98th Annual Meeting DOG 2000

K 790

Therapeutic strategies for the ocular manifestations of Marfan syndrome

M. Ladewig1, P. N. Robinson2, M. H. Foerster1

Background: Ocular anomalies associated with Marfan syndrome (MFS) include ectopia lentis and lens dislocation, megalocornea, myopia, hypoplasia of the iris and of the sphincter muscle, retinal detachment and secondary glaucoma. Here, we present results and therapeutic options for MFS patients with ectopia lentis and/or retinal detachment.

Patients and Method: Operations were performed on 12 eyes of 11 patients with MFS who were referred between 1993 and 1998 for ectopia lentis or retinal detachment. The mean age of the patients at the time of surgery was 33 years (4-69). The surgical method was chosen according to the individual findings.

Results: At the initial presentation, 3 eyes were amaurotic and in 3 eyes visual acuity was reduced to hand motions. In all patients, lens dislocation was the initial ocular manifestation, and was often associated with retinal detachment. In 2 cases, spontaneous retinal detachment associated with lens dislocation occurred. In 3 cases, retinal detachment occurred subsequent to lens surgery. In one other case, retinal detachment occurred three years following pars plana lentectomy. Retinal detachment was observed in a total of 7 eyes. Retinal reattachment was performed successfully in all cases. The average follow-up time was 14 months (2-60).

Pars plana lentectomy with posterior-chamber lens reattachment was performed on 7 eyes because of refractive errors which were not optically correctable.

In 2 eyes, secondary, phakolytic glaucoma associated with lens dislocation or ectopia lentis persisted even after vitrectomy. In one eye, secondary glaucoma requiring medical therapy developed following four vitrectomies which had been performed because of retinal detachment. The mean preoperative visual acuity was 0.25 (intact LP-0.6) and the mean postoperative visual acuity was 0.5 (0.04-1.0).

Conclusions: The treatment of MFS-associated disorders of the eye is challenging because of the great variability of findings. Secondary lens implantation should be performed depending on the retinal status of the affected eye and the other eye. If vision is preserved in only one eye, the indication for operation for lens dislocation should be made with caution.

1Department of Ophthalmology, Benjamin Franklin University Hospital, Free University of Berlin
Department of General Pediatrics, Charité University Hospital, Berlin