Abstract
Purpose :
Ectopia lentis is associated with a number of genetic disorders, most commonly Marfan syndrome and homocystinuria. Surgical advances have improved the effectiveness of retinal detachment repair, but literature describing surgical outcomes among those with connective tissue disturbances is scarce. We evaluated anatomical and functional success rates of retinal detachment repair in patients with ectopia lentis at a single institution.
Methods :
This retrospective study included patients with ectopia lentis who were surgically treated for retinal detachment at the Wilmer Eye Institute between 2012 and 2023. The primary outcome was single-operation success (SOS) at 12 months. Secondary outcomes included change in best-corrected visual acuity (BCVA) and complications such as cataract, proliferative vitreoretinopathy (PVR), or epiretinal membrane (ERM).
Results :
A total of 44 eyes (20 OD, 24 OS) from 44 patients (22 male, 22 female) were included. The mean age at time of operation was 59 years (range, 18 to 92). PVR was present preoperatively in 13 eyes, ERM was noted in 7, and 25 had posterior vitreous detachment. Macular involvement was noted in 29 cases (66%), and three or more quadrants were involved in 15 cases (34%). 28 eyes received pars plana vitrectomy (PPV) alone, 3 received scleral buckle (SB) alone, and 13 received a combination of PPV plus SB. Initial anatomical success was achieved in all cases, and 36 remained completely attached at 12 months without reoperation. PVR was observed post-operatively in 7 eyes, 6 of which were among those that did require reoperation. ERM was seen in 6 eyes, vitreous hemorrhage was seen in 6 eyes, and cataract occurred in 5 of 8 phakic eyes (63%). Intraocular pressure became elevated in 22 eyes. After excluding eyes with incomplete visual acuity data and pre-existing PVR, 82% of remaining eyes either had an improvement of 0.3 logMAR or achieved a visual acuity equal or superior to 0.3 logMAR (20/40 Snellen) at 12 months. In this group, the median BCVA at 12 months was 0.3 logMAR, and the mean was 0.76 logMAR (1.06 for macula off versus 0.64 for macula on, p=0.275).
Conclusions :
Surgical treatment of retinal detachments in patients with ectopia lentis produces anatomical outcomes comparable to those of the general population. Visual function also improved substantially in most patients despite a relatively high frequency of existing comorbidities and post-operative complications.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.