Presentation of Infratentorial Ependymomas in Children

Symptoms and Signs

• Elevated ICP: Presenting signs and symptoms are most frequently related to obstructive hydrocephalus. They include nausea and vomiting. 
• Irritation of the floor of the fourth ventricle: Frequent vomiting can be due to direct stimulation of the emetic center at the floor of the fourth ventricle. Nystagmus also may be present.
• Other posterior fossa signs: The patient may have ataxia. Young patients may present with irritability alone.
• Signs of compression at cervicomedullary junction: Nuchal rigidity and pain, torticollis, and head tilt can be found due to tumor extension from the fourth ventricle to the cervical spine. Infiltration of the brainstem or direct tumor involvement within lateral brainstem recesses may cause cranial nerve symptoms (2, 9).  Signs and symptoms of spinal cord compression or nerve root damage can be observed in the presence of spinal metastasis of infratentorial ependymomas (9, 10).

Time for evolution

• 2–3 months: The median duration of symptoms is 2 to 3 months for patients with ependymoma.

Evaluation at Presentation

See EVALUATION

Intervention

• Admission to ICU: Any signs or symptoms of obstructive hydrocephalus or rapidly worsening neurological examination require close observation pending treatment.
• Dexamethasone: Dexamethasone should be started on admission (0.5–1 mg/kg IV loading dose, up to 10 mg, then 0.25–0.5 mg/kg/day IV or PO divided every 6 hours). Antacids should be considered for GI protection when dexamethasone is started.
• Preparation for surgery: A type and cross-match of packed red blood cells should be performed in preparation for surgery.  Malignant tumors may be quite vascular, and young children may have extensive venous sinuses in the posterior fossa dura.
• Imaging: MRI of the brain and total spine, with and without gadolinium contrast, should be obtained preoperatively, if possible.

Hydrocephalus

• Dexamethasone: Steroid administration will commonly alleviate the symptoms of elevated ICP due to obstruction of the fourth ventricle by the tumor. Steroids are accompanied by an antacid for gastrointestinal protection, admission to an ICU, and timely tumor resection.
• EVD: Can be placed emergently for lethargy, bradycardia, or neurological deterioration.  One should avoid rapid decompression of the ventricles. The drainage set is set no lower than 15 cm H2O above the external auditory meatus to minimize the risk of upward herniation.
• ETV prior to tumor resection: This is an area of controversy.  ETV may reduce the incidence of postoperative hydrocephalus (21). However, only a minority of children require CSF diversion after tumor resection (20).
• Shunt placement: Shunting is not currently recommended, as only a minority of patients require permanent CSF diversion, and it exposes patients to a lifetime of shunt dependency and shunt-related complications.