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David M. Benglis and David I. Sandberg

✓Cervicomedullary compression at the foramen magnum in patients with achondroplasia can be associated with apnea, neurological deficits, and sudden death. Decompressive operations are often performed in symptomatic patients. In asymptomatic patients, the indications for prophylactic decompression are controversial. The authors present the case of a previously neurologically intact 4-month-old girl with achondroplasia who presented with severe hemiparesis after a low-velocity motor vehicle accident. Imaging studies demonstrated osseous compression of the medulla and upper cervical spinal cord with associated parenchymal signal changes. To the authors' knowledge this is the first reported case of a new neurological deficit after a minor trauma in this patient population. The authors review the relevant literature, focusing on the indications for cervicomedullary decompression in infants with achondroplasia. They propose that asymptomatic patients with achondroplasia and osseous compression at the foramen magnum should be offered prophy-lactic surgery if T2-weighted magnetic resonance imaging signal changes in the spinal cord are observed. Prophylactic surgery can be considered an option in patients whose imaging studies do not show signal changes in the spinal cord but demonstrate significant osseous compression and absence of visible subarachnoid spaces.

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David I. Sandberg, J. Gordon McComb and Mark D. Krieger

Object

The authors analyzed the chemical composition of intracranial arachnoid cyst fluid to gain greater understanding of potential causes of cyst growth.

Methods

The authors studied 54 pediatric patients who underwent craniotomies for arachnoid cyst fenestration. Cyst fluid was analyzed and results were compared with expected values (EVs) for cerebrospinal fluid (CSF).

The arachnoid cyst fluid values were as follows: protein (median 37 mg/dl, mean 178.2 mg/dl, and EV 25.6 mg/dl), osmolality (median 284 mOsm/kg, mean 285.5 mOsm/kg, and EV 287.2 mOsm/kg), potassium (median 2.6 mEq/L, mean 2.68 mEq/L, and EV 2.88 mEq/L), sodium (median 140 mEq/L, mean 142.2 mEq/L, and EV 143.3 mEq/L), and chloride (median 122 mEq/L, mean 120.77 mEq/L, and EV 120.3 mEq/L). Median glucose was 51 mg/dl (range 26–98 mg/dl). The median white blood cell count in cyst fluid was 1/mm3, and the median red blood cell (RBC) count was 1.5/mm3. All Gram stains and cultures were negative.

No statistically significant correlations were found between arachnoid cyst fluid protein level and elevated RBC count, patient age, or the subsequent need for a shunt. Elevated RBC count in cyst fluid was also not associated with the subsequent need for a shunt.

Conclusions

Fluid from arachnoid cysts has a chemical composition similar to that of CSF, but some of them have significantly elevated protein levels. The authors hypothesize that elevated protein content may contribute to arachnoid cyst expansion in some patients.

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Yoshua Esquenazi, David I. Sandberg and Harold L. Rekate

Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a 7-year-old girl who was diagnosed with an HH due to precocious puberty and was treated medically with a gonadotropin-releasing hormone analog for 3 years. Despite normalization of her plasma levels of luteinizing hormone, follicle-stimulating hormone, and estradiol and arrest of her precocious puberty, the patient developed progressive weight gain associated with extreme hyperphagia and morbid obesity by the age of 10 years. Her compulsive eating patterns were refractory to counseling and other interventions attempted by her parents and physicians. After resection of the HH, her hyperphagia resolved and her weight stabilized. To the authors' knowledge, this is the first report describing resection of an HH for the purpose of treating hyperphagia and obesity.

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Anitha Nimmagadda, David I. Sandberg and John Ragheb

✓ The authors report the case of a newborn presenting at birth with macrocephaly and a large pineal region hemorrhagic cyst without neurological deficit. No neurosurgical intervention was performed, and subsequent imaging studies demonstrated complete involution of the cyst.

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David I. Sandberg, Mark A. Edgar and Mark M. Souweidane

Object. Convection-enhanced delivery (CED) can be used safely to achieve high local infusate concentrations within the brain and spinal cord. The use of CED in the brainstem has not been previously reported and may offer an alternative method for treating diffuse pontine gliomas. In the present study the authors tested CED within the rat brainstem to assess its safety and establish distribution parameters.

Methods. Eighteen rats underwent stereotactic cannula placement into the pontine nucleus oralis without subsequent infusions. Twenty rats underwent stereotactic cannula placement followed by infusion of fluorescein isothiocyanate (FITC)—dextran at a constant rate (0.1 µl/minute) until various total volumes of infusion (Vis) were reached: 0.5, 1, 2, and 4 µl. Additional rats underwent FITC—dextran infusion (Vi 4 µl) and were observed for 48 hours (five animals) or 14 days (five animals). Serial (20-µm thick) brain sections were imaged using confocal microscopy with ultraviolet illumination, and the volume of distribution (Vd) was calculated using computer image analysis. Histological analysis was performed on adjacent sections.

No animal exhibited a postoperative neurological deficit, and there was no histological evidence of tissue disruption. The Vd increased linearly (range 15.4–55.8 mm3) along with increasing Vi, with statistically significant correlations for all groups that were compared (p < 0.022). The Vd/Vi ratio ranged from 14 to 30.9. The maximum cross-sectional area of fluorescence (range 9.8–20.9 mm2) and the craniocaudal extent of fluorescence (range 2.8–5.1 mm) increased with increasing Vi.

Conclusions. Convection-enhanced delivery can be safely applied to the rat brainstem with substantial and predictable Vds. This study provides the basis for investigating delivery of various candidate agents for the treatment of diffuse pontine gliomas.

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Garrett K. Zoeller, Carole D. Brathwaite and David I. Sandberg

Optic pathway gliomas (OPGs) arise from the optic nerves, optic chiasm, and/or hypothalamus and most commonly occur in childhood. Although these tumors can be quite challenging to manage, they are typically low-grade astrocytomas histologically, most commonly pilocytic astrocytomas. The few previously reported cases of malignant degeneration of an OPG occurred after external beam radiation therapy. The authors report the first case in the English literature of an OPG that transformed from a low-grade astrocytoma, with features most consistent with a pilocytic astrocytoma, to a malignant glioma without any exposure to radiation therapy.

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Eliel N. Arrey, Marcia L. Kerr, Stephen Fletcher, Charles S. Cox Jr. and David I. Sandberg

OBJECT

In this study the authors reviewed clinical management and outcomes in a large series of children with isolated linear nondisplaced skull fractures (NDSFs). Factors associated with hospitalization of these patients and costs of management were also reviewed.

METHODS

After institutional review board approval, the authors retrospectively reviewed clinical records and imaging studies for patients between the ages of 0 and 16 years who were evaluated for NDSFs at a single children’s hospital between January 2009 and December 2013. Patients were excluded if the fracture was open or comminuted. Additional exclusion criteria included intracranial hemorrhage, more than 1 skull fracture, or pneumocephalus.

RESULTS

Three hundred twenty-six patients met inclusion criteria. The median patient age was 19 months (range 2 weeks to 15 years). One hundred ninety-three patients (59%) were male and 133 (41%) were female. One hundred eighty-four patients (56%) were placed under 23-hour observation, 87 (27%) were admitted to the hospital, and 55 patients (17%) were discharged from the emergency department. Two hundred seventy-eight patients (85%) arrived by ambulance, 36 (11%) arrived by car, and 12 (4%) were airlifted by helicopter. Two hundred fifty-seven patients (79%) were transferred from another institution. The mean hospital stay for patients admitted to the hospital was 46 hours (range 7–395 hours). The mean hospital stay for patients placed under 23-hour observation status was 18 hours (range 2–43 hours). The reasons for hospitalization longer than 1 day included Child Protective Services involvement in 24 patients and other injuries in 11 patients. Thirteen percent (n = 45) had altered mental status or loss of consciousness by history. No patient had any neurological deficits on examination, and none required neurosurgical intervention. Less than 16% (n = 50) had subsequent outpatient follow-up. These patients were all neurologically intact at the follow-up visit.

CONCLUSIONS

Hospitalization is not necessary for many children with NDSFs. Patients with mental status changes, additional injuries, or possible nonaccidental injury may require observation.

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Faiz Ahmad, Marc K. Rosenblum, Gabriel Chamyan and David I. Sandberg

Neurocytomas are typically intraventricular in location, and extraventricular neurocytomas are uncommon. The authors report the unique case of a 15-year-old girl who was found to have a low-grade neurocytoma infiltrating the brainstem and cerebellum and spreading along the CSF pathways to the lateral and third ventricles. The patient underwent endoscopic third ventriculostomy to treat associated hydrocephalus, and biopsy specimens from intraventricular tumor nodules were obtained. Because of the low-grade pathology, the fact that the lesion was not amenable to resection, and the extensive radiation field required for radiation therapy, she has been treated conservatively with close follow-up. Over the course of almost 4 years since diagnosis, no additional treatment has been required. Neurocytoma with widespread infiltration of the brainstem and cerebellum has not been previously reported.