Øyesykdom ved dystrophia myotonica type 1.

Myotonic dystrophy type 1 is one of the most common genetic neuromuscular diseases in adults. The disease not only affects the musculoskeletal system, but is multisystemic, and ocular involvement with cataract formation is a frequent additional finding. To avoid recurrence of secondary opacification that is difficult to treat, the cataract should not be treated with traditional lens replacement. This clinical review article presents ophthalmological findings in cases of myotonic dystrophy type 1 and describes a new surgical method for cataracts in this patient group.

Bag-in-the-lens intraocular lens in paediatric cataract surgery: intraoperative and postoperative outcomes.

PURPOSE: To report intra- and postoperative surgical outcome using the bag-in-the-lens (BIL) technique in paediatric cataract surgery. METHODS: In a retrospective case series, we studied the outcomes of children aged <12 years operated for cataract with the bag-in-the-lens intraocular lens (IOL), with a minimum of 6 months of follow-up. RESULTS: Since 2013, 50 eyes in 30 patients <12 years (20 bilateral and 10 unilateral) have been operated at our department with the BIL technique, with a median follow-up time of 33.5 months (range 6-77). Median age at surgery was 49.5 months (4-139). In one case, the IOL luxated through the capsulorhexes to the vitreous, but could be secured and repositioned as planned without further difficulties. Anterior vitrectomy was necessary in one case due to prolapse of vitreous to the anterior chamber during surgery. No other intraoperative complications occurred. Visual axis opacification (VAO) developed in four eyes (8%). So far, only one of these has needed a reoperation with clearing of the secondary cataract. A complete absence of VAO was thus seen throughout the study period in 92%. In two eyes, postoperative iris capture occurred. In both cases, surgical repositioning of the iris was needed. No eyes developed secondary glaucoma during the study period. CONCLUSION: The BIL technique seems to be a safe surgical procedure in paediatric cataract, with significantly less complications and need for additional surgery compared with the conventional lens-in-the-bag technique.