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  • Author or Editor: Hugh J. L. Garton x
  • Journal of Neurosurgery: Pediatrics 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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Jennifer Strahle, Andrew J. Odden, Cormac O. Maher and Hugh J. L. Garton

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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.

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Jeffrey L. Nadel, D. Andrew Wilkinson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

OBJECTIVE

The goal of this study was to determine the rates of screening and surgery for foramen magnum stenosis in children with achondroplasia in a large, privately insured healthcare network.

METHODS

Rates of screening and surgery for foramen magnum stenosis in children with achondroplasia were determined using de-identified insurance claims data from a large, privately insured healthcare network of over 58 million beneficiaries across the United States between 2001 and 2014. Cases of achondroplasia and screening and surgery claims were identified using a combination of International Classification of Diseases diagnosis codes and Current Procedural Terminology codes. American Academy of Pediatrics (AAP) practice guidelines were used to determine screening trends.

RESULTS

The search yielded 3577 children age 19 years or younger with achondroplasia. Of them, 236 met criteria for inclusion in the screening analysis. Among the screening cohort, 41.9% received some form of screening for foramen magnum stenosis, whereas 13.9% of patients were fully and appropriately screened according to the 2005 guidelines from the AAP. The screening rate significantly increased after the issuance of the AAP guidelines. Among all children in the cohort, 25 underwent cervicomedullary decompression for foramen magnum stenosis. The incidence rate of undergoing cervicomedullary decompression was highest in infancy (28 per 1000 patient-years) and decreased with age (5 per 1000 patient-years for all other ages combined).

CONCLUSIONS

Children with achondroplasia continue to be underscreened for foramen magnum stenosis, although screening rates have improved since the release of the 2005 AAP surveillance guidelines. The incidence of surgery was highest in infants and decreased with age.

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Jennifer Strahle, Béla J. Selzer, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

Object

The authors investigated the effect of a tablet computer on performance-level settings of a programmable shunt valve.

Methods

Magnetic field strength near the tablet computer with and without a cover was recorded at distances between 0 and 100 mm. Programmable valves were exposed to the tablet device at distances of less than 1 cm, 1–2.5 cm, 2.5–5 cm, 5–10 cm, and greater than 10 cm. For each distance tested, the valves were exposed 100 times to the tablet with the cover, resulting in 500 total valve exposures. The tablet alone, without the cover, was also tested at distances of less than 1 cm for 30 valve exposures. Changes in valve performance-level settings were recorded.

Results

The maximum recorded magnetic flux density of a tablet with a cover was 17.0 mT, and the maximum recorded magnetic flux density of the tablet alone was 7.6 mT. In 100 exposures at distances between 0 and 1 cm, 58% of valves had different settings following exposure. At distances greater than 1 cm but less than 2.5 cm, 5% of valves in 100 exposures had setting changes. Only a single setting change was noted in 100 exposures at distances greater than 2.5 cm but less than 5 cm. No setting changes were noted at distances greater than 5 cm, including 100 exposures between 5 and 10 cm, and 100 exposures of more than 10 cm. For the 30 valve exposures to the tablet without a cover, 20 valve performance-level changes (67%) were noted.

Conclusions

Based on these results, exposure to tablet devices may alter programmable shunt valve settings.

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Wajd N. Al-Holou, Cormac O. Maher, Karin M. Muraszko and Hugh J. L. Garton

Object

The authors reviewed their experience with pediatric pineal cysts to define the natural history and clinical relevance of this common intracranial finding.

Methods

The authors identified all patients with pineal cysts who had been clinically evaluated at their institution over an 11.5-year interval and were < 25 years of age at the time of diagnosis. All inclusion criteria were met in 106 patients, and included repeated MR imaging as well as repeated clinical evaluation over at least a 6-month interval.

Results

The mean age at diagnosis was 11.7 ± 7.2 years. Forty-two patients were male and 64 were female. On follow-up MR imaging evaluation at a mean interval of 3.0 years from the initial study, 98 pineal cysts had no increase in size and no change in imaging appearance. Six pineal cysts increased in size and 2 others had a change in imaging characteristics without associated growth. Younger age was associated with cyst change or growth on follow-up imaging (p = 0.02). The mean age of patients with cysts that changed or grew was 5.5 years, and the mean age of patients with stable pineal cysts was 12.2 years. Initial cyst size and appearance on MR imaging were not significant predictors of growth or change in imaging appearance at follow-up. Similarly, the patient's sex was not a significant predictor of growth or change in imaging characteristics.

Conclusions

Follow-up imaging and neurosurgical evaluation may be considered optional in older children with pineal cysts.

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D. Andrew Wilkinson, Kyle Johnson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

OBJECTIVE

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

Analysis of a large nationwide health care network showed recently increasing rates of CMD in children and adults over the past 14 years.

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Jennifer Strahle, Karin M. Muraszko, Joseph Kapurch, J. Rajiv Bapuraj, Hugh J. L. Garton and Cormac O. Maher

Object

Chiari malformation Type I (CM-I) with an associated spinal syrinx is a common pediatric diagnosis. A better understanding of the relative age-related prevalence and MR imaging characteristics of these associated conditions may lead to improved treatment decisions.

Methods

The authors performed a retrospective review of 14,116 consecutive individuals 18 years of age or younger who had undergone brain or cervical spine MR imaging at the University of Michigan between November 1997 and August 2008. In the patients with CM-I, demographic, clinical, and radiographic information was recorded.

Results

Five hundred nine children (3.6%) with CM-I were identified. Among these patients, 23% also had a spinal cord syrinx, and 86% of the syringes were found in the cervical spine. The MR imaging prevalence of CM-I with a syrinx was 1.2% in girls and 0.5% in boys (p < 0.0001). The severity of impaired CSF flow at the foramen magnum was associated with the amount of tonsillar herniation (p < 0.0001) and conformation of the tonsils (p < 0.0001). Patients with CM-I were treated surgically in 35% of cases; these patients exhibited more severe tonsillar herniation (p < 0.0001) and impaired CSF flow (p < 0.0001) as compared with those who did not undergo surgery. On imaging, 32% of all the patients with CM-I were considered symptomatic by the treating physician. Patients were more likely to be considered symptomatic if they were female, had a syrinx, displayed abnormal tonsillar pulsations, or had a greater amount of tonsillar herniation.

Conclusions

In this study the authors describe the age-related prevalence and MR imaging characteristics of CM-I and its association with a syrinx and other abnormalities in a large group of children who underwent MR imaging for any indication. Syringes are more common in older children, in girls, and in patients with a greater degree of tonsillar descent and CSF flow impairment.

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Jennifer Strahle, Karin M. Muraszko, Joseph Kapurch, J. Rajiv Bapuraj, Hugh J. L. Garton and Cormac O. Maher

Object

The natural history of the Chiari malformation Type I (CM-I) is incompletely understood. The authors report on the outcome of a large group of patients with CM-I that were initially selected for nonsurgical management.

Methods

The authors identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis.

Results

Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not.

Conclusions

In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen.

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Cormac O. Maher, Hugh J. L. Garton, Wajd N. Al-Holou, Jonathan D. Trobe, Karin M. Muraszko and Eric M. Jackson

Object

Arachnoid cysts may occasionally be associated with subdural hygromas. The management of these concurrent findings is controversial.

Methods

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.

Results

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.

Conclusions

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.