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.

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.

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.

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.

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.

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.

Prenatal diagnosis of terminal myelocystocele in the fetal surgery era: case report.

OBJECTIVE AND IMPORTANCE: We report a case of a child with terminal myelocystocele (TMC). This case exemplifies the importance of performing a multidisciplinary evaluation and magnetic resonance imaging in the patient with suspected meningomyelocele when fetal surgery is being considered. CLINICAL PRESENTATION: This patient was evaluated at the Center for Fetal Diagnosis and Treatment and considered for fetal surgery to repair meningomyelocele. TECHNIQUE: A follow-up based on both ultrasound and magnetic resonance imaging was performed throughout pregnancy. CONCLUSION: Although a definitive diagnosis of TMC was not established prenatally, the patient was not offered fetal surgery, based on multiple selection criteria. We correlate the pathophysiology of TMC with the radiographic and amniocentesis findings. TMC must be considered in the differential diagnosis in a fetus with a dysraphic defect.

Revision rate of pediatric ventriculoperitoneal shunts after 15 years.

OBJECT: Ventriculoperitoneal (VP) shunt placement remains the mainstay treatment for pediatric hydrocephalus. These devices have a relatively high complication and failure rate, often requiring multiple revisions. The authors present a single institution's experience of pediatric patients treated with VP shunts. With an average follow-up time of 20 years, this study is among the longest reports of VP shunt revision in the literature to date. Hydrocephalus origins, shunt revision rates, and causes of shunt failure are described. Patients who underwent their first shunt revision more than 10 years after initial shunt placement were also explored. METHODS: A retrospective chart review was performed on all pediatric patients who underwent VP shunt placement from January 1990 through November 1996 at the University of Rochester Medical Center. Only patients who had at least 15 years of follow-up since their initial shunting procedure were included. RESULTS: A total of 234 procedures were performed on 64 patients, with a mean follow-up of 19.9 years. Patients ranged from a few days to 17.2 years old when they received their original shunt, with a median age of 4 months; 84.5% of the patients required 1 or more shunt revisions and 4.7% required 10 or more. Congenital defects, Chiari Type II malformations, tumors, and intraventricular hemorrhage were the most common causes of hydrocephalus. Overall, patients averaged 2.66 revisions, with proximal (27%) and distal (15%) catheter occlusion, disconnection (11%), and infection (9%) comprising the most common reasons for shunt malfunction. Notably, 12.5% of patients did not require their first shunt revision until more than 10 years after initial device placement, a previously undescribed finding due to the short follow-up duration in previous studies. CONCLUSIONS: This long-term retrospective analysis of pediatric VP shunt placement revealed a relatively high rate of complications with need for shunt revision as late as 17 years after initial placement. Catheter occlusion represented a significant percentage of shunt failures. Cerebrospinal fluid shunting has a propensity for mechanical failure and patients with VP shunts should receive follow-up through the transition to adulthood.

Hemorrhage of a cavernous malformation associated with accidental electrocution: Case report and review of the literature.

BACKGROUND: Cavernous malformations (CMs) are the second most common intracranial vascular lesions. They typically present after hemorrhage or as incidental findings. Several risk factors have been identified for hemorrhage, however, electrocution as a cause has not been described. We performed a literature review of electrocution associated with CM hemorrhage and of the mechanisms of pathological injury in the central nervous system (CNS) secondary to electrocution. We found no cases of hemorrhage of CMs associated with electrocution. CASE DESCRIPTION: A 19-year-old male electrician was accidentally electrocuted with 277 V of alternating current (AC) at a job site. He suffered no trauma or physical injuries and reported no immediate abnormal findings. He then experienced progressive nausea, emesis, and lethargy until he presented to the emergency department (ED) where it was discovered that he had a left thalamic/midbrain hemorrhage with hydrocephalus. His hydrocephalus was treated and he began to improve. Subsequent magnetic resonance imaging (MRI) of his head demonstrated characteristic features of a CM. CONCLUSIONS: There are several proposed mechanisms in the literature by which electrocution may cause CNS damage. It is conceivable that given the pathology of CMs and the proposed mechanisms of electrical injury, these lesions may have an increased risk of hemorrhage as result of electrocution and we are reporting the first case of such an association.

Leber congenital amaurosis associated with Chiari I malformation: Two cases and a review of the literature.

OBJECTIVE: Leber congenital amaurosis (LCA) is a rare, clinically and genetically heterogeneous disorder characterized by severe loss of vision in the first year of life, affecting approximately 3000 people in the United States. Some LCA patients manifest developmental abnormalities of the central nervous system (CNS) and neuroradiological studies have revealed a variety of cerebral anomalies in association with LCA; however, Chiari I malformations (CMI) have never been described. CASE DESCRIPTION: We report two sisters who were referred to the pediatric neurosurgery clinic for evaluation of CMI. The elder sister presented with convergence nystagmus from 3 months of age and magnetic resonance imaging (MRI) demonstrated evidence of significant CMI. Her younger sister began developing nystagmus at 4 months of age. Both had symptomatic progression and underwent suboccipital decompression. Both were subsequently diagnosed with LCA. Case specifics and imaging findings are presented. CONCLUSIONS: CMI have been found in association with several genetic syndromes, but not with LCA. These patients represent the first reported cases of CMI with LCA and suggest an additional potential CNS anomaly. The unique occurrence in siblings and the association with another inherited disorder are suggestive of a genetic basis for CMI.

Delayed sub-aponeurotic fluid collections in infancy: Three cases and a review of the literature.

BACKGROUND: Sub-aponeurotic fluid collections (SFCs) in the neonatal period are poorly described in the literature. We describe the occurrence, possible etiologies and treatment of sub-aponeurotic fluid collections following the neonatal period. CASE DESCRIPTION: We present 3 cases of previously healthy children who developed soft, fluctuant, extracranial masses several weeks after birth. All 3 children were seen by a pediatric neurosurgeon after parents noticed scalp masses between 5 and 9 weeks of age. All 3 children were found to be otherwise healthy. Two of the children were born via C-section and 1 child was born vaginally. The vaginal delivery was described as difficult and utilized vacuum assist. Scalp electrodes were placed in all 3 children for intensive monitoring during labor. These children received plain skull x-rays to assess for abnormalities, and 2 of the children underwent a non-contrast brain CT scan to better characterize the fluid collection. Plain x-rays and CT scans showed no abnormalities of the skull or ventricles. In both patients who underwent a CT scan, a soft tissue prominence was noted with a Hounsfield unit similar to water. All cases resolved between 5 and 9 weeks after initial presentation, with no long-term sequelae. CONCLUSION: SFCs presenting after the neonatal period are usually associated with benign soft tissue swellings. Use of fetal scalp electrodes has been shown to cause cerebrospinal fluid (CSF) leakage in the neonatal period and may result in delayed SFC. This condition is benign, and the recommended course of treatment is conservative management.

Cervical laminoplasty as a management option for patients with cervical spondylotic myelopathy: a series of 40 patients.

BACKGROUND: Cervical spondylotic myelopathy (CSM) is one of the leading causes of spinal cord dysfunction in the adult population. Laminoplasty is an effective decompressive procedure for the treatment of CSM. OBJECTIVE: We present our experience with 40 patients who underwent cervical laminoplasty using titanium miniplates for CSM. METHODS: We performed a retrospective review of the medical records of a consecutive series of patients with CSM treated with laminoplasty at the University of Rochester Medical Center or Rochester General Hospital. We documented patient demographic data, presenting symptoms, and postoperative outcome. Data are also presented regarding the general cost of constructs for a hypothetical 3-level fusion. RESULTS: Forty patients underwent cervical laminoplasty; all were available for follow-up. The mean number of levels was 4. All patients were myelopathic, and 17 (42.5%) had signs of radiculopathy preoperatively. Preoperatively, 62.5% of patients had a Nurick grade of 2 or worse. The average follow-up was 31.3 months. The median length of stay was 48 hours. On clinical evaluation, 36 of 40 patients demonstrated an improvement in their myelopathic symptoms; 4 were unchanged. Postoperative kyphosis did not develop in any patients. CONCLUSION: The management of CSM for each of its etiologies remains controversial. As demonstrated in our series, laminoplasty is a cost-effective, decompressive procedure for the treatment of CSM, providing a less destabilizing alternative to laminectomy while preserving mobility. Cervical laminoplasty should be considered in the management of multilevel spondylosis because of its ease of exposure, ability to decompress, effective preservation of motion, maintenance of spinal stability, and overall cost.

Aneurysmal bone cyst: surgical management in the pediatric cervical spine.

STUDY DESIGN: Case report. OBJECTIVE: To present 2 cases of aneurysmal bone cyst in the cervical spine of children who were aggressively treated with resection, fusion, and stabilization with hardware to achieve complete cure and future stability. SUMMARY OF BACKGROUND DATA: Aneurysmal bone cyst is a rapidly expanding and destructive bone lesion of unknown etiology. Current approaches to treatment include surgical en bloc excision, complete curettage, selective arterial embolization, radiotherapy, and intralesional injection with a sclerosing agent. Fusion and stabilization with instrumentation have often been avoided in children due to concerns over instability following future axial skeleton growth. METHODS: The authors present 2 case reports of aneurysmal bone cyst in the cervical spine of children, both demonstrating deeply involved lesions with extensive bone destruction. Both were treated aggressively with resection, fusion, and stabilization with instrumentation. Additionally, the relevant background literature is briefly discussed. RESULTS: Both patients had an uncomplicated postoperative course. At 36- and 18-month follow-up, both patients have no cervical spine instability or recurrence of tumor. CONCLUSION: Treatment of aneurysmal bone cyst in the cervical spine is challenging when it occurs in close proximity to neural and vascular structures. Future stability of the cervical spine is a concern, especially when there is significant involvement of the vertebral body and posterior elements. At 36- and 18-month follow-up, the surgical approach to aneurysmal bone cyst presented here provided these patients with complete curative treatment and preservation of cervical spine stability.