Inducing further dislocation of the crystalline lens in Marfan syndrome

ByUday Devgan, MD

In my last blog entry, I wrote about presenting challenging cases during the upcoming South African Society of Cataract and Refractive Surgery meeting in August. Bill Woolf, MD, from Mesa, Ariz., e-mailed me with a novel approach to help a Marfan syndrome patient. He writes:

I have a solution to your interesting case. I am sure there will be options with our current technology, but here is one that is more than just a theoretical possibility.

About 23 years ago, I had a young man present with this same picture bilaterally. He lived in my neighborhood and was the same age as some of my sons. He would ride his bicycle with his head in a chin-down position and his eyes squinted partially closed. It wasn't until he came to the office some years later that I determined the reason for the unusual head and lid positions. If he kept his head in a natural position, his eyes fully open, he was essentially aphakic. Someone had given him two pairs of glasses, one to use if he was looking through the edge of the lens and one to use if he was looking through the "aphakic" visual axis. He didn't like to wear either pair! He had discovered that if he changed his head position and squinted, he could see well enough to function at school or play. He also discovered that if he just lifted his lower lid with his finger his vision greatly improved. After my examination, I referred him to my retinal consultant to consider the possibility of doing a pars plana lensectomy. At the time there weren't any capsular tension rings, and transscleral fixation IOL procedures were just beginning to be done. Also, IOLs weren't even approved for use in teenagers. Complicating the issue was the fact that he and his family were uninsured, so they were not financially able or willing to have a vitrectomy/lensectomy. Their goal was to have him be able to have a normal head position, sans squint, and have reasonable vision.

Searching the literature, I found an article by Tchah et al describing a technique (Tchah HW, Larson RS, Nichols BD, Lindstrom RL. Neodymium:YAG laser zonulysis for treatment of lens subluxation. Ophthalmology. 1989;96(2):230-235).

My plan was to use the Nd:YAG laser (which at the time was a relatively new tool) to lyse the remaining zonules inferiorly, allowing the lens to move up and out of the visual axis altogether. I would treat away from the lens so as not to create a break in the lens capsule, causing a traumatic, hydrating cataract. If my plan was successful, I could then fit him with an aphakic contact lens for distance and he could wear glasses to read or do monovision. I discussed my plan with Dr. Jack Sipperly, my retinal colleague, and the boy's parents, emphasizing that my plan may not be successful. Armed with the knowledge that Jack agreed that it might just work, with little risk, having the parents' consent, I proceeded. I will say that it is easier to see the zonules with the transilluminating slit lamp beam than it is through a decentered Abraham contact lens! Today, we have many more options of contact lenses to use with our various laser procedures.

My treatment was indeed successful. The lens did further dislocate superiorly and out of his visual axis. He had no problems with inflammation, anterior movement of vitreous, increased IOP or retinal detachment, etc. Soon after the procedure, I was able to fit him with a soft aphakic contact lens, and he has done fine for the past 23 years. He has been seen at the Marfan syndrome clinic at Stanford, after which the doctors requested my records to document his successful treatment. Now married with children, my patient works as a graphic artist illustrating video games. The lens has not dislocated any further. I have discussed the option that he consider a lensectomy and sewn-in posterior chamber IOL, but he is happy with his vision and at present hasn't felt the need to take any additional risk.

I don't know what the participants at your meeting will come up with, but this is one option that was successful with no invasive procedure and a "happy patient" outcome.

Sincerely,

William A. Woolf, MD

Mesa, Ariz.