- Macrocephaly: There can be an enlarged head due to associated hydrocephalus (75).
- Developmental/psychomotor delay (75).
- Elevated ICP: Signs of raised ICP: headaches, irritability, nausea and vomiting.
Less common presentation
- Bobble head doll syndrome: Can occur when the tumor is located in the third ventricle (69).
- Drop attacks: Can occur when the tumor is located in the third ventricle (69).
- Dyspnea: Can occur when the tumor is located in the fourth ventricle.
- Hiccups: Can occur when the tumor is located in the fourth ventricle (18).
- Sudden death (88).
- Hemorrhagic presentation in less than 5% of cases.
Patterns of evolution
- Onset of symptoms due to development of hydrocephalus: The tumor is usually diagnosed when it is big enough to lead to secondary hydrocephalus. Symptoms of hydrocephalus and elevated ICP are therefore usually the first symptoms to be picked up, and the evolution is linked to the importance of hydrocephalus.
Time for evolution
- Variable but diagnosed usually at point of tolerance of hydrocephalus: The evolution of the tumor depends on its nature. Choroid plexus papillomas most often are slow growing. However, because they are usually diagnosed when they are large and cause hydrocephalus, they need to be treated rapidly. Choroid plexus carcinomas are more aggressive and therefore might present more acutely, with a decreased level of consciousness that necessitates emergency treatment.
Evaluation at Presentation
- See EVALUATION
- Elevation in ICP: Hydrocephalus might need to be dealt with to stabilize the child before dealing with the tumor if the child is lethargic or shows signs of severely elevated ICP.
Preparation for definitive intervention, nonemergent
- Imaging: MRI and angiography should be done.
- Plan surgery: After imaging, a surgical approach can be chosen and the case discussed with the parents. Surgery can be done as a semi-elective case in the OR.
Preparation for definitive intervention, emergent
- Imaging: Imaging should be done. If the child is unstable, a CT scan will suffice to assess the pathology and the hydrocephalus.
- EVD: An EVD should be inserted. If the tumor is anterior and bulges in the third ventricle, blocking one or both foramina of Monro, it might be necessary to insert a drain in each lateral ventricle or do an endoscopic septostomy to allow a single EVD.
Standard admission orders are used in preparing child for surgery.
CSF drainage parameters
- Don’t overdrain: When an EVD is inserted, it should be left at a height of 12-15 cm H2O.