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Also known as pseudotumor cerebri, it is a condition characterized by elevated intracranial pressure (ICP) of unknown cause with normal neuroimaging. The pressure may involve the optic nerve, causing swelling and vision loss, and it is a common cause of preventable childhood vision loss that I encounter here at Children's Hospital Boston. We have a dedicated Neuro-Ophthalmology Service within our Department
of Ophthalmology, and we work closely with our colleagues in Neurology and Neurosurgery to care for children with this condition. The disease is treatable with early intervention, so a prompt diagnosis and multidisciplinary collaboration are essential to ensuring a good outcome.
One main symptom is a persistent headache which is worse laying down and which may be accompanied by double vision or by some degree of vision loss or impairment. An eye exam can detect telltale papilledema or optic nerve swelling in the setting of elevated ICP. In addition to neuroimaging to exclude a true tumor, a lumbar puncture confirms the diagnosis by determining that the ICP is elevated.
There are a few possible avenues. Patients are treated either medically with a pressure- lowering diuretic such as acetazolamide (Diamox), or surgically (if there is a profound threat to the child's vision, or headaches that are refractory
to treatment).
Here at Children's, we can surgically treat some cases of idiopathic intracranial hypertension with the optic nerve fenestration procedure, where we make a small incision in the membrane of the nerve and drain the excess fluid. It's minimally invasive, the recovery time is short and the success rate is excellent. When optic nerve fenestration isn't an option, our neurosurgical colleagues can insert a shunt to route the buildup of fluid out of the brain and into the abdomen or the peritoneal cavity.
Children experiencing both persistent headaches and vision impairment should be referred to a specialist to screen for idiopathic intracranial hypertension. When the disease is diagnosed and treated early, the majority of children respond very well. The headaches and vision problems dissipate as their intracranial pressure stabilizes. We follow up with regular eye exams and ICP readings to monitor their progress over the
long term.
— Gena Heidary, MD, PhD, is a staff pediatric ophthalmologist and pediatric neuro-ophthalmologist at Children's Hospital Boston.
Make a referral to Children's Neuro-Ophthalmology Service: 617-355-6401
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