Blunt prenatal trauma is known to have consequences to the developing brain, and can result in subdural hematoma (SDH) or epidural hematoma (EDH). The authors present a case of blunt prenatal trauma resulting in a fetal SDH, intraparenchymal hematoma, and intraventricular hemorrhage, and perform a systematic review of the literature. This systematic review was conducted according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Relevant studies (up to April 2016) that reported on cases of fetal SDH or EDH after blunt prenatal trauma were identified from the PubMed database. The primary outcome was fetal mortality, and the secondary outcome was neurological outcome. Fourteen studies were included in the analysis, comprising a total of 14 patients including the present case. The average gestational age at discovery of hemorrhage was 30.1 weeks. Nine mothers were in a motor vehicle collision and 3 were assaulted; the mechanism of injury for 2 mothers was not defined. Twelve patients had SDH, 1 had EDH, and 1 had conflicting reports. Three patients had intrauterine fetal demise, and 3 died in the neonatal period after birth. Three patients had persistent neurological deficit, and 5 were neurologically intact. Fetal SDH or EDH after blunt trauma to the mother trauma is rare and is associated with mortality. However, a significant number of patients can have good neurological outcomes.
Jacob R. Joseph, Brandon W. Smith and Hugh J. L. Garton
Zhe Guan, Todd Hollon, J. Nicole Bentley and Hugh J. L. Garton
Epidermoid cysts (ECs) are uncommon pediatric tumors that often occur in the cerebellopontine angle. Although cyst rupture is a recognized complication, the radiographic evolution of an EC following rupture and the resultant parenchymal brainstem edema have not been reported. The authors present the case of a 13-year-old female with a newly diagnosed cerebellopontine angle EC who presented with worsening headaches, photophobia, and emesis. Magnetic resonance imaging demonstrated significant pericystic brainstem edema and mass effect with effacement of the fourth ventricle. Refractory symptoms prompted repeat imaging, revealing cyst enlargement and dense rim enhancement. Resection of the EC resolved both her symptoms and the brainstem edema. This case documents the radiographic evolution of EC rupture and subsequent clinical course.
Brandon W. Smith, Jennifer Strahle, Erick Kazarian, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher
It is unclear if there is a relationship between Chiari malformation Type I (CM-I) and body mass index (BMI). The aim of this study was to identify the relationship between BMI and cerebellar tonsil position in a random sample of people.
Cerebellar tonsil position in 2400 subjects from a cohort of patients undergoing MRI was measured. Three hundred patients were randomly selected from each of 8 age groups (from 0 to 80 years). A subject was then excluded if he or she had a posterior fossa mass or previous posterior fossa decompression or if height and weight information within 1 year of MRI was not recorded in the electronic medical record.
There were 1310 subjects (54.6%) with BMI records from within 1 year of the measured scan. Of these subjects, 534 (40.8%) were male and 776 (59.2%) were female. The average BMI of the group was 26.4 kg/m2, and the average tonsil position was 0.87 mm above the level of the foramen magnum. There were 46 subjects (3.5%) with a tonsil position ≥ 5 mm below the level of the foramen magnum. In the group as a whole, there was no correlation (R2 = 0.004) between BMI and cerebellar tonsil position.
In this examination of 1310 subjects undergoing MRI for any reason, there was no relationship between BMI and the level of the cerebellar tonsils or the diagnosis of CM-I on imaging.
D. Andrew Wilkinson, Kyle Johnson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher
The goal of this analysis was to define temporal and geographic trends in the surgical treatment of Chiari malformation Type I (CM-I) in a large, privately insured health care network.
The authors examined de-identified insurance claims data from a large, privately insured health care network of over 58 million beneficiaries throughout the United States for the period between 2001 and 2014 for all patients undergoing surgical treatment of CM-I. Using a combination of International Classification of Diseases (ICD) diagnosis codes and Current Procedural Terminology (CPT) codes, the authors identified CM-I and associated diagnoses and procedures over a 14-year period, highlighting temporal and geographic trends in the performance of CM-I decompression (CMD) surgery as well as commonly associated procedures.
There were 2434 surgical procedures performed for CMD among the beneficiaries during the 14-year interval; 34% were performed in patients younger than 20 years of age. The rate of CMD increased 51% from the first half to the second half of the study period among younger patients (p < 0.001) and increased 28% among adult patients between 20 and 65 years of age (p < 0.001). A large sex difference was noted among adult patients; 78% of adult patients undergoing CMD were female compared with only 53% of the children. Pediatric patients undergoing CMD were more likely to be white with a higher household net worth. Regional variability was identified among rates of CMD as well. The average annual rate of surgery ranged from 0.8 surgeries per 100,000 insured person-years in the Pacific census division to 2.0 surgeries per 100,000 insured person-years in the East South Central census division.
Analysis of a large nationwide health care network showed recently increasing rates of CMD in children and adults over the past 14 years.
Jennifer Strahle, Brandon W. Smith, Melaine Martinez, J. Rajiv Bapuraj, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher
Chiari malformation Type I (CM-I) is often found in patients with scoliosis. Most previous reports of CM-I and scoliosis have focused on patients with CM-I and a spinal syrinx. The relationship between CM-I and scoliosis in the absence of a syrinx has never been defined clearly. The authors sought to determine if there is an independent association between CM-I and scoliosis when controlling for syrinx status.
The medical records of 14,118 consecutive patients aged ≤ 18 years who underwent brain or cervical spine MRI at a single institution in an 11-year span were reviewed to identify patients with CM-I, scoliosis, and/or syrinx. The relationship between CM-I and scoliosis was analyzed by using multivariate regression analysis and controlling for age, sex, CM-I status, and syrinx status.
In this cohort, 509 patients had CM-I, 1740 patients had scoliosis, and 243 patients had a spinal syrinx. The presence of CM-I, the presence of syrinx, older age, and female sex were each significantly associated with scoliosis in the univariate analysis. In the multivariate regression analysis, older age (OR 1.02 [95% CI 1.01–1.03]; p < 0.0001), female sex (OR 1.71 [95% CI 1.54–1.90]; p < 0.0001), and syrinx (OR 9.08 [95% CI 6.82–12.10]; p < 0.0001) were each independently associated with scoliosis. CM-I was not independently associated with scoliosis when controlling for these other variables (OR 0.99 [95% CI 0.79–1.29]; p = 0.9).
A syrinx was independently associated with scoliosis in a large pediatric population undergoing MRI. CM-I was not independently associated with scoliosis when controlling for age, sex, and syrinx status. Because CM-I is not independently associated with scoliosis, scoliosis should not necessarily be considered a symptom of low cerebellar tonsil position in patients without a syrinx.
Jennifer Strahle, Béla J. Selzer, Ndi Geh, Dushyanth Srinivasan, MaryKathryn Strahle, Meleine Martinez-Sosa, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher
There is currently no consensus on the safety of sports participation for patients with an intracranial arachnoid cyst (AC). The authors' goal was to define the risk of sports participation for children with this imaging finding.
A survey was prospectively administered to 185 patients with ACs during a 46-month period at a single institution. Cyst size and location, treatment, sports participation, and any injuries were recorded. Eighty patients completed at least 1 subsequent survey following their initial entry into the registry, and these patients were included in a prospective registry with a mean prospective follow-up interval of 15.9 ± 8.8 months.
A total 112 patients with ACs participated in 261 sports for a cumulative duration of 4410 months or 1470 seasons. Of these, 94 patients participated in 190 contact sports for a cumulative duration of 2818 months or 939 seasons. There were no serious or catastrophic neurological injuries. Two patients presented with symptomatic subdural hygromas following minor sports injuries. In the prospective cohort, there were no neurological injuries
Permanent or catastrophic neurological injuries are very unusual in AC patients who participate in athletic activities. In most cases, sports participation by these patients is safe.
Jennifer Strahle, Karin M. Muraszko, Steven R. Buchman, Joseph Kapurch, Hugh J. L. Garton and Cormac O. Maher
Chiari malformation (CM) Type I is frequently associated with craniosynostosis. Optimal management of CM in patients with craniosynostosis is not well-established. The goal of this study was to report on a series of pediatric patients with both craniosynostosis and CM and discuss their management.
The authors searched the medical records of 383 consecutive patients treated for craniosynostosis at a single institution over a 15-year period to identify those with CM. They recorded demographic data as well as surgical treatment and outcomes for these patients. When MR imaging was performed, cerebellar tonsillar descent was recorded and any other associated findings, such as hydrocephalus or spinal syringes, were noted.
A total of 29 patients with both CM and craniosynostosis were identified. Of these cases, 28% had associated occipital venous abnormalities, 45% were syndromic, and 52% also had hydrocephalus. Chiari malformation was more likely to be present in those patients with isolated lambdoid synostosis (55%), multisuture synostosis (35%), and pansynostosis (80%), compared with patients with coronal synostosis (6%) or sagittal synostosis (3%). All patients underwent surgical repair of craniosynostosis: 16 had craniosynostosis repair as well as CM decompression, and 13 patients did not undergo CM decompression. Of the 7 patients in whom craniosynostosis repair alone was performed, 5 had decreased tonsillar ectopia postoperatively and 5 had improved CSF flow studies postoperatively. Both patients with a spinal syrinx had imaging-documented syrinx regression after craniosynostosis repair. In 12 patients in whom CM was diagnosed after primary craniosynostosis repair, 5 had multiple cranial vault expansions and evidence of elevated intracranial pressure. In 5 cases, de novo CM development was documented following craniosynostosis repair at a mean of 3.5 years after surgery.
Chiari malformation is frequently seen in patients with both multi- and single-suture lambdoid craniosynostosis. Chiari malformation, and even a spinal cord syrinx, will occasionally resolve following craniofacial repair. De novo development of CM after craniosynostosis repair is not unusual.
Jennifer Strahle, Karin M. Muraszko, Hugh J. L. Garton, Brandon W. Smith, Jordan Starr, Joseph R. Kapurch II, and Cormac O. Maher
Syrinx size and location within the spinal cord may differ based on etiology or associated conditions of the brain and spine. These differences have not been clearly defined.
All patients with a syrinx were identified from 14,118 patients undergoing brain or cervical spine imaging at a single institution over an 11-year interval. Syrinx width, length, and location in the spinal cord were recorded. Patients were grouped according to associated brain and spine conditions including Chiari malformation Type I (CM-I), secondary CM (2°CM), Chiari malformation Type 0 (CM-0), tethered cord, other closed dysraphism, and spinal tumors. Syringes not associated with any known brain or spinal cord condition were considered idiopathic. Syrinx characteristics were compared between groups.
A total of 271 patients with a syrinx were identified. The most common associated condition was CM-I (occurring in 117 patients [43.2%]), followed by spinal dysraphism (20 [7.4%]), tumor (15 [5.5%]), and tethered cord (13 [4.8%]). Eighty-three patients (30.6%) did not have any associated condition of the brain or spinal cord and their syringes were considered idiopathic. Syringes in patients with CM-I were wide (7.8 ± 3.9 mm) compared with idiopathic syringes (3.9 ± 1.0, p < 0.0001) and those associated with tethered cord (4.2 ± 0.9, p < 0.01). When considering CM-I–associated and idiopathic syringes, the authors found that CM-I–associated syringes were more likely to have their cranial extent in the cervical spine (88%), compared with idiopathic syringes (43%; p < 0.0001). The combination of syrinx width greater than 5 mm and cranial extent in the cervical spine had 99% specificity (95% CI 0.92–0.99) for CM-I–associated syrinx.
Syrinx morphology differs according to syrinx etiology. The combination of width greater than 5 mm and cranial extent in the cervical spine is highly specific for CM-I–associated syringes. This may have relevance when determining the clinical significance of syringes in patients with low cerebellar tonsil position.
Cormac O. Maher, Hugh J. L. Garton, Wajd N. Al-Holou, Jonathan D. Trobe, Karin M. Muraszko and Eric M. Jackson
Arachnoid cysts may occasionally be associated with subdural hygromas. The management of these concurrent findings is controversial.
The authors reviewed their experience with arachnoid cysts and identified 8 patients with intracranial arachnoid cysts and an associated subdural hygroma. The medical records and images for these patients were also examined.
In total, 8 patients presented with concurrent subdural hygroma and arachnoid cyst. Of these 8 patients, 6 presented with headaches and 4 had nausea and vomiting. Six patients had a history of trauma. One patient was treated surgically at the time of initial presentation, and 7 patients were managed without surgery. All patients experienced complete resolution of their presenting signs and symptoms.
Subdural hygroma may lead to symptomatic presentation for otherwise asymptomatic arachnoid cysts. The natural course of cyst-associated subdural hygromas, even when symptomatic, is generally benign, and symptom resolution can be expected in most cases. The authors suggest that symptomatic hygroma is not an absolute indication for surgical treatment and that expectant management can result in good outcomes in many cases.
Shawn L. Hervey-Jumper, Hugh J. L. Garton, Darryl Lau, David Altshuler, Douglas J. Quint, Patricia L. Robertson, Karin M. Muraszko and Cormac O. Maher
Vascular endothelial growth factor (VEGF) is the major proangiogenic factor in many solid tumors. Vascular endothelial growth factor receptor (VEGFR) is expressed in abundance in pediatric patients with medulloblastoma and is associated with tumor metastasis, poor prognosis, and proliferation. Gadolinium enhancement on MRI has been suggested to have prognostic significance for some tumors. The association of VEGF/VEGFR and Gd enhancement in medulloblastoma has never been closely examined. The authors therefore sought to evaluate whether Gd-enhancing medulloblastomas have higher levels of VEGFR and CD31. Outcomes and survival in patients with enhancing and nonenhancing tumors were also compared.
A retrospective analysis of patients with enhancing, nonenhancing, and partially enhancing medulloblastomas was performed. Primary end points included risk stratification, extent of resection, and perioperative complications. A cohort of 3 enhancing and 3 nonenhancing tumors was selected for VEGFR and CD31 analysis as well as microvessel density measurements.
Fifty-eight patients were analyzed, and 20.7% of the medulloblastomas in these patients were nonenhancing. Enhancing medulloblastomas exhibited strong VEGFR1/2 and CD31 expression relative to nonenhancing tumors. There was no significant difference in perioperative complications or patient survival between the 2 groups.
These results suggest that in patients with medulloblastoma the presence of enhancement on MRI may correlate with increased vascularity and angiogenesis, but does not correlate with worse patient prognosis in the short or long term.