| Affiliation |
(AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (JA) Baylor College of Medicine, Houston, Texas |
| Transcript |
Today we're going to be talking about a very special form of idiopathic intracranial hypertension called fulminant, fulminant idiopathic intracranial hypertension. So, of course you still have to meet the modified Dandy criteria, you still have to have an MRI and an MR venogram with contrast that's negative, you still have to have a lumbar puncture that shows only elevated opening pressure and a normal CSF content. So, the normal criteria that we have for IIH all still apply. But the fulminant refers to the speed and severity of the IIH. So, normally IIH is slow, might be months or years even, but in fulminant it's got two kinds of key criteria. The first is it's fast and normally that means it's days to weeks-so less than two weeks-but it can be days, could even be hours, and the severity, it's usually bad. And that means either bad vision, visual acuity, or field. So, the reason it's dangerous is fulminant IIH, we really shouldn't be fooling around with Diamox, that just needs surgery. So, some things just need surgery. You can give Diamox, but it's going to take too long. Certainly giving low dose and ramping it up, all the usual things that we do for regular IIH, we really probably shouldn't be doing for fulminant. So, fulminant IIH is IIH but it's too fast and too bad. You're going to give Diamox but I would recommend you admit this patient to the hospital. We're going to do the workup if they don't have the diagnosis yet but if they do have the diagnosisal ready we're going todo a lumbar drain, and that lumbar drain is to temporize the ICP until we can get a definitive surgical procedure done. Our choices are optic nerve sheath fenestration - I prefer that for patients who present with just the vision loss -or a shunting procedure, a CSF diversion procedure. I prefer stereotactic programmable valve, ventricul operitoneal shunt. However, no one would fault you if you did an LP shunt here. I think stent is a less desirable option. Once we admit the patient we're going to give high-dose intravenous Diamox, you can add intravenous steroids onto here because it's going to be hard to tell the difference between fulminant IIH and other optic neuropathies that are acute and bilateral. So, those people still need to be covered as if it's inflammatory optic neuritis until we get all the scans and stuff done. So, as opposed to regular IIH, we really need to have the orbit in the fulminant IIH so we can see if there's enhancement of the optic nerve. There's no enhancement, then we're looking for the radiographic features of IIH. That's going to be fluid in the sheath, flattening the globe, venous sinus narrowing at the distal transverse and sigmoid sinus, and empty sella. If, however, we don't see any enhancement of the nerve, get the lumbar drain, call the surgeons. This just depends on what your hospital has available to it. So, use what you got. So, fulminant IIH generates a lot of medical legal problems for neurology and ophthalmology and neurosurgery alike. Please don't fool around with it. Admit, hit him hard with the medical therapy, do the lumbar drain to temporize, and get ready for surgery." |
