an ipsilateral hypotonia, resulting in falling to the affected side Herniation of the cerebellar tonsil can cause head tilt toward the tumor and neck pain or stiffness Supratentorial masses may present with signs and symptoms derived from the involved anatomic locations Tumors near the optic chiasm may present with vision deficits Craniopharyngiomas, located in the pituitary gland, can cause visual disturbances, headache, and alterations in endocrine function Growth hormone deficiency or hypothyroidism may cause delayed growth Pineal tumors may cause hydrocephalus by obstructing the aquaduct of Sylvius or can cause Parinaud syndrome (deficits in upward gaze, convergence nystagmus, and impaired pupillary response) Hypothalamic tumors may cause diencephalic syndrome, which includes failure to thrive, wasting, and unusual euphoria If a tumor is located near the third ventricle, hydrocephalus and symptoms of increased ICP can result Masses within the cerebral hemispheres themselves usually present with focal motor dysfunction or seizure The spinal cord may be the site of a primary tumor or, in some cases, the site of a “dropped metastasis” from a primary lesion within the brain These can present with focal neurologic deficits attributable to areas of the spinal cord inferior to the lesion (see “Tumors in and Around the Spinal Cord” section) TABLE 98.4 TUMORS AFFECTING THE CENTRAL NERVOUS SYSTEM Location Specific tumor types Presenting signs and symptoms Comments Supratentorial hemispheric Low-grade gliomas Pilocytic Fibrillary High-grade glioma Mixed neuronal-glial neoplasms Ganglioglioma Ependymoma Choroid plexus tumors Primitive neuroectodermal tumor (PNET) Chiasmatic/hypothalamic glioma Craniopharyngioma Germinoma/malignant germ cell tumors Pineoblastoma PNET Varies with site Hemiparesis Hemisensory deficits Seizure Hemianopsia Outcome for these tumors usually improved by extensive resection Vision deficits Diencephalic syndrome Neuroendocrine symptoms Hydrocephalus Parinaud syndrome Germinoma and germ cell tumors require biopsy; treatment usually chemotherapy not surgery Medulloblastoma Cerebellar astrocytoma Ependymoma Diffuse malignant brainstem glioma Benign focal brainstem glioma Cranial nerve palsies Cerebellar signs Ataxia Dysmetria Brainstem signs Weakness Unsteady gait Increased intracranial pressure Supratentorial midline Infratentorial Brain tumors can cause increased ICP by blocking CSF drainage The symptoms of increased ICP vary based on patient age Infants with an open fontanelle can sometimes present with bulging of the fontanelle as well as seizure, vomiting, irritability, or loss of acquired skills Older children often have headache and early morning vomiting Sixth nerve palsies are common Sometimes increased ICP is detected only on imaging of the brain that reveals enlargement of the ventricles or effacement of the gyri Seizures developing in the setting of a child with cancer, whether due to a CNS tumor or another malignancy, should be managed as described in Chapter 72 Seizures Brain tumors should be considered in the differential diagnosis of newonset seizures Patients with brain tumors are at risk for several metabolic complications The presence of an intracranial tumor may cause for cerebral salt wasting or SIADH DI can result from tumor involvement of the pituitary gland Patients should be screened for these abnormalities with serum chemistries However, these complications are not managed uniquely because of the brain tumors Chapter 89 Endocrine Emergencies provides guidelines on evaluation and management Clinical Assessment After ensuring that the airway, breathing, and circulation are stable, the evaluation in the ED should focus on a thorough history and physical examination, assessing for any neurologic symptoms and screening for complications described above A complete physical examination should include a thorough ophthalmologic and neurologic assessment and an evaluation of the patient’s external genitalia for precocious puberty or virilization, since some pediatric brain tumors may be hormone secreting A rectal examination to evaluate the anal “wink” is also useful as a screen for spinal cord compression If the history or physical examination raises concern for increased ICP or spinal cord compression, therapy should be initiated as described below A CT scan is useful to rule out hemorrhage and assess for increased ICP, and can sometimes visualize a brain tumor However, a CT scan may miss infratentorial masses so in most cases, magnetic resonance imaging (MRI) with gadolinium will ultimately be needed Laboratory evaluation should include serum electrolytes to evaluate for SIADH, salt wasting, or DI A CBC is also useful to ensure the patient’s hematocrit and platelet count are adequate for any upcoming procedures Management The emergency management of increased ICP is critical as patients often present with signs and symptoms of this condition at the time of diagnosis If increased ICP is known or suspected, a lumbar puncture should be avoided, as this theoretically may precipitate a rapid change in ICP followed by herniation of the brain The pressure may be relieved using steroids such as dexamethasone at a dose of to mg every hours Mannitol or hypertonic (3%) saline may be useful in decreasing ICP In some situations, intubation may be needed for both airway protection and to allow for hyperventilation to lower PCO when signs of herniation are present Neurosurgical consultation can address the appropriateness of a ventriculostomy or debulking procedure The decision about where to admit a patient with a newly diagnosed brain tumor hinges primarily on the neurologic status Patients may have altered airway, breathing, or circulation due to the tumor compressing the brainstem Cranial nerve dysfunction may compromise a patient’s ability to eat normally The tumor may cause symptoms such as headache, nausea, or vomiting, which require inpatient management Functional deficits may make discharge problematic In any of these cases, patients should be admitted to the hospital with prompt consultation with pediatric oncology, pediatric neurology, and pediatric neurosurgery for definitive management TUMORS OF THE HEAD AND NECK Goals of Treatment Children with head and neck tumors should be rapidly assessed for airway impingement, breathing compromise, and cervical spinal cord compression CLINICAL PEARL AND PITFALLS Cervical lymphadenopathy is common in childhood and rarely due to cancer Characteristics that make malignancy more likely include nontender masses, very firm/hard texture, diameter more than cm, adherence to other structures, irregular margins, and absence of signs or symptoms of infection Retinoblastoma often presents with leukocoria (white pupil) first noticed by parents Current Evidence Head and neck tumors represent a diverse range of conditions and, with the exception of retinoblastoma and neuroblastoma, occur most commonly in older children and teenagers Aerodigestive tract malignancies include sarcomas, lymphoid tumors, and carcinomas While the latter are commonly seen in the adult population, carcinoma is rare in pediatrics Clinical Considerations ... patients should be admitted to the hospital with prompt consultation with pediatric oncology, pediatric neurology, and pediatric neurosurgery for definitive management TUMORS OF THE HEAD AND NECK... patient’s hematocrit and platelet count are adequate for any upcoming procedures Management The emergency management of increased ICP is critical as patients often present with signs and symptoms... evaluation of the patient’s external genitalia for precocious puberty or virilization, since some pediatric brain tumors may be hormone secreting A rectal examination to evaluate the anal “wink”