The management of large and giant arteriovenous malformations (AVMs) in patients presenting with nonhemorrhagic neurological deficits secondary to vascular steal phenomenon is challenging and controversial. In many cases, large AVMs cannot be completely excised or cured, leaving patients with residual or partially treated AVMs, the natural history of which is unknown. Additionally, large, diffuse vascular malformations with multiple, small feeders, slow flow, or so-called cerebral proliferative angiopathy represent a related but distinct clinical and angiographic entity that may require a different therapeutic approach than traditional brain AVMs. The current management of children with other conditions of chronic cerebral hypoperfusion, such as moyamoya disease, involves consideration of surgical revascularization to enhance blood flow to the compromised hemisphere. Here, the authors present the case of a young child with a large thalamic vascular malformation who presented with clinical and radiological features of vascular steal and ischemia. In an effort to augment flow to the hypoperfused brain and protect against future ischemia, the authors treated the child with unilateral pial synangiosis. At 12 months, postoperative angiography demonstrated robust neovascularization, and the child has not sustained any further ischemic events. The authors discuss concept of vascular malformation–related hypoperfusion and the utility of indirect revascularization for inoperable vascular malformations presenting with ischemic symptoms.
Michael J. Ellis, Derek Armstrong and Peter B. Dirks
Rinchen Phuntsok, Benjamin J. Ellis, Michael R. Herron, Chase W. Provost, Andrew T. Dailey and Douglas L. Brockmeyer
There is contradictory evidence regarding the relative contribution of the key stabilizing ligaments of the occipitoatlantal (OA) joint. Cadaveric studies are limited by the nature and the number of injury scenarios that can be tested to identify OA stabilizing ligaments. Finite element (FE) analysis can overcome these limitations and provide valuable data in this area. The authors completed an FE analysis of 5 subject-specific craniocervical junction (CCJ) models to investigate the biomechanics of the OA joint and identify the ligamentous structures essential for stability.
Isolated and combined injury scenarios were simulated under physiological loads for 5 validated CCJ FE models to assess the relative role of key ligamentous structures on OA joint stability. Each model was tested in flexion-extension, axial rotation, and lateral bending in various injury scenarios. Isolated ligamentous injury scenarios consisted of either decreasing the stiffness of the OA capsular ligaments (OACLs) or completely removing the transverse ligament (TL), tectorial membrane (TM), or alar ligaments (ALs). Combination scenarios were also evaluated.
An isolated OACL injury resulted in the largest percentage increase in all ranges of motion (ROMs) at the OA joint compared with the other isolated injuries. Flexion, extension, lateral bending, and axial rotation significantly increased by 12.4% ± 7.4%, 11.1% ± 10.3%, 83.6% ± 14.4%, and 81.9% ± 9.4%, respectively (p ≤ 0.05 for all). Among combination injuries, OACL+TM+TL injury resulted in the most consistent significant increases in ROM for both the OA joint and the CCJ during all loading scenarios. OACL+AL injury caused the most significant percentage increase for OA joint axial rotation.
These results demonstrate that the OACLs are the key stabilizing ligamentous structures of the OA joint. Injury of these primary stabilizing ligaments is necessary to cause OA instability. Isolated injuries of TL, TM, or AL are unlikely to result in appreciable instability at the OA joint.
Peter M. Grossi, Michael J. Ellis, Thomas J. Cummings, Linda L. Gray, Takanori Fukushima and John H. Sampson
✓Cholesterol granulomas (CGs) are benign lesions resulting from an inflammatory reaction to cholesterol and hemosiderin. These masses most often arise within the temporal bone or nasal sinuses; intracerebral CGs are extremely rare. In this report the authors present an unusual case of a CG arising within the lateral ventricle.
The patient presented with transient hemiparesis and numbness. Computed tomography and magnetic resonance imaging demonstrated a cystic partially enhancing midline mass within the right lateral ventricle, expanding the ventricle and displacing the septum pellucidum. The patient underwent an interhemispheric, transcallosal resection of the lesion. Microscopic examination revealed a granulomatous inflammatory lesion containing cholesterol clefts, macrophages, and hemosiderin. Embedded within the granulomatous response were foci of tiny cystlike structures lined by nonciliated flattened cuboidal epithelium, consistent with the diagnosis of CG.
To the authors' knowledge this is the first reported case of CG presenting as an intraventricular mass. The origin of this lesion is unclear, but it may relate to prior traumatic brain injury. The authors describe the presentation, imaging findings, histopathological characteristics, and surgical treatment of this rare lesion and related pathological entities.
Gerald Dieter Griffin
Michael J. Ellis, Dean Cordingley, Sara Vis, Karen Reimer, Jeff Leiter and Kelly Russell
The objective of this study was 2-fold: 1) to examine the prevalence of vestibulo-ocular dysfunction (VOD) among children and adolescents with acute sports-related concussion (SRC) and postconcussion syndrome (PCS) who were referred to a multidisciplinary pediatric concussion program; and 2) to determine if VOD is associated with the development of PCS in this cohort.
The authors conducted a retrospective review of all patients with acute SRC (presenting 30 days or less postinjury) and PCS (3 or more symptoms for at least 1 month) referred to a multidisciplinary pediatric concussion program between September 2013 and July 2014. Initial assessment included clinical history, physical examination, and Post-Concussion Symptom Scale assessment. Patients were also assessed for VOD, which was defined as more than one subjective vestibular and oculomotor complaint (dizziness, blurred vision, and so on) and more than one objective physical examination finding (abnormal smooth pursuits, saccades, vestibulo-ocular reflex, and so on). This study was approved by the local institutional ethics review board.
A total of 101 patients (mean age 14.2 years, SD 2.3 years; 63 male and 38 female patients) participated, including 77 (76.2%) with acute SRC and 24 (23.8%) with PCS. Twenty-two of the 77 patients (28.6%) with acute SRC and 15 of the 24 (62.5%) with PCS met the clinical criteria for VOD. The median duration of symptoms was 40 days (interquartile range [IQR] 28.5–54 days) for patients with acute SRC who had VOD compared with 21 days (IQR 13–32 days) for those without VOD (p = 0.0001). There was a statistically significant increase in the adjusted odds of developing PCS among patients with acute SRC who had VOD compared with those without VOD (adjusted OR 4.10; 95% CI 1.04–16.16).
Evidence of VOD was detected in a significant proportion of children and adolescents with acute SRC and PCS who were referred to a multidisciplinary pediatric concussion program. This clinical feature was a significant risk factor for the subsequent development of PCS in this pediatric acute SRC cohort.
Michael J. Ellis, Dean M. Cordingley, Sara Vis, Karen M. Reimer, Jeff Leiter and Kelly Russell
There were 2 objectives of this study. The first objective was to identify clinical variables associated with vestibulo-ocular dysfunction (VOD) detected at initial consultation among pediatric patients with acute sports-related concussion (SRC) and postconcussion syndrome (PCS). The second objective was to reexamine the prevalence of VOD in this clinical cohort and evaluate the effect of VOD on length of recovery and the development of PCS.
A retrospective review was conducted for all patients with acute SRC and PCS who were evaluated at a pediatric multidisciplinary concussion program from September 2013 to May 2015. Acute SRS was defined as presenting < 30 days postinjury, and PCS was defined according to the International Classification of Diseases, 10th Revision criteria and included being symptomatic 30 days or longer postinjury. The initial assessment included clinical history and physical examination performed by 1 neurosurgeon. Patients were assessed for VOD, defined as the presence of more than 1 subjective vestibular and oculomotor complaint (dizziness, diplopia, blurred vision, etc.) and more than 1 objective physical examination finding (abnormal near point of convergence, smooth pursuits, saccades, or vestibulo-ocular reflex testing). Poisson regression analysis was used to identify factors that increased the risk of VOD at initial presentation and the development of PCS.
Three hundred ninety-nine children, including 306 patients with acute SRC and 93 with PCS, were included. Of these patients, 30.1% of those with acute SRC (65.0% male, mean age 13.9 years) and 43.0% of those with PCS (41.9% male, mean age 15.4 years) met the criteria for VOD at initial consultation. Independent predictors of VOD at initial consultation included female sex, preinjury history of depression, posttraumatic amnesia, and presence of dizziness, blurred vision, or difficulty focusing at the time of injury. Independent predictors of PCS among patients with acute SRC included the presence of VOD at initial consultation, preinjury history of depression, and posttraumatic amnesia at the time of injury.
This study identified important potential risk factors for the development of VOD following pediatric SRC. These results provide confirmatory evidence that VOD at initial consultation is associated with prolonged recovery and is an independent predictor for the development of PCS. Future studies examining clinical prediction rules in pediatric concussion should include VOD. Additional research is needed to elucidate the natural history of VOD following SRC and establish evidence-based indications for targeted vestibular rehabilitation.
Kelly Russell, Erin Selci, Brian Black and Michael J. Ellis
The longitudinal effects of sports-related concussion (SRC) in adolescents on health-related quality of life (HRQOL) remain poorly understood. Hence, the authors established two objectives of this study: 1) compare HRQOL outcomes among adolescents with an acute SRC or a sports-related extremity fracture (SREF) who were followed up until physician-documented clinical recovery; and 2) identify the clinical variables associated with worse HRQOL among adolescent SRC patients.
The authors conducted a prospective cohort study of adolescents with acute SRC and those with acute SREF who underwent clinical assessment and follow-up at tertiary subspecialty clinics. Longitudinal patient-reported HRQOL was measured at the time of initial assessment and at each follow-up appointment by using the adolescent version (age 13–18 years) of the Pediatric Quality of Life Inventory (PedsQL) Generic Core Scale and Cognitive Functioning Scale.
A total of 135 patients with SRC (60.0% male; mean age 14.7 years; time from injury to initial assessment 6 days) and 96 patients with SREF (59.4% male; mean age 14.1 years; time from injury to initial assessment 8 days) participated in the study. At the initial assessment, the SRC patients demonstrated significantly worse cognitive HRQOL and clinically meaningful impairments in school and overall HRQOL compared to the SREF patients. Clinical variables associated with a worse HRQOL among SRC patients differed by domain but were significantly affected by the patients’ initial symptom burden and the development of delayed physician-documented clinical recovery (> 28 days postinjury). No persistent impairments in HRQOL were observed among SRC patients who were followed up until physician-documented clinical recovery.
Adolescent SRC is associated with temporary impairments in HRQOL that have been shown to resolve in patients who are followed up until physician-documented clinical recovery. Future studies are needed to identify the clinicopathological features that are associated with impaired HRQOL and to assess whether the initiation of multidisciplinary, targeted rehabilitation strategies would lead to an improvement in HRQOL.
Michael J. Ellis, Colin J. Kazina, Marc R. Del Bigio and Patrick J. McDonald
✓Shunt failure is commonly associated with infection or mechanical obstruction of the shunt system. The presence of eosinophilia in the cerebrospinal fluid (CSF) has been associated with CSF shunt failure and may be related to both latex and shunt allergies. The authors describe the case of a child with a latex allergy who presented with 10 episodes of shunt failure over a period of 93 months. Cerebrospinal fluid sampling demonstrated persistent eosinophilia (3–36%) and negative cultures. Pathological examination of the ventricular catheter on 3 occasions demonstrated mechanical obstruction by inflammatory debris consisting largely of eosinophils and multinucleated giant cells. On the suspicion that the child might have some uncharacterized allergy to the shunt hardware, shunt replacement was performed using an “extracted” shunt system. The child has remained free of shunt malfunction for > 2 years since the last surgery. Immune responses to unpolymerized silicone are discussed.
Michael J. Ellis, Samuel Cheshier, Sunjay Sharma, Derek Armstrong, Cynthia Hawkins, Eric Bouffet, James T. Rutka and Michael D. Taylor
Among the neoplastic conditions that affect patients with neurofibromatosis Type 1 (NF1) are malignant peripheral nerve sheath tumors (MPNSTs), which typically arise from peripheral nerves of the limbs, trunk, and lumbar and brachial plexuses. Ionizing radiation is an established risk factor for MPNST development, especially in susceptible patients such as those with NF1. Patients with NF1 are also at risk for intracranial aneurysms, which are increasingly being successfully managed with endovascular therapies. The authors describe the case of a 9-year-old, previously healthy girl who presented in extremis with a right frontal intracerebral hemorrhage resulting from a ruptured right middle cerebral artery (MCA) trifurcation aneurysm. Following urgent decompressive craniectomy, the patient underwent endovascular coil embolization of the MCA aneurysm without complication. Given her mother's history of NF1, the child underwent genetic testing, which disclosed signs positive for NF1. The patient recovered well, but follow-up MR imaging and MR angiography performed at 14 months demonstrated a large frontotemporal mass encasing the right MCA trifurcation. The patient underwent frontotemporal craniotomy and subtotal resection of the mass, which was histologically found to be an intracranial MPNST. The patient received chemotherapy and focal radiation therapy and remains alive at 6 months postresection. To the authors' knowledge, this represents the only known case of intracranial neoplasm arising in the region of an intracranial aneurysm repaired by endovascular coil embolization. While patients with NF1 represent a population with genetic susceptibility to radiation-induced tumors, the pathogenesis of intracerebral MPNSTs remains poorly understood.
Michael J. Ellis, Patrick J. McDonald, Dean Cordingley, Behzad Mansouri, Marco Essig and Lesley Ritchie
The decision to advise an athlete to retire from sports following sports-related concussion (SRC) remains a persistent challenge for physicians. In the absence of strong empirical evidence to support recommendations, clinical decision making must be individualized and should involve a multidisciplinary team of experts in concussion and traumatic brain injury. Although previous authors have advocated for a more conservative approach to these issues in child and adolescent athletes, there are few reports outlining considerations for this process among this unique population. Here, the authors use multiple case illustrations to discuss 3 subgroups of clinical considerations for sports retirement among pediatric SRC patients including the following: those with structural brain abnormalities identified on neuroimaging, those presenting with focal neurological deficits and abnormalities on physical examination, and those in whom the cumulative or prolonged effects of concussion are suspected or demonstrated. The authors' evolving multidisciplinary institutional approach to return-to-play and retirement decision making in pediatric SRC is also presented.