Abstract
Purpose :
Stickler syndrome (SS) is an inherited, progressive collagenopathy first described in 1965 that is the leading cause of pediatric retinal tears and detachments. Retinal tears or detachments in SS have been shown to occur as early as 8 months, and SS patients require frequent examinations to detect retinal detachments (RD) and prevent development of proliferative vitreoretinopathy which can result in severe vision loss. The purpose of this study is to compare the visual acuity (VA) and odds of RD among patients with SS who received prophylactic laser to those who did not.
Methods :
Retrospective chart review included patients with SS at University of Chicago Medical Center or Retina Consultants, Ltd. between 1/1/2006 and 10/6/2020. Patients older than 3 years of age underwent laser indirect ophthalmoscopy and received a pattern of about 10 rows of 360 degrees of laser prophylaxis from the ora serrata to the equator (complete laser). The association between RD and laser was investigated using Pearson chi-square / Fisher’s exact test. Frequency weighting to control for 2 eyes of the same patient was used to calculate median logmar visual acuity (VA) and interquartile range (IQR). To compare logmar visual acuity by laser treatment groups, a mixed effects logistic regression controlling for 2 eyes of the same patient; nonverbal patients were excluded from visual acuity regression analysis.
Results :
A total of 230 eyes of 124 patients were included. Of those, 90 (39%) eyes had no laser, 7 (3%) received inadequate laser elsewhere, and 133 (58%) received complete laser. Of the eyes who received complete laser, only 5 eyes (4%) had an RD, compared to the 69 eyes (71%) which had inadequate or no laser (p<0.001). Among 204 eyes of 103 patients, patients with complete laser had about 6 lines better vision compared to patients without laser or with inadequate laser (-0.55 logmar, CI -0.79- -0.30, p< 0.001). The odds of low vision were 2.1 (CI 1.1-3.0, p<0.001) times lower for eyes with complete laser.
Conclusions :
Complete laser prophylaxis seems to reduce the chance of retinal detachment and improve visual outcomes in patients with SS. Laser prophylaxis should be considered in all patients with confirmed SS after the age of 3.
This is a 2021 ARVO Annual Meeting abstract.