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Round table: Pediatric pseudotumor cerebri more prevalent than believed
In this fifth excerpt from a round table conducted at the 2011 AAPOS meeting, members of the OSN Pediatrics/Strabismus Section address pediatric pseudotumor cerebri.
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 Robert S. Gold |
Robert S. Gold, MD: Let’s talk about something that we hopefully rarely see in the office: pediatric pseudotumor cerebri.
Roberto Warman, MD: This is not a rare condition in the pediatric ophthalmologist’s office, and we really should give it more attention. Many cases are slightly subclinical, and it is a problem.
I work in a very big center, with a very big pediatric neurology referral group, but I have one to two new patients a week with active pseudotumor cerebri, which is an amazing number. Not all of them are dangerous conditions; many resolve after one episode. That is typical of pediatric cases. But some cases are very difficult to handle, and there are other problems. We have patients with documented, repetitive, high opening pressures in the 30s with absolutely no papilledema. In some, even venous pulsations are present, making management very difficult. Fortunately, most patients have good visual function, but I have seen enough disaster cases in which vision deteriorates quickly.
I am not sure in another setting how common this is, but I do get pediatric ophthalmologists sending me patients for second opinions, so I know the patients are out there. This is a condition to pay a lot of attention to, and if you are not comfortable handling these cases, do not do it. Get help from your neuro-ophthalmologist.
Dr. Gold: Many of us get consultations or referrals from neurologists or neuro-surgeons weekly to rule out papilledema in those cases that they may be treating for headaches and they want to rule out pseudotumor cerebri. Fortunately, most cases are negative, but what are the alarming symptoms?
 Roberto Warman |
Dr. Warman: Children will not complain of transient obscuration of vision. Maybe a teenager you can specifically ask, but the young kids do not talk about that. Diplopia is a problem and teenagers may tell you, but the younger kids tend to suppress it or ignore it and they will not mention it. Most patients with pseudotumor cerebri have bilateral papilledema that is clear-cut and noticeable. There is always that tough case, though, that we send for imaging. It is usually a relatively acute episode in a young child.
The other issue is that children do not have obesity if they are prepubescent. None at all. They are absolutely little thin kids, and boys and girls are affected equally. They are not necessarily chronic cases, although there are enough of those. Therefore, the typical picture of an obese female is only valid after puberty, and their absence does not exclude the possibility of pseudotumor cerebri.
 Scott E. Olitsky |
Scott E. Olitsky, MD: One of the interesting symptoms, which is also problematic for many reasons, is vision loss. Although we are traditionally taught that patients with papilledema do not lose vision early, I see some of these children who come in with profound vision loss, and other ophthalmologists often assume optic neuritis when they look at the nerves of these children. We have had several cases of very high pressures and very acute presentations that have needed shunting procedures to save vision. So the fact that patients come in with profound loss of vision does not necessarily mean that it is not papilledema when there is a swollen nerve.
Dr. Warman: Venous sinus thrombosis is an important factor to consider in pseudotumor cerebri. Now, pediatric neurologists, by standard, once they order an MRI, also order an MRA and MRV because the child is asleep and they want to rule out the diagnosis.
I have seen several patients with idiopathic thrombocytopenia and pseudotumor cerebri, and I have seen patients on protocols of leukemia treatment who have pseudotumor cerebri. I am not sure why, but it may have to do with some of the chemotherapeutic agents used. There are also patients treated for acne both with tetracycline derivatives and/or Retin-A and also patients on growth hormone who can develop pseudotumor cerebri.
- Robert S. Gold, MD, can be reached at 790 Concourse Parkway South, Suite 200, Maitland, FL 32751; 407-767-6411; fax: 407- 767-8160; email: rsgeye@gmail.com.
- Scott E. Olitsky, MD, can be reached at Children’s Mercy Hospital and Clinics, 2401 Gillham Road, Kansas City, MO 64108; 816-234-3000; fax; 816-346-1375; email: seolitsky@cmh.edu.
- Roberto Warman, MD, can be reached at Miami Children’s Hospital, 3200 SW 60th Court, Suite 103, Miami, FL 33155-4072; 305-662-8390; fax: 305-661-7862; email: rwarman@eyes4kids.com.
- Disclosure: No products or companies are mentioned that would require financial disclosure.