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Wajd N. Al-Holou, Andrew Y. Yew, Zackary E. Boomsaad, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

Object

Arachnoid cysts are a frequent finding on intracranial imaging in children. The prevalence and natural history of these cysts are not well defined. The authors studied a large consecutive series of children undergoing MR imaging to better define both the MR imaging–demonstrated prevalence and behavior of these lesions over time.

Methods

The authors reviewed a consecutive series of 11,738 patients who were 18 years of age or younger and had undergone brain MR imaging at a single institution during an 11-year period. In the patients in whom intracranial arachnoid cysts were identified, clinical and demographic information was recorded and imaging characteristics, such as cyst size and location, were evaluated. Prevalence data were analyzed using univariate and multivariate logistic regression, linear regression, and ANOVA. All patients with sufficient data (repeat MR imaging studies as well as repeated clinical evaluation over at least 5 months) for a natural history analysis were identified. This group was assessed for any change in symptoms or imaging appearance during the follow-up interval.

Results

Three hundred nine arachnoid cysts (2.6% prevalence rate) were identified. There was an increased prevalence of arachnoid cysts in males (p < 0.000001). One hundred eleven patients met all criteria for inclusion in the natural history analysis. After a mean follow-up of 3.5 years, 11 arachnoid cysts increased in size, 13 decreased, and 87 remained stable. A younger age at presentation was significantly associated with cyst enlargement (p = 0.001) and the need for surgery (p = 0.05). No patient older than 4 years of age at the time of initial diagnosis had cyst enlargement, demonstrated new symptoms, or underwent surgical treatment.

Conclusions

Arachnoid cysts are a common incidental finding on intracranial imaging in pediatric patients. An older age at the time of presentation is associated with a lack of clinical or imaging changes over time.

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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.

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Javier Márquez and Mónica Rivero

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

Object

Pineal cysts are a frequent incidental finding on intracranial imaging. In adults, the prevalence of pineal cysts is estimated to be 1.1–4.3%. However, the prevalence is not well established in younger patients.

Methods

The authors retrospectively reviewed a consecutive series of 14,516 patients 25 years of age and younger, who underwent brain MR imaging at a single institution over an 11-year period. In patients identified with pineal cysts, the authors analyzed the images according to cyst size, signal characteristics, enhancement pattern, and evidence of local mass effect. Patient characteristics including demographics and other intracranial diagnoses were collected in the pineal cyst population and compared with a randomly selected age- and sex-matched control patient population. The data were evaluated using univariate and multivariate logistic regression, linear regression, and ANOVA.

Results

The authors identified 288 pineal region cysts (2.0%). The prevalence of pineal cysts was higher in female (2.4%) than in male patients (1.5%; p < 0.001). Pineal cysts were identified in patients of all ages, with an increased prevalence found in older patients (p < 0.001). Pineal cyst size was similar for all age and sex groups.

Conclusions

Pineal cysts are common in the pediatric population, with an increased prevalence in girls and in older patients.

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Cordelie E. Witt, Anthony C. Wang, Cormac O. Maher, Khoi D. Than, Hugh J. L. Garton and Karin M. Muraszko

In this report, the authors describe the first known case of inducible hemifacial weakness in a patient with Chiari malformation Type I (CM-I). The patient was a 14-year-old girl with a 1-year history of right facial paresis induced by sustained leftward head rotation. These episodes were characterized by weak activation of her right facial muscles with preserved eye opening and closure. Additionally, she had hypernasal speech, persistent headaches, and intermittent left arm twitching. Magnetic resonance imaging demonstrated a CM-I. A suboccipital craniectomy and C-1 laminectomy were performed for decompression of the CM-I, with duraplasty and coagulation of the pial surface of the cerebellar tonsils. At the 9-month follow-up, the patient's inducible hemifacial weakness had completely resolved. Her symptoms were thought to have resulted from the CM-I, perhaps due to traction on the right facial nerve by the ectopic tonsils with head rotation.

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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

Object

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.

Methods

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.

Results

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.

Conclusions

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.

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Michael J. Cools, Wajd N. Al-Holou, William R. Stetler Jr., Thomas J. Wilson, Karin M. Muraszko, Mohannad Ibrahim, Frank La Marca, Hugh J. L. Garton and Cormac O. Maher

Object

Filum terminale lipomas (FTLs) are being identified with increasing frequency due to the increasing utilization of MRI. Although an FTL may be associated with tethered cord syndrome (TCS), in many cases FTLs are diagnosed incidentally in patients without any symptoms of TCS. The natural history of FTLs is not well defined.

Methods

The authors searched the clinical and imaging records at a single institution over a 14-year interval to identify patients with FTLs. For patients with an FTL, the clinical records were reviewed for indication for imaging, presenting symptoms, perceived need for surgery, and clinical outcome. A natural history analysis was performed using all patients with more than 6 months of clinical follow-up.

Results

A total of 436 patients with FTL were identified. There were 217 males and 219 females. Of these patients, 282 (65%) were adults and 154 (35%) were children. Symptoms of TCS were present in 22 patients (5%). Fifty-two patients underwent surgery for FTL (12%). Sixty-four patients (15%) had a low-lying conus and 21 (5%) had a syrinx. The natural history analysis included 249 patients with a mean follow-up time of 3.5 years. In the follow-up period, only 1 patient developed new symptoms.

Conclusions

Filum terminale lipomas are a common incidental finding on spinal MRI, and most patients present without associated symptoms. The untreated natural history is generally benign for asymptomatic patients.

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Shawn L. Hervey-Jumper, Gail M. Annich, Andrea R. Yancon, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

Object

Extracorporeal membrane oxygenation (ECMO) is a potentially life-saving treatment for patients in refractory cardiorespiratory failure. Neurological complications that result from ECMO treatment are known to significantly impact patient survival and quality of life. The purpose of this study was to review the incidence of neurological complications of ECMO in the pediatric population and the role of neurosurgery in the treatment of these patients.

Methods

Data were obtained from the national Extracorporeal Life Support Organization (ELSO) Registry for the years 1990 to 2009. The neurological complications recorded by the registry include CNS hemorrhage, CNS infarction, and seizure. The ECMO Registry at the authors' institution was then searched, and 3 pediatric patients who had undergone craniotomy during ECMO treatment were identified.

Results

Children in the ELSO Registry who were treated with ECMO survived to hospital discharge in 65% of cases. Intracranial hemorrhage occurred in 7.4% of the ECMO-treated patients, with 36% of those surviving to hospital discharge. Hemorrhage was more likely in patients younger than 30 days old and in those requiring ECMO for cardiac indications. Cerebral infarction occurred in 5.7% of all ECMO-treated patients. Clinically diagnosed seizures occurred in 8.4% of all ECMO-treated patients. The ECMO Registry at the authors' institution revealed that 1898 patients were treated there. Intracranial hemorrhage was diagnosed in 81 patients (5.8%), and 3 of these patients were treated with craniotomy. Two of the patients were alive with minimal neurological impairment and normal school performance at 10 and 16 years of follow-up.

Conclusions

Intracranial hemorrhage is a serious complication of ECMO treatment. While the surgical risk is substantial, there may be a role for surgical evacuation of hemorrhage in well-selected patients.

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John R. W. Kestle, Hugh J. L. Garton, William E. Whitehead, James M. Drake, Abhaya V. Kulkarni, D. Douglas Cochrane, Cheryl Muszynski and Marion L. Walker

Object

Approximately 10% of cerebrospinal fluid (CSF) shunt operations are associated with infection and require removal or externalization of the shunt, in-hospital treatment with antibiotic agents, and insertion of a new shunt. In a previous survey, the authors identified substantial variation in the duration of antibiotic therapy as well as the duration of hospital stay. The present multicenter pilot study was undertaken to evaluate current strategies in the treatment of shunt infection.

Methods

Patients were enrolled in the study if they had a successful treatment of a CSF shunt infection proved by culture of a CSF specimen. Details of their care and the incidence of culture-proved reinfection were recorded.

Seventy patients from 10 centers were followed up for 1 year after their CSF shunt infection. The initial management of the infection was shunt externalization in 17 patients, shunt removal and external ventricular drain insertion in 50, and antibiotic treatment alone in three. Reinfection occurred in 18 patients (26%). Twelve of the 18 reinfections were caused by the same organism and six were due to new organisms. The treatment time varied from 4 to 47 days, with a mean of 17.4 days for those who later experienced a reinfection compared with 16.2 days for those who did not. The most common organism (Staphylococcus epidermidis, 34 patients) was associated with a reinfection rate of 29% and a mean treatment time of 12.8 days for those who suffered reinfection and 12.5 days for those who did not.

Conclusions

Reinfection after treatment of a CSF shunt infection is alarmingly common. According to the data available, the incidence of reinfection does not appear to be related to the duration of antibiotic therapy.

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Jennifer Strahle, Ndi Geh, Béla J. Selzer, Regina Bower, Mai Himedan, MaryKathryn Strahle, Nicholas M. Wetjen, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

OBJECT

There is currently no consensus on the safety of sports participation for patients with Chiari I malformation (CM-I). The authors' goal was to define the risk of sports participation for children with the imaging finding of CM-I.

METHODS

A prospective survey was administered to 503 CM-I patients at 2 sites over a 46-month period. Data were gathered on imaging characteristics, treatment, sports participation, and any sport-related injuries. Additionally, 81 patients completed at least 1 subsequent survey following their initial entry into the registry and were included in a prospective group, with a mean prospective follow-up period of 11 months.

RESULTS

Of the 503 CM-I patients, 328 participated in sports for a cumulative duration of 4641 seasons; 205 of these patients participated in contact sports. There were no serious or catastrophic neurological injuries. One patient had temporary extremity paresthesias that resolved within hours, and this was not definitely considered to be related to the CM-I. In the prospective cohort, there were no permanent neurological injuries.

CONCLUSIONS

No permanent or catastrophic neurological injuries were observed in CM-I patients participating in athletic activities. The authors believe that the risk of such injuries is low and that, in most cases, sports participation by children with CM-I is safe.