital LOS was 22.5 days (median 16; range 1-174) and EVD duration was 7.8 days (median 7; range 1-37). Trauma (43.3%) and neoplasms (32.2%) were the most common indications. Approximately, one quarter of the patients were unable to become EVD independent and, therefore, required a VPS. Only patients receiving an EVD for traumatic brain injury were significantly less likely to be converted to a shunt than other causes of hydrocephalus (relative risk, RR = 0.18, 95% confidence interval, CI = 0.07-0.46). Conversely, patients with neoplasms (RR = 3.56, 95% CI = 1.74-7.31), obstructive hydrocephalus (RR = 5.48, 95% CI = 1.37-22.0) and intraventricular hemorrhage (RR = 9.86, 95% CI = 1.05-92.3) were more likely to need a shunt. CONCLUSION: Traumatic brain injuries and neoplasms represent the most common indications for EVD placement in the pediatric population. While patients with neoplasm were much more likely to need conversion to a VPS for long-term cerebrospinal fluid diversion, the majority of EVDs in patients with head injuries were successfully weaned.

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.

Ventriculosubgaleal shunting--a strategy to reduce the incidence of shunt revisions and slit ventricles: an institutional experience and review of the literature.

BACKGROUND/AIMS: Slit ventricles and multiple episodes of shunt failure are problematic in many infants and preterm neonates shunted for hydrocephalus. We utilized ventriculosubgaleal (VSG) shunting as the initial neurosurgical intervention in neonates with hydrocephalus associated with intraventricular hemorrhage and infants with myelomeningocele. METHODS: We conducted a chart review of 21 children initially treated with a VSG shunt between November 2002 and July 2009. Patient records and imaging studies were reviewed. Demographics, case data and clinical outcome were collected. RESULTS: Five patients (27.8%) required a revision after conversion to a ventriculoperitoneal (VP) shunt. There were 9 cases of radiographic slit ventricles (45%). Average follow-up was 59.5 months (range 12-97 months). Average time interval to shunt conversion was 81.5 days. Two patients have not required conversion to a VP shunt (one with an 8-year follow-up). To date, none of these patients has required a subtemporal window or cranial vault expansion. CONCLUSION: Based on our results, initial management of selected hydrocephalic infants with a VSG shunt may prove to be advantageous in the long run for these children as the number of shunt revisions and the incidence of slit ventricles are significantly less than those reported in the literature.

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.

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.

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.

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.

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.