Background: Retinal detachment (RD) associated with aniridia due to globe rupture (GR) is an uncommon condition with a severe prognosis. Surgical technique must address anterior and posterior segment issues secondary to the altered compartmentalization and increased risk for corneal toxicity. The purpose of this paper is to report a series of GR patients undergoing combined pars plana vitrectomy (PPV) and artificial iris diaphragm (AID) implant for the repair of RD associated to aniridia.
Methods: The authors retrospectively reviewed 12 consecutive patients operated on by a single surgeon. Surgery consisted of a standard three-port PPV with extensive bimanual dissection of vitreous base and ciliary body membrane and combined AID implant. Office visits included Snellen visual acuity (VA), intraocular pressure measurement, biomicroscopy and indirect ophthalmoscopy. AID prosthesis included aniridic IOLs, Heimann's PMMA and silicone diaphragm.
Results: Mean age was 53 years and mean follow-up was 19 months. At the end of follow-up, seven patients gained more than two lines (58.3%), two lost their vision (16.6%) and three were unchanged (25%). Seven patients (58.3%) had a VA better than 20/400 and one (8%) 20/40 vision. Eight patients (66.6%) retained a clear cornea, two (16.6%) had minimal corneal oedema and two (16.6%) corneal decompensation. Implanted prosthesis included two silicone diaphragms, four PMMA diaphragms and six aniridic IOLs. After an average 1.6 operations, the retina was completely attached in six patients (50%), partially attached in four (33.3%) and detached in two (16.6%).
Conclusion: RD associated to GR carries a guarded prognosis both due to RD complexity and hypotony. The combined repair of RD and aniridia after GR offers the advantage of addressing all issues at one time allowing correct eye compartmentalization and better tamponade effect. Successful anatomical and functional results can be achieved although multiple surgeries are often needed.