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  • Author or Editor: Hugh J. L. Garton x
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Jacob R. Joseph, Brandon W. Smith and Hugh J. L. Garton

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.

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Brandon W. Smith, Jennifer Strahle, Erick Kazarian, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

OBJECT

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

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Brandon W. Smith, Jennifer Strahle, J. Rajiv Bapuraj, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

Object

Prior attempts to define normal cerebellar tonsil position have been limited by small numbers of patients precluding analysis of normal distribution by age group. The authors' objective in the present study was to analyze cerebellar tonsil location in every age range.

Methods

Two thousand four hundred patients were randomly selected from a database of 62,533 consecutive patients undergoing MRI and were organized into 8 age groups. Magnetic resonance images were directly examined for tonsil location, morphology, and other features. Patients with a history or imaging findings of posterior fossa abnormalities unrelated to Chiari malformation (CM) were excluded from analysis. The caudal extent of the cerebellar tonsils was measured at the midsagittal and lowest parasagittal positions.

Results

The mean tonsil height decreased slightly with advancing age into young adulthood and increased with advancing age in the adult age range. An increasing age in the adult age range was associated with a decreased likelihood of a tonsil position 5 mm or more below the foramen magnum (p = 0.0004). In general, the lowest tonsil position in each age group was normally distributed. Patients with pegged morphology were more likely to have a tonsil location at least 5 mm below the foramen magnum (85%), as compared with those having intermediate (38%) or rounded (2%) morphology (p < 0.0001). Female sex was associated with a lower mean tonsil position (p < 0.0001). Patients with a lower tonsil position also tended to have an asymmetrical tonsil position, usually lower on the right (p < 0.0001).

Conclusions

Cerebellar tonsil position follows an essentially normal distribution and varies significantly by age. This finding has implications for advancing our understanding of CM.

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Jennifer Strahle, Brandon W. Smith, Melaine Martinez, J. Rajiv Bapuraj, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

OBJECT

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.

METHODS

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.

RESULTS

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).

CONCLUSIONS

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.

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Jennifer Strahle, Karin M. Muraszko, Hugh J. L. Garton, Brandon W. Smith, Jordan Starr, Joseph R. Kapurch II, and Cormac O. Maher

OBJECT

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.