Abstract
Purpose :
Genetic connective tissue diseases such as Stickler, Marfan, and Ehlers Danlos Syndrome predispose children to rhegmatogenous retinal detachments. However, controversy exists regarding the use of prophylactic laser photocoagulation to prevent retinal detachments (RD) in patients with these syndromes. We hypothesize that prophylactic laser therapy decreases the rate of RD in patients with these connective tissue disorders.
Methods :
We performed a retrospective, observational clinical study to determine the natural history of retinal detachments/tears in patients with Ehlers Danlos, Marfan Syndrome, and Stickler Syndrome. The electronic medical record (EMR) was queried for the respective ICD codes and for the words “Stickler”, “Marfan”, and “Ehler Danlos”. A total of 95 patients were identified from the year 2000 to present. We compared the rate of RD in subjects with a RD in one eye compared to the fellow eye and between subjects who received prophylactic laser therapy and those who did not receive prophylactic laser therapy to determine whether laser therapy reduced the rate of RDs/tears.
Results :
Preliminary results identified 43 patients with Stickler syndrome, of which 21 were female. Of the 86 eyes identified with Stickler syndrome the rate of RD was 6.9% (n=6 eyes). Of the eyes receiving prophylactic laser therapy (n=64), 1.6% (n=1) developed a RD or tear. Of the eyes that did not receive prophylactic laser (n=22), 22% (n=5) developed a RD or tear. Additional data from patients seen at Children’s Hospital of Minnesota ENT and eye clinics is currently being analyzed to further investigate prophylactic laser and retinal detachment rates. Additional analysis will investigate RD rates in patients with Marfan and Ehler Danlos.
Conclusions :
Preliminary results suggest a 6.9% detachment in patients with Stickler Syndrome. This detachment rate is higher compared to eyes that have undergone prophylactic laser therapy, consistent with our working hypothesis. We aim to further assess the effect of prophylactic laser therapy on detachment rate among pediatric patients with connective tissue disorders.
This is a 2020 ARVO Annual Meeting abstract.