Sunday, April 12, 2009

Cataract surgery and Behcet's

If you have Behcet's-related eye involvement with inflammation that's lasted for months or years, OR

If you've had NO Behcet's eye involvement, but have been using prednisone and/or other corticosteroid-based meds for a long time, then this post is for you. Here are the facts:
  1. Long-term eye inflammation can create a cataract in the affected eye(s) -- whether you have Behcet's uveitis or some other inflammatory eye disease.

  2. And prednisone use can also cause cataracts in BOTH eyes, even if you have uveitis in only one of those eyes. As a matter of fact, prednisone use can cause cataracts in both eyes even if you've never had uveitis.
It's not a great situation. What are you supposed to do if cataracts start to affect your vision? Some surgeons will refuse to do cataract surgery on Behcet's patients, either because they've never had a BD patient before, or they're afraid of complications from the surgery (and/or possible liability). And some surgeons will tell you that the operation isn't a problem for Behcet's patients; these are the surgeons to be worried about. Ask how many BD patients they've operated on, and what their results were.

One way or another, though, if your vision has gone downhill due to a cataract, it's time to look for solutions.

I've been through two cataract removals and IOL (intraocular lens) implants. One of the surgeries was very complicated, and required creating a new pupil at the same time as the cataract removal (it's a long story). Remembering that I'm not medically trained, and that your doctor(s) are the best source of information, here are my suggestions:
  1. Cataract surgery in Behcet's patients should only be done by a surgeon who has experience in handling the potentially-explosive complications that can come with eye surgery in BD patients. Find out how much experience your surgeon has in this area, how many Behcet's cataract surgeries s/he has done, and how experienced s/he is in using immunosuppressive meds to handle serious post-surgical inflammation. I was in excellent hands with my surgeon (Dr. Foster, at MERSI in Boston) but several other surgeons I'd seen first had refused to do the operation because of the possible complications. Keep in mind though, that even with a good surgeon, no one can guarantee what the outcome will be.

  2. Cataract surgery should only be done when the affected eye has been totally quiet for at least 3 months. No cells in the vitreous, and no evidence of inflammation.

  3. The outcome of the surgery (how well you'll be able to see) really depends on how much damage has already happened to your retinas/optic nerve/etc. Replacing a cataract with an IOL will only you give good vision if your retina is still in good shape. If your cataract is very dense and your vision is already poor, it may be hard for your ophthalmologist to know what's going on with your retina(s) before the surgery. There were a lot of doubts about whether an IOL would improve the vision in one of my eyes since I had so much retinal scar tissue, but it turned out very well. Of course I didn't know for sure, though, until the bandages came off the day after surgery. And then it was like a miracle -- I went from counting fingers and basic light/dark vision, to 20/60 overnight...and it kept improving over the following weeks till it reached 20/20 -- where it is today (10+ years later :-)

  4. If you have cataracts in BOTH eyes, you'll need two separate surgeries: If there are complications during the first surgery and you lose some vision as a result, you'll still have one "good" eye left.

  5. Patients with Behçet's uveitis should receive immunosuppressive/steroid meds before any eye surgery to reduce the chance of inflammation after the operation.
Dr. Foster has a couple of short background articles on his website that you might find helpful. You can see them here:
There are also several medical journal articles about cataract removal in BD patients. If your surgeon is good, s/he should already know all of this information (and more). If you'd like to read the summaries (abstracts), I've included the links below:
  1. Curr Opin Ophthalmol. 2009 Jan;20(1):42-5
    Cataract surgery in the setting of uveitis
    Van Gelder RN, Leveque TK.
    The basics:
    If the special challenges of cataract surgery for uveitis patients are taken care of, excellent vision can be achieved in most patients. Recent research shows high rates of uveitis flareups after surgery in some diseases, though, suggesting that medications should be prescribed in advance of the surgery to prevent flareups afterward. Although excellent outcomes can be achieved with many types of intraocular lenses, several recent studies have suggested that complications after surgery may be lower in patients who receive intraocular lenses made of acrylic instead of other materials.
    PMID: 19077828

  2. J Cataract Refract Surg. 2007 Feb;33(2):305-9
    Phacoemulsification cataract extraction and intraocular lens implantation in patients with uveitis.
    Kawaguchi T, Mochizuki M, Miyata K, Miyata N.
    The basics: Cataract removal and IOL implant in patients with uveitis was satisfactory. Patients with Behçet's- related eye inflammation, however, appeared to have a higher risk for complications, and therefore worse outcomes, than patients with other types of uveitis.
    PMID: 17276275

  3. Ophthalmic Surg Lasers Imaging. 2004 May-Jun;35(3):215-8.
    Phacoemulsification cataract extraction and intraocular lens implantation in patients with Behçet's disease.
    Berker N, Soykan E, Elgin U, Ozkan SS.
    The basics: Vision got better in 29 eyes (72.5%) after surgery and was 20/40 or better in 18 eyes (45%). Vision got worse in 7 eyes (17.5%), resulting in vision of 20/400 or worse. The most frequent complication after surgery was posterior capsular opacification [a cloudy lens], which developed in 15 eyes (37.5%). Other complications were posterior synechiae formation [a pupil that won't move, because the iris is stuck to the lens of the eye] in 7 eyes (17.5%), severe inflammation in 5 eyes (12.5%), cystoid macular edema in 5 eyes (12.5%), epiretinal membrane [a membrane over the retina that can cause distorted vision] in 3 eyes (7.5%), and optic atrophy [an optic nerve that's wasting away] in 2 eyes (5%).
    PMID: 15185789

  4. J Cataract Refract Surg. 2002 Feb;28(2):316-20.
    surgery in patients with Behçet's disease.

    Kadayifçilar S, Gedik S, Eldem B, Irkeç M.
    The basics: In patients with Behçet's disease, inflammation after surgery was mild when surgery was performed after at least 3 months of no inflammatory signs. The vision after surgery depended on how much damage had already been done to the back of the eye from previous inflammations.
    PMID: 11821216

  5. Ophthalmologica 2001;215:179–182
    Ocular attacks after phacoemulsification and intraocular lens implantation in patients with Behcet's disease
    Toshihiko Matsuoa, Makiko Takahashia, Yasushi Inoueb, Kuniaki Egic, Yasunori Kuwataa, Akihiro Yamaokaa
    The basics: The development of inflammation after cataract surgery was related to inflammation in the affected eye at any point within 1 year before the surgery. The authors recommend a minimum 6-month inflammation-free time period before removing a cataract and implanting an IOL.
    PMID: 11340387

  6. Acta Ophthalmol Scand. 2000 Dec;78(6):680-3.
    The results of cataract extraction and intraocular lens implantation in patients with Behçet's disease.
    Süllü Y, Oge I, Erkan D.
    The basics: For patients with chronic Behçet's uveitis who had cataract removal and IOL implants, the patients had low vision after surgery due to problems that already existed before the operation: optic atrophy, inflammation that caused scar tissue, or macula edema.
    Full article available here:
    PMID: 11167232
And finally, here's a general overview article on BD-related eye problems at "Update on Ocular Behcet's Disease"

I've had no regrets about either of my cataract surgeries. After 30 years of Behcet's-related eye involvement, I'm still able to see, drive, work, and handle my life on a day-to-day basis. Not everyone with BD is that lucky (and my situation could change tomorrow) but I credit excellent eye care for my good vision. If you haven't seen a uveitis specialist yet, please do it! There's a list of U.S. and international uveitis specialists here:

All the best,
Joanne Z.