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Abstract #15471 Published in IGR 1-3
Marfan Syndrome, lens subluxation, and open angle glaucoma.
Krupin ThJournal of Glaucoma 1999; 8: 396-399
In this "cases of controversy" the author Krupin presents a case of Marfan's Syndrome, lens subluxation and open angle glaucoma to Gentile, Melamed and LeBlanc. The case concerned is a 31 year old woman with Marfan Syndrome who has lost her left eye and has serious glaucoma in her right eye. The best vision is 20/60. But there is considerable fluctuation. The lens subluxed superiorly and temporally. The lens equator is close to the visual axis. Her IOP is 29 mmHg and the disc is tilted and difficult to evaluate and Goldmann perimetry reveals large central and temporal islands. The axial length is 27.5 mm. She is on maximum medical treatment including oral acetazolamide 500 mg twice daily and still has a pressure of close to 30 mmHg. The question is asked are: 1. How is your surgical management. 2. How would you approach cataract surgery, 3. What are the risks of retinal detachment.The first answer comes from Gentile, a retinal specialist. Based on the available information this patient has a significant risk for a retinal detachment in her remaining eye. In Marfan Syndrome retinal detachment is related to axial myopia, lens subluxation and aphakia. He wants to know more about the cause and type of detachment in the left eye, family history, and status of the vitreous and vitreo-retinal interface. This author would perform prophylactic ARGON laser photocoagulation to totally surround lattice degeneration. He wants to do this several weeks before any planned intra-ocular surgery. If there are any other risk factors he would also consider a prophylactic scleral buckle with encircling element. The superior conjunctiva could be spared if needed. Regarding the visual situation this surgeon is very hesitant about cataract removal because of the high risk of complications. If such a procedure is decided he would do it by a pars plana approach accompanied by a pars plana vitrectomy. He would not place an intra-ocular lens implant. In case a drainage implant is necessary he would like to have it in the inferior position.The preference of Melamed is to postpone cataract surgery as long as possible. If necessary he would prefer a pars plana lensectomy or an anterior approach by a scleral tunnel to be followed by an anterior chamber intra-ocular lens. So far as intra-ocular filtering surgery is concerned this may be associated with lens dislocation, vitreous incarceration into the sclerostomy, choroidal effusion, subchoroidal hemorrhage and retinal detachment. This author recommends non-penetrating deep sclerectomy although he admits that he has no experience with this procedure in Marfan's Syndrome. LeBlanc also feels that non-penetrating trabeculectomy may be the procedure of choice although he too has no experience in these particular cases. Low dose mitomycine would be desirable, but the risk of hypotony is very real. A peripheral iridectomy might be avoided. He also would like to avoid cataract surgery. If necessary he would do a pars plana lensectomy and vitrectomy. He considers vitrectomy with endolaser therapy. Eventually tube-shunt surgery might be necessary.The editor of this series points out that controlled randomized clinical studies on non-penetrating deep sclerectomies are not available. He feels that the theoretical advantages are limited in this patient. Cyclodestruction is not advisable because of possible macular complications. He would prefer a trabeculectomy with 5-fluorouracil and tight sutures. Improvement of visual acuity could be done by pars plana removal of the lens without intraocular lens implant.
Theodore Krupin MD, Clinical Professor of Ophthalmology, Northwestern University, 676 North St. Clair, Suite 320, Chicago, IL 60611, USA
Classification:
9.4.4.3 Glaucomas associated with lens dislocation (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.4 Glaucomas associated with disorders of the lens)
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
