Pediatric Pseudotumor Cerebri
What is pseudotumor cerebri?
Pseudotumor cerebri is a condition in which patients have elevated intracranial pressure without the presence of hydrocephalus, a tumor or other mass lesion in the brain. In some patients, the condition can be associated with anomalies of the veins of the brain that lead to increased pressure.
Although pseudotumor cerebri can occur in persons of normal weight, it is more commonly found in obese patients. It is more common in females than males.
What are the symptoms of pseudotumor cerebri?
The most common symptom is headache, which may be severe and difficult to treat. Patients may also experience visual loss, which can progress to blindness in severe cases.
How is pseudotumor cerebri diagnosed?
An MRI of the brain can confirm the absence of a mass lesion, hydrocephalus or other cause of a patient’s symptoms. A magnetic resonance venogram (MRV) may be used to screen for anomalies of the large veins of the brain (called venous sinuses). Ophthalmology evaluation, along with formal visual-field testing, is important to assess for visual loss. The diagnosis of pseudotumor cerebri is often confirmed by elevated opening pressures on lumbar puncture (spinal tap).
How is pseudotumor cerebri treated?
In patients who are overweight, the best treatment is an aggressive weight loss program. This should be coordinated by the patient’s pediatrician and/or a nutritionist.
Additional medical treatment options include:
- Acetazolamide, a medication that may be effective in decreasing pressure in the brain for some pseudotumor cerebri patients
- Serial lumbar punctures to drain cerebrospinal fluid and decrease the pressure in the brain
If medical management is not effective, a patient may require surgical treatments, which can include:
- Placement of a shunt from the fluid spaces of the brain (the ventricles) into the abdominal cavity
- A lumboperitoneal shunt to divert fluid from the lumbar spinal canal into the abdomen
- Optic nerve sheath fenestration, which may be performed with the goal of saving deteriorating vision
Patients with anomalies of the veins of the brain may be treated with endovascular procedures in which the venous drainage of the brain is improved by using a catheter threaded from an artery in the leg into the sinuses.
What follow-up is necessary after treatment?
Because of the possibility of permanent visual loss, patients should be followed closely by a pediatric ophthalmologist and a pediatric neurosurgeon. Care of patients with pseudotumor cerebri may also be managed by pediatric neurologists.
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