The ocular findings in patients with
homocystinuria due to
cystathionine-beta-
synthetase (
CBS) deficiency are reviewed and a new approach to lens surgery is presented. Lens surgery in
CBS deficiency was reported to be accompanied by a high risk of secondary complications like vitreal
prolapse and
retinal detachment. So far an operative procedure for these patients that allows a complete preservation of the anterior vitreous cortex, the posterior lens
capsule and an implantation of an artificial lens is not established. A minimal invasive method of lens surgery was developed in vitro and applied in the case of a 10-year-old patient with an uncorrectable refractive situation due to progressed
lens dislocation. Principle steps of the evolved operative procedure are (1) visco-elastic stabilization of the anterior chamber; (2) small (1.5 mm) peripheral
capsulorhexis; (3) manual endocapsular aspiration of lens material and removal of lens epithelium; (4) ab externo introduction of a transcleral fixation
suture; (5) positioning of the haptic into the ciliary sulcus in the inferior position; (6) resection of the anterior lens
capsule; and (7) positioning of the second haptic into the ciliary sulcus. The first patient treated according to this method presented a stable position of the pseudophakos during the follow up of 1 year. Visual acuity improved from a level of 20/200 (O.D.) and 10/200 (O.S.) to 18/20 in both eyes. The
surgical procedure presented in this report seems to be useful for dealing with dislocated
lenses due to zonular deficiency in patients with
CBS deficiency and hopefully with Marfan disease. It allows a minimal invasive removal of the lens, a complete preservation of the anterior vitreous cortex and at the same time a stable fixation of an artificial
intraocular lens.