Presentation of Supratentorial High-Grade Gliomas in Children

Symptoms

Infants

• Developmental delay: These tumors are commonly associated with developmental delay and loss of developmental milestones.
• Macrocrania: Accelerated head growth, a bulging anterior fontanelle, and splayed cranial sutures are commonly present.  The head occasionally may be asymmetric, with the side of the tumor being larger than the normal side.
• Hydrocephalus: This may or may not be present.  The infant’s ability to expand the head will minimize associated signs of intracranial hypertension.
• Intracranial hypertension: In late stages there may be lethargy, vomiting, and failure to thrive when the tumor reaches a large size.
• Large tumors:  A very young child with a patent anterior fontanelle and sutures that have not fused may present with a supratentorial high-grade tumor much larger than signs and symptoms suggest. The elastic expansion of the calvaria, plasticity of the young brain to accommodate mass effect, inability to verbally communicate accurately, and difficulty examining these babies lends to shockingly large, aggressive-appearing lesions at diagnosis.

Young children

• Macrocrania:  A large head, splayed sutures, and a thin calvaria are signs of long-standing increased ICP, even in the absence of papilledema or abducens nerve palsies.
• Intracranial hypertension symptoms more common: Headache, vomiting, and altered mental status become more common as the child develops.
• Focal symptoms more common: Ataxia, hemiparesis, and visual disturbances are all more evident in the child who has started to walk and talk.
• Seizures: New-onset seizures because of supratentorial tumors are uncommon.

Older children

• Diagnoses earlier:  Older children can communicate and articulate better, and they may present with more subtle findings such as blurry vision or diplopia, headaches, and nausea prior to vomiting.
• Seizures more common:  Older children more likely present with seizures from cerebral hemispheric tumors than do younger children.

Time for evolution

• AA intermediate: Symptoms of AA may be slower, over months to years, and they more commonly present in older children.
• GBM rapid: The rapid growth of GBM, particularly in young children, may lead to the rapid development of symptoms, over days or weeks (14).

Evaluation at Presentation

• See EVALUATION

Intervention

Stabilization

• ABCs: As with any child presenting to the hospital, the basic evaluation of airway, blood pressure, and potential bleeding should be addressed. If the airway is compromised, or there is concern for imminent neurological deterioration, intubation with appropriate control of pCO2 may be required. The administration of appropriate IV fluids, typically based on normal saline (NS) or blood products if necessary, may be required to maintain adequate blood pressure and cerebral perfusion.
• Treat elevation in ICP: Acute neurological deterioration with loss of consciousness and/or signs of increased ICP from hydrocephalus, tumor size and edema, concomitant hemorrhage, or all of these, may require mild hyperventilation, placing the head above the level of the heart, and the administration of hypertonic saline or an osmotic diuretic.
• Treat mass effect of hydrocephalus or acute hematoma: The unstable child with a HGG is typically one with symptomatic hydrocephalus. More malignant brain tumors, including gliomas, infrequently may present with acute hemorrhage requiring urgent treatment.

Preparation for definitive intervention, nonemergent

• Routine preoperative evaluation: Preparation of the patient who will be treated in an elective or semi-elective fashion will depend on the size and location of the tumor and the findings on initial imaging. Appropriate blood work to verify adequate hematologic parameters (hematocrit, platelet count, coagulation profile), specific location-related concerns (endocrine function, tumor marker profiles, metabolic profile), and a sample to the blood bank for preparation of blood products should be done on admission.
• Preoperative imaging: Appropriate radiologic studies, including CT, MRI, fMRI, and DTI, should be obtained prior to surgery. If the tumor is considered to be vascular on the basis of these studies, although not common for HGGs, formal catheter angiography for purposes of tumor embolization may be considered. This would allow for more controlled surgery, decreased blood loss, and improved outcome.
• Consultations: If time allows and depending on presenting symptoms and tumor location, neuropsychological, ophthalmologic, and endocrinologic consultations may be helpful. In addition, the oncology team may be consulted in advance.

Preparation for definitive intervention, emergent

• Simplified imaging: Typically, acute intervention is based on the emergent imaging studies, usually a simple, unenhanced CT. If indeed a child is in extremis, neurologically compromised because of increased ICP, emergent intervention may be required without the benefit of more sophisticated and helpful images.
• Minimal laboratory investigation: At a minimum, the child’s coagulation status and hematologic status is necessary prior to skin incision.
• Emergent relief of obstructive hydrocephalus: If hydrocephalus is the primary cause of urgent symptoms, ventricular drainage alone should be secured, allowing for a more thorough and thoughtful work up prior to definitive biopsy or resection.
• Craniotomy to relieve elevated ICP due to swelling: If there is malignant tumor swelling or intratumoral hemorrhage, formal craniotomy will be required to assure intraoperative control of both swelling and bleeding.

Admission Orders

• HOB, positioning, and activity: Unless the child is completely asymptomatic and the HGG was found incidentally, the HOB should generally be kept above 30 degrees. If signs and symptoms are focal with no evidence for increased ICP, the child may be free to sleep at 0 degrees, depending on deficit and independence, allowed to ambulate, with or without assistance, and generally be out of bed. Any concern for a child falling, stumbling, or exacerbating presenting symptoms should prompt a strict bed rest order.
• VS: If admitted to the ICU for concerns of increased ICP, nursing neurological assessment should be every 1–2 hours, depending on the child and the symptoms. If the child is ill, intubated, and in critical condition, blood pressure should be monitored continuously by arterial line. Oxygenation is monitored by pulse oximetry.
• BP parameters: In an otherwise healthy child, blood pressure should not be iatrogenically altered. If there is evidence for neurological decline or alteration in consciousness secondary to hypotension, appropriate fluid/blood product resuscitation is indicated.
• IVF and rate: Normal or half- normal saline is used at maintenance rates ((0-10 kg, 10 ml/kg/hour; 11-20 kg, 40 ml/hour+2 ml/kg/hour; and > 20 kg, 60 ml/hour+1 ml/kg/hour).
• CSF drainage parameters: If an EVD is in place and the ICP is felt to be stable in a normal range, the drain can be clamped and left to continuous transduced monitoring. Typically, depending on opening pressure and ventricular size, the drainage should be set at a level of 15-20 cmH20, depending on each individual child. The ICP should be transduced 1-2 times each hour.